MEDICAL RADIOLOGY Diagnostic Imaging

Transcription

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2 MEDICAL RADIOLOGY Dignostic Imging Editors: A. L. Bert, Leuven M. Knuth, Göttingen K. Srtor, Heidelberg

3 Goetz Benndorf Durl Cvernous Sinus Fistuls Dignostic nd Endovsculr Therpy Foreword by K. Srtor With 178 Figures in 755 Seprte Illustrtions, 540 in Color nd 19 Tbles 1 3

4 Goetz Benndorf, MD, PhD Associte Professor, Deprtment of Rdiology Bylor College of Medicine Director of Interventionl Neurordiology Ben Tub Generl Hospitl One Bylor Plz, MS 360 Houston, TX USA Medicl Rdiology Dignostic Imging nd Rdition Oncology Series Editors: A. L. Bert L. W. Brdy H.-P. Heilmnn M. Knuth M. Molls C. Nieder K. Srtor Continution of Hndbuch der medizinischen Rdiologie Encyclopedi of Medicl Rdiology ISBN e-isbn DOI / Medicl Rdiology Dignostic Imging nd Rdition Oncology ISSN Librry of Congress Control Number: , Springer-Verlg Berlin Heidelberg This work is subject to copyright. All rights re reserved, whether the whole or prt of the mteril is concerned, specificlly the rights of trnsltion, reprinting, reuse of illustrtions, recittions, brodcsting, reproduction on microfilm or in ny other wy, nd storge in dt bnks. Dupliction of this publiction or prts thereof is permitted only under the provisions of the Germn Copyright Lw of September 9, 1965, in its current version, nd permission for use must lwys be obtined from Springer-Verlg. Violtions re lible for prosecution under the Germn Copyright Lw. The use of generl descriptive nmes, trdemrks, etc. in this publiction does not imply, even in the bsence of specific sttement, tht such nmes re exempt from the relevnt protective lws nd regultions nd therefore free for generl use. Product libility: The publishers cnnot gurntee the ccurcy of ny informtion bout dosge nd ppliction contined in this book. In every individul cse the user must check such informtion by consulting the relevnt literture. Cover-Design nd Lyout: PublishingServices Teichmnn, Muer, Germny Printed on cid-free pper 21/3180xq springer.com

5 Dedicted to my prents, Dorothe nd Eberhrd Benndorf

6 Foreword Of the durl venous sinuses, the cvernous sinus is ntomiclly the most complex. It hs n intimte topogrphicl reltionship with the internl crotid rtery nd the sixth crnil nerve (both of which pss through its meshwork) nd houses the crnil nerves III, IV nd V1-2 in its lterl wll. Medilly it buts the pituitry glnd, while lterlly it ners the temporl lobe; Meckel s cve with the trigeminl gnglion lies immeditely posterior to it. Locted essentilly t the center of the skull bse, the cvernous sinus connects with numerous durl sinuses nd veins of ll three crnil fosse, s well s the orbit. Furthermore, there is n bundnce of durl rteries in the sellr region tht re interconnected nd derive from the crotid system. Due to its specific vsculr ntomy, the cvernous sinus is common site of vrious types of rteriovenous (AV) fistuls, which re essentilly benign lesions but which my endnger both vision nd crnil nerve function. These fistuls, including their lrgely endovsculr interventionl tretment, re the topic of Götz Benndorf s monogrph. After describing the relevnt ntomy of the cvernous sinus in gret detil, the uthor continues by explining the ntomic nd hemodynmic clssifictions of AV fistuls of the cvernous sinus. This is followed by importnt informtion on etiology, pthogenesis nd prevlence of the vrious lesions. Before embrking on the min prt of his book, dignostic nd therpeutic rdiology of AV cvernous fistuls, Benndorf devotes n entire chpter to clinicl, lrgely neuro-ophthlmologicl symptoms nd signs. The rdiologicl chpters, ll beutifully illustrted nd contining tretise on hemodynmics, will undoubtedly convince reders of the immense dignostic nd therpeutic experience of the uthor in his chosen topic. The tretment focuses on trnsvenous emboliztion rther thn the trnsrteril pproches. I m unwre of ny publiction tht covers the dignostic nd interventionl rdiology of AV fistuls of the cvernous sinus in such cler, systemtic nd complete wy s Benndorf s book. Any interventionl neurordiologist deling with skull bse lesions needs copy, s does ny skull bse surgeon. In ddition, the book would not look out of plce on neurologist s bookshelf. Heidelberg Klus Srtor

7 Prefce This volume of Medicl Rdiology is bsed to lrge degree on my Ph.D. Thesis t Chrité, Humboldt University (Berlin, 2002) nd contins most of the originl text nd imging mteril. During my subsequent yers (2003 to 2009) t the deprtments of rdiology, Bylor College of Medicine (BCM) nd The Methodist Hospitl (TMH) in Houston, this work grew substntilly, nd thus required more time to complete thn nticipted. Nevertheless, I hope, the result represents hppy ending tht ws worth witing for. The monogrph stnds for more thn 13 yers of personl experience in performing endovsculr tretment nd clinicl mngement of ptients with vrious types of intrcrnil rteriovenous shunting lesions, in prticulr fistuls of durl origin involving the cvernous sinus. It is intended s prcticl guide nd reference for those involved in the dignosis nd tretment of these lesions. Completing this book would hve been impossible without the motivtion, help nd support from my techers, collegues nd friends. Acknowledgements: I m very thnkful to Christine Kgel (University Greifswld) nd Christine Poehls (Helios Klinikum Berlin-Buch), who between 1989 nd 1991, tught me the bsic principles of dignostic cerebrl ngiogrphy nd vsculr interventionl rdiology. Horst Peter Molsen (Chrité, Berlin), my INR fellowship director from 1991 to 1993, introduced the techniques of trnsvenous ctheteriztion nd emboliztion to me while we were treting the first CSF ptients together. My most sincere grtitude goes to Jcques Moret (Foundtion Rothschild, Pris) for his generous permission to use some of his mteril for my thesis. During his numerous visits nd lectures t the Benjmin Frnklin Hospitl, Free University Berlin from 1992 to 1997, Pierre Lsjunis (, Kremlin Bicetre, Pris) ws not only brillint techer, but lso gret inspirer in the studies of the vsculr ntomy of the cvernous sinus region. I m very much indebted to Wolfgng Lnksch (Chrité, Berlin), chir of the deprtment of neurosurgery, who ws my clinicl mentor nd stedy supporter of my work from 1991 to I owe mny specil thnks to the entire INR tem t Rudolf-Virchow-Hospitl, Chrité, but mostly to two nurses, Angelik Wehner nd Petr Schlecht, whose loyl devotion nd outstnding ssistnce over mny yers plyed key role in the successful performnce of complex endovsculr procedures. Christof Brner, immensely experienced nd dedicted neuronesthesiologist t Chrité, becme n indispensble collegue for chieving good clinicl outcomes. I lso wish to thnk Horst Menneking (mxillo-fcil surgeon, Chrité) for his exceptionlly skillful surgicl work in those cses, where trnsophthlmic SOV pproches becme necessry. My collegue nd friend, Andres Bender (Chrité until 1998), one of the most tlented interventionl neurordiologists I hd the good fortune to meet, helped me to mster the tretment chllenges of severl of my erly ptients. Stephnie Schmidt, ophthlmologist t Chrité until 2003, ws n outstnding clinicin nd most plesnt collegue to work with. The mjority of presented cse reports re of ptients, for whom stff members from the deprtments of neurosurgery, neurology nd ophthlmology t Chrité provided excellent cre.

8 X Prefce The following collegues nd friends contributed dditionl interesting nd vluble imging mteril: Jcques Moret (Foundtion Rothschild, Pris), Alessndr Biondi (Pitié-Slpêtrière University Hospitl, Pris); Gyul Gl (University Hospitl Odense); Michel Mwd (BCM, Houston); Jcques Dion (Emory University Hospitl, Atlnt); Richrd Klucznik (TMH, Houston); Mri Angeles De Miquel (Hospitl Universitri de Bellvitge, Brcelon); Michel Soedermn (Krolinsk University Hospitl, Stockholm); Winston Lim (Singpore Generl Hospitl); Adrin Cmpi (Ospedle Sn Rffele, University of Miln); Rob De Keizer (University Hospitl, Leiden); Chrbel Mounyer (Foundtion Rothschild, Pris); Rimund Prsche (Ruppiner Kliniken); Bernhrd Snder (MRI Prxis, Berlin) nd Ullrich Schweiger (Ullsteinhusklinik, Berlin). Specil credit goes to Alessndr Biondi, Gyul Gl, Stefnie Schmidt, Dine Nino (BCM Houston), Phillip Rndll (TMH Houston) nd Mi Crlson (MDA, Houston) for review, suggestions nd corrections of the text. The superb clinicl photogrphs were tken by Frnz Hffner nd Peter Behrend (Chrité, Berlin); Dvid Gee (Houston) produced the high-qulity dry skull pictures. Corinn Nujok (Chrité, Berlin), Chrlie Thrn (TMH, Houston) nd Scott Weldon (BCM, Houston) helped to crete the nicely colored illustrtions. Dirk Emmel (Chrité, Berlin) provided invluble support for the digitl storge of the imge mteril. Richrd Klucznik (TMH, Houston) ws very helpful in cquiring high-resolution 3D-dt for the imging studies of the cvernous sinus. Lst, but not lest, I m truly grteful to Ursul Dvis (Heidelberg), Christine Schefer (Hemsbch), Kurt Teichmnn (Muer) nd Klus Srtor (Heppenheim), whose endless ptience nd understnding llowed me to produce nd finish this volume. Houston Goetz Benndorf

9 Contents 1 Introduction Historicl Considertions Arteriovenous Fistul nd Pulsting Exophthlmos Angiogrphy Therpeutic Mesures Emboliztion References Antomy of the Cvernous Sinus nd Relted Structures Osseous Antomy Antomy of the Dur Mter nd the Crnil Nerves Vsculr Antomy References Clssifiction of Cvernous Sinus Fistuls (CSFs) nd Durl Arteriovenous Fistuls (DAVFs) Antomic Clssifiction Etiologic Clssifiction Hemodynmic Clssifiction References Etiology, Prevlence nd Nturl History of Durl Cvernous Sinus Fistuls (DCSFs) Etiology nd Pthogenesis of Type A Fistuls Etiology nd Pthogenesis Type B D Fistuls Prevlence References Neuro-Ophthlmology in Durl Cvernous Sinus Fistuls (DCSFs) Extrorbitl Oculr Symptoms Orbitl Symptoms Other nd Neurologicl Symptoms Differentil Dignosis References

10 XII Contents 7 Rdiologicl Dignosis of DCSFs Non-invsive Imging Techniques Intr-rteril Digitl Subtrction Angiogrphy (DSA) References Endovsculr Tretment Techniques of Trnsvenous Ctheteriztion Embolic Agents Results of Trnsvenous Emboliztions Discussion of Trnsvenous Occlusions Conclusion References Alterntive Tretment Options Spontneous Thrombosis Mnul Compression Therpy Controlled Hypotension Rdiotherpy Surgery References Hemodynmic Aspects of DCSFs Introduction Bsic Hemodynmic Principles Invsive Assessment of Hemodynmics Flow Velocity nd Pressure Mesurements in Brin AVMs nd DAVFs Hemodynmics nd Pthophysiology in CSFs Flow Velocity nd Pressure Mesurements in DCSFs References Summry Subject Index

11 Glossry ACC Anterior condylr confluens ACT Angiogrphic computed tomogrphy (contrst enhnced DynCT; bsed on C-rm mounted Flt pnel technology) ACV Anterior condylr vein AFR Artery of the formen rotundum AMA Accessory meningel rtery APA Ascending phryngel rtery AVM Arteriovenous mlformtion BP Bsilr plexus BSC Boston Scientific Corportion CCF(s) Crotid cvernous fistul (direct fistul) CN(s) Crnil nerve(s) CS(s) Cvernous sinus(es) CSF(s) Cvernous sinus fistul(s) (AV shunt involving the cvernous sinus in generl) CTA Computed tomogrphic ngiogrphy (bsed on conventionl CT technology) CVD Corticl venous dringe DAVF(s) Durl rteriovenous fistul DAVS Durl rteriovenous shunt DCCF Durl crotid cvernous fistul DCSF(s) Durl cvernous sinus fistul(s) (indirect fistul) DSA Digitl subtrction ngiogrphy DynCT Siemens term for cross sectionl (CT-like) imging using rotting C-rms ECA Externl crotid rtery F French (Chrrière; 1 french = 1/3 mm) FLP Formen lcerum plexus FrV Frontl vein FV Fcil vein i.. Intrrteril i.v. Intrvenous ICA Internl crotid rtery ICAVP Internl crotid rtery venous plexus ICS Intercvernous sinus IJV Internl jugulr vein ILT Inferolterl trunk IMA Internl mxillry rtery IOF Inferior orbitl fissure (infrorbitl) IOV Inferior ophthlmic vein IPCV Inferior petroclivl vein IPS Inferior petrosl sinus IPV Intrpetrosl vein

12 XIV Glossry JB LCS LCV LVD MHT MIP MMA MPR MRA MTA MTV NBCA OA PCP PCV PP PPF PVP RAFL SMCV SOF SOV SPPS SPS SS SSD STA TAE TVO UV VA ViA VRT Jugulr bulb Lterocvernous sinus Lterl condylr vein Leptomeningel venous dringe Meningohypophysel trunk Mximum Intensity projections Middle meningel rtery Multiplnr reconstructions Mgnetic resonnce ngiogrphy Mrginl tentoril rtery (medil tentoril rtery) Middle Temporl vein N-butyl-cynocrylte Ophthlmic rtery Posterior clinoid process Posterior condylr vein Pterygoid plexus Pterygopltine foss Prevertebrl plexus Recurrent rtery of the formen lcerum Superficil middle cerebrl vein Superior orbitl fissure (suprorbitl) Superior ophthlmic vein Sphenoprietl sinus Superior petrosl sinus Sigmoid sinus Surfce shded Disply Superficil temporl rtery Trnsrteril emboliztion Trnsvenous occlusion Uncl vein Vertebrl rtery Vidin rtery Volume rendering technique

13 Introduction 1 In reference to cvernous sinus fistuls (CSFs) cusing pulsting exophthlmos, Wlter Dndy (1937) wrote: The study of crotid-cvernous neurysm the clinicl ensemble the vrition nd cpricious results of tretment hve been told nd retold, nd most dmirbly. Medicl literture cn scrcely clim more ccurte nd thorough studies thn upon this subject. More thn 70 yers lter, similr sttement cn be mde relting to subgroup of CSFs, the rteriovenous shunts between smll durl brnches rising from the externl nd internl crotid rteries nd the cvernous sinus, lso clled durl cvernous sinus fistuls (DCSFs). Indeed much hs been written bout these fistuls, which were recognized reltively lte s seprte entity mong CSFs, nd which cn be cliniclly perplexing nd sometimes quite difficult to dignose or to tret. The cure of ptients, on the other hnd, is one of the most rewrding in the spectrum of modern neuroendovsculr tretments. The initil ngiogrphic descriptions by Cstigne et l. (1966), Newton nd Hoyt (1970) nd Djindjn et l. (1968) focused minly on their peculir rteril supply, which lter becme the bsis for widely used ntomic clssifiction (Brrow et l. 1985). The cvernous sinus itself represents rther complex venous reservoir, embedded in the bse of the skull nd trversed by the cvernous crotid rtery nd four crnil nerves. It functions s confluens, receiving multiple cerebrl nd intr crnil fferent veins (tributries) nd drins into vrious efferent veins or durl sinuses. Despite numerous studies, etiology, pthophysiology nd clinicl course of these fistuls re to dte only prtilly understood. Becuse the rteriovenous shunts develop within the durl wlls of the cvernous sinus (CS), their flow is usully directed towrds the superior ophthlmic vein (SOV), cusing signs nd symptoms very similr, lbeit milder, to those observed in ptients with direct high-flow crotid cvernous fistuls (CCFs). Significnt improvements in ngiogrphic imging technology over the lst 15 yers, such s the introduction of three-dimensionl digitl subtrcted ngiogrphy (3D-DSA), hve resulted in better understnding of the specific rteril nd venous ntomy, opening the doors for novel tretment options. In combintion with the dvnces mde in endovsculr tools nd devices, trnsvenous occlusion using vrious trnsfemorl or percutneous ccess routes hs become incresingly populr. Numerous cse reports nd smll cse series hve been published, creting welth of informtion in the medicl literture. However, the dt scttered through journls of vrious clinicl disciplines nmely neurordiology, neurosurgery, neurology nd ophthlmology. Regrding therpeutic options for ptients with CSFs, Hmby (1966) stted: The best possibility theoreticlly would be to induce thrombosis tht would close the sinus completely. This ppers to be hrdly possibly, by currently known techniques, in the fce of the tremendous rteril inflow of blood. This concept ws reiterted by Mulln (1974), nd 40 yers lter, trnsvenous occlusion (TVO) techniques ply dominnt role in the mngement of ptients with DCSFs. Becuse TVO of DCSFs cn often be performed successfully tody with high efficcy nd low morbidity, it hs widely replced microneurosurgery. On the other hnd some controversy bout its proper indiction, ssocited compliction rtes nd the use of therpeutic lterntives persists. The purpose of this monogrph ws to collect nd discuss much of the rdiologicl nd imging informtion vilble. It ims to summrize nd

14 2 1 Introduction fcilitte ccess to currently existing knowledge on these complex, incompletely understood, nd sometimes chllenging lesions. Views nd opinions stted below reflect personl experience in clinicl nd endovsculr mngement of ptients with DCSFs, demonstrting the evolution of miniml invsive techniques, prticulrly the incresing use of trnsvenous pproches to the CS. Insights into ll spects of these interesting cerebrovsculr lesions, including their ntomy, etiology, clssifiction, clinicl presenttion, imging techniques nd hemodynmics, re provided. Vrious current tretment options nd their role in ptient mngement re described, such s conservtive mngement, mnul compression, controlled hypotension, rdiosurgery, surgery, but foremost endovsculr therpy. Percutneous ctheteriztion techniques re covered in greter detil with gret emphsis on trnsvenous ccess routes nd the progress tht hs been mde since Hlbch et l. (1989) published the first relevnt series. This volume is intended s reference nd guide for neurordiologists, neurosurgeons, neurologists nd ophthlmologists, who see ptients with DCSFs in their prctice. References Dndy W (1937) Crotid-cvernous neurysms (pulsting exophthlmos). Zentrlbl Neurochir 2: Cstigne P, Lplne D, Djindjin R, Bories J, Augustin P (1966) Spontneous rteriovenous communiction between the externl crotid nd the cvernous sinus. Rev Neurol (Pris) 114:5 14 Newton TH, Hoyt WF (1970) Durl rteriovenous shunts in the region of the cvernous sinus. Neurordiology 1:71 81 Djindjin R, Cophignon J, Comoy J, Rey J, Houdrt R (1968) Neuro-rdiologic polymorphism of crotido-cvernous fistuls. Neurochirurgie 14: Djindjin R, Mnelfe C, Picrd L (1973) Externl crotid-cvernous sinus, rteriovenous fistule: ngiogrphic study of 6 cses nd review of the literture. Neurochirurgie 19: Newton TH, Hoyt WF (1968) Spontneous rteriovenous fistul between durl brnches of the internl mxillry rtery nd the posterior cvernous sinus. Rdiology 91: Brrow DL, Spector RH, Brun IF, Lndmn JA, Tindll SC, Tindll GT (1985) Clssifiction nd tretment of spontneous crotid-cvernous sinus fistuls. J Neurosurg 62: Hmby W (1966) Crotid-cvernous fistul. Springfield Mulln S (1974) Experiences with surgicl thrombosis of intrcrnil berry neurysms nd crotid cvernous fistuls. J Neurosurg 41: Hlbch VV, Higshid RT, Hieshim GB, Hrdin CW, Pribrm H (1989) Trnsvenous emboliztion of durl fistuls involving the cvernous sinus. AJNR Am J Neurordiol 10:

15 Historicl Considertions 2 CONTENTS 2.1 Arteriovenous Fistul nd Pulsting Exophthlmos Angiogrphy Therpeutic Mesures Emboliztion 10 References Arteriovenous Fistul nd Pulsting Exophthlmos Willim Hunter in 1757, is credited with recognizing n rteriovenous neurysm s direct communiction between the rtery nd vein, while previous observers hving interpreted the lesion s simple neurysm (Hunter 1762). He studied two ptients in whose rms the vessels hd been injured by phlebotomy, n opertion extensively prcticed t tht time not only by physicins but lso chrltns nd brbers. His first ccurte pprisl of n rteriovenous communiction, described not only the bruit nd the plpble thrill t the site of communiction but lso the mrked dilttion nd tortuosity of the rtery t the site of the fistul: In former pper upon neurysm, I took notice of species of tht complint, which, so fr s I know, hd not been mentioned by ny other uthor; where there is n nstomosis or immedite connection between the rtery nd the vein t the prt where the ptient let blood in consequence of the rtery being wounded through the vein; so tht blood psses immeditely from the trunk of the rtery into the trunk of the vein nd so bck into the hert. It will differ in its symptoms from the spurious neurysm principlly thus. The vein will be dilted or become vri- cose nd it will hve pulstile jrring motion on ccount of the strem from the rtery. It will mke hissing noise, which will be found to correspond with the pulse for the sme reson. The blood of the tumor will be ltogether or lmost entirely fluid becuse of its constnt motion. Cleghorn (1769) suggested the nme neurisml vrix for the direct communiction. Without postmortem evidence for his conclusions, Benjmin Trvers in 1809 described first pulsting exophthlmos nd designted it s Aneurysm per nstomosin or cirsoid neurysms of the orbit (Fig. 2.1) (Trvers 1811). Three yers lter on April 7, 1813, Dlrymple (1815) operted second, similr cse of pulsting exophthlmos nd followed Trvers explntion for its etiology, s did the mjority of subsequent writers until In these yers the French ntomist Breschet (1829) studied in detil the vsculr ntomy of the brin nd skull, s well s the hed nd neck re nd provided color pltes in most outstnding qulity (Fig. 2.2). Guthrie (1827) recorded during the sme period the first necropsy of ptient with pulsting exophthlmos nd found insted of cirsoid neurysm n neurysm of the ophthlmic rtery: On the deth of the ptient n neurism of the ophthlmic rtery ws discovered on ech side, of bout the size of lrge nut. Thus, he ws led to believe erroneously tht it ws the usul lesion in pulsting exophthlmos nd dvocted this s etiology of ll reported cses of pulsting exophthlmos. This ws supported by Busk s (1839) utopsy findings of nother cse. However, in Frnce, four yers prior to the Busk report, it ws Bron (1835) who is given credit for being the first to discover direct communiction between the internl crotid nd the cvernous sinus. Even though his report ws so brief tht it ws

16 4 2 Historicl Considertions Fig The first description of pulsting exophthlmos. Engrvings of Trvers ptient before nd fter opertion. (From Med Chir Trns 1813) not noticed by mny of his collegues, he in fct estblished very erly on the most importnt spect in the etiology of pulsting exophthlmos, nmely its intrcrnil cuse. Gendrin (1841) found the sme communiction, nd in 1856, Nelton (Henry 1959) reported the similr finding. In 1851, Nelton (1857) lso described the conversion of n rteriovenous neurysm into simple neurysm due to thrombophlebitis nd reported tht the simple neurysm ws cured by proximl ligtion. Broc (1856) pplied the nme vricose neurysm to the direct communiction by wy of flse neurysm sc interposed between rtery nd vein. Guttnus (1785) is given credit for the first cure of brchil neurysml vrix by combintion of direct nd indirect methods of compression. In 1833, Breschet described two cses of rteriovenous fistul treted by ligtion of the rtery proximl to the opening, followed in ech instnce by gngrene of the limb beyond the fistul. Bron (1835) first showed tht the essentil defect of crotid cvernous sinus fistul ws n opening between the internl crotid rtery nd the cvernous sinus. Delens (1870) found in cdvers tht the crotid rtery, injected under pressure, ruptured most often in the segment coursing within the cvernous sinus. It ws lso found tht, in certin cses, preexisting neurysms of the ICA hd ruptured into the CS either spontneously or s result of trum. Direct trum to the crotid cvernous sinus re by knitting needles, bullets, umbrell stves nd similr shrp-pointed objects ws lso reported to hve produced the lesion. Interestingly, the concept of ruptured crotid rtery in its course through the cvernous sinus, s supported by Delens, Nelton nd other French uthors, ws only reluctntly ccepted in Englnd. Trvers, Dlrymple, nd fter them mny English physicins, considered pulsting exophthlmos cused by n introrbitl neurysm. This ws in prt due to Hulke (1859) nd Bowmn (1860), who observed cse in which they found ll signs of pulsting exophthlmos including dilted ophthlmic vein on utopsy, but no ltertions in the crotid rtery. Thus, they continued to suggest the theory of ophthlmic rtery neurysms bsed on the erlier observtion of Guthrie (1827). Nunneley, Chief Surgeon of the Leeds Eye nd Er Infirmry, ws the first English uthor who ccepted tht pulsting exophthlmos might indeed hve n intrcrnil origin Nunneley (1864). The excellent thesis of Delens (1870), nd the lectures of Timothy Holmes (Holmes 1873) before the

17 2.1 Arteriovenous Fistul nd Pulsting Exophthlmos 5 b c Fig. 2.2 c. Vsculr ntomy of the hed nd neck re s seen by Breschet in 1829, from Recherches Antomique, Physiologique sur le Systeme Veineux et Specilment sur le Cnux Veineux des Os. Superficil veins of the hed nd neck. Shown is the extensive network of sclp veins, minly the superficil nd middle temporl vein, frontl veins, ngulr vein, nd fcil vein drining into the externl jugulr vein. b Deep veins of the hed nd neck nd their connection to the introrbitl veins. The pterygopltine foss is shown with the pterygoid plexus tht drins vi the retromndibulr vein into the externl jugulr vein. The internl jugulr vein, mrkedly lrger in cliber, lies underneth nd nterior to the externl jugulr vein. c Cerebrl veins nd intrcrnil sinuses. The ICA is shown with the cvernous sinus, intercvernous sinus, the inferior petrosl sinus nd bsilr plexus. The drwing lso shows the communiction between jugulr bulb, intrspinl veins nd vertebrl plexus. Note the more plexiform ppernce of both, the CS nd the IPS Royl College of Surgeons in Englnd between on the The Surgicl Tretment of Aneurism in Its Vrious Forms helped the slow cceptnce of the intrcrnil cuse for n orbitl symptomtology s rteriovenous neurysm within the cvernous sinus (Fig. 2.3). Sttler, Professor of Ophthlmology in Erlngen, provided the first detiled review of 106 cses collected to tht dte (Sttler 1880). He ws opposed to the reports of Guthrie, Busk nd Hulke nd supported the etiology of n rteriovenous communiction tht in his opinion must hve been frequently overlooked in previous descriptions. One of the interesting findings in his work is tht in some cses significnt thrombosis of the cvernous sinus or the superior ophthlmic vein ws found. Nevertheless, he never ssumed this per se could cuse or trigger the typicl symptoms of pulsting Exophthlmos. Most reports nd studies of the following 50 yers (including Dndys work) re bsed on the initil mteril provided by Sttler (1920, 1930), who continued his work nd reported on 322 ptients.

18 6 2 Historicl Considertions Dilted frontl vein (FV) Dilted superior ophthlmic vein (SOV) Optic nerve Ophthlmic rtery (OA) Internl crotid rtery (ICA) Dilted cvernous sinus (CS), cut open Ter in the crotid wll (ICA cut open) Optic nerve Dilted superior petrosl sinus (SPS) Frctured nd dislocted portion of the petrous pyrmid Optic nerve Internl crotid rtery (ICA) Dilted intercvernous sinus (ICS) Internl crotid rtery (ICA) Frcture line t the dorsum selle Fig Delens (1870) cse. The second illustrted cse of trumtic CCF from the clinic of Nelton fter n utopsy report of 17-yer-old girl who developed pulsting exophthlmos fter fll out of crrige in July The ptient presented 6 months lter nd died 7 dys following n unsuccessful opertion due to pyemi. A frcture line running through the posterior nd middle crnil foss is seen, s well s portion of the petrous pex tht is detched nd likely penetrted the crotid rtery. Dringe into the enlrged intercvernous sinus, superior ophthlmic vein nd superior petrosl sinus. (From Sttler 1920, 1930: Pulsierender Exophthlmus. In: Hndbuch der Gesmten Augenheilkunde, Springer, pp ) de Schweinitz nd Hollowy (1908) in n nlysis of 313 previously reported cses found tht n bnorml communiction between the internl crotid nd cvernous sinus ws the most frequent cuse of unilterl exophthlmos, but tht n neurysm of the ophthlmic, or crotid rteries or even tumor of the orbit could produce the sme symptoms (Fig. 2.4). Locke (1924) comprehensively reviewed 588 cses of pulsting exophthlmos, of which 126 occurred spontneously nd 418 were of trumtic origin. Conducting creful necropsies, he lso found mong 33 spontneous cses tht only 16 were cused by communiction between the rtery nd sinus. Seven cses occurred due to tumor, three due to neurysm of the ICA, nd three due to neurysm of the ophthlmic rtery. In one cse no lesion ws found. In 17 cses of trumtic origin, utopsies showed tht 16 were due to direct communiction between the crotid rtery nd the cvernous sinus nd only one cse ws cused by n neurysm. Dndy (1935) nd others found in reviews of postmortem exmintions tht the opening between the rtery nd sinus hd vried in size from 1 mm to 10 mm nd (Loehr 1937) tht in few instnces the rtery hd been completely severed, the two ends lying within the sinus but seprted by centimeter or more. In some cses the crotid wll ppered to hve wekened by rteriosclerosis nd given wy, s do vessels in the brin. 2.2 Angiogrphy Only one yer fter the invention of X-rys by Conrd Roentgen in 1895, Hscheck nd Lindenthl (1896) performed the first ngiogrm on n mputted hnd using chlk s contrst gent. On the 28th of June, 1927 during his ninth ttempt, Egz Moniz (Moniz 1927) performed the first cerebrl ngiogrm ever, using strontium bromide in 20-yer-old boy who ws blind becuse of tumor in the sellr region. This procedure required direct contrst injection fter surgicl exposure nd temporry ligtion of the crotid rtery. It ws the first in vivo rdiogrm of the cerebrl rteries showing displcement nd stretching of the vessels by the tumor nd represents milestone in the development of dignostic rdiology. Terry nd Mysel (1934) performed the first ngiogrm in ptient with pulsting exophthlmos by injecting thorium dioxide sol. They were ble to demonstrte the AV fistul between the internl crotid rtery nd the internl jugulr vein in the upper prt of the neck. In the sme yer, Ziedses des Plntes (1934) presented thesis in which he introduced two mjor elements of the twentieth century rdiology, film tomogrphy nd film subtrction ngiogrphy. The

19 2.2 Angiogrphy 7 ltter, which ws the predecessor to X-ry digitl subtrction ngiogrphy (DSA), ws n importnt step forwrds since it llowed for seprting the relevnt vessel informtion from superimposing osseous bckground. Some of the first ngiogrphic imges of ptients with pulsting exophthlmos were presented by Loehr (1936) nd Toennis et l. (1936) in Berlin. Loehr (1936) initilly did not express much enthusism when he stted: Contrry to genuine neurysms, the trumtic forms t the bse of the crnium cnnot be demonstrted so well by rteriogrphy, since the blood nd with it the contrst substnce cn pss into the sinus cvernous without coming into the hemisphere. Dndy (1937) who felt the clinicl signs of pulsting exophthlmos re so striking tht confirmtion by nother dignostic procedure is not required, ws initilly very reluctnt to see the need for cerebrl ngiogrphy. Referring to the work of Terry nd Mysel he stted Since the clinicl picture of these neurysms is unmistkble, one cnnot be justified in unnecessry procedures merely to disply them more grphiclly. There hs been noteworthy contribution to the subject by Wolff nd Schmid (1939) from Wuerzburg in 1939, who crefully studied the venous dringe pttern of crotid cvernous fistuls nd developed the first clssifiction for CCFs (Figs. 2.5 nd 4.2) tht brought more cceptnce to cerebrl ngiogrphy. List nd Hodges (1945) confirmed this work nd were followed by others ( Rmos nd Mount 1953; Rney et l. 1948; Alpers et l. 1951). Rney nd Rney (1948) recognized the vlue of rteriogrphy in crotid cvernous fistuls for ssessment of the collterl circultion through the circle of Willis; in cses where no dye would fill cerebrl vessels, trpping procedure could probbly be performed sfely. Flconer nd Hore (1951) reported on ptient with trumtic crotico-cvernous fistul tht ws mintined through the externl crotid e d b c d m.r.s. b l g h h h m g c j e b k l m Fig. 2.4,b. Veins of the orbit fter Festl: Recherches ntomiques sur les veines de l orbite, leurs nstmoses vec les veines des regiones voisins, Thesis, Pris In Die Erkrnkungen der Orbit, Birch-Hirschfeld, The illustrtions show nicely the introrbitl ntomy nd the reltionship between eye bulb, muscles nd veins. The communictions of the superior ophthlmic vein with the ngulr vein nd fcil vein, frontl nd suprorbitl vein re demonstrted. In ddition, the superior nd inferior root of the superior ophthlmic vein, s discussed in more detil lter (Chp. 3), is shown

20 8 2 Historicl Considertions Fig Probbly one of the first ngiogrms of cvernous sinus fistul, obtined nd published in 1939 (Wolff nd Schmidt 1939). The uthors described the leptomeningel dringe vi the bsl vein of Rosenthl (6) nd n nstomotic vein (5) which they were unble to identify s lterl (or nterior?) mesencephlic vein, probbly filled vi the SPS rtery. They were ble to demonstrte ngiogrphic evidence of cerebrl rteries filled vi the internl mxillry nd ophthlmic rtery nstomoses. Prsons et l. (1954) reported the first spontneous occlusion of CSF following ngiogrphy. Higzi nd El-Bhnwy (1963) demonstrted the vlue of cerebrl ngiogrphy for differentil dignosis in cses where other diseses my present the typicl clinicl picture of clssicl crotid cvernous fistul. The uthors emphsized its vlue in severl spects: the presence of fistul, its exct site, the differentition between unilterl nd bilterl fistul etc. Hyes (1958) suggested the differentition in high-flow nd low-flow fistuls. It becme more obvious tht ngiogrphy hd significnt vlue not only s new dignostic tool but lso s mesure for efficcy of tretment methods nd for development of new strtegies. A few yers lter (Hyes 1963) he showed ngiogrphic evidence in severl cses of crotid cvernous fistul tht reoccurred fter ligtion due to collterl flow through externl crotid brnches. Possibly the first who observed DCSF ngiogrphiclly ws Lie in 1961, when he performed n ngiogrm in ptient with non-pulsting exophthlmos (Lie 1968, Fig. 28 there). He noted bilterl bnorml network ner the sellr region, supplied by brnches of the internl mxillry nd middle meningel rtery. Although he could not identify fistul to the CS he commented this must hve been n bnorml form of crotid cvernous shunt. Cstigne et l. (1966) were probbly the first to recognize nd demonstrte the ngiogrphic chrcteristics of durl cvernous sinus fistul. By 1939, Steinberg (1939) nd Cstellnos et l. (1937) hd lredy investigted the possibility of performing iodine-enhnced ngiogrphy using intrvenous injection. In the 1970s the development of rel-time digitl fluoroscopic imge processors ws begun nd led to the introduction of three systems in 1980 (Mistrett nd Grist 1998). Becuse intrvenous DSA provided inconsistent imge qulity nd often required repeted injection to overcome rtery-vein overlp, rdiologist recognized tht digitl rel time subtrction cpbilities could be used with intr-rteril injections (intr-rteril DSA). Intr-rteril DSA hs dvnced nd improved remrkbly since, nd despite significnt technologicl efforts nd investments in non-invsive imging technique, is still the gold stndrd for vsculr dignosis due to its superior sptil resolution, unmtched time resolution nd overll imge qulity. The development of three-dimensionl DSA in the lte 1990s hs opened wide rnge of new possibilities for visuliztion of cerebrl vsculr ntomy. Recently, the introduction of FD technology pushes gin the limits of high qulity vsculr imging (see more detil in Sect ).

21 2.3 Therpeutic Mesures Therpeutic Mesures When Trvers ws demonstrtor of ntomy t Guys Hospitl in London in 1811, he ligted successfully the left common crotid rtery for crotid cvernous sinus fistul. Thus he becme not only the first to describe the condition but lso the first to describe its therpy even before the discovery of nesthesi. Frnce of Guys Hospitl, in 1853 ws the first to describe spontneous thrombosis of the fistul (Frnce 1855) without digitl compression or ligtion. Gioppi (1858) of Pdu suggested intermittent digitl compression of the crotid rtery in 1856, nd he is credited with being the first to use it successfully. Vnzetti (1858) very shortly fterwrd described this method in more detil. Brinrd, Professor of Surgery t Rush Medicl School in Chicgo in 1852 ttempted s one of the first to cure n erectile tumor of the orbit by injecting lctte of iron nd puncture with hot needles in 35-yer-old ptient (Brinrd 1853) in whom ligtion yer erlier hd filed (Kosry et l. 1968). Similr ttempts were mde t the sme time in Europe by Girldes ( ) nd Prvez et l. (1853), who used perchloride of iron for injection, which ws, however, followed by gngrene. These ttempts represented different therpeutic pproch, nmely ignoring the inflow trcts nd ttempting to obliterte the outflow trct of crotid cvernous complex. Petrequin (1846), Bourguet (1855), nd Ciniselli (1868) suggested electropuncture using cupuncture needles mde of pltinum with n iron tip. Lnsdown (1875), surgeon t Bristol Generl Hospitl, ttempted to cure trumtic cse with ligtion nd removl of the vricose ophthlmic veins. This technique ws lso performed nd dvocted by Sttler (1880, 1905) (Fig. 2.6). In Berlin, Zeller (1911) questioned ligtion of the crotid rtery s suitble tretment nd opposed to the dngerous bilterl ligtion in cses of retrogrde filling of the fistul vi collterl vessels through the circle of Willis or the externl crotid system. He suggested new pproch nmely the voellige Ausschltung des Arterienstueckes in dem ds Loch sitzt, us dem rteriellen Kreisluf durch proximle nd distle Ligtur, the trpping of the fistulous crotid by proximl ligtion in the neck nd distl ligtion proximl or immeditely distl to the origin of the ophthlmic rtery. After studies in cdvers he ws ble to demonstrte in 1908 tht this procedure ws effective. Even though his ptient died due to intropertive rupture of the ICA cused by n unfortunte mistke of his ssistnt, this pproch ws dopted lter by Hmby nd Grdner (1933), Dndy (1937) nd others (Toennis 1937). Locke (1924) described n pprtus for externl compression of the lrge vessels in the neck, consisting of wooden frme nd rubber bnd (Fig. 2.7). Brooks (1931), in discussing pper by Nolnd nd Tylor before the Southern Surgicl Assocition, reported unique method for oblitertion of CCF: He opened the crotid nd pcked strip of muscle between the clmps. The incision of the rtery ws then closed nd the clmps removed so tht the blood strem would force the muscle piece downstrem into the fistul site. The ptient lost vision but otherwise recovered. This procedure, lthough never relly proven, hs since been considered the first successful rteril emboliztion. Whether or not the muscle piece in fct plugged the fistul or just occluded the crotid remins unknown. Brooks himself discussed criticlly in his originl description: Owing to the mrked curvture of the bony cnl through which the crotid rtery enters the intrcrnil cvity, it is of course, difficult to be sure tht our ttempt to obliterte the rtery t the site of fistulous opening ws successful. We believe however this ws ccomplished. The procedure ws modified by Grdner (Hmby nd Grdner 1933) on My 15, 1931 who used piece of muscle the size of pe (5 mm) nd ttched silver clip for rdiogrphic locliztion. The ide here ws to mke the embolus smll enough to enter the venous component (sinus) of the fistul where it would be n ctive nucleus for the rpid production of thrombus tht would close the opening. The bruit immeditely cesed nd proptosis improved until it reoccurred 8 dys lter nd the ptient underwent ligtion. In 1934, Dndy occluded the intrcrnil ICA with silver clip (Dndy 1937), followed by Wlker nd Allegre (1956), Adson (1942) nd Toennis et l. (1937). Gurdjin (1938) reported cse in which externl nd internl crotid rteries were ligted nd cm muscle plugs were introduced to remove the crotid opening t the site of the lesion. This re-

22 10 2 Historicl Considertions b c d Fig. 2.6 d. One of Sttler s ptients (Cse Wiesinger, 1903): Trumtic CCF in 38-yer-old mle who presented in 1903 with bilterl pulsting exophthlmos of 5 yers stnding. Extreme vricose diltion of frontl nd suprorbitl veins (,b). Three weeks fter bilterl ligtion nd resection of the SOV (c) nd 17 months fter cure of the fistul (d) sulted in improvement of proptosis, ophthlmoplegi nd vision. Jeger (1949) reported the successful combintion of intrcrnil clipping of the internl crotid, muscle embolus nd ligtion of the crotid in the neck in 12-yer-old boy 7 yers erlier. He ws ble to control the position of the embolus using X-ry identifiction of the silver clip t the crotid cvernous opening. The ptient ws immeditely nd completely cured when seen gin for 7-yers follow-up. In 1959 he reported on six cses being successfully treted using the sme technique. Prkinson (1963,b) devised the direct surgicl pproch to the cvernous sinus using hyperthermi nd crdic rrest. Although his first ptient died due to pulmonry complictions, this new pproch opened the door for microsurgicl techniques in the tretment of crotid cvernous fistuls, some of which re employed until tody in intrctble cses. 2.4 Emboliztion On September, 25th in 1963, Lng nd Bucy (1965) were ble to successfully tret cse with free emboliztion of muscle piece with silver clip nd referred to severl dditionl previous reports in the literture. While ll of those hd to undergo intrcrnil ligtion becuse of persisting symptoms, the cse of Lng nd Bucy ws cured by embolism

23 2.4 Emboliztion 11 Fig Instrument employed to compress the common crotid rtery ginst the trnsverse process of the cervicl vertebre, from Locke (1924): Intrcrnil rteriovenous neurysm or pulsting exophthlmos. The frme ws plced round the neck nd the rubber bnd ws then stretched over the screw lone. The control ngiogrm confirmed complete thrombosis of the ipsilterl internl crotid while good cross flow ws preserved from the contrlterl territory. Kosry et l. (1968) reported the successful emboliztion of CCF using porcelin beds. To void distl migrtion of the embolus, Arutiunov et l. (1968) hd developed prticulr technique, consisting of clipped muscle embolus ttched to nylon string. This llowed controlling of the embolus to the fistul site under X-ry, nd securing it in plce by nchoring it to the ICA ligtion. This technique ws performed in 13 ptients with 100% success nd cn be considered the precursor of detchble blloons introduced few yers lter. Prolo nd Hnbery performed in October 1969 n occlusion of CCF using nylon blloon ctheter through n rteriotomy in the common crotid rtery chieving complete cure. Ismt et l. (1970) ingeniously embolized the fistul nd preserved the ptency of the crotid rtery using previously mgnetized metl clip on muscle embolus. The uthors thought of guiding the embolus by n electromgnet over the skin covering the superior-nterior prt of the cvernous sinus of the left zygom, which ws however not necessry becuse the pressure nd flow ws such s to conduct the embolus properly into the fistulous opening in its venous side without interfering the ptency of the crotid. In 1973, Blck et l. followed this concept of flow-directed muscle emboliztion nd were ble to mintin the ptency of the crotid rtery. Serbinenko (1971) published n rticle bout the use of detchble blloons for occlusion of cerebrl vessels. This first nd quite significnt contribution ws published only in the Russin literture nd ws initilly missed by most Westerners. It ws fter his second report, this time published in the English literture (Serbinenko 1974), tht collegues like Chermet et l. (1977) followed in his footsteps. Peterson et l. (1969) deserves credit for the first retrogrde venous pssge through the SOV using copper wire nd positive current for electrocogultion of CCF. Kerber et l. (1979) pioneered the use cynocrylte to occlude crotid cvernous fistul with preservtion of the crotid rtery flow. He used prticulr clibrted lek blloon microctheter in three ptients nd ws ble to occlude the fistul but with persistent neurologicl compliction in one. These nd other pioneering efforts opened the door to new er of therpeutic mngement of crotid cvernous fistuls: endovsculr tretment using rteril emboliztion. Miniml invsive mngement hs been firmly estblished since, hs continuously dvnced over the following yers, nd hs eventully been ugmented by trnsvenous occlusion (TVO) techniques in the 1990s (more in Chp. 8). References Adson A (1942) Surgicl tretment of vsculr disese ltering function of the eyes. Am J Ophthlmol 25:824 Alpers B, Schlezinger N, Tssmn I (1951) Bilterl internl crotid neurysm involving cvernous sinus, right crotid rtery-cvernous fistul nd left scculr neurysm. Arch Ophthl 46: Arutiunov AI, Serbinenko FA, Shlykov AA (1968) Surgicl tretment of crotid-cvernous fistuls. Prog Brin Res 30: Bron M (1835) Comptu rendu des trvux de l societe ntomique pendnt l nnee Bull Acd Med. Pris 1:178 Blck P, et l. (1971) Crotid-cvernous fistul: technique for occlusion of fistul with preservtion of crotid blood flow. Trns Am Neurol Assoc. 96:

24 12 2 Historicl Considertions Blck P, et l. (1973) Crotid-cvernous fistul: controlled embolus technique for occlusion of fistul with preservtion of crotid blood flow. Technicl note. J Neurosurg. 38(1): Bourguet (1855). Note sur un cs d nevrisme de l rtere ophthlmique et de ses principles brnches gueri du moyen des injections de perchlorure de fer. Gz. med. de Pris 49:772 Bowmn (1860) Med Times Gz II Brinrd (1853) Cse of erectile tumor of the orbit, cured by iniltrtion with the solution of lctte of iron nd puncture with hot needles fter ligtion of the crotid rtery hd filed etc. Lncet August 20th:162 Breschet G (1829) Recherches ntomique, physiologiques sur le systeme veineux et specilment sur les cnux veineux des os. Pris, Vileret et Rouen Breschet, G. (1833) Memiore sur les neurysmes. Mem Acd Roy Med (Pris). 3:101 Broc P (1856) Des nevrismes et de leur tritment. Pris, 1856 Brooks, B. (1930) The tretment of trumtic rteriovenous fistul. South. med. J. 23: Brooks B (1931) Discussion of Nolnd L nd Tylor AS. Trns South Surg Ass 43: Busk (1839) Med Chir Trns xxii:124 Cstigne P, Lplne D, Djindjin R, Bories J, Augustin P (1966) Spontneous rteriovenous communiction between the externl crotid nd the cvernous sinus. Rev Neurol (Pris) 114:5 14 Cstellnos A, Pereirs R, Grci A (1937) L ngiocrdiogrfi rdiopqu. Arch Hbn 31: Chermet M, Cbnis EA, Debrun G, Hut J (1977) Crotidocvernous fistul treted with infltble blloons. Bull Soc Ophtlmol Fr 77: Ciniselli (1868) Dell electropunctur nell cur degli neurismi dell Aort thorcic. Gz Med Itlin Lombrd 39 Cleghorn G (1769) A cse of neurysml vrix. Med Obs Soc Phys (London) 3:110 Dlrymple W (1815) A cse of neurysm by nstomosis in the left orbit, cured by tying the common trunk of the left crotid rtery. Trns Med Chir Soc Edinburg 6: Dndy W (1935) The tretment of crotid cvernous neurysms. Ann Surg 102: Dndy W (1937) Crotid-cvernous neurysms (pulsting exophthlmos). Zentrl Bl Neurochir 2: Dndy WE. (1957) The tretment of crotid cvernous rteriovenous neurysms. In: Selected writings of Wlter E. Dndy, C.E. Trolnd nd F.J. Otensek, Editors. Chrles Thoms: Springfield Ill. p , De Schweinitz G, Hollowy T (1908) Pulsting Exophthlmos: its etiology, symptomtology, pthogenesis nd tretment. Sunders, Phildelphi Delens E (1870) De l communiction de l crotide interne et du sinus cverneux. Pris Flconer M, Hore R (1951) Crotico-cvernous fistul cusing pulsting exophthlmos with cerebrl blood flow mintined through the externl crotid rtery. Proc R Soc Med Frnce J (1855) Cse of pulsting swelling of the orbit. Guys Hosp Rep Series 3:58 Gendrin MAN (1841) Lecons sur l mldies du coeur. Billiere (Pris) 1: Gioppi G (1858) Aneurism dell rteri oftlmic. Giornle d oftlmol Itlin Girldes M ( ) Presenttion de mlde. Bull Soc Chir (Pris) 4:22 Guttnus C (1785) De externis neurysmtibus. Scriptorium Ltinorum de Aneurysmtibus. Collectio. 101:234 Gurdjin ES (1938) Pcking of the internl crotid rtery with muscle in tretmentment of crotid-cvernous rteriovenous neurysms. Arch Ophthl 19: Guthrie (1827) Opertive surgery of the eye, 2nd edn. Burgess nd Hill, London Hmby W, Grdner W (1933) Tretment of pulsting exophthlmos with report of 2 cses. Arch Surg 27: Hmby, WB, (1966) Crotid-cvernous fistul. Vol. III. Springfield: Chrles C Thoms. 9 15; Hschek E, Lindenthl T (1896) Ein Beitrg zur prktischen Verwertung der Photogrphie nch Roentgen. Wien Klin Wochenschr 9:63 64 Hyes GJ (1958) Crotid cvernous fistuls: dignosis nd surgicl mngement. Am Surg 24: Hyes GJ (1963) Externl crotid-cvernous sinus fistuls. J Neurosurg 20: Henry (1956) Considertion sur l nevrisme rteriosoveineux. Pris, p 13 Henry, J (1959) Contribution l'etude de l ntomie des visseux de l orbite et de l loge cverneuse pr injection de mtieres plstiques, du tendon de Zinn et de l cpsule de Tenon. These. Pris: Typescript no Higzi, I. nd A. El-Bnhwy (1964). The Vlue of Angiogrphy in the Differentil Dignosis of Pulsting Exophthlmos; Report of 3 Cses. J Neurosurg 21: Holmes T (1873) Holmes lectures. Lncet ii:142 Hulke (1859) All the cpitl signs of orbitl neurism present, in mrked degree, but independently of neurism or ny erectile tumor. Ophthlmic Hosp Rep II:6 Hunter W (1762) Further observtions upon prticulr species of neurism. Med Observtions Inquiries 2: Hunter, W (1757) The history of n neurysm of the ort, with some remrks on neurysms in generl. Med Observ Inquir. (1):323 Ismt F, Sllers V, Mirnd AM (1970) Artificil emboliztion of crotid-cvernous fistul with post-opertive ptency of internl crotid rtery. J Neurol Neurosurg Psychitr 33: Jeger R (1949) Intrcrnil neurysms. South Surg 15: Kerber CW, Bnk WO, Cromwell LD (1979) Cynocrylte occlusion of crotid-cvernous fistul with preservtion of crotid rtery flow. Neurosurgery 4: Kosry IZ, Lerner MA, Mozes M, Lzr M (1968) Artificil embolic occlusion of the terminl internl crotid rtery in the tretment of crotid-cvernous fistul. Technicl note. J Neurosurg 28: Lng ER, Bucy PC (1965). Tretment of Crotid-Cvernous Fistul by Muscle Emboliztion Alone: The Brooks Method. J Neurosurg 22: Lnsdown F (1875) A cse of vricose neurism of the left orbit, cured by ligture of the disesed vessels. Brit Med J 1: Lie, T (1968) Congenitl nomlies of the crotid rteries. An ngiogrphic study nd review of the literture. Monogrph. Excerpt Medicl Foundtion. Amsterdm:

25 References 13 List C, Hodges F (1945) Intrcrnil ngiogrphy I. J Neurosurg 3:25 45 Locke C (1924) Intrcrnil rteriovenous neurysm or pulsting exophthlmos. Ann Surg 80:1 24 Loehr (1937) Schlefenlppentumoren, ihre Klinik und rteriogrphische Dignostik. Zbl. Neurochir. 2: p Loehr, W (1936) Hirngefessverletzungen in rteriogrphischer Drstellung. Zbl. Chir Loehr W, Jcobi W (1933) Die kombinierte Enzephlrteriogrphie. Fortschritte uf dem Gebiet der Roentgenstrhlen und Nuklermedizin. 44 Mistrett CA, Grist TM (1998) X-ry digitl subtrction ngiogrphy to mgnetic resonnce-digitl subtrction ngiogrphy using three-dimensionl TRICKS. Historicl perspective nd computer simultions: review. Invest Rdiol 33: Moniz E (1927) Rdiogrfi ds rteris cerebris. J Soc Ciencis Med Lisbo XCL:8 Nelton (1857) Cons. sur l nevrsime rterio-veneux, These d Henry, Pris Nunnely (1864) On vsculr protrusion of the eyebll, being second series of three cses nd two postmortem exmintions of so-clled neurism by nstomosis of the orbit with some observtions of the ffection. Med Times Gz 752:602 Prkinson D (1963) Norml ntomy of cvernous crotid nd its surgicl significnce. Presented t Annul Meeting of Hrvey Cushing Society, Phildelphi Prkinson D (1963b) Crotid cvernous fistul with pulsting exophthlmos: fortuitous cure. Cn J Surg 6: Prsons TC, Guller EJ, Wolff HG, Dunbr HS (1954) Cerebrl ngiogrphy in crotid cvernous communictions. Neurology 4:65 68 Peterson E, Vlberg J, Whittinghm D (1969) Electriclly induced thrombosis of the cvernous sinus in the tretment of crotid-cvernous-fistul. Presented t Fourth Interntionl Congress of Neurologicl Surgery nd Ninth Interntionl Congress of Neurology, Amsterdm, New York, London Petrequin (1846) Memoire sur une nouvelle methode pour guerir certins nevrisme. Gz Med Pris Petrequin (1845) Anevrisme de l`rtere opthlm. etc., in Comptes rendu de l`cdemie de science: Pris Prvez M, Girldes M, Debout M (1853) Revue Medico- Chirurgicle de Pris Prolo DJ, Hnbery JW (1971) Intrluminl occlusion of crotid-cvernous sinus fistul with blloon ctheter. Technicl note. J Neurosurg. 35(2): Rmos M, Mount L (1953) Crotid cvernous fistul with signs on contrlterl side. Cse Report. J Neurosurg 10: Rney R, Rney AA, et l. (1949). The role of complete cerebrl ngiogrphy in neurosurgery. J Neurosurg 6(3): Robb G, Steinberg I (1939) Visuliztion of the chmbers of the hert, the pulmonry circultion, nd the gret blood vessels in mn. AJR Am J Roentgenol 41:1 17 Sttler H (1880) Pulsierender exophthlmus. In: Grefe A, Semisch T (eds) Hndbuch der Gesmten Augenheilkunde. Engelmnn, Leipzig, pp Sttler H (1905) Ueber ein neues Verfhren bei der Behndlung des pulsierenden Exophthlmus. Klin Montsbl Augenheilk 4:1 6 Sttler, H (1920) Pulsierender Exophthlmus, in Hndbuch der Gesmten Augenheilkunde, A. Grefe nd T. Semisch, Editors. Julius Springer: Berlin. p Sttler, H (1930) Pulsierender Exophthlmus. In: Grefe A, Semisch T (eds) Hndbuch der Gesmten Augenheilkunde. Leipzig: Engelmnn, p Serbinenko F (1971) Ctheteriztion nd occlusion of mjor cerebrl vessels nd prospects for the development of vsculr neurosurgery [in Russin]. Vopr Neirokhir 35:17 27 Serbinenko FA (1974) Blloon ctheteriztion nd occlusion of mjor cerebrl blood vessels. J Neurosurg 41: Terry T, Mysel P (1934) Pulsting exophtlmos due to internl crotid-jugulr neurysm: the use of thorium dioxide sol in locliztion. JAMA 103: Toennis W (1936) Aneurysm rterio-venosusm, in Gefessmissbildungen und Gefessgeschwuelste des Gehirns, H. Bergstrnd nd H. Olivecron, Editors. Thieme: Leipzig. p Toennis W (1961) Zur Entstehung der Rezidive bei der Behndlung der Crotis-Sinus-Cvernosus-Aneurysmen und ihre Verhuetung. Arch. klin. Chir. (295): p Trvers B (1811) A cse of neurysm by nstomosis in the orbit, cured by the ligture of the common crotid rtery. Med Chir Tr 2:1 16 Vnzetti (1858) Secondo cso di neurism dell rteri oftlmic gurito coll compressione digitli dell crotide, e cenni prtici intorno questo metodo di curre gli neurismi. Pdov, nd Annli universli di medicin. CLXV:151 Wlker AE, Allegre GF (1956) Crotid-cvernous fistuls. Surgery 39: Wolff H, Schmid B (1939) Ds Arteriogrmm des pulsierenden exophthmus. Zbl Neurochir 4: Zeller O (1911) Die chirurgische Behndlung des durch neurysm rterio-venosum der crotis int. im Sinus cvernosus hervorgerufenen pulsirenden Exophthlmus Ein neues Verfhren. Deutsche Ztschr Chir 111:1 39 Ziedses des Plntes B (1934) Plnigrfie en Subtrctie. Thesis. The University of Utrecht, The Netherlnds

26 Antomy of the Cvernous Sinus 3 nd Relted Structures CONTENTS 3.1 Osseous Antomy Orbit Antomy of the Dur Mter nd the Crnil Nerves Autonomic Nervous System Vsculr Antomy Arteril Antomy Internl Crotid Artery Externl Crotid Artery Venous Antomy The Cvernous Sinus, Receptculum, Sinus Croticus (Rektorzik), Confluens Sinuum Anterius, Sinus Spheno-Prietle (Cruveilhier), Cvernous Plexus, Lterl Sellr Comprtment Tributries of the Cvernous Sinus (Afferent Veins) Dringe of the Cvernous Sinus (Efferent Veins) Other Veins of Importnce for the CS Dringe or for Trnsvenous Access to the CS 44 References Osseous Antomy (Figs. 3.1, 3.2) The cvernous sinus (CS) is closely relted to the osseous structures of the middle crnil foss such s the sphenoid bone nd the sell turcic. The sphenoid bone is situted in the bse of the skull nd consists of cuboid corpus contining the two sphenoid sinuses. The greter wings rise from the side of the corpus of the sphenoid bone nd project trnsversely, bending superiorly in their nterior portion. The lesser wings re two thin tringulr pltes of bone rising from the nterior spect of the sphenoid bone extending nerly horizontlly nd lterlly. The lterl extremity of the smller wing, slender nd pointed, pproches the greter wing but s rule ctully never touches it ( Trotter nd Peterson 1996). The superior surfce is slightly smooth nd concve forming the posterior prt of the floor of the nterior foss. The inferior surfce constitutes portion of the superior wll of the orbit nd overhngs the superior orbitl fissure (Trotter nd Peterson 1996), the elongted opening between the wings. The posterior border of the lesser wings forms prt of the boundry between the nterior nd middle crnil foss nd is prolonged t its medil extremity to form the nterior clinoid process. The superior surfce of the sphenoid corpus contins the groove for the pituitry glnd, Turkish sddle (sell turcic) ending nteriorly in the rounded elevtion of the tuberculum selle. The posterior boundry of the sell turcic is formed by qudrilterl plte of bone, the dorsum selle; its posterior surfce is sloped in continution with the dorsl surfce of the bsilr prt of the occipitl bone nd supports the pons nd the bsilr rtery. The superior ngles of the dorsum selle re surrounded by the posterior clinoid process which give ttchment to the tentorium cerebelli ( Trotter nd Peterson 1996). On the lterl surfce of the sphenoid, superior to the ttchment of the greter wings is the crotid groove (sulcus croticus), lodging the petrous nd cvernous segment of the internl crotid rtery (ICA). This sulcus is only posteriorly well formed where the rtery enters from the pex of the petrous bone. Medilly, the sulcus hs border, n osseous process, while lterlly bony projection, the lingul, continues posteriorly cross the formen lcerum (Keller et l. 1997). The middle clinoid processes re less well-defined nd lie posterolterl to the optic cnl. An interclinoid osseous or fibrous bridge between the nterior nd posterior clinoid processes cn be found in 4% 9% (Borb nd Al-Mefty 2000). A bony bridge between the nterior nd middle

27 16 3 Antomy of the Cvernous Sinus nd Relted Structures clinoid process cn form so-clled croticoclinoid formen (Henle). The ICA psses through this formen during its course from the posterior to the nterior cvernous segment (C5 nd C4 segment, see below) nd to the suprclinoid portion. Keller et l. (1997) found such formen in 18 of 135 skulls (13%), of which 6 were bilterl. The interclinoid formen, ccording to Keyes (1935), cn be divided into complete type, contct type nd incomplete type. These osseous detils re minly of interest for neurosurgeons when performing open surgicl procedures for tretment of CS lesions. They re of minor importnce in the context of endovsculr tretment (EVT). More noteworthy insted is n understnding of the ntomic reltionships between the CS nd its connecting vsculr structures, s well s of the relted bony cnls nd formin in the middle crnil foss nd the skull bse. They my serve s potentil routes for EVT of DCSF, nd re described below in more detil. The formen rotundum is short horizontl cnl (cnlis rotundus), locted in the nteromedil portion of the greter sphenoid wing nd is trversed by the mxillry nerve, the rtery of the formen rotundum nd smll emissry veins to the pterygoid foss. It lies directly below the medil end of the superior orbitl fissure nd is intimtely relted to the lterl wll of the sphenoid sinus. The formen is usully 3.4 mm long nd hs size of 3 3 mm to 4 5 mm (Lindblom 1936). The formen is best visible on rdiogrphs (Figs ) in Cldwell nd Wters projections (Shpiro nd Robinson 1967). Asymmetric enlrgement nd communictions with the superior orbitl fissure re rrely seen. However, postntl chnges in its dimensions nd shifts of its xis re described (Lng 1983). During postntl life the width of the cnl increses lmost continuously from 2.06 mm in neontes to 3.34 mm in dults. The intrcrnil opening cn be round or ovl (Sondheimer 1971) nd the formen rotundum does not in fct form n lmost circulr shpe, s initilly stted by Luschk (1967). The formen ovle is commonly n ovl shped hole in the greter sphenoid wing slightly lterl nd posterior to the formen rotundum (Shpiro nd Robinson 1967). It trnsmits the mndibulr division of the trigeminl nerve, the ccessory meningel rtery nd in the bsence of the cnliculus innomintus, the lesser petrosl nerve. As stted by Shpiro nd Robinson (1967), the formen ovle lso trnsmits emissry veins when the formen Veslii is not present. Lindblom (1936) found n verge dimeter of 5 8 mm. The distnce from the midline verges mm on the right nd mm on the left (Lng 1983). Shpiro nd Robinson (1967) further pointed out tht wide rnge of vritions exist, including bsence nd incomplete formtion, which determines whether or not the vsculr chnnels pssing through them exist or not. The sphenoidl emissry formen (formen venosum Veslii, sphenoid emissry formen, formen of Veslius, cnliculus sphenoidlis) is smll inconstnt perture in the greter wing, slightly nterior nd medil to the formen ovle nd mediodorsl to the formen rotundum. It trnsmits smll nerve (nervulus sphenoidlis lterlis) nd smll vein (bsl emissry vein) (Lng 1983) which connects the cvernous sinus with the pterygoid plexus ( Shpiro nd Robinson 1967). The formen hs n verge dimeter of 1.14 mm nd ws present in 49% on the right nd 36% on the left (Lng 1983). Occsionlly, the formen ovle my communicte with the formen venosum (Veslii). The venous segment my be seprted from the reminder by bony spur locted nteriorly nd medilly, producing duplicted formen (Lng 1983). The smll circulr formen spinosum is situted directly lterl nd posterior to the formen ovle nd gives pssge to the middle meningel rtery (MMA), ccompnying veins nd recurrent brnch of the mndibulr nerve. The formen spinosum my be bsent, symmetric, incompletely formed or remin confluent with the formen ovle ( Shpiro nd Robinson 1967). The formen spinosum nd the formen ovle re seprted by n verge distnce of 3.2 mm nd they lie n verge of 4.4 mm nterolterl to the crotid cnl (Borb nd Al-Mefty 2000). The distnce from the midline rnges from mm on the right nd from mm on the left (Lng 1983). Strictly speking the formen spinosum, like the formen rotundum, is bony cnl with length of bout 7 mm. The formen lcerum is short cnl (ctully not true formen) situted t the posterior end of the crotid groove, posteromedil to the formen ovle bounded behind by the petrous pex, in front of the body nd posterior border of the greter wing. It is pproximtely 1 cm long nd contins the ICA, meningel brnches of the scending phryngel rtery nd ccompnying sympthetic nd venous plexus. Some uthors found, however, tht no structures ctully pss through the formen (Pullus et l. 1977) nd the crotid rtery psses only through the upper hlf of it.

28 3.1 Osseous Antomy Fig. 3.1 c. Osseus ntomy of the prsellr region, the middle crnil foss nd the inner skull bse. Crnil/posterior view. Most horizontl perspective from posterior showing the opening of the suprorbitl fissure, the optic cnl nd the formen rotundum. b, c More verticl views from bove showing the formen ovle, spinosum, lcerum nd mgnum. 1 Anterior clinoid process 2 Lesser wing of sphenoid 3 Posterior clinoid process nd rough surfced upper prt of the clivus 4 Sell turcic 5 Optic cnl (intrcrnil perture) 6 Clivus 7 Pyrmid pex 8 Formen ovle 9 Formen rotundum 10 Superior orbitl fissure 11 Jugulr formen 12 Formen mgnum 13 Petroclivl fissure (sphenopetrosl synchondrosis), groove for IPS 14 Formen spinosum 15 Internl coustic porus 16 Crotid cnl, terminl portion of trnsverse petrosl prt 17 Formen lcerum 18 Formen venosum (of Veslius) 19 Greter wing of the sphenoid 20 Crist glli 21 Cribo-ethmoidl formen b c

29 18 3 Antomy of the Cvernous Sinus nd Relted Structures The cnliculus innomintus (cnl of Arnold) is minute cnl, posterior to the formen ovle nd medil to the formen spinosum (Shpiro nd Robinson 1967). The formen for the ophthlmomeningel vein, (Hyrtl) is situted in the greter wing, usully in the lterl hlf of the orbit (Shpiro nd Robinson 1967). The ophthlmomeningel vein connects orbitl with cerebrl veins nd commonly drins into the CS. A meningolcriml rtery my lso pss through this formen nd supply prt of the lcriml territory (Lsjunis et l. 1975). The jugulr formen is lrge perture nd lies posterolterl to the crotid cnl between the petrous, temporl nd occipitl bones. The formen is configured round the sigmoid sinus nd the IPS. Trversing structures re the sigmoid sinus nd jugulr bulb, inferior petrosl sinus (IPS), meningel brnches of APA nd occipitl rteries, crnil nerves (CNs) IX XI, tympnic brnch of CN IX (Jcobson s nerve), uriculr brnch of CN X nd the cochler queduct. It is generlly lrger on the right thn on the left side (68%) (Ktsut et l. 1997), nd my be subdivided into two comprtments (Inserr et l. 2004). The posterolterl prs venos, which contins the jugulr bulb nd the CNs X nd XI crnil nerves, is seprted from the nteromedil prs nervos, contining the IPS nd CN IX, either by bone, fibrous tissue or thin connective tissue. More often, however, there is no septtion nd the jugulr formen exists s one comprtment. The IPS, coursing from the cvernous sinus, empties into the medil spect of the jugulr bulb. The IPS often psses between the CN IX nteriorly nd the CNs X nd XI posteriorly Orbit (Fig. 3.2) The orbit is shped s qudrilterl pyrmid with its bse in plne with the orbitl rim. Seven bones conjoin to form the orbitl structure. The orbitl process of the frontl bone nd the lesser wing of the sphenoid form the orbitl roof. The orbitl plte of the mxill joins the orbitl plte of the zygom nd the orbitl plte of the pltine bones to form the floor. Medilly, the orbitl wll consists of the frontl process of the mxill, the lcriml bone, the sphenoid, nd the thin lmin ppyrce of the ethmoid. The lterl wll is formed by the lesser nd greter wings of the sphenoid nd the zygom. The mjor nerves nd vessels to the orbit nd the globe enter through three openings. The superior orbitl fissure (SOF) represents the elongted opening between the wings nd is situted between the orbits roof nd lterl wll. In its medil prt it is wide, but lterlly it nrrows nd turns upwrds. Its upper border is formed by the lower surfce of the lesser wing. Its medil boundry is formed by the inferior root of the lesser wing nd by prt of the sphenoid body, while the inferior border of the SOF belongs to the greter wing of the sphenoid. It cn be subdivided into medil nd lterl prt by the spine of the lterl rectus muscle. The lterl boundry of the SOF is situted n verge of 34 mm from the frontozygomtic suture. The gretest width of the fissure is usully found in the medil prt (Lng 1983). The lesser wing of the sphenoid hs durl lyer up to 3 mm thick, which covers it posteriorly together with the sphenoprietl sinus. The rmus communicns between the lcriml rtery nd the frontl brnch of the MMA is enclosed into this durl lyer. Above the common tendinous ring, the trochler nerve runs medilly nd forwrds, lterlly it is ccompnied by the lcriml nd frontl nerves. The course of the SOV vries but is usully through the lterl prt, occsionlly piercing the tendinous ring. Arising from the frontl brnch of the MMA, the communicting brnch with the lcriml rtery runs in the lterl ngle of the SOF or even through the greter wing. As rule it nstomoses with the lcriml rtery immeditely fter the ltter s origin from the ophthlmic rtery (OA). This communicting brnch cn be bsent or completely replce the OA, or the entire lcriml rtery cn rise from the MMA. Not infrequently, brnch from this nstomotic region runs bckwrds through the SOF to tke prt in the blood supply of the nerves, bone nd dur of the CS [nstomoses with the nteromedil brnch of the inferolterl trunk (ILT), see Sect ]. The inferior orbitl fissure (IOF) is formed by the greter wings of the sphenoid, the mxill, nd the pltines bones. Inferior orbitl fissure Infrorbitl nerve Zygomtic nerve Prsympthetic nerves to lcriml glnd Infrorbitl rtery Infrorbitl vein Inferior ophthlmic vein brnch to pterygoid plexus The optic cnl is locted t the pex of the orbit nd formed by the sphenoid bone.

30 3.1 Osseous Antomy b 14 Fig. 3.2,b. Osseous ntomy of the orbit. Anterior view. 1 Superior orbitl fissure 2 Optic cnl 3 Inferior orbitl fissure 4 Lesser sphenoid wing 5 Greter sphenoid wing 6 Nsion 7 Anterior ethmoidl formen 8 Posterior ethmoidl formen 9 Frontl formen 10 Infrorbitl formen 11 Ethmoid 12 Lcriml bone 13 Mxillry bone 14 Frontl bone 15 Zygom 16 Lcriml formen (Hyrtl) Optic cnl Optic nerve Ophthlmic rtery Centrl retinl vein The contents of these bony openings re s follows: Superior orbitl fissure Crnil nerves (CNs) III, IV, nd VI Lcriml nerve Frontl nerve Nsociliry nerve Orbitl brnch of middle meningel rtery Recurrent brnch of lcriml rtery Superior ophthlmic vein Inferior ophthlmic vein As rule the SOV runs lterl to the nerve group nd lterl to the common tendinous ring nd enters the CS from below (Lng 1983). In the medil wll of the orbit long the frontoethmoidl suture line lie the nterior nd posterior ethmoid formin through which the nterior nd posterior ethmoidl rteries pss.

31 20 3 Antomy of the Cvernous Sinus nd Relted Structures 3.2 Antomy of the Dur Mter nd the Crnil Nerves (Figs. 3.3, 3.4) In most textbooks it is ccepted tht the CSs re locted between the two lyers of the dur mter: the periostel (endostel) lyer forming the floor nd most of the medil wll of the CS, nd the durl lyer forming its roof, lterl wll, nd the upper prt of the medil wll (Umnsky nd Nthn 1987). According to Keller et l. (1997), only three surfces re covered by dur mter: the superior, the lterl nd the medil surfce. The dur mter of the medil surfce cn be very thin or fenestrted. The lower surfce is covered by the periost of the floor of the middle crnil foss. The roof of the CS is formed by the nterior extension of the tentorium nd the lterl extension of the diphrgm selle, while the lterl wll is formed by the dur propri of the middle crnil foss. Vrious concepts nd contrdictory descriptions of the lterl wll of the CS exist (Umnsky nd Nthn 1987). While most clssicl textbooks (Wrwick nd Willims 1973; Christensen nd Telford 1978) describe CNs III, IV nd V1, V2 s being embedded in the lterl wll, others (Pturet 1964; Rouviere 1970) differentite deep nd superficil lyer of the lterl wll, dividing the CS into two comprtments by septum nd Fig From Professior Elliot Smiths Textbook of Antomy, drwn from dissection in the Moorfield Pthologicl Museum 1 Infundibulum 2 Hypophysis 3 Diphrgm selle 4 Optic chism 5 Internl crotid rtery 6 Intercvernous sinus 7 3rd crnil nerve (superior division) 8 3rd crnil nerve( inferior division) 9 Lesser sphenoid wing 10 Trochler nerve 11 Internl crotid rtery 12 1st division, 5th crnil nerve 13 6th crnil nerve 14 2nd division, 5th crnil nerve 15 Sphenoid sinus 16 Cvernous sinus Fig Intrcrnil course of 3rd, 4th, nd 5th crnil nerves (from dissection of Wolff, E: Antomy of the Orbit, 1940) 1 Anterior clinoid process 2 Middle cerebrl rtery 3 Third nerve 4 Posterior cerebrl rtery 5 Fourth nerve 6 Superior orbitl fissure 7 1st division, 5th crnil nerve 8 6th crnil nerve 9 2nd division, 5th crnil nerve 10 3rd division, 5th crnil nerve 11 Middle meningel rtery 12 Gsserion gnglion 13 Internl crotid rtery 14 Sensory nd motor roots of 5th CN 15 Superior cerebellr rtery 16 Cerebellum

32 3.2 Antomy of the Dur Mter nd the Crnil Nerves 21 contining the ICA nd the sixth nerve in the deep nd running the CNs III, IV nd V1, V2 through this septum but not in the superficil lyer. Hrris nd Rhoton (1976) found two durl lyers of the lterl wll nd the nerves III, IV nd V1 running between them. Umnsky nd Nthn (1982) studied the lterl wll of the CS in 70 specimens nd found neither septum dividing the CS, nor single durl lyer. They observed superficil nd deep lyer tht were loosely ttched to ech other. The sheths of the CNs III, IV nd V1, V2 nd n often incomplete reticulr membrne extending between the sheths, formed the deep lyer. The CN III penetrtes the posterior CS vi the oculomotor formen (Umnsky et l. 1994; Inoue et l. 1990) nd courses nteriorly long the inferior surfce of the nterior clinoid process to rech the SOF through the nnulus of Zinn (Keller et l. 1997). The CN IV enters the CS in durl opening between the nterior nd posterior petroclinoid ligments (Umnsky nd Nthn 1982, 1987; Umnsky et l. 1994) nd crosses the III CN before entering the SOF without coursing through the Annulus of Zinn (Keller et l. 1997). The CN V1 courses from the Gsserin gnglion to the SOF inferior nd lterl to CN IV nd VI nd lies t the level of the SOF lterl to CN VI (Keller et l. 1997). The CN VI exits the pontomedullry junction nd courses through the subrchnoid spce to rech Dorello s cnl, smll tringulr spce formed beneth the petroclinoid ligment (Gruber s ligment) from the petrous pex to the posterior clinoid process (PCP) (Keller et l. 1997). This cnl contins the CN VI, usully lying lterlly to the IPS nd the dorsl meningel rtery from the meningohypophysel trunk (MHT). The bducens nerve is considered the only true intrcvernous nerve becuse of its course long the lterl surfce of the ICA fter leving Dorellos cnl (Keller et l. 1997). The rteril blood supply to these CNs is provided by multiple smll brnches minly rising from the inferolterl trunk (ILT) nd the MHT nd in prt rising from durl brnches of the ECA territory (see below). The development nd incresing use of trnsrteril emboliztions of DCSFs in the lte 1980s nd erly 1990s required us to study in more detil the rteril blood supply of the CNs in the CS region (Knosp et l. 1987b). At the present time this knowledge plys minor role due to the introduction of trnsvenous occlusion techniques, being incresingly employed to tret DCSFs since Autonomic Nervous System A detiled knowledge of the sympthetic nd prsympthetic nervous systems within the CS is still missing. It is known tht the sympthetic fibers course long the extrcrnil ICA, through the crotid cnl, to rech the petrous nd cvernous portion where they form plexus (Keller et l. 1997; Pullus et l. 1997; Mitchel 1953). Recently, prsympthetic fibers nd gngli hve been found in the CS s well, probbly connected with rmi orbitlis rising from the sphenopltine gnglion nd coursing through the SOF (Suzuki nd Hrdebo 1993). Bleys et l. (2001); while performing immunohistochemicl studies in rts, found tht the cvernous sinus gngli, consisting of the pterygopltine gnglion nd smll cvernous gngli, contribute to prsympthetic cerebrovsculr innervtion nd tht the cvernous nerve plexus nd bducens nerve re involved in the pthwy from these gngli to the cerebrl rteries. It cn be ssumed tht not only the sympthetic but lso prsympthetic innervtions ply role in the tone regultion of cerebrl vessels (Suzuki nd Hrdebo 1993). 3.3 Vsculr Antomy Arteril Antomy (Figs ) Internl Crotid Artery The ICA provides the nterior circultion tht supplies the lrgest prt of the cerebrum, the eye nd other introrbitl structures. The ICA lso gives rise to the brnches of the forehed nd the nose. The rtery begins t the bifurction of the common crotid rtery (CCA), usully t the level of the fourth cervicl vertebr, where it is enlrged to form the so-clled crotid sinus. Fischer (1938) divided the ICA into four segments: the cervicl, the petrous, the cvernous nd the cerebrl segment (Fig. 3.5). The cervicl segment extends lmost verticlly to the bse of the skull to rech the Apertur extern (externl perture) of the Cnlis croticus (crotid cnl) to enter the petrous bone. Just before entering

33 22 3 Antomy of the Cvernous Sinus nd Relted Structures C3 C1 C2 C4 C5 Cisternl (cerebrl) segment (C1 C2) Cvernous segment (C3 C5) Fig Originl clssifiction of ICA segments fter Fisher (1938, modified fter Kryenbuehl nd Ysrgil, 1997). Although somewht limited in ccurcy, it is still used by mny neurordiologists nd neurosurgeons. Cvernous segment: Between pex of petrous pyrmid nd bse of nterior clinoid process. Petrous segment: Between entrnce into the skull bse nd pex of petrous pyrmid. C5: Gnglion segment fter Fisher, becuse the rtery lies in close proximity to the frontl pole of the trigeminl gnglion. C4: Horizontl nd lterl to the pituitry glnd. C3: Below the bse of the clinoid process ( crotid knee ). C2: After penetrtion of the dur lterl to clinoid process, beneth the optic nerve. C1: Ascends nd divides t the circle of Willis. Crotid siphon fter Moniz: Curved portion within nd bove the cvernous sinus (C2 C5). The MHT most commonly rises from the C5 segment. Petrous cnl Petrous segment Cervicl segment C7 C6 C5 C4 Petrous cnl C3 C2 C1 C7: Communicting segment C6: Ophthlmic segment C5: Clinoid segment C4: Cvernous segment C3: Lcerum segment C2: Petrous segment C1: Cervicl segment Fig New clssifiction of ICA segments fter Bouthillier (1996). The new clssifiction divides the internl crotid rtery ccording to its course through the skull bse from proximl to distl. This pproch, using more logicl numericl scle, my be more prcticl since it follows the blood flow nd helps identify pertinent prts of ntomy, given current techniques of crnil bse surgery nd identified bnormlities. (Ogilvy, comment in Neurosurgery, Volume 38 (3), Mrch 1996, pp ). Trnsitions: C2 to C2: Vginl process of crotid cnl. C2 to C3: End of crotid cnl t the posterolterl mrgin of the formen lcerum. C3 to C4: Level of the petrolingul ligment. C4 to C5: Proximl durl ring. C5 to C6: Distl durl ring. C6 to C7: Origin of posterior communicting rtery. the cnl, the ICA forms medil convex curve. The cervicl segment lies medil to the internl jugulr vein (IJV), the vgus nerve usully between both vessels. The ICA enters the crnium by pssing through the crotid cnl. This cnl is lined by periosteum nd is locted in the petrous portion of the temporl bone. The posterior orifice of the cnl opens onto the posterior wll of the formen lcerum, djcent to the jugulr formen. The nterior or internl orifice of the cnl is locted t the petrous pex (Miller N 1998). The petrous segment cn be divided into verticl nd horizontl portion with course depending on the configurtion nd development of the skull bse, prticulrly on the shpe of the petrous bone. The totl length of this segment is bout mm. The verticl portion courses 6 15 mm in the verticl direction then turns medilly nd nteriorly to form genu nd becomes the horizontl portion. The horizontl portion courses nteriorly nd medilly bove the formen lcerum to eventully leve the bony cnl ner the petrous pex. The petrous segment cn give rise to two smll rteril brnches

34 3.3 Vsculr Antomy 23 in 38% of the cses. The vidin rtery, usully rising from the internl mxillry rtery (see below), cn lso rise from the petrous ICA (30%) (Pullus et l. 1977). The smll croticotympnic rtery, previously reported to be the most common brnch, is thought to enter the tympnic cvity through formen in the wll of the crotid cnl, ws not found by Pullus et l. (1977), but often seen by Lng (1983). The ltter lso sw periostel twigs rmifying in the periosteum of the crotid cnl nd the neighborhood of the formen lcerum, which hve been described only by Lzorthes (1961). They my be responsible for retrogrde filling of the petrous ICA in ptients with ICA occlusions. The cvernous segment begins t the superior mrgin of the petrolingul ligment t the posterior spect of the CS nd ends t the root of the nterior clinoid process. This segment lies within the CS, surrounded by its venous spces nd by some trbeculr connective tissue. From the petrous pex medil nd rostrl towrds the lterl side of the sphenoid bone, the ICA is seprted from the Gsserin gnglion by thin osseous or connective tissue septum. Becuse the posterior limits of the cvernous segment vry nd re difficult to exctly define, this segment ws lso divided into presellr nd juxtsellr segment by some uthors (Dilenge nd Heon 1974). Above the formen lcerum the ICA courses lmost perpendiculrly crnil in groove long the lterl side of the sphenoid bone nd lies directly djcent to the frontl pole of the Gsserin gnglion [C5 fter Fischer (1938) or scending segment]. From here the ICA psses rostrlly to rech the root of the nterior clinoid process, to which it lies lterlly. Lterl to the pituitry foss the ICA lies in shllow groove of the lterl sphenoid bone (C4, or horizontl segment). The medil wll of the ICA lies within the CS, which is seprted from the pituitry glnd by thin sheet of the dur mter. Underneth the root of the nterior clinoid process,the ICA forms the so-clled knee of the crotid, shrp, nteriorly convex curve (C3 or clinoid segment). After piercing the dur nd the rchnoid membrne t the medil mrgin of the nterior clinoid process, the ICA courses within the subrchnoid spce (cerebrl or cisternl segment, C2) upwrd nd posteriorly underneth the opticl nerve tht enters the optic cnl. Finlly, the ICA scends to rech its bifurction into the middle nd nterior cerebrl rtery (MCA nd ACA) to form the circle of Willis (C1, terminl segment fter Fischer). In this mnner the segments C3 C5 form the cvernous nd C1 C2 form the cerebrl segment of the ICA. The double or S-shpe curve of the ICA within nd bove the CS is, ccording to Moniz (1927), clled the crotid siphon. After Kryenbühl nd Ysrgil (1997), the shpe of this crotid siphon my differ remrkbly: U-shpe, V-shpe, Arcus-shpe nd Omegshpe, double siphon, megsiphon or dolichosiphon cn be seen. The first three types re seen more frequently, while in older ptients (51 74 yers) the omeg type occurs more often. Tody, Fischer s system is considered somewht limited s it is ntomiclly inccurte nd numbers the ICA segments opposite the direction of blood flow. It hs been modified or replced by severl others, e.g. by Bouthillier et l. (1996), who divided the ICA into seven segments using numericl scle in the direction of the blood flow: C1 = cervicl, C2 = petrous, C3 = lcerum, C4 = cvernous, C5 = clinoid, C6 = ophthlmic nd C7 = terminl segment (Fig. 3.6). Lsjunis et l. (2001) hve pointed out tht the morphologicl continuity of the ICA obscures significnt differences between vrious segments which cn lso be mrked by the origins of embryonic vessels (see below). In clinicl prctice, however, the clssifiction of Fischer is still widely used Brnches of the ICA Brnches of the Cvernous Segment (Fig. 3.7 nd Figures in Sects ) Within the CS, the C5 segment usully gives rise to one (MHT), nd the C4 segment gives rise to two smll but importnt brnches (ILT, cpsulr rteries). Meningohypophysel Trunk (MHT) Luschk (1860) first described the Arteri hypophysilis inferior. Mc Conell (1953) studied the rteril supply of the pituitry glnd by smll rteril trunk, of which the inferior hypophysel rtery is the lrgest. Lter on, Schnüerer nd Stttin (1963), Prkinson (1965), nd Rhoton nd Inoue (1991) gve more detiled description of these ntomic vsculr reltionships. Origin nd brnching of the smll rteries vry significntly. Most frequently, 0.75-mm meningohypophysel rmus rises from the dorsl circumference of the C5 segment, just immeditely before the verticl prt turns into the horizontl prt. This vessel is in the English literture commonly nmed the

35 24 3 Antomy of the Cvernous Sinus nd Relted Structures meningohypophysel trunk (MHT) ccording to Prkinson (1965, 1990), the inferior hypophysel rtery ccording to Mc Connell (1953), the dorsl min stem rtery ccording to Schuerer nd Stttin (1963) or the posterior trunk ccording to Trn-Dinh (1987). The clssifiction of Prkinson (1984) differentited initilly three min brnches of the MHT, the rdiologicl ppernce of which ws described by Pribm et l. (1966). The mrginl tentoril rtery (rtery of the free mrgin of the tentorium cerebelli, tentoril rtery, medil tentoril rtery), first described by Bernsconi nd Cssinri (1956) scends to the roof of the CS, courses long the free edge of the tentorium nd gives two brnches for the third nd fourth crnil nerve nd to the roof of the CS. It supplies the medil third of the tentorium nd my nstomose with meningel brnch of the ophthlmic rtery, with corresponding contrlterl brnch nd meningel brnch of the scending phryngel rtery. This rtery ws found by Lsjunis et l. (1977) to rise from the superior brnch of the ILT in 8/20 cses (see below). The lterl clivl rtery (dorsl meningel rtery or dorsl clivl rtery) rose from the MHT in 90% of 50 studied cdvers (Hrris nd Rhoton 1976), supplies the sixth crnil nerve nd nstomoses with the contrlterl side nd brnches of the vertebrl rtery nd the jugulr rmus of the APA. This rtery supplies the dur of the dorsum selle nd clivus nd is most often involved in the rteril supply of DCSF (Brrow et l. 1985). The inferior hypophysel rtery (posteroinferior hypophysel rtery) crosses the CS medilly, divides into superior nd inferior brnches, connects with the corresponding rtery of the contrlterl side nd supplies the posterior lobe of the pituitry glnd, the dur of the posterior clinoid process, the floor of the sell nd prts of the posterior CS. According to Mrtins et l. (2005) this durl territory is supplied by medil clivl rtery, which cn lso rise directly from the ICA nd nstomoses with the hypoglossl rmus of the APA. The inferior hypophysel rtery is often superimposed on ngiogrms nd difficult to identify (Lsjunis et l. 2001). Further described re: A bsl tentoril brnch (lterl tentoril rtery) by Schnüerer nd Stttin (1963) nd Lsjunis et l. (1978) which cn rise from common trunk with medil tentoril rtery (Mrtins et l. 2005). A medil clivl rtery hs been described by Lsjunis et l. (2001) nd Mrtins et l. (2005) (see bove). It should be mentioned tht Pribrm et l. (1966) hve lredy emphsized tht the clssic MHT rising s single trunk is not constntly seen. The existence of singulr trunk ws observed by Lsjunis et l. (1978) in only 10% of the cses. He suggested insted tht these brnches more often rise independently s single vessels corresponding to the remnnts of two trnsient embryonic vessels, the primitive mxillry nd the primitive trigeminl rtery. The former gives rise to the inferior hypophysel rtery, the ltter to the medil nd lterl clivl rteries s well s to the bsl tentoril rtery. Lsjunis et l. (1978) furthermore pointed out tht the mrginl tentoril rtery of Bernsconi my originte from eight different pedicles, [including the ccessory meningel (Silvel nd Zmrron 1978), middle meningel, introrbitl ophthlmic nd lcriml rteries], of which the C5 siphon is only one. The term MHT is nevertheless widely used, lthough it not only supplies the meninges nd the pituitry glnd but lso the oculomotor, trochler nd bducens nerves. In over 200 cvernous crotid dissections it ws identified in 100% of the cses (Prkinson 1965). Mrtins et l. (2005) hve recently provided comprehensive review of the ntomy of durl rteries within the CS region nd differentite the MHT into the following components: 1. Tentoril trunk Medil tentoril rtery (mrginl tentoril rtery, Bernsconi) Lterl tentoril rtery (bsl tentoril rtery) 2. Dorsl meningel rtery (lterl clivl rtery) Medil brnch Medil clivl brnch Lterl brnch 3. Inferior hypophysel rtery Hypophysel circle Medil clivl rtery The inferolterl trunk (ILT) ccording to Lsjunis et l. (1977), lso nmed rtery of the inferior cvernous sinus (Miller 1998; Prkinson 1965), the lterl min stem (Schnürer nd Stttin 1963) or the lterl trunk (Trn-Dinh 1987) in the

36 3.3 Vsculr Antomy 25 Fig. 3.7.=Arteril ntomy in the cvernous sinus region (rtist s drwing of smll durl rteries rising from ICA nd ECA, considered so-clled dngerous nstomoses, in the cvernosus sinus region, lterl view.) The durl rteries of the ICA nd ECA connecting both territories in the cvernous sinus region re lso referred to s dngerous nstomoses. Becuse of their smll cliber these brnches re often not (or not completely) visulized in dignostic rteriogrms, unless they re enlrged due to incresed flow cused by AV-shunting lesions, rteril occlusions or tumors. The numerous possibilities of indvertent migrtion of embolic mteril into the cerebrl circultion during trns rteril emboliztion of ECA brnches re obvious. However, it is not the vessel per se which is dngerous, but lck of knowledge or negligence of the prticulr ntomy in this region. In cses of rteriovenous shunts developing within or djcent to the cvernous sinus, these brnches become supplying feeders nd re recruited from ipsi- nd contrlterl ECA nd ICA territory. Even in cse of successful positioning of microctheter in the ILT or MHT, reflux of embolic mteril such s prticles or glue my esily occur nd poses risk for neurologicl complictions b c (9) d Internl crotid rtery (ICA, C5) 2 Internl crotid rtery (C4) 3 Ophthlmic rtery (OA) 4 Meningohypophysel trunk (MHT) 5 Inferior hypophysel rtery 6 Lterl clivl rtery 7 Medil clivl rtery 8 Bsl tentoril rtery 9 Mrginl tentoril rtery 10 Inferolterl trunk (ILT) 11 Superior rmus 12 Anteromedil rmus 13 Anterolterl rmus 14 Posterolterl rmus 15 Posteromedil rmus 16 Cpsulr rteries 17 Externl crotid rtery (ECA) 18 Ascendending phryngel rtery (APA) 19 Superficil temporl rtery (STA) 20 Internl mxillry rtery (IMA) 21 Middle meningel rtery(mma) 22 Accessory meningel rtery (AMA) 23 Sphenopltine rtery 24 Artery of the formen rotundum (AFR) 25 Recurrent rtery of the formen lcerum (RAFL) 26 Artery of the pterygoid cnl (vidin rtery) Suprorbitl fissure b Formen rotundum c Formen ovle d Formen spinosum English literture, corresponds to the remnnt of the dorsl ophthlmic rtery ( Lsjunis et l. 1977). It rises from the lterl spect of the horizontl portion of the cvernous portion of the ICA distl to the origin of the MHT (C4 segment). It usully curves over the CN VI (96%) nd divides into three min brnches: The superior rmus (rmus tentorii mrginlis, mrginl tentoril rtery) supplies the roof of the CS nd the proximl prt of CN III nd IV. It cn replce or nstomose with the tentoril rtery of the MHT. The nterior rmus divides into lterl nd medil brnch. The nteromedil rmus psses to the suprorbitl fissure nd supplies the distl prts of the CN III nd IV. It ends s deep recurrent ophthlmic rtery (or dorsl ophthlmic rtery) nd nstomoses with the introrbitl ophthlmic rtery. The nterolterl rmus courses together with the second division of

37 26 3 Antomy of the Cvernous Sinus nd Relted Structures the trigeminl nerve (V/2) through the formen rotundum nd nstomoses with the rtery of the formen rotundum of the internl mxillry rtery (IMA). The posterior rmus divides into lterl nd medil brnch. The posteromedil rmus courses together with the third division of CN V towrds the formen ovle nd nstomoses with the ccessory meningel rtery (AMA) brnch of the IMA. It lso supplies the CN VI, the medil third of the Gsserin gnglion nd the motor roots of the CN V3. The posterolterl rmus reches the formen spinosum nd supplies the middle nd lterl third of the Gsserin gnglion nd nstomoses with brnch of the middle meningel rtery (MMA). Prkinson (1965) ws ble to identify the ILT in 80% of the cses, others in 65% 84% (Hrris nd Rhoton 1976; Trn-Dinh 1987; Lsjunis nd Berenstein 1987; Rhoton et l. 1979). Unlike the MHT, the ILT ws not observed to show vrints nd lwys rose s single trunk (Trn-Dinh 1987; Willinsky et l. 1987). According to Lng nd Schfer (1976), the MHT is clled Truncus crotico-cvernosus posterior nd the ILT cn be clled Truncus crotico-cvernosus lterlis. As mentioned bove, Lsjunis et l. (1977, 1978,b) nd Willinsky et l. (1987) s well s Brssier (1987) hve suggested different concept tht considers the intrcvernous ICA brnches embryologicl remnnts, ssuming tht observed norml dispositions nd vrints rther represent vrious phses in the embryologicl development of the primitive mxillry nd trigeminl rteries, possibly influenced by individul hemodynmic blnce between ICA nd ECA, right nd left or nterior nd posterior. Trn-Dinh (1987) who studied the vrious clssifictions nd terminology extensively proposed simplifiction into primry nd secondry brnches. The fourth Nomencltur Antomic (1977) contins list of intrcvernous ICA brnches but no systemtic clssifiction. Tble 3.1 provides n overview of vrious terms used for the nstomotic brnches of the C4 nd C5 segment. Cpsulr rteries ccording to Mc Connell (1953) nd Prkinson (1965), nd medil group ccording to Trn-Dinh (1987) cn ngiogrphiclly be identified in less thn 30% of cses (Osborn 1991) nd were seen in cdver dissections in 28% of the cses (Hrris nd Rhoton 1976). The inferior cpsulr rtery courses inferomedilly to supply the floor of the sell turcic nd nstomoses with the contrlterl side nd with brnches of the inferior hypophysel rtery. The superior cpsulr rtery psses long the roof of the sell. The cpsulr rteries my lso rise from the inferior hypophysel rtery. They do not contribute to the supply of the CNs nd usully ply no role in the supply of DCSFs. Recurrent rtery of the formen lcerum (RAFL), smll brnch of the lterl surfce of the verticl portion of the C5 segment, descends to the formen lcerum nd usully nstomoses with the crotid brnch of the APA. It supplies the pericrotid utonomic nerve plexus nd the crotid wll (Mrtins et l. 2005) nd my connect with the posterior rmus of the ILT (cvernous brnch of MMA) (Lsjunis et l. 2001). Ophthlmic Artery The ophthlmic rtery (OA) is the most proximl mjor intrcrnil brnch of the ICA nd rises just s this vessel is emerging from the cvernous sinus on the medil side of the nterior clinoid process, nd enters the orbitl cvity through the optic formen, below nd lterl to the optic nerve. The origin is intrdurl in bout 90% nd extrdurl in bout 10% of the time, from either the cvernous or the clinoid segment of the ICA (Punt 1979). The most commonly reported vrints re the origins from the MMA (Heyre 1974) nd from the ACA (Lsjunis et l. 2001). Other possible origins re the ccessory meningel rtery nd the bsilr rtery (Lsjunis et l. 2001), the MMA, the nterior deep temporl rtery or directly from the ECA ( Newton nd Potts 1974). The OA usully courses nterolterlly below the optic nerve nd enters the optic cnl where it pierces the durl sheth of the optic nerve, usully inferolterl but sometimes directly below the nerve (Hyreh 1962). The introrbitl portion of the OA my be divided into three segments, the first extending from its entry to where the rtery crosses under the nerve. The second segment crosses the nerve (in 80% from lterl to medil), nd the third extends from here to its termintion. The OA gives off number of brnches including the short nd long posterior ciliry rteries nd the centrl retinl rtery (CRA). The centrl retinl rtery hs dimeter of bout 200 microns nd my hve tortuous course long the inferior surfce of the optic nerve before it pierces the optic nerve sheths mm posterior to the globe nd runs for 1 3 mm with

38 3.3 Vsculr Antomy 27 Tble 3.1. Vrious terms for cvernous ICA brnches (modified fter Trn-Dinh 1987) Luschk 1860 Inferior hypophysel rtery Mc Connell 1953 Inferior hypophysel rtery Cpsulr rteries Bernsconi nd Cssinri 1956 Tentoril (mrginl) rtery Schnurer nd Stttin 1963 Dorsl min stem (Bsl tentoril brnch, Clivl brnches, Inferior hypophysel rtery) Lterl min stem (Mrginl tentoril brnch) Pribrm et l Prkinson 1964, 1984 Meningohypophysel trunk (Inferior hypophysel rtery, dorsl meningel rtery, tentoril rtery) Meningohypophysel trunk (Inferior hypophysel rtery, dorsl meningel rtery, tentoril (mrginl) rtery) Artery of the inferior CS Artery to Meckel s Cve Cpsulr rteries Lsjunis 1977, 1978 Primitive mxillry Dorsl ophthlmic rtery Cpsulr rteries Willinsky et l (Posterior inferior hypophysel rtery) Trigeminl rtery (Lterl clivl rtery) Medil clivl rtery Bsl tentoril rtery (Inferolterl trunk) Trn-Dinh 1987 Posterior trunk Lterl trunk Medil group Mrtins et l MHT (Tentoril trunk, medil tentoril rtery, lterl tentoril rtery, medil clivl rtery) ILT Cpsulr rteries the subrchnoid spce of the optic nerve (Miller 1998). The CRA gives numerous smll brnches to the optic nerve nd finlly psses through the retrolminr portion of the nerve where it gives off its terminl brnches to supply the inner lyers of the retin (Hyreh 1963). The introrbitl course of the OA nd its brnching pttern ws studied in detil by Hyreh (1962b). He found mjor vritions, depending on whether the OA crosses over or under the nerve. In the former cse, the first mjor brnch is the centrl retinl rtery, followed by the lterl posterior ciliry rtery, lcriml rtery, musculr rteries, medil posterior ciliry rteries, suprorbitl rtery, nterior nd posterior ethmoidl rteries nd medil plpebrl rtery. In the ltter cse, the OA sends smll perforting brnches to the optic nerve, followed by the lterl posterior ciliry, centrl retinl rtery, musculr rteries, medil posterior ciliry rteries, lcriml rtery, posterior ethmoidl rtery, suprorbitl rtery nd nterior ethmoidl nd medil plpebrl rtery (Hyreh 1962b). The OA lso gives rise to smll meningel rteries of which the nterior flx rtery origintes from the nterior ethmoidl rtery. The terminl brnches re the suprtrochler nd the dorsl nsl rteries (Miller N 1998). The posterior ciliry rteries vry in number nd form the nstomosing ring, the Circle of Zinn nd Hller (Hyreh 1962b). They re clled short posterior ciliry rteries nd give rise to the cilioretinl rteries which supply the retin in the region of the optic disc (Rndll 1887). The long posterior ciliry rteries supply the internl structure of the nterior portion of the eye (Ducsse et l. 1986). Importnt nstomoses re formed by the nterior nd posterior ethmoidl rteries, the lcriml rtery nd the deep nd superficil recurrent ophthlmic rteries. The deep recurrent ophthlmic rtery usully rises from the first prt of the introrbitl OA, courses bckwrds through the SOF nd consistently nstomoses with the nteromedil rmus of the ILT. The ngiogrphic ppernce of this vessel is chrcteristic when it projects below the C3 nd C4 portion of the ICA (Lsjunis et l. 1978b). The superficil recurrent ophthlmic rtery my rise from the introrbitl OA or from

39 28 3 Antomy of the Cvernous Sinus nd Relted Structures the lcriml rtery nd usully courses through the most lterl prt of the SOF to supply the intrdurl prts of CN III nd IV nd continues s n rtery of the free mrgin of the tentorium (Lsjunis et l. 1978b). Two others cn estblish nstomoses between OA nd MMA, the recurrent meningel rtery nd the meningolcriml rtery (Lsjunis et l. 1975b). The recurrent meningel rtery is n dult remnnt of the common meningoorbitl vsculr system, courses through the SOF nd connects the lcriml rtery with the nterior brnch of the MMA. The meningolcriml rtery lso rises from the nterior brnch MMA to enter the orbit through the meningolcriml formen (formen of Hyrtl, lso clled crnioorbitl, meningoorbitl, stpedilophthlmo-lcriml formen), which usully lies just lterl to the SOF (Moret et l. 1977). Ethmoidl Arteries The ethmoidl rteries rise from the ophthlmic rtery nd cn be divided into n nterior nd posterior group. Their origin, course nd supplied regions were studied on 30 injected dult heds by Lng nd Schefer (1979). After brnching off, the nterior ethmoidl rtery normlly turns in single loop by first coursing forwrds nd then, reversing towrds the nterior ethmoidl formen, it psses into the cnl portion. Occsionlly, common ethmoidl rtery or common source for the ethmoidl rteries is present. As rule, the smller posterior ethmoidl rtery rises from the ophthlmic rtery; occsionlly it is bsent or cn even very rrely rise from the MMA (Lng nd Schfer 1979). The rtery usully courses over the superior oblique muscle, psses through the posterior ethmoid cnl, supplies the posterior ethmoidl cells nd enters the crnium, the dur mter nd gives off brnches to descend to the nsl cvity through the cribriform plte where they nstomose with brnches from the sphenopltine rtery (Gry 1918). Before or while entering the olfctory foss the rtery usully gives off brnches to supply the dur nd bone of the plnum sphenoidle, lesser wing of the sphenoid nd djcent dur of the nterior crnil foss. The nterior ethmoidl rtery ccompnies the nsociliry nerve through the nterior ethmoidl cnl, supplying the nterior nd middle ethmoidl cells nd frontl sinus. It then enters the crnium to supply the dur mter with the nterior meningel rtery, which gives off the nterior flx rtery nd nsl brnches to supply the lterl wll nd the septum of the nose (Gry 1918). The ethmoidl rteries re usully involved in the rteril supply of DAVFs locted on the floor of the nterior crnil foss, but my lso contribute to the rteril supply of DCSFs. Tble 3.2. ICA nd ECA brnches relevnt for supply of DCSF nd their min nstomoses to djcent territories (modified fter Mrtins et l. 2005) MHT Tentoril trunk Medil tentoril rtery (to contrlterl, lterl tentoril, dorsl meningel, MMA, ILT) Lterl tentoril rtery (to medil tentoril, dorsl meningel, posterior meningel, mstoid) Dorsl meningel rtery (to contrlterl, medil clivl, tentoril, MMA, APA) Inferior hypophysel rtery (to contrlterl, cpsulr) Medil clivl rtery (to contrlterl, dorsl meningel, medil tentoril) ILT Superior rmus (to medil tentoril) Anterior Rmus Anteromedil rmus (to deep recurrent ophthlmic) Anterolterl rmus (to AFR) Posterior Rmus Posteromedil rmus (to AMA) Posterolterl rmus (to MMA) Ophthlmic rtery Anterior ethmoidl rteries Posterior ethmoidl rteries Deep recurrent ophthlmic rtery (to ILT) Superficil recurrent ophthlmic rtery (to medil tentoril) Recurrent meningel rtery (to MMA) Recurrent rtery of the formen lcerum (to APA, ILT, vidin) ECA Ascending phryngel rtery Crotid rmus (to RAFL) Jugulr rmus (to lterl clivl) Hypoglossl rmus (to medil clivl) Mxillry rtery Artery of pterygoid cnl (to AMA, APA, C5 segment, RAFL) Pterygovginl rtery Middle meningel rtery (to ILT, AMA, OA) Accessory meningel rtery (to MMA, ILT, medil tentoril)

40 3.3 Vsculr Antomy Externl Crotid Artery The externl crotid rtery (ECA) gives off four mjor rteries, the brnches of which contribute to the supply of the CS nd the CNs Ascending Phryngel Artery The scending phryngel rtery (APA), the smllest brnch of the ECA, is rther grcil, long vessel. It rises close to the origin of the ECA t its dorsl circumference nd scends between ICA nd the phryngel wll to rech the bse of the skull. Its meningel brnches re very smll vessels, supplying the dur mter nd hve been described in detil by Lsjunis nd Moret (1976). One brnch enters the skull through the formen lcerum, nother through the jugulr formen, nd sometimes third one through the hypoglossl cnl. The first, the crotid rmus (rmus croticus), ccompnies the ICA in its cnl. It nstomoses t the level of the formen lcerum with smll brnch of the C5-portion (recurrent rtery of the formen lcerum). The second, the jugulr rmus (rmus jugulris), psses with CNs 9 11 through the jugulr formen nd reches the dur mter. It nstomoses within the sigmoid sinus with the dorsl brnch of the occipitl rtery nd within the inferior petrous sinus with the medil brnch of the lterl clivl rtery. The third, the hypoglossl rmus (rmus hypoglossus), ccompnies nd supplies the hypoglossl nerve. It brnches further within the dur of the formen mgnum nd nstomoses with the medil clivl rtery rising from the MHT (Lsjunis nd Moret 1976). This rmus does sometimes not exist, in which cse connection between the extrdurl course of the vertebrl rtery nd corresponding brnch cn be found Internl Mxillry Artery The internl mxillry rtery (IMA), or mxillry rtery ccording to current terminology, is the lrgest terminl brnch of the ECA consisting of mndibulr, pterygoid nd pterygopltine segment giving rise to t lest 16 terminl brnches. Becuse of their nstomoses with brnches of the C4 segment of the ICA, the middle meningel rtery nd the ccessory meningel rtery (both rising from the second, the pterygoid segment), re of greter importnce in the context of this monogrph. The brnches of the third portion leve the pterygopltine foss through corresponding formin nd fissures (Allen et l. 1973) which ccount for reltive constnt ngiogrphic pttern. Among the nteriorly directed brnches re the posterior superior lveolr rtery, the infrorbitl rtery nd the greter (descending) pltine rtery. The sphenopltine rtery is considered the terminl brnch of the mxillry rtery nd leves the pterygopltine foss through the sphenopltine formen (Allen et l. 1973). The posteriorly directed brnches re from medil to lterl the pterygovginl rtery, the rtery of the pterygoid cnl (Vidin) nd the rtery of the formen rotundum (see lso Figs ). The rtery of the formen rotundum (AFR), becuse of its connection with the ILT, hs prticulr importnce (Djindjin nd Merlnd 1973), lying within the pterygoid foss nd leving it through the formen rotundum s the most lterlly coursing brnch (Allen et l. 1973). It nstomoses with the nterolterl brnch of the ILT (in older literture, rtery of the inferior cvernous sinus). This very smll rtery hs dimeter of bout 150 microns (Lng 1979) nd is usully not visulized on stndrd ngiogrms, unless its dimeter is incresed (Ribeiro et l. 1984; Allen et l. 1974). Its course is usully oblique, posterior nd crnil through the cnl. It represents one of the most prominent brnches in the supply of DCSFs, nd my lso ply role s collterl supply to the circle of Willis in cse of ICA occlusions or s tumor feeding vessel for meningioms of the sphenoid wing (Ribeiro et l. 1984). The vidin rtery (Arteri cnlis pterygoidei Vidii, rtery of the pterygoid cnl) rising within the pterygopltine foss s brnch of the distl mxillry rtery, courses within the vidin cnl towrds the formen lcerum nd my nstomose within the orophryngel roof with brnches of the AMA nd APA (Lsjunis 1984). It cn rise from the greter pltine rtery nd my continue nd nstomose with the petrous segment of the ICA. The vidin rtery is remnnt of the first ortic rch nd hs been demonstrted to rise from the inferior (55%) or nteroinferior (35%) spect of the internl crotid rtery within the petrous bone in 30% of ntomic specimens (Pullus et l. 1977; Quisling nd Rhoton 1979). It runs here for n verge of 7 mm long the nterior wll of the crotid cnl before it emerges from the skull through the crtilge

41 30 3 Antomy of the Cvernous Sinus nd Relted Structures of the formen lcerum nd enters the pterygoid cnl nd hs n verge dimeter of less thn 0.5 mm (Lng 1983). The vidin rtery supplies the lterl phryngel recess, giving rise to brnches supplying the uditory tube nd the tympnic region (Allen et l. 1973). This rtery cn be involved in the rteril supply of DCSFs (Osborn 1980b). In contrst the pterygovginl rtery rises from the distl prt of the mxillry rtery nd psses posteriorly through the pterygovginl cnl long the roof of the nsophrynx nd nstomoses with the inferomedil eustchin brnch of the ccessory meningel rtery, the Eustchin brnch of the APA nd the mndibulr brnch of the ICA ( Lsjunis et l. 2001). This most medil coursing brnch is lso clled the phryngel rtery nd supplies the chones, the phrynx nd eustchin tube. As mentioned by Allen et l. (1974), other collterl brnches my trverse the lterl spect of the superior orbitl fissure nd re clled A. nstomotice fter Lie (1968). The ltter erroneously described these brnches s ll pssing through the suprorbitl fissure nd did not recognize the rtery of the formen rotundum. Although neglected in severl textbooks, modern high-resolution DSA nd, in prticulr, 3D-DSA revel tht in some DCSFs these A. nstomotice, pssing through the SOF, re indeed identifible. They course more crnilly bove the rtery of the formen rotundum nd often contribute to the supply of DCSFs (see Chp. 7) Middle Meningel Artery The middle meningel rtery (MMA) is the second scending brnch of the IMA nd the lrgest of the durl rteries. Its min brnch courses through the formen spinosum to enter the skull. Of the four groups of brnches (extrcrnil, bsl, nterior nd posterior) the extrcrnil nd bsl rteries re of specil interest. The extrcrnil brnches supply locl structures, in prticulr the AMA with brnch to the formen ovle nd the bove lying meninges. The bsl group supplies the crnil foss (Lsjunis nd Theron 1976). Of the ltter, the inferior meningel brnches supplying the middle crnil foss, s well s their nstomotic brnches to the extrcrnil rteries, re of importnce: to the APA vi the crotid cnl nd the formen lcerum, to the vidin rtery vi the formen lcerum, to the AMA vi the formen ovle nd to the IMA vi the formen rotundum. Other nstomotic vessel re medil brnches to the Gsserin gnglion nd to the cvernous segment nd their nstomoses to the ICA nd other brnches supplying the CS; finlly nterior brnches supplying the superior orbitl fissure nd their nstomoses. Sometimes one lrger brnch of the temporl segment of the MMA curves posteriorly nd medilly nd contributes to the supply of the CS or even the tentorium. The MMA usully hs nstomoses with the posterolterl brnch of the MMA nd my lso give rise to the medil (mrginl) tentoril rtery (Benndorf 2008) Accessory Meningel Artery The ccessory meningel rtery (AMA) supplies the phrynx nd the eustchin tube, sometimes the meninges. According to Bumel nd Berd (1961) it rises lmost s frequently from the IMA s from the MMA, depending on if the ltter is of the deep or superficil vrint. It never origintes before the MMA. In the superficil vrition (IMA lterl to the lterl pterygoid muscle), the AMA origintes from the MMA, in the deep vrition (IMA medil to the lterl pterygoid muscle), it stems directly from the IMA (Lsjunis nd Theron 1976; Vitek 1989) in 60% of cses, immeditely following the origin of the MMA, nd in the remining cses from the middle portion of the pterygoid segment. In 24% of the cses it consists of more thn one vessel. The AMA courses prllel to the superior boundry of the medil pterygoid muscle in fscil plne between the medil nd lterl pterygoid muscles (Vitek 1989). The rtery scends through the interponeurotic spce nd divides below the skull bse into its nterior nd posterior rmi. The nterior rmus courses long the eustchin tube, the posterior meningel rmus courses in circ 10% through the formen ovle (Lng 1979) or the formen of Veslius to supply the lterl wll of the CS, the Gsserin gnglion nd the ntero-superior surfce of the petrous bone. It my lso contribute to the supply of the sphenoid sinus. In cse of dominnt supply of the CS region by trnscrnil brnches of the IMA, the AMA my supply the entire re, giving rise to four brnches usully belonging to the ILT in 20% of the cses (Lsjunis et l. 2001). Lng (1979b) describes the AMA s brnch of the MMA, contributing to the rich vsculr network on the surfce of the dur mter tht is provided minly by the ltter (Djindjin nd Merlnd 1973). Other smll

42 3.3 Vsculr Antomy 31 rteries in the neighborhood my lso be involved such s the APA nd OA. Other smll connections re provided by rterioles of the intrcvernous nd suprcvernous portion of the ICA tht supply the sell, the Gsserin gnglion nd the tentorium. The rterioles lso supply the lterl wll of the CS by AMA brnches nd brnches of the vidin rtery nd of the rtery of the formen rotundum. As lredy discussed by Bumel nd Berd (1961), there is discrepncy between the nomenclture of this rtery nd the territory supplied is meningel in only 10% (Vitek 1989). Therefore, Vitek (1989) suggested more pproprite term, the pterygomeningel rtery. According to Lsjunis nd Berenstein (1987), the AMA cn hve four brnches, the posterior, inferomedil, inferopltine nd intrcrnil brnch. The ltter, lso nmed the intrcrnil scending rmus, courses through the formen ovle nd nstomoses with the posteromedil rmus of the ILT. Brssier et l. (1987) emphsized the importnce of these rteril nstomoses in the region of the CS nd the intrcvernous ICA Venous Antomy (Figs nd Figs. in Sects ) The Cvernous Sinus, Receptculum, Sinus Croticus (Rektorzik), Confluens Sinuum Anterius, Sinus Spheno-Prietle (Cruveilhier), Cvernous Plexus, Lterl Sellr Comprtment Embryology The role of the CS s drining pthwy chnges during life. As described by Pdget (1956b), the CS forms during the 40-mm stge of embryonic development s plexiform extension of the prootic sinus nd the ventrl myelencephlic vein. The primitive mxillry vein, initilly drining into the prootic sinus, connects with the CS nd becomes the superior ophthlmic vein. At the 60-mm embryonic stge, the CS is still not involved in the cerebrl venous dringe nd receives blood only from the ophthlmic veins drining it into the IJV vi the IPS. During this time, the peri (internl) crotid venous plexus my prticipte insted, bringing blood from the CS through the crotid cnl (Knosp et l. 1987). Pdget considered the CS nd the IPS, secondry sinuses nd n intrcrnil detour through the bse of the skull between two extrcrnil veins, the SOV nd the IJV. In typicl infnt stge (3rd month) the CS nd the IPS hve no connection with cerebrl veins, the superficil middle cerebrl vein (SMCV) still drins through the embryonic tentoril sinus nd the superior petrosl sinus (SPS) is still not conjoined, drining only cerebellr veins. Wheres only in typicl dult stge the CS my receive blood from the SMCV, the sphenoprietl sinus (SPPS) nd the SOV nd drins into the inferior petrosl sinus (IPS), pterygoid plexus (PP) nd SPS (Suzuki nd Mtsumoto 2000). The condition tht the CS does not prticipte in the cerebrl venous dringe my persist, but frequently the secondry nstomoses involving the CS occur before dult life (Pdget 1956b). This concept is not in full greement with more recent studies on fetl skull bses by Knosp et l. (1987) who found tht in 20% the superficil middle cerebrl vein drins into the CS, or the tentoril sinus hs no connection to the CS. The uthors further observed the SPS in 60% connected with the CS, however minor functionl significnce, demonstrting the prentl existence of cerebrl venous blood flow through the CS. A recent study on 270 ptients using CT-ngiogrphy showed tht in pproximtely 27% of the dult popultion the SMCV my not be connected to the CS (Suzuki nd Mtsumoto 2000). Bsed on this stged embryologic development nd vrious possibilities of persistence in dult life, the pttern of venous tributries nd dringe of the CS seen on cerebrl ngiogrms cn vry considerbly Antomy nd Topogrphy The cvernous sinus (CS) belongs to the group of the gret durl sinuses (sinus dure mtris) which, becuse of their prticulr ntomicl structure, re not nmed veins. An endothelil tube is surrounded by firm connective collgenous tissue without vlves, but numerous lcunes nd trbecule. The CS surrounds the sphenoid bone nd the pituitry glnd. It forms in the middle crnil foss centrl collector for the blood coming from the meningel veins (MV), the sphenoprietl sinus (SPPS), the superficil middle cerebrl vein (SMCV) nd the ophthlmic veins. The CS is locted t the lterl side of the sphenoid bone, is tringulr in shpe nd its medil border is formed by connective tissue lyer of the hypophysis. The CS hs length of pproximtely

43 32 3 Antomy of the Cvernous Sinus nd Relted Structures 20 mm nd width of 29 mm (Lng 1979), extends from the petrous pex to the bsl roots of the lesser sphenoid wing nd reches the medil prt of the superior orbitl fissure. Since its very first description, controversy concerning the true ntomic structure of the CS hs repetedly occurred nd persists to some extent until tody. The cvernous sinus ws probbly first described by Ridley (1695) s follows: Another I discovered by hving these veins injected with wx, running round the pituitry glnd on its upper side, forwrdly within the duplicture of the dur mter, bckwrdly between the dur mter nd pi mter, then somewht loosely stretched over the subjcent glnd itself nd lterlly in sort of cnl mde up of the dur mter bove nd the crotid rtery on ech outside of the glnd, which by being fstened to the dur mter bove, nd below t the bsis of the skull, leves only little interstice betwixt itself b Fig. 3.8,b. The complex ntomy of the cvernous sinus nd its communicting venous structures cn be pprecited. The min tributries re the superior ophthlmic vein, sphenoprietl sinus, superficil middle cerebrl vein nd the uncl vein. The Sylvin vein drins into the sphenoprietl sinus or directly into the venous plexus of the formen ovle (FOP). The most importnt drining vessels re the plexus of the formn ovle which is connected to the pterygoid plexus, the inferior petrosl sinus nd the superior petrosl sinus. The course of the inferior petrosl sinus to the jugulr formen long the petroclivl fissure nd the course of the superior petrosl sinus to the sigmoid sinus long the petrous ridge re demonstrted. Becuse of its stright nd short course, the inferior petrosl sinus represents the most suitble venous pproch to the cvernous sinus in the mjority of cses. Note lso: The internl crotid rtery venous plexus (ICAVP, Rektorzik) is not illustrted. The mrginl sinus tht receives the blood from the BP is not shown. The IPCV nd the ACC would not be visible in this perspective. Osseus ntomy of the sellr region. Crnil nd posterior view onto the middle crnil foss nd the inner skull bse. 1 Anterior clinoid process 2 Sphenoid bone 3 Posterior clinoid process 4 Sell turcic 5 Optic cnl 6 Clivus 7 Pyrmid pex 8 Formen ovle 9 Formen rotundum 10 Superior orbitl fissure 11 Jugulr formen 12 Formen mgnum 13 Petroclivl fissure b Osseus nd venous ntomy of the sellr region. Venous tributries nd dringe of the cvernous sinus (rtist s drwing). 1 Sphenoprietl sinus (SPPS) 2 Superficil middle cerebrl vein (SMCV) 3 Emissry vein of the formen rotundum 4 Superior ophthlmic vein (SOV) 5 Uncl vein (UV) 6 Formen ovle plexus (FOP) 7 Superior petrosl sinus (SPS) 8 Inferior petrosl sinus (Prs verticlis) 9 Inferior petrosl sinus (Prs horizontlis) 10 Sigmoid sinus (SS) 11 Bsilr plexus (BP) 12 Cvernous sinus (nterius) 13 Cvernous sinus (posterius) 14 Intercvernous sinus (ICS)

44 3.3 Vsculr Antomy b c Fig Venous ntomy of the cvernous sinus region (rtist s drwing) with its tributries nd drining veins (see lso Chp. 7). The most importnt tributries re the superior ophthlmic vein, the Sylvin vein nd the sphenoprietl sinus. The uncl vein is in some ntomicl textbooks neglected, however in mny rteriogrms visible (c. 2/3 of the cses). For the venous dringe of the cvernous sinus, the inferior petrosl sinus nd the pterygoid plexus re of mjor importnce. In generl, tributries s well s drining veins, my serve s endovsculr ccess routes to the cvernous sinus, depending on individul ngiorchitecture of the fistul, hemodynmics nd ssocited thrombosis cusing occlusion. Note: the internl crotid rtery venous plexus (ICAVP, Rektorzik) is usully not esily visible on 2D ngiogrms due to its very thin circulr lumen in tngentil projections. The inferior petroclivl vein (IPCV) is in lterl ngiogrphic views difficult to identify, becuse the sinus is often superimposed onto the IPS (see Figs ). Both veins re neglected in mny textbooks. Furthermore, the nterior condylr confluens (ACC) lies nterior nd medil to the jugulr bulb, is collector tht nstomoses with the internl nd externl vertebrl venous plexus nd is not shown here (see Figure 3.12.). 14 Tributries (fferent veins): 1 Superior ophthlmic vein (SOV) 2 Inferior ophthlmic vein (IOV) 3 Superficil middle cerebrl vein (SMCV) 4 Uncl vein (UV) 5 Sphenoprietl sinus (SPPS) 6 Meningel veins Dringe (efferent veins): 7 Formen ovle plexus (FOP) 8 Vein of the formen rotundum 9 Pterygoid plexus (PP) 10 Bsilr plexus (BP) 11 Superior petrosl sinus (SPS) 12 Inferior petrosl sinus (IPS) 13 Intercvernous sinus (ICS) 14 Internl jugulr vein (IJV) 14 Jugulr bulb (JB) 15 Cvernous sinus (CS) 16 Sigmoid sinus (SS) 17 Fcil vein (FV) 18 Middle temporl vein (MTV) 19 Angulr vein (AV) 20 Retromndibulr vein 21 Superior root of SOV 22 Inferior root of SOV 23 Suprorbitl vein 24 Frontl vein (FrV) 25 Inferior petroclivl vein (IPCV) 26 Internl crotid rtery venous plexus (ICAVP, Rektorzik) Other: 27 Cerebellr vein 28 Lterl mesencephlic vein 29 Bsl vein of Rosenthl 30 Vein of Glen Superior orbitl 31 Stright sinus b Formen rotundum 32 Trnsverse sinus c Formen ovle

45 34 3 Antomy of the Cvernous Sinus nd Relted Structures nd the glnd. Winslow (1732) found the internl crotid rtery bthed in the blood of the sinus together with the third, fourth, fifth nd sixth pirs of nerves. It ws Winslow who nmed the sinus cvernous becuse of its spongious (cvernous) structure tht seemed to be formed by numerous fibers nd sept of connective tissue. At the beginning of the lst century, most ntomicl textbooks (Huber 1930; Sobott 1928; Splteholz 1933), s well clinicl contributions (Cmpbell 1933; Pce 1941) repetedly emphsized tht the lumen of the CS is crossed by numerous fibrous lmine, termed trbecule. Knott (1882) observed tht the CS is crossed by fibrous trbecule from some which villous process hng into the current of venous blood. This so-clled cvernous structure hs been lter on incresingly questioned. Butler (1957) found tht only the extended cvernous sinus in dults contins miniml filments nd those re only found close to the connections with tributries. He did not find trbecule in fetl sinuses. Prkinson (1965, 1967) initilly perpetuted the concept of the CS s one lrge venous spce, but found in lter study using venous corrosion specimens tht it represents n irregulr plexus of vrying sized venous chnnels, dividing nd colescing nd incompletely surrounding the crotid rtery (Prkinson 1973). Also Bonnet (1957) expressed the opinion tht the CS per se does not exist nd the spce between the durl sheets is filled by the crotid lumen, surrounded by plexus of veins nd nerves. The trbecule seen in cdvers were probbly cut wlls of the smll veins. Bedford (1966) exmined 34 cvernous sinuses nd found in 80% n unbroken venous chnnel. She therefore proposed not to cll this sinus cvernous but insted sinus orbito-temporlis, or s lredy suggested by Ridley (1695) the circulr sinus. This concept ws initilly defended lso by Hrris nd Rhoton (1976) fter detiled studies on 50 cdvers. Lter on, Rhoton et l. (1984) found the CS not ppering n unbroken cvern, but composed of severl nstomosing venous chnnels formed by the convergence of multiple veins nd durl venous sinuses. These venous chnnels would converge to form three min venous spces, identified by their reltionship to the ICA in medil, nteroinferior nd posterosuperior comprtments (Inoue et l. 1990; Rhoton et l. 1984; Ok et l. 1985). These three comprtments re substntilly lrger thn the spce between the ICA nd the lterl wll. The smll lterl spce cn be so nrrow tht the CN VI is dherent to the crotid wll medilly nd to the lterl sinus wll lterlly (Bedford 1966). Bedford (1966) further found tht the internl crotid rtery nd the CN VI were outside the lumen of the sinus in 77% of the dult specimens. To consider the CS obstructive in nture nd tht it would predispose to thrombus formtion ws ccordingly incorrect. Even though one my expect tht thrombus formtion would be seen more often in sinuses with dense trbecule, Turner nd Reynolds (1926) found only few trbecule in their studies on CS thrombosis. Knosp et l. (1987) focused in his studies on fetl cvernous sinus nd preferred the term cvernous venous plexus referring to the CS s network of distinct, individul veins. There is otherwise no doubt tht trbecule hve been found in some CS. Through the CS psses the ICA, surrounded by plexus of sympthetic nerves. These structures re seprted from the blood by lyer of endothelium. According to Lng (1979), in n embryo there exists venous plexus tht is in 70% of the cses replced by single blood spce, which cn be trversed by trbecule. In 27% of the cses the CS consists of different lrge venous lkes tht nstomose with ech other. Browder nd Kpln (1976) found the outer nd inner wlls of the CS reltively fixed by irregulrly plced strnds of fibrocollgenous tissues which did not form recognizble pttern. They further emphsized tht vlve-like membrnes were not observed t or ner the orifice of ny of the tributries. Tpts (1987) described the cvernous sinus s network of smll cliber extrdurl veins nd not s single trbeculted venous cnl. He gve n excellent historicl overview nd compred the English nd French literture of the lst century with Germn uthors, reveling the numerous discrepncies in reports nd ntomic textbooks between Lnger (1884) nd Rouviere (1985). It is stonishing, s it remins difficult to understnd why for more thn 100 yers, prticulrly in the English nd French literture, the concept of lrge trbeculted sinus previled. As speculted by Tpts (1987), it my in prt be due to the most convenient explntion of Nelton s first observtion of pulsting exophthlmos by ruptured crotid wll nd shunting blood into lrge single lumen cvern. The lck of precise ngiogrphic imging certinly contributed to this long-lsting misconception. The controversil terminology is continued by others (Knosp et l. 1987; Srm nd ter Brugge

46 3.3 Vsculr Antomy ; Lsjunis 1997), suggesting the term cvernous plexus becuse of its resemblnce to other plexiform structures in the neighborhood, such s the retroclivl (bsilr) plexus, the venous plexus long the nterior mrgin of the formen mgnum (mrginl sinus) nd the spinl venous plexus (Brown et l. 2005). Prkinson (2000) in more recent communiction emphsized tht the term cvernous sinus remins one of the gretest obstcles to understnding the ntomy of the sellr nd prsellr region. He suggests the term lterl sellr comprtment s n enlrged segment of the extrdurl neurl xis comprtment (EDNAC). This extends from the coccyx to the orbit nd contins vlueless veins through which blood my run freely in either direction in ddition to nervous, rteril nd venous elements tht my either leve or enter the comprtment in its vrious segments. The CS is probbly neither n unbroken trbeculted venous cvern (Hrris nd Rhoton 1976; Inoue et l. 1990; Bedford 1966) nor plexus of vrious-sized veins (Prkinson 1965, 1973; Bonnet 1957), but rther complex venous comprtment where numerous durl sinuses nd veins converge to form lrger venous spces round the crotid rtery which could be termed cverns (Rhoton et l. 1984). This concept finds, t lest in prt, support in the ngiogrphic pttern of the CS in norml crotid rtery venogrms nd in the ngiorchitecture of mny DCSFs or CCFs. The ngiogrphic pttern of the CS vries significntly in size nd shpe but is often one of more or less single vsculr spce on ech side of the sphenoid bone. In most direct highflow crotid cvernous fistuls (CCFs), the blood seems to shunt into single more or less enlrged cvity in which lrge detchble blloons my esily migrte fter being detched. Finlly, in the mjority of low-flow DCSF with usully multiple feeders, the shunting zone often ppers rther s single communicting venous spce which cn often (lthough not lwys) be pproched from both sides using vrious CS tributries or drining chnnels. A true ntomicl comprtmentliztion of the CS (Chloupk et l. 1993) my occur, but remins seldom nd likely represents often misinterpreted thrombosed CS. Pltinum coils, used for trnsvenous occlusions, usully tke configurtion tht resembles rther one single venous spce thn true plexus of multiple veins. Hrris nd Rhoton (1976) nd others (Inoue et l. 1990; Ok et l. 1985) hve described three or four min venous spces within the CS, which they termed ccording to their reltionship with the ICA s medil, nteroinferior, posterosuperior nd lterl comprtments. The medil comprtment lies between the pituitry glnd nd the ICA, the nteroinferior comprtment lies in the concvity below the first curve of the ICA, the posterosuperior comprtment between the ICA nd the posterior roof of the CS (Miller 1998). The CSs communicte vi intercvernous sinuses (ICSs), which hve been described lredy by Winslow (1732) s the inferior circulr sinus lying underneth the pituitry glnd. Knott (1882) reported these connections in 6 of 44 specimens nd described two intercvernous sinuses, locted nteriorly nd posteriorly to the hypophysis. This intrsellr venous connection through the midline hs been further studied by Renn nd Rhoton (1975) who concluded tht n intrsellr communiction between both CS my or my not be present. Typicl re the nterior nd posterior ICS (see below), which re nmed by other uthors s coronry sinuses (Rbischong et l. 1974). It is noteworthy tht Sn Milln Ruiz et l. (1999) recently described venous chnnel seprte from the CS but enclosed in its lterl wll, which ws found in 24% of the cses. This so-clled lterocvernous sinus ws found to drin the SMCVs in 22% of the cses nd ws seprted from the CS ntomiclly nd ngiogrphiclly (Gilloud et l. 2000). Knowledge of this lterocvernous sinus my become crucil in cse of DCSF tht my be not ccessible. employing common trnsvenous pproches vi the IPS or the SOV becuse communictions between CS nd lterocvernous sinus seem to be rre (3 cses in 58 specimens) (Sn Milln Ruiz et l. 1999). Besides the SMCVs, the ophthlmic veins nd some inferior veins of the brin re connected with the CS. The sphenoprietl sinus (SPPS) t the lower surfce of the sphenoid wing, fter receiving blood from severl smll veins of the dur mter nd the nterior trunk of the middle meningel vein (MMV), lso drins into the CS. The CS communictes with the trnsverse sinus vi the superior petrosl sinus, with the IJV vi the inferior petrosl sinus (IPS), with the bsilr plexus (BP), internl crotid rtery venous plexus (ICAVP) nd pterygoid plexus (PP) vi veins through the sphenoid emissry formen, thin veins of the cnlis rotundis, lrger veins of the formen ovle, lcerum, nd formen venosum (Veslii), nd with the fcil vein (FV) vi the supe-

47 36 3 Antomy of the Cvernous Sinus nd Relted Structures rior ophthlmic vein (SOV). In ddition, communictions with the IJV vi the ICAVP cn be found. The venous vessels, connected with the CS, my be divided into tributries nd drining veins, of which their functions my be chnged under pthologicl conditions such s thrombosis, tumors or rteriovenous shunts (Miller N 1998). The SOV, IOV, SMCV, smll hypophysel veins, nd occsionlly the uncl vein re considered fferent veins (tributries). The efferent veins re the emissry veins, the IPS, nd the petro-occipitl sinus (if present) (Doyon et l. 1974). The SPS cn drin into both the sigmoid sinus (SS) nd the CS, depending on the hemodynmic blnce. It is believed tht the ICA pulstions support indirectly the venous flow in the CS. In cse of crotid rtery occlusion this flow is slowed down nd the flow in the CS my become more continuous (Doyon et l. 1974). Tble 3.3. Cvernous sinus nd its min fferent (tributries) nd efferent (drining) veins CS Afferent veins Orbitl veins Superior ophthlmic vein Inferior ophthlmic vein Centrl retinl vein (to SOV, CS) Angulr vein (to SOV) Superficil middle cerebrl vein (SMCV) Uncl vein (UV) Sphenoprietl sinus (SPPS) Intrcvernous sinus (ICS) Meningel veins Veins of the formen rotundum Efferent veins Superior petrosl sinus (SPS) Inferior petrosl sinus (IPS) Bsilr plexus (BP) Pterygoid plexus (PP) Inferior petroclivl vein (IPCV) Petro-occipitl sinus Internl crotid venous plexus Formen ovle plexus (FOP) Formen lcerum plexus (Trnsverse occipitl sinus) Tributries of the Cvernous Sinus (Afferent Veins) Orbitl Veins The orbit is drined by the superior nd inferior ophthlmic veins. These veins nd their tributries re unusul in tht they re without vlves nd re mrkedly tortuous with mny plexiform nstomoses (Doyon et l. 1974). The complex ntomy of the orbitl veins, their numerous tributries, nstomoses nd dringe pthwys hs been studied erly on by both detiled ntomic dissections (Henry 1959),nd specificlly developed rdiogrphic technique, orbitl phlebogrphy. The ltter, mjor dignostic method for introrbitl tumors nd infectious diseses in the pre CT MRI er provided highly detiled ntomic knowledge, tht still cnnot be obtined tody even when using high-resolution MRI or DSA (Doyon et l. 1974; Fischgold et l. 1952; Hnfee et l. 1968; Lombrdi nd Psserini 1967, 1968, 1969; Cly nd Vignud 1974; Cly et l. 1976; Vignud nd Cly 1974; Vignud et l. 1972, 1974; Theron 1972; Gozet et l. 1974). In 1755, Zinn described the superior ophthlmic vein, its course through the superior orbitl fissure, nd its venous nstomoses in the orbit. According to tht uthor, Veslius ws the first to describe the ophthlmic vein s beginning t the medil ngle of the eye nd Fllopin ws the first to describe its nstomoses with the fcil vein. Wlter first correctly described the ntomic reltion of the superior ophthlmic vein, inferior ophthlmic vein nd fcil veins in In their reports, Gurwitsch (1883) nd, in 1887, Festl described firly ccurtely the orbitl veins nd their reltion with the cvernous sinus, the oculr muscles, the optic nerve nd the ophthlmic rtery. By dissection of the veins in ten orbits tht hd been injected previously with geltin, Henry (1959) described the orbitl veins most ccurtely (Doyon et l. 1974). Superior Ophthlmic Vein (Fig. 3.10) The superior ophthlmic vein (SOV) is connected with ll other introrbitl veins through numerous direct nd indirect collterls. The vein origintes t the junction of its inferior nd superior roots, lso described s inferior nd superior tributries (Doyon et l. 1974). The vein mesures 2 mm nteriorly nd pproximtely 3.5 mm posteriorly, nd increses in dimeter t the junction of ech tributry. As described by Doyon et l. (1974), the inferior root cn be considered the true initil segment of the SOV belonging to the ngulr vein, while the superior root represents continution of the frontl vein. Some uthors (Dutton 1994) consider the superior root the suprtrochler vein nd the true SOV emerging t the junction between the infrtrochler nd the suprorbitl vein. As stressed by Biondi et l. (2003), this prticulr ntomy my

48 3.3 Vsculr Antomy 37 SIII Intrconic SOV SII Extrconic portion SI Frontl v. Superior root of SOV Sup. oblique pulley Vorticose v. Lcriml v. SOV Sup. rectus Levtor plp. Superior root Sup. oblique Ophthlmic. Cvernous sinus Inferior root of SOV Lcriml glnd Inferior root Apsidl v. Angulr v. Apsidl v. (nt. post.) Int. psidl v. Optic n. Sup. orbitl fissure v. IOV Fcil v. b Inf. oblique Brnch of IOV Trnsverse nstomosis between psidl vv. Fig. 3.10,b. Segments of the superior ophthlmic vein. (From Doyon et l., 1974). Note the prticulr rrngment of superior nd inferior roots of the SOV, not demonstrted in ll textbooks. Being wre of this prticulr ntomy cn become importnt when nvigting microctheter through the ngulr vein during fcil vein/superior ophthlmic vein pproch (Biondi et l. 2003) (see lso Fig d) become importnt when performing trnsfcil or trnsophthlmic pproches. Sesemnn (1869) s well s Gurwitsch (1883) divided the SOV into two or three brnches tht unify lter on. The SOV enlrges during their course nd nrrows fter pssing the SOF, indicting tht dringe is minly posterior towrds the CS. The slight enlrgement before entering the CS is sometimes described s the ophthlmic sinus (Sinus ophthlmicus). Hnfee et l. (1968) nd others (Doyon et l. 1974) mde the suggestion to clssify the SOV into three segments. The nterior segment (first segment, Doyon et l. 1974) forms underneth the superior oblique muscle by unifiction of two brnches, of which one comes from the suprorbitl vein (often lrger) nd psses through the suprorbitl incisur (superior root). The other origintes from the ngulr vein (inferior root) nd psses long the medil orbitl wll. The nterior prt of the SOV lies initilly djcent to the orbitl roof, lies outside the muscle cone but is fixed by its reltion to the superior oblique muscle. The vein then enters the muscle cone bout 5 mm behind the posterior pole of the globe nd courses s the middle (second) segment lterl nd below the superior rectus muscle nd the levtor plpebre muscle dorslly to rech the superior orbitl fissure (SOF). As the vein pproches the lterl wll of the orbit, it crosses the underlying optic nerve nd ophthlmic rtery nd receives the lcriml vein. In this segment the vein is not fixed nd cn be esily displced by introrbitl msses. The posterior (third) segment begins when the vein turns medilly, posteriorly nd slightly downwrds. The SOV leves the muscle cone, courses between the tendinous portions of the superior rectus nd levtor muscles nd the lterl rectus muscle inferiorly. It leves the orbit by following the lterl edge of the nnulus of Zinn nd then descending long the inferior edge of the superior portion of the SOF (Doyon et l. 1974). When the vein psses the fissure nd enters the nterior CS its lumen is usully nrrowed. On ngiogrms, this segment is best visible in lterl projections, strting where the vein turns downwrds, nd then psses through the fissure nd ending t the CS (Hnfee et l. 1968; Brismr 1974). The SOV hs connections with the veins of the fce nd forehed (ngulr vein, fcil vein, frontl nd temporl veins) nd receives blood from the territory of the ophthlmic rtery (four vortex veins of the globe, ciliry veins, centrl retinl veins) when pssing through the orbit. The dimeter of the SOV vries considerbly nd symmetricl rrngement of the vein is frequently seen without underlying pthology (BrismR 1974). The SOV receives blood minly from two lrge ethmoidl veins, four vortex veins nd lrge lcriml vein, which my occsionlly drin into the CS seprtely (Miller N 1998). Inferior Ophthlmic Vein The inferior ophthlmic vein (IOV) is much smller nd often not visible on ngiogrms. It courses over the floor of the orbit, tkes off brnches from the inferior oblique muscle s well s from the inferior rectus muscle, drins veins of the lcriml sc nd the lower eye lid nd two lower vortex veins, nd

49 38 3 Antomy of the Cvernous Sinus nd Relted Structures then psses with the inferior rectus muscle bckwrds through the orbit. It drins in the orbit into the SOV or gives off connecting brnch nd empties fter pssing the SOF into the CS (Lombrdi nd Psserini 1967, 1968). According to Hnfee et l. (1986), the IOV drins directly into the PP. Browder nd Kpln (1976) found the SOV ws the lrgest venous trunk joining the CS. According to Gurwitsch (1883) this vein my be bsent in one-third of the cses nd is usully n inconspicuous structure tht is hs been given numerous other nmes (Lng 1983). The existence of third introrbitl vein, the middle ophthlmic vein, is doubtful (Lombrdi nd Psserini 1967). Centrl Retinl Vein (No Direct CS Tributry) The centrl retinl vein (CRV) drins the retin nd the introrbitl prts of the optic nerve nd is of specil clinicl significnce. Formed by the union of vrious retinl veins t the level of the lmin cribros, it runs through the core of the optic nerve in compny with the centrl retinl rtery. Within the lmin cribros the centrl retinl vein nstomoses through lterl twigs with the choroidl venous plexus. In ptients with occlusion or chronic compression of the CRV (Miller N 1998), these smll nstomotic chnnels my enlrge to become optociliry veins tht shunt blood from the retinl to the choroidl circultion The CRV exits from the optic nerve bout 10 mm posterior to the globe, usully in compny with the CRA. The vein normlly courses for 4 8 mm through the vginl spces surrounding the nerve before it pierces the dur to exit the nerve 1 2 mm dorsl to the point of entry of the rtery. The CRV my drin into the SOV, into some other orbitl veins or directly into the CS (Lng 1983). It is believed tht the CRV hs no functionlly dequte collterls within the eyebll. Occlusion of the vein will therefore result into serious circultory disorders with retinl venous infrcts or hemorrhges nd subsequently secondry glucom. Superficil Middle Cerebrl Vein, Sylvin Vein The superficil middle cerebrl vein (SMCV), lso clled the superficil Sylvin vein (Sylvii) ( Glligioni et l. 1969), origintes in the posterior segment of the lterl fissure, nd courses long the fissure downwrds nd nteriorly to rech the regio pterionlis. According to trditionl views, the vein penetrtes the dur nd becomes venous sinus (the sphenoidl prt of the Breschet` sinus, sphenoprietl sinus) which courses medilly long the lesser sphenoid wing to the nterior prt of the CS. The existence of this connection ws recently questioned by Ruiz et l. (2004) who studied the crnil venous system of 15 nonfixed humn specimens. These uthors could not find connection between the SMCV nd the sphenoprietl sinus in their series nd, referring to the originl work of Breschet, stress tht this connection ws in fct never illustrted in his work (see below). Though the connection of the SMCV with the CS is not developed t birth, it forms in lter stge of life. Typiclly, t infnt stge the vein drins posteriorly into the tentoril sinus to rech the trnsverse sinus. Under norml conditions nd fter complete development, the SMCV drins the insul, the cortex on both sides of the lterl fissure, s well s prts of the occipitl nd frontl lobes. It nstomoses with the deep venous system vi the uncl vein, the insulr vein nd the bsilr veins nd other superficil cerebrl nd durl veins. It communictes lso with the fcil veins vi the venous circultion of the orbit (Glligioni et l. 1969). It my further tke off blood from the middle meningel veins, which re connected with diploic veins nd the superior sgittl sinus. The dringe system of the SMCV my show numerous vritions. As rule the SMCV s vein of durl origin usully does not drin into the bsl vein of Rosenthl, since the ltter nd its tributries re derived from veins of pil origin (Hung nd Wolf 1974). It my however be joined by the first or second segment of the bsl vein nd continue into durl sinus. Bisri (1985). who studied the course of the SMCV in 140 humn cdvers, found the following vritions in its dringe: 51% into both SPPS, 6% into SPPS nd CS, 13% in SPPS nd middle meningel veins. In 14% the vein drined into the CS lone, in 5% into the CS nd meningel veins. In one cse it drined into vein in the formen lcerum nd the SPPS, in one cse into the SPPS nd the SPS, in nother into the middle meningel vein on either side. The uthors found one unusul dringe into the SSS nd emphsize tht prior to their study, the mjority of the work on the termintion of the SMCV ws bsed on roentgenogrphic findings rther thn on dissections. Bsed on CT ngiogrms, Suzuki nd Mtsumoto (2000) found seven different types of dringe of which the most frequent were the sphenoprietl sinus (54%), the cvernous sinus (7%) nd n emissry vein of the formen ovle

50 3.3 Vsculr Antomy 39 (12%). The superficil nd deep SMCVs my lso drin directly into the lterocvernous sinus (Sn Milln et l. 1999). This work ws recently complemented by Tnoue et l who, by mens of MRI exmintions, simply clssified the SMCV into four min types of vrition. A direct connection with the SPPS nd nterior CS ws found in 39%, connection with the lterl spect of the CS independently in 30%, direct communiction with the PP in 11% nd posterior course cross the petrous ridge to drin into the SPS or trnsverse sinus in 8% of their cses. Uncl Vein, Uncinte Vein The uncl vein (UV) is smll, but importnt vein tht cn be ngiogrphiclly identified in two out of three cses (Gozet et l. 1974). Surprisingly, this vein however, is neglected in schemtic drwings of mny textbooks. It psses the medil temporl lobe long the nterior mrgin of the uncus nd drins either in the SMCV, the SPPS or directly in the CS. According to Wolf et l. (1955) this vessel mrks the medil spect of the temporl lobe nd therefore hs dignostic importnce. Bisri (1985) found it in only 5% of the specimens pointing to the fct tht this vein hs been not described hitherto in mny ntomicl textbooks. The uncl vein is derivte of the deep telencephlic vein, one of three primitive veins forming the bsl vein of Rosenthl (Hung nd Wolf 1974; Pdget 1956) by longitudinl nstomoses, nd joins the SMCV in the neonte stte, both drining into the CS. Filure of these longitudinl nstomoses is most frequent between the first nd second segments of the bsl vein resulting into common stem formed by tributries of the strite veins nd posterior insulr veins tht drin nteroinferior into the SPPS, CS or PCS (Hung nd Wolf 1974). This vein hs been designted the uncl vein (Wolf et l. 1963) nd my be responsible for redirection of corticl venous dringe cusing intrcerebrl venous hemorrhge in DCSF ( Tkzw et l. 2005). Vrious secondry nstomoses between the SMCV, SPPS, SOV, CS nd the uncl vein cn occur (Hung nd Wolf 1974). Sphenoprietl sinus (Breschet), Sinus le prve, Sinus sphenoidles superior (Sir C. Bell) The sphenoprietl sinus (SPPS) is the lrgest of the meningel veins, usully coursing with the nterior brnch of the MMA bove the level of the pterion (Ok et l. 1985). The clssic description of the SPPS is n ntero-inferior continution of the meningel sinus following the rc of the sphenoid wing nd reching the nterior CS. The term sphenoprietl sinus ws introduced originlly by Breschet (1829) nd hs been widely used in ntomicl textbooks. According to him, the nterior brnch of the middle meningel vein joins smll durl sinus, the sinus of the lesser sphenoid wing (Ruiz et l. 2004). In mny textbooks (Gry 1918; Lng 1979; Osborn 1980) nd erly originl ppers (Wolf 1963), this sinus is considered the min dringe of the SMCV to rech the cvernous sinus which is probbly due to the close ntomicl reltionship of both vessels while coursing long the sphenoid wing. As pointed out by Pdget, this sinus is not clerly defined nd redily confused with conspicuous remnnt of the tentoril sinus drining the SMCV while following the lesser sphenoid wing. A true constnt connection between the SMCV nd the SPPS ws lso questioned in recent study of Ruiz et l. (2004) who suggested insted tht both veins pss next to ech other but independently under the sphenoid wing to rech the cvernous sinus. This is supported by reevlution of originl descriptions of the French ntomists Breschet (1829), Trolrd (1890) nd others. The uthors suggest tht the prietl portion of the SPPS should be rther considered continution of the nterior middle meningel vein wheres the sphenoidl portion of SPPS would represent distinct durl sinus coursing under the lesser sphenoid wing with its own tributries. This rrngement would be more ppropritely termed sinus of the lesser sphenoid wing s erlier suggested by Wolf et l. (1963). The SPPS my join the sphenoidl emissry veins to rech the PP, or pss further posteriorly to rech the SPS or the trnsverse sinus (TS; lterl sinus, LS). The former vrint is clled sphenobsl sinus, the ltter sphenopetrosl sinus. Becuse both re remnnts of the embryonic tentoril sinus, the SMCV my lso empty into these sinuses when the SPPS is bsent (Ok et l. 1985). The course of the SMCV lterlly over the middle crnil foss is lso nmed prcvernous sinus (Sn Milln Ruiz et l. 1999). Intercvernous Sinus, Sinus intercvernosus, Sinus circulris (Ridley), Sinus ellipticus, Sinus coronrius, Sinus clinoideus (Sir C. Bell), Sinus trnsversus selle equine (Hller) The two cvernous sinuses re connected by mens of one or more trnsverse vessels, intercvernous sinuses (ICS) which cross the pituitry foss

51 40 3 Antomy of the Cvernous Sinus nd Relted Structures (Knott 1882). There re usully two ICS with dimeter of up to 8 mm (Lng nd Weigel 1983), connecting the CS nteriorly nd posteriorly to the hypophysis nd forming venous circle (elliptic sinus, circulr sinus, clinoid sinus). The nterior ICS is lrger; the posterior cn be completely bsent. Knott (1882) found its bsence in 26/44 cses (ntomicl studies). In two cses he found the posterior ICS ws lrger nd in one cse it ws the only one present. He found 3 trnsverse ICS in 15/44 cses. Smll irregulr venous sinuses coursing beneth the pituitry glnd nd drining into the ICS were lredy described by Winslow s soclled inferior circulr sinus. Knott (1882) found this rrngement in only 6 cses, wheres in 12 others he found single intercvernous vein beneth the pituitry body. In ddition to the two ICS, Doyon et l. (1974) nd others (Lsjunis et l. 2001) hve described posterior communiction vi n occipitl trnsverse sinus which probbly corresponds to the bsilr plexus. Ysud et l. (2004, 2005) in his recent study of the medil wll of the CS lso described n inferior intercvernous sinus tht connects the pired CS. These ICS extend cross the midline between the meningel durl lyer covering the inferior spect of the pituitry glnd nd the endostel lyer covering the floor of the sell. Meningel Veins The meningel veins (MV) re often developed s pirs, enclosed by dur sheets. Commonly, they ccompny meningel rteries nd provide venous dringe for the crnil dur mter nd re considered meningel sinuses by some uthors (Ok et l. 1985). They course between the rteries nd the overlying bone which led to compression of their lumen nd the typicl rdiogrphic ppernce of prllel chnnels (Ok et l. 1985). They communicte with the superior sgittl sinus nd unify to form two trunci nteriorly nd posteriorly, which ccompny the brnches of the meningel rteries. At the bse of the skull they pss through the formen spinosum nd ovle to connect with the pterygoid plexus. The veins ccompnying the nterior brnch of the MMA drin into the SPPS, the CS or emissry veins (Ok et l. 1985). Veins of the Formen Rotundum, Emissry Vein Knott (1882) observed in 2 cses (2/44) n dditionl smll tributry in the form of n emissry vein pssing through the cnlis rotundus ccompnying the mxillry division of the trigeminl nerve. It is connected with the extrcrnil pterygoid plexus nd is considered by Browder nd Kpln (1976) drining vein (rrely severl smll veins). Knott (1882) found in 23/44 subjects n inconstnt vein lying in the dur mter on the inner surfce of the greter sphenoid wing. On rre occsions, the bsl vein or the inferior ventriculr vein ws found to nstomose with the lterl wll of the CS (Lng 1983) Dringe of the Cvernous Sinus (Efferent Veins) Superior Petrosl Sinus, Sinus petrobsilris (Lnger), Sinus tentorii lterlis (Weber), Sinus petrosus superficilis The superior petrosl sinus (SPS) courses between the lyers of the tentorium cerebelli over the superior spect of the petrous bone nd connects the posterior superior ngle of the CS with the proximl sigmoid sinus or the trnsverse sinus (Ok et l. 1985). It receives blood from the cerebellr veins, the lterl mesencephlic vein, the petrosl vein, the prcvernous sinus, the bsl vein nd the rcute vein. Although often considered chnnel drining the CS, the SPS my ctully not drin blood from the CS. The blood flow is frequently directed from posterior to nterior, towrds confluence t the petrous pex, which is formed by the posterior prt of the CS, (trnsverse) communicting sinus, connecting the posterior CS nd the IPS, drining the blood towrds the IJV (Kryenbühl nd Ysrgil 1997). In contrst to Theron (1972), in our experience the posterior CS is reltively often visible in vertebrl ngiogrms, which is explined by this direction of the flowing blood within the SPS. Knott (1882) found complete bsence of this sinus in 3/45 cses. Inferior Petrosl Sinus, Sinus petrosus profundus, Sinus petro-occipitlis superior (Trolrd) The inferior petrosl sinus (IPS), shorter but lrger thn the SPS, represents the min posterior dringe of the CS towrds the bulb of the internl jugulr vein. It courses long the lower edge of the petrous bone nd the petro-occipitl suture, lying between the petrous pyrmid nd the clivus in the petroclivl fissure. It reches the jugulr formen nd psses together with the glossophryngel, vgus nd ccessory nerve to enter the internl jugulr vein (IJV). It receives blood from veins of the lbyrinth, the

52 3.3 Vsculr Antomy 41 cochler queduct, medull, pons nd cerebellum. The IPS hs close reltionship with the bducens nerve, which is imbedded in the wll of its sgittl segment. According to Boskovic et l. (1963) this sinus hs dimeter of 7 10 mm in 93% of cses nd hs horizontl nd sgittl prts. According to Lng (1983) it enters the posterior foss 2 6 mm medil to the trigeminl pore (Dorello s cnl) with dimeter of 7 10 mm in its longitudinl nd mm in its trnsverse prt. The ctul entry is usully situted below the superior sphenopetrosl ligment nd lso contins the sixth CN in its lterl ngle. Shiu et l. (1968) studied 346 ptients undergoing petrosl sinus smpling nd described 4 different types of junction between the IPS nd the IJV (Fig. 3.11): Type I: 45% directly into the IJV Type II: 24% into vein, which connects the deep cervicl plexus with the IJV Type III: 24% vi plexus into the IJV Type IV: 7% directly into the cervicl plexus Miller et l. (1993) modified this clssifiction nd dded n incomplete type IV which is chrcterized by smll vein between the IJV nd the point t which the IPS nstomoses with the vertebrl venous plexus. It needs to be remembered, however, tht these descriptions re exclusively bsed on retrogrde venous ngiogrms nd therefore my, due to vrible venous hemodynmics, thrombosis of the IPS nd suboptiml imge qulity, not fully reflect the true ntomy in this re. Thus, some vsculr detils like the IPCV ws not recognized s such, lthough opcified in some of the figures (Fig. 2C therein). Knott (1882) nd others (Lblette 1891; Sppey 1888; Theile 1843) described in the nineteenth century connection between the IPS nd the IJV below the skull bse (3/8 of n inch). Lng nd Weigel (1983) found such vrint in 10% of their specimens. The uthors describe in ddition n inferior petrosl sinus cnl in 2% 3% of the cses. Surprisingly, this vrint is not included in the clssicl description of Shiu et l. (1968). Recent studies hve shown tht the IPS, s n ntomicl vrint, my lso enter the IJV fr below the skull bse (up 4 5 cm) (Gilloud et l. 1997). Gilloud et l. (1997) hve nmed this deep termintion of the IPS s the ccessory jugulr vein. We hve observed this sitution in three ptients with vsculr lesion of the CS (see Fig. 7.37) nd were ble to successfully ctheterize Cvernous sinus Inf. petrosl sinus Inf. jugulr v. Deep cervicl vv. Fig Four min types of IPS-IJV connections s described by Shiu (from Shiu et l., 1968). This widely used clssifiction is bsed on 2D ngiogrms from the pre-dsa er. It does not disply ll ntomic detils in this re, which is in prt due to the technique used for the cvernous sinus venogrphy. Retrogrde opcifiction of the IPS nd CS cn be limited by locl hemodynmics nd thrombotic processes. Furthermore, modern bi-plne DSA imging provides significnly better sptil resolution (see Chp. 7). The inferior petroclivl vein nd the internl crotid rtery venous plexus re not illustrted, lthough visulized in modern high-qulity phlebogrms. In AP view the vein lies next to the IPS nd follows n lmost prllel course. Neither the fct tht the IPS my be thrombosed nd thus only prtilly filled, nor the possibility of n bbernt inferior petrosl sinus (deep termintion below the skull bse) ws known or tken into ccount t tht time this berrnt inferior petrosl sinus (Benndorf nd Cmpi 2001). According to Ktsut et l. (1997) the IPS, in its course long the petroclivl fissure, lso hs connections with the CVP through the intrpetrosl veins nd with the venous chnnel, clled the inferior petroclivl vein (IPCV, see below) which courses long the extrcrnil surfce of the petroclivl fissure [probbly identicl to the petro-occipitl sinus described by Aubin et l. (1974) nd others (Cly nd Vignud 1974)]. The IPS forms within the jugulr formen venous confluens of 2 3 mm dimeter, receiving blood from the venous plexus of the hypoglossus cnl, the inferior petroclivl vein

53 42 3 Antomy of the Cvernous Sinus nd Relted Structures nd tributries from the vertebrl venous plexus nd the posterior condylr vein. Sometimes two min chnnels connect the petrosl confluens with the jugulr bulb (Ktsut et l. 1997). Venous Plexus of the Hypoglossl Cnl, Anterior Condylr Vein This plexus is lso referred to s the nterior condylr vein (ACV), which connects the mrginl sinus with the JB. It is crossed by trbecule nd my empty into the lower end of the IPS or directly into the IJV. Occsionlly it drins into the lower end of the sigmoid sinus nd my communicte with the posterior condylr vein nd the vertebrl plexus (Ktsut et l. 1997). Posterior Condylr Vein The posterior condylr vein (PCV) psses through the posterior condylr formen nd courses in the posterior condylr cnl to connect the vertebrl venous plexus with the junction of the sigmoid sinus nd the JB. The posterior condylr formen opens into the posteromedil junction of the sigmoid sinus nd the JB. It hs communictions with the confluens t the lower end of the IPS nd vi bony chnnel with the nterior condylr vein (venous plexus of the hypoglossl cnl) (Ktsut et l. 1997). Lterl Condylr Vein The lterl condylr vein (LCV) runs between the inferior spect of the externl orifice of the hypoglossl cnl (nterior condylr cnl) nd the suboccipitl cvernous sinus s described by Sn Milln Ruiz et l. (2002) nd Arnutovic et l. (1997). Inferior Petroclivl Vein The inferior petroclivl vein (IPCV) (Fig. 3.12, see lso Figs ) is, ccording to Ktsut et l. (1997), mirror imge to the IPS coursing long the extrcrnil surfce of the petroclivl fissure nd empties into the confluens t the lower end of the IPS (Rhoton 2000). This vein is in some studies, probbly erroneously clled inferior petro-occiptl vein (Sn Milln Ruiz et l. 2002). Petro-occipitl Sinus, Sinus petro-occipitlis inferior, petro-occipitl vein (Pdget) This very smll sinus, described by Trolrd (1890), lso origintes from the CS, courses prllel to the IPS, but outside the skull, long the petro-occipitl suture, enters the IPS just before its connection with the IJV nd cn be seen in phlebogrms of the jugulr vein (Aubin et l. 1974) or in orbitl phlebogrms (Cly nd Vignud 1974). Becuse of its smll cliber nd its proximity to the IPS it my be esily superimposed nd is best visulized in the projection fter Aubin et l. (1974) nd cn be missed on stndrd ngiogrphic projections (see Chp. 7). The projection is clled fter Hirtz nd ws suggested by Aubin. Both, the petro-occipitl sinus nd the inferior petroclivl vein re possibly referring to the sme vsculr structure. Trnsverse Occipitl Sinus (Doyen) Doyen et l. (1974), while reviewing the ophthlmic veins, described sinus s trnsverse occipitl sinus tht connects both posterior CSs horizontlly long the upper surfce of the clivus. In nother figure (Fig therein) this sinus trvels cudlly to rech the formen mgnum. I hve not be ble to find this sinus mentioned elsewhere other thn in Aubin et l. (1974) who describe it s the communiction of the two posterior CS with the nterior segments of the IPS nd the SPS. Bsilr Plexus (Virchow) The bsilr plexus (BP) consists of severl communicting venous chnnels between the durl sheets bove the dorsum selle of the sphenoid bone nd the bony skull bse. The lrgest nd most constnt connection cross the midline between the CS (Renn nd Rhoton 1975) extends from the dorsum selle nd the prs bsilris of the sphenoid bone downwrds, to become the mrginl sinus. Its upper prt, lying over the rostrl end of the clivus, is connected with the posterior CS nd both SPS. Its lower prt is connected with both IPS nd with the nterior internl vertebrl venous plexus. According to Lng (1979) it regulrly contins clivl rmi of the MHT. Mrginl Sinus This sinus lies between the lyers of the dur mter djcent to the nterior inner surfce of the formen mgnum. It receives blood from the bsilr plexus nd communictes with the occipitl sinus nd the nterior internl venous plexus. Tubbs et l. (2006) recently studied further the ntomy nd found significnt communiction between the mrginl sinus nd the veins of the hypoglossl cnl, nd tht the vertebrl rtery ws noted to pierce this sinus in the mjority of the cses.

54 3.3 Vsculr Antomy 43 Internl Crotid Artery Venous Plexus, Sinus Venous Croticus (Hike), Crotid Sinus, Pericrotid Plexus The internl crotid rtery venous plexus (ICAVP) ws first described by Rektorzik in 1858 s prs intrcnlem sinus croticus, nd descends from the inferior cvernous sinus enclosing the crotid rtery more or less completely t the lower prt of the crotid cnl (Knott 1882) (Fig. 3.12). This plexus converges to form one or more trunks which open finlly into the IJV nd ws found by Knott (1882) in ech cse of his series. Hike (1902) found tht the sinus resembles the rchitecture of the CS, consisting of numerous smll vsculr spces in children tht confluent to lrger lcunes with ge. To him it ppered in some cses more like plexus, in others more like sinus. Knosp et l. (1987) considers the plexus in mny cses very importnt dringe system nd found it more prominent in the fetus when compred to the dult condition. Lng (1983) found this plexus running together with the rtery, sympthetic nerves nd tissue of the rteril sheth to form two longitudinl veins t the externl perture of the crotid cnl which originte ner the bend of the ICA. The initil prt of the plexus communictes with the IPS vi medil intrpetrosl vein in bout 46% nd vi lterl intrpetrosl vein in 50% of the cses. The former termintes bout 11 mm bove the jugulr formen, the ltter termintes in the vicinity of the formen (Lng nd Weigel 1983). Pullus et l. (1977) found this (perirteril venous) plexus in 76% of the cses, in 24% it ws poorly developed or bsent. It extended n verge of 7.6 mm into the cnl nd ws locted between the floor of the middle crnil foss nd the rtery in only 4%. A fistul between the ICA nd this plexus could mimic true CCF. This sinus is difficult to identify on ngiogrms or phlebogrms nd my be superimposed by the IPS or IPCV, nd thus is not shown by severl uthors investigting the ngiogrphic ntomy in the CS IPS region (Hnfee et l. 1968; Shiu et l. 1968; Miller DL et l. 1993). Aubin (1974) demonstrted its visibility in xil projections, lthough it my be superimposed by the petro-occipitl sinus (IPCV). Modern high-resolution DSA nd threedimensionl DSA proves vluble in visulizing Rektorzik plexus (Chp. 7). Formen Ovle Plexus (Trigeminl Sinus), Sphenoid Emissry, Rete of the Formen Ovle Nuhn (in Knott 1882) described pir of smll veins, which pss through the formen ovle to rech the pterygoid plexus. Knott found this plexus quite vrible: in 18/45 cses he found pir of veins on both sides, in 10 cses he found pir on one side nd single vein on the other, in 11 cses there ws single vein on ech side nd in 5/45 cses totl bsence on one side. Henderson (1966) clled this sinus trigeminl sinus. Browder nd Kpln (1976) found number of veins drining the pchymeninx of the inferior prt of the cerebrl hemispheres converging to the middle crnil foss nd coursing to the formen ovle, where chnnels from the ventrolterl spect of the CS join them. Pdget (1956b) ssumed tht this plexus my not necessrily be concerned with the venous dringe, especilly in the young. In dults, however, we see this plexus often drins the SMCV directly, bypsses the CS nd my rech dimeter of up 6 mm (roughly the dimeter of the formen). Vein of the Sphenoid Formen (Formen Venosum, Formen of Veslius) A very smll, inconstnt vein supplementing the formen ovle plexus when pssing through the formen venosum the mrgins of which re usully not ossified t birth (Pdget 1956b). Formen Lcerum Plexus Knott (1882) constntly found vrying number of smll veins pssing through the formen lcerum nd lwys being connected with the cvernous sinus. The emissry veins connect with the pterygoid plexus but lso with the inferior petroclivl vein. Pterygoid Plexus The pterygoid plexus (PP) is n extensive network of smll venous chnnels in the neighborhood of the IMA nd lies lterl nd medil to the lterl pterygoid muscle nd is connected with the CS vi sphenobsl emissry chnnels pssing through the formin in the middle crnil foss. It receives blood from the trnsbsl veins (middle meningel veins, formen ovle plexus, crotid venous plexus, vein of the formen rotundum, vein of the formen venosum). The pterygoid plexus lso communictes with the ophthlmic veins through the inferior orbitl fissure, with the nterior fcil vein vi deep fcil brnch nd receives tributries corresponding to brnches of the pterygopltine mxillry rtery segment (Osborn 1981). It usully drins posteriorly into the mxillry vein, which forms together with the superficil temporl vein the retromndibulr vein. Although the ltter vessel is usully

55 44 3 Antomy of the Cvernous Sinus nd Relted Structures mjor tributry of the externl jugulr vein (EJV) the pterygoid plexus my lso drin vi posterior nd common fcil veins into the IJV (Osborn 1981; Goss 1966). In some cses, the SMCV drins not vi the CS but directly into the PP, disposition identified by Osborn (1981) s the sphenobsl pttern nd found in 34% of the cses. This pttern ws differentited from the sphenopetrosl pttern vi the sphenopetrosl vein into the trnsverse sinus (13%) or combintion of both (24%) Other Veins of Importnce for the CS Dringe or for Trnsvenous Access to the CS Fcil Vein The ntomy of the fcil vein (FV) hs been described in prt bove (see under SOV) nd hs some importnce for retrogrde trnsvenous pproches. This vein drins the nterior portion of the sclp nd the soft tissue of the fce nd begins t the medil plpebrl ngle s direct continution of the ngulr vein (Osborn 1981). The FV receives the suprtrochler nd suprorbitl veins nd descends obliquely downwrds crossing the fce nd the msseteric muscle behind the fcil rtery nd until it reches the body of the mndible. Slightly inferior nd nterior to the ngle of the mndible, it is joined by the nterior portion of the retromndibulr vein nd forms the common fcil vein, which usully drins into the internl jugulr vein (Peuker et l. 2001). In some cses it my lso drin into the EJV (Osborn 1981) nd under rre circumstnces even into the superficil temporl vein (Peuker et l. 2001). The FV receives blood from the l nsi, the deep fcil vein (connection to the pterygoid plexus), inferior plpebrl, superior nd inferior lbil, buccintor, protid nd msseteric veins. Under the mndible the submentl nd the submndibulr veins join the FV. Frontl Vein The frontl vein (FrV) begins on the forehed in venous plexus which communictes with the frontl brnches of the superficil temporl vein (Gry 1918). The veins converge to form single trunk, which runs downwrd ner the middle line of the forehed prllel with the vein of the opposite side. The two FrVs re joined t the root of the nose by trnsverse brnch, clled the nsl rch, which receives some smll veins from the dorsum of the nose. At the root of the nose the veins diverge nd t the medil ngle of the orbit ech of them joins the suprorbitl vein to form the ngulr vein. Occsionlly, the frontl veins join to form single trunk, which bifurctes t the root of the nose into the two ngulr veins. The min stem of the FV usully forms the superior tributry to the ngulr vein (AV) (Doyon et l. 1974) consisting of two prts: the superior root of the SOV nd the internl frontl vein. The former is ccompnied by the suprorbitl nerve nd rtery, which pss through the suprorbitl notch (incisur suprorbitlis) nd penetrte the orbitl septum bove the trochle of the superior orbitl muscle. The ltter is direct superior extension of the ngulr vein nd hs been used s common pproch to perform orbitl phlebogrphy (Doyon et l. 1974). Therefore, in cse of DCSF with prominent nterior venous dringe, it cn lso be used s percutneous pproch for ctheteriztion of the SOV nd CS (Venturi et l. 2003). Angulr Vein The ngulr vein (AV) represents n nstomosis between the fcil vein nd the SOV nd is formed by the confluence of the suprorbitl nd frontl veins. Its subcutneous course is down the side of the nose, lterl to the ngulr rtery. The vein then crosses the nsl edge of the medil plpebrl ligment pproximtely 8 mm from the internl cnthus. The ngulr vein is continuous below with the fcil vein (Doyon et l. 1974). The ngulr vein hs three min tributries: medil or prensl rch, the inferior root of the SOV nd the internl frontl vein (Doyon et l. 1974). Middle Temporl Vein The middle temporl vein (MTV) rises ner the eye, hs n lmost horizontl course bove the zygomtic process nd is connected with plpebrl veins, the suprorbitl vein nd the fcil vein. The vessel joins the superficil temporl vein to form the retromndibulr vein. According to Hyrtl (1885) this vein is often found s plexus. On ngiogrms it usully occurs s single vein tht hs typicl cute ngle when it psses over the zygomtic rch. Becuse it nstomoses with the ngulr vein, this vessel my lso be involved in the dringe of DCSF nd cn be used s n pproch for EVT (Cheng et l. 2003). Internl Jugulr Vein The internl jugulr vein (IJV) is the mjor dringe pthwy for the cerebrovsculr system, collecting blood from the brin, the superficil prt of the fce,

56 3.3 Vsculr Antomy 45 nd from the neck (Gry 1918). The IJV begins in the posterior comprtment of the jugulr formen, t the bse of the skull, being continuous with the sigmoid sinus. Its origin is somewht dilted, clled the jugulr bulb (JB, or superior bulb) (Gry 1918). It runs down the side of the neck, lying t first lterl to the ICA nd then lterl to the CCA. Above it lies the rectus cpitis lterlis, behind the ICA nd the nerves pssing through the jugulr formen. Lower down, the IJV nd ICA lie in the sme plne, the CNs IX nd XII pssing forwrd between them. The CN X descends between nd behind the vein nd the rtery in the sme sheth nd the CN XI runs obliquely bckwrd, superficil or deep to the vein. At the bse of the neck the right IJV is locted t little distnce from the CCA nd crosses the first prt of the subclvin rtery while the left IJV usully overlps the CCA. The left IJV is usully smller nd ech vein contins pir of vlves pproximtely 2.5 cm bove their termintion. The most importnt superficil reltionship of the IJV is the sternomstoid muscle, which is lterl to the vein in its upper prt nd covers it in its lower prt. The IJV unites t the bse of the neck with the subclvin vein to form the innominte vein with little second dilttion bove, clled the inferior bulb. The IJV receives blood from the sigmoid sinus, inferior petrosl sinus, fcil, lingul, phryngel superior thyroid, middle thyroid veins nd sometimes the occipitl veins. In clinicl sense the most crucil reltionship is with the VA nd CCA/ICA becuse indvertent puncture (usully of the ICA) cn be serious compliction (see below). The Externl Jugulr Vein The externl jugulr (EJV) vein drins the greter prt of the blood from the exterior of the crnium nd the deep prts of the fce. It is formed by the junction of the posterior division of the posterior fcil vein nd the posterior uriculr vein. This vessel origintes in the substnce of the protid glnd t the level of the ngle of the mndible nd runs perpendiculrly down the neck to end in the subclvin vein (Gry 1918). It usully tkes the retromndibulr vein s mjor tributry, which is formed by the mxillry vein (drining the PP, see bove) nd the superficil temporl vein. There ppers to be n inverse correltion between the size of the externl nd internl jugulr vein thus the presence of lrge EJV my be n indictor of smll IJV nd therefore of potentilly more difficult IJV puncture (Stickle nd McFrlne 1997). Vertebrl Vein, Vertebrl Artery Venous Plexus The vertebrl vein is formed in the sub-occipitl ngle, from numerous smll tributries of the internl vertebrl venous plexuses, minly by the confluences of the nterior nd posterior condylr veins (roots) which join to form the plexus of the vertebrl vein, the supply functions of which cn be modified by the presence of the mstoid nstomotic emissry veins (Brum nd Tournde 1977). The nterior root of the vertebrl vein origintes t the level of the junction of the nterior nd middle thirds of the occipitl venous plexus from the nterior condylr vein (venous plexus of the hypoglossl cnl). It follows n oblique, outwrd nd forwrd course, crossing the nterior condylr cnl before it joins the posterior root of the vertebrl vein. The posterior root origintes t the level of the posterior third of the occipitl venous plexus, crosses the tlntooccipitl membrne nd termintes by joining the nterior root to form the vertebrl vein (Brun nd Tournde 1977). The veins form dense plexus round the vertebrl rtery, which descends in the cnl formed by the formin trnsversri of the sixth cervicl vertebre. This vertebrl rtery venous plexus ends in single trunk which emerges from the formen trnsversrium of the sixth vertebr nd empties into the bck prt of the innominte vein with pir of vlves (Gry 1918). In the sub-occipitl ngle the vein communictes with the nterior internl vertebrl venous plexuses with the deep cervicl, nd occipitl veins, nd is joined by veins coming from the recti nd oblique muscles nd from the pericrnium. Arnutovic et l. (1997) studied the venous ntomy of the suboccipitl region in prticulr round the third segment (V3) of the vertebrl rtery nd found n stonishing resemblnce with the cvernous sinus, nming it ccordingly suboccipitl cvernous sinus. Deep Cervicl Vein The deep cervicl vein, lrger thn the vertebrl vein, psses down the neck posterior to the cervicl trnsverse processes. It corresponds to the deep cervicl rtery from which it is seprted by the semispinlis cervicis muscle. It begins in the posterior vertebrl venous plexus nd receives tributries from the deep muscles of the neck. It communictes or entirely drins the occipitl vein by brnch tht perfortes the trpezius muscle. The deep cervicl vein then psses forwrd beneth the trnsverse process of the seventh cervicl vertebr to open into the innominte vein ner the vertebrl vein, or into

57 46 3 Antomy of the Cvernous Sinus nd Relted Structures the ltter its termintion. Its orifice is gurded by pir of vlves (Gry 1918). Anterior Condylr Confluent (Confluens Condyloideum Anterius, Trolrd 1868) The nterior condylr confluent (ACC) (Fig. 3.12) ws initilly observed by Trolrd (1868), but ws to lrge extent ignored in the literture. Sn Milln Ruiz et l. (2002) recently rediscovered this short, venous structure locted extrcrnilly in front of the perture of the hypoglossl cnl t the level of the skull bse. It provides communiction between the cerebrl venous system nd the internl nd externl vertebrl venous plexus vi six min chnnels: the nterior condylr vein, the lterl condylr vein, the IJV, the IPS, the venous plexus of Rektorzik, nd the prevertebrl plexus. Knott (1882) sw venous plexus surrounding the hypoglossl nerve s it psses through the inner prt of the nterior condyloid formen, (the circellus venosus hypoglossi fter Luschk) nd found two veins proceeding from this plexus, one of which communictes with the vertebrl plexus, the other with the IPS. He did not observe consistent confluent. Ktsut et l. (1997), however, described in their study on microsurgicl ntomy of the jugulr formen (see therein) confluens tht connects the nterior condylr vein, the posterior condylr vein, the IPS, the inferior petroclivl nd the vertebrl venous plexus vein with the JB. This chnnel likely corresponds to the ACC illustrted by Sn Milln Ruiz et l. (2002). Detiled knowledge of the complex nd vrint ntomy in this region is importnt, not only to understnd of bsic physiology of the venous circultion in the crnio-cervicl region but lso to perform petrosl sinus smpling. It certinly fcilittes trnsvenous ctheteriztions for trnsvenous occlusion of DCSF. The ACC my form nstomoses with the IPS t vrible distnce from its termintion into the IJV. In some cses the direct communiction between IPS nd IJV my be smll or non-existent nd the route through the ACC is the lterntive. See more detils in Chp. 7. References Allen WE III, Kier EL, Rothmn SL (1973) The mxillry rtery: norml rteriogrphic ntomy. Am J Roentgenol Rdium Ther Nucl Med 118: Allen WE III, Kier EL, Rothmn SL (1974) The mxillry rtery in crniofcil pthology. Am J Roentgenol Rdium Ther Nucl Med 121: Arnutovic KI, Al-Mefty O, Pit TG, Krisht AF, Husin MM (1997) The suboccipitl cvernous sinus. J Neurosurg 86: Aubin ML, Pleirc R, Trserr J (1974) Rdiontomy of the jugulr sinus nd its nterior collterls. Ann Rdiol (Pris) 17: Brrow DL, Spector RH, Brun IF, Lndmn JA, Tindll SC, Tindll GT (1985) Clssifiction nd tretment of spontneous crotid-cvernous sinus fistuls. J Neurosurg 62: Bumel JJ, Berd D (1961) The ccessory meningel rtery of mn. J Ant 95: Bedford MA (1966) The cvernous sinus. Brit J Ophthl 50:41 46 Benndorf G, Cmpi A (2001) The berrnt inferior petrsol sinus: n unusul pproch to the cvernous sinus. Neurordiology DOI /s Benndorf G (2008) Anomlous origin of the mrginl tentoril rtery: detection by contrst-enhnced ngiogrphic computed ngiogrphy. Clin Neurord 4:1 4 Bernsconi V, Cssinri V (1956) Un sengo crotidogrfico tipico di meningiom del tentorio. Chirurgi 11: Biondi A, Mile D, Cognrd C, Riccirdi GK, Bonneville F, vn Effenterre R (2003) Cvernous sinus durl fistule treted by trnsvenous pproch through the fcil vein: report of seven cses nd review of the literture. AJNR Am J Neurordiol 24: Bisri KK (1985) The superficil sylvin vein in humns: with specil reference to its termintion. Ant Rec 212: Bleys RL, Thrsivoulou C, Cowen T (2001) Cvernous sinus gngli re sources for prsympthetic innervtion of cerebrl rteries in rt. J Cereb Blood Flow Metb 21: Bonnet P (1957) Les syndromes de l loge cverneuse. Rev Oto-Neuro-Ophth Borb LAB, Al-Mefty O (2000) Norml ntomy of the cvernous sinus. In: Eisenberg MB, Al Mefty O (eds) The cvernous sinus. Lippincott Willims &Willims, Phildelphi, pp Boskovic M, Svic V, Josifov J (1963) Über die Sinus petrosi und ihre Zuflüsse. Gegenburs Morphol Jhrb 104: Bouthillier A, vn Loveren HR, Keller JT (1996) Segments of the internl crotid rtery: new clssifiction. Neurosurgery 38: ; discussion Brssier G, P. Lsjunis, et l. (1987) Microsurgicl ntomy of collterl brnches of the intrcvernous internl crotid rtery. In: Dolenc VV (ed) The cvernous sinus: multidisciplinry pproch to vsculr nd tumorous lesions. Springer: Wien-New York, pp Brun JP, Tournde A (1977) Venous dringe in the crniocervicl region. Neurordiology 13: Breschet G (1829) Recherches ntomique, physiologiques sur le systeme veineux et specilment sur les cnux veineux des os. Pris, Vileret et Rouen Brismr J (1974) Orbitl phlebogrphy. II. Antomy of superior ophthlmic vein nd its tributries. Act Rdiol Dign (Stockh) 15: Browder J, Kpln H (1976) Cerebrl durl sinuses nd their tributries. Chrles C. Thoms, Springfield

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62 Clssifiction of Cvernous Sinus Fistuls (CSFs) 4 nd Durl Arteriovenous Fistuls (DAVFs) CONTENTS Introduction Antomic Clssifiction Durl Arteriovenous Fistuls (DAVFs) Cvernous Sinus Fistuls (CSFs) Etiologic Clssifiction Hemodynmic Clssifiction 60 References 62 Cvernous sinus fistuls (CSF), like other rteriovenous fistuls, represent bnorml communictions between the rteril nd venous blood circultion, either directly between the ICA lumen nd the CS or indirectly between brnches of the ICA nd/or ECA nd the CS. The vsculr nomlies involving the cvernous sinus nd the internl crotid rtery re ccordingly nmed crotid cvernous fistuls (CCFs) (Prkinson 1987), crotid cvernous sinus fistuls (CCSF) (Brrow et l. 1985), cvernouscrotid fistuls (Yoo nd Krisht 2000), or crotid rtery cvernous fistuls (Phtouros et l. 2000). Likewise, communictions linking the cvernous sinus nd durl rteril supply re ptly nmed, durl cvernous sinus rteriovenous mlformtions (Houdrt et l. 1993), durl crotid cvernous fistuls (durl CCF) ( Meyers et l. 2002), cvernous sinus durl rteriovenous mlformtion (CSDAVM) (Stiebel-Klish et l. 2002; Brcio-Slorio et l. 2000), cvernous sinus durl rteriovenous fistule (CSDAVF) (Stomi et l. 2005; Suh et l. 2005) or durl cvernous sinus fistuls (DCSFs) (Peeters nd Kroger 1979). This inconsistent terminology reflects need to elucidte further the etiology, pthophysiology nd nturl history of these fistuls to stndrdize nd simplify their clssifiction. In this monogrph, the term CSF is used for ll rteriovenous fistuls involving the CS regrdless of their ntomy, etiology, prognosis or hemodynmic chrcteristics. In generl, CSFs re clssified ntomiclly, bsed on their etiology, or ccording to their specific hemodynmic chrcteristics. Introduction 4.1 Antomic Clssifiction Durl Arteriovenous Fistuls (DAVFs) Becuse of their vsculr supply by durl rteries, Type B D fistuls re ctegorized by some uthors into the lrge group of durl rteriovenous fistuls (DAVF) (Mlek et l, 2000; Chung et l. 2002; Klisch et l. 2003; Tsi et l. 2004; Mironov 1995; Cognrd et l. 1995; Srm nd ter Brugge 2003; Dvies et l. 1997), which re locted t the gret cerebrl sinuses such s the sigmoid, trnsverse, superior sgittl, inferior nd superior petrosl sinus. DAVFs hve been ntomiclly clssified ccording either to their specific loction, or ccording to their ntomic fetures seen on cerebrl ngiogrms ( Tble 4.1). The severity or ggressiveness of DAVFs hs been outlined previously using severl clssifiction schemes (Cognrd et l. 1995; Dvies et l. 1996; Borden et l. 1995; Llwni et l. 1993; Mironov 1995). All DAVF clssifictions re bsed on specific ngiogrphic fetures of the venous dringe pttern including venous stenosis, occlusions or corticl (leptomeningel) dringe. One of the first ttempts to clssify these lesion ws mde by Aminoff et l. (1973) who divided them

63 52 4 Clssifiction of Cvernous Sinus Fistuls (CSFs) nd Durl Arteriovenous Fistuls (DAVFs) Tble 4.1. Antomic clssifictions of DAVFs Author Type/ grde/ group Type of venous dringe Cstigne (1975) 1 Sinus, direct or indirect (SSS, SPS, CS) 2 Sinus vi venous ectsi 3 Corticl vein Djindjin (1978) 1 Into sinus or meningel vein 2 Sinus dringe with reflux into cerebrl veins 3 Solely into corticl veins 4 With supr- or infrtentoril venous lkes Llwni (1993) 1 Anterogrde sinus, no venous restriction, no corticl dringe 2 Anterogrde nd retrogrde sinus, no venous restriction, no corticl dringe 3 Retrogrde nd corticl dringe without ntegrde venous dringe 4 Corticl venous dringe only Borden (1995) I Sinus only II Sinus nd subrchnoid veins III Subrchnoid veins only Cognrd (1995) I Sinus, without reflux II Sinus with reflux (insufficient ntegrde flow) IIb Sinus with reflux into corticl veins II+b Reflux into sinus nd corticl veins III Corticl vein without ectsi IV Corticl vein with venous ectsis (> 5 mm, 3 > drining vein) V Spinl perimedullry veins Author Type/ grde/ group Loction of the AV-shunt Amonoff (1973) 1 Anterior-inferior (cvernous sinus) 2 Superior-posterior (trnsverse nd signoid sinus) Picrd (1987) 1 Lterl sinus 2 Cvernous sinus 3 Superior sgittl sinus 4 Other: tentoril, flcine, convexity Awd (1990) 1 Trnsverse sigmoid sinus (62%) 2 Cvernous sinus (11.9%) 3 Tentoril incisures (8.4%) 4 Convexity superior sgittl sinus (7.4%) 5 Orbitl nterior flx (5.8%) 6 Sylvin middle crnil foss (3.7%) 7 Others: mrginl sinus Mironov (1995) 1 Durl sinuses (40.6%) 2 Cvernous sinus (30.2%) 3 Glen s system (10.4%) 4 Venous plexus t the bse of the skull (9.3%) 5 Prsinusl corticl veins (9.3%) Moret (2006) 1 Osteo Durl 2 Duro Durl 3 Duro Archnoidl 4 Duro Pil Geibprsert (2008) Anterior epidurl (includes CS region) Dorsl epidurl Lterl epidurl Note tht mny ntomicl clssifictions include the cvernous sinus region

64 4.1 Antomic Clssifiction 53 into two groups, nterior nd posterior, depending on their venous dringe into the cvernous sinus or into the lterl sinus. Cstigne et l. (1976) studied 13 cses of meningel rteriovenous fistuls with venous nd corticl dringe nd suggested dividing them into three groups: Group 1 would drin directly or vi meningel vein into the sinus, Group 2, rrely seen would drin into lrge venous sc, nd Group 3 would drin into corticl vein. Group 3 represented specil entity becuse only these fistuls would produce neurologicl symptoms. Djindjin nd Merlnd (1978) extended this clssifiction nd subdivided DAVFs into four types: (1) fistuls drining into sinus or meningel vein (most frequent), (2) fistuls drining directly into sinus without significnt reflux into corticl veins, (3) fistuls drining into corticl veins, nd (4) fistuls with lrge supr- or infrtentoril lkes. Type 2 fistuls, lthough possibly developing from the preceding Type 1, were considered seprte group becuse of their potentil to cuse neurologicl complictions. Type 3 fistuls were considered specific group becuse of their constnt production of neurologicl signs. According to Cstigne et l. (1975), more thn 42% nd ccording to Mlek et l. (2000) 33% of the fistuls with corticl venous dringe were ssocited with intrcrnil hemorrhge. The clssifiction of Cognrd et l. (1995) ws bsed on 205 ptients nd modifiction of the one provided by Djindjin nd Merlnd (1978). The uthors subdivided Grde II DAVFs nd further specified the dringe into corticl veins with nd without ectsis. They found tht in their group, venous ectsis nd corticl dringe represent significnt risk fctors. It hs recently been suggested tht ptients who develop reversible dementi due to bilterl venous hypertension should receive Type VI clssifiction; however, the usefulness hs not been estblished yet (Houdrt 2005). Mironov (1995) divided DAVFs into five groups: Type 1 DAVFs of the durl sinuses; Type 2 DAVFs of the cvernous sinus; Type 3 DAVFs of Glen s system; Type 4 DAVFs of the venous plexus t the bse of the skull; Type 5 DAVFs of the prsinusl corticl veins. He studied ngiogrms in 96 ptients nd found tht the documenttion of cusl sinus thrombosis depends on the loction of the DAVF: in 72% of the cses with Type 1 DAVFs there ws thrombosis t the time of the investigtion, but no thrombosis ws proven in cses with Type 4 DAVFs. Mironov concluded tht the morphologicl development of DAVFs seems to depend on the flow volume of the venous recipient. A pronounced development of pthologicl AV shunts tkes plce t the level of the lrge bsl durl sinuses. A delyed development of pthologicl AV shunts with low shunt volume occurs in venous recipient with low AV pressure grdient. Llwni et l. (1993) nd Mlek et l. (2000) proposed grding system for DAVFs in four grdes: fistuls of grde 1 show no venous restriction nd norml ntegrde dringe, grde 2 show nterond retrogrde dringe with or without corticl component. Grde 4 fistuls show exclusively corticl venous dringe. They were ble to demonstrte tht ptients with grde 3 or grde 4 fistuls hve significntly higher risk for intrcrnil hemorrhge or visul symptoms. Tble 4.1 shows severl clssifictions bsed on venous dringe nd ssocited risk of intrcrnil hemorrhge. However, this clssifiction hs limited ppliction to DCSFs, becuse most of the ltter re Type 1 2 nd corticl dringe is not often seen (10% 31%) (Hlbch et l. 1987; Cognrd et l. 1995; Tomsick 1997; Meyers 2002). Even when corticl dringe is present, ssocited intrcrnil hemorrhge seems to occur less frequently thn DAVFs. On the other hnd, in clinicl prctice most groups consider corticl drining veins in DCSF n indiction for endovsculr tretment. Moret et l. (2006) hve proposed new ntomicl clssifiction, dividing DAVFs without DCSFs, ccording to their reltionship to the djcent tissue components into four types. The osteo-durl nd duro-durl fistuls re cused by pthology of the dur mter while the duro-rchnoidl nd duropil fistuls re cused by pthology of the perivsculr spce. In their series of more thn 200 DAVFs, the osteo-durl type of DAVF is the most frequently seen nd cn be cured in up to 94% of the cses. Duro-durl or murl type fistuls re reltively rre in comprison. Typicl of the duro-rchnoidl type is the frequent hemorrhgic complictions (up to 50%). Attempts to ctegorize nd clssify DAVFs continue s is demonstrted by the recent effort of Geibprsert et l. (2008), who divided, bsed on embryologicl spects, durl rteriovenous shunts (DAVS) into three groups: nterior epidurl, posterior epidurl nd lterl epidurl shunts. It is suggested tht this clssifiction llows prediction of the venous dringe nd possibly fcilittes tretment decisions tht still depend on the presence of

65 54 4 Clssifiction of Cvernous Sinus Fistuls (CSFs) nd Durl Arteriovenous Fistuls (DAVFs) corticl or leptomeningel venous dringe (CVD, LVD). It is interesting to note tht DCSFs would be grouped into the nterior epidurl shunts in which reflux into corticl/leptomeningel veins usully does not occur, unless there is venous outflow restriction due to thrombosis or high-flow condition. DAVFs of the nterior foss, such s ethmoidl DAVFs re ntomiclly closely relted to DCSFs but re grouped into the lterl epidurl shunts. They drin in up to 100% into corticl veins nd re ssocited with bleeding rte of 61% (AGID 2009). This corresponds with ggressive symptoms seen in 86% of these fistuls, versus 92% benign symptoms observed in nterior epidurl shunts. Whether or not DCSFs with present CVD/LVD hve to be treted with the sme urgency remins uncler, nd the fct tht they ctully rrely bleed, even when reflux into cerebrl veins is present (up to 31%, Meyers 2002) is still not explined. Although the reference to embryology provides new insights into potentil mechnisms of development nd morphologicl nd clinicl presenttions of DAVFs, the usefulness of this clssifiction for clinicl prctice needs to be proven Cvernous Sinus Fistuls (CSFs) The prticulr ntomic rrngement in the cvernous region cuses the ptho-ntomicl sequele of CSFs. While the venous component of the fistulous connection is invribly provided by the cvernous sinus, the rteril supply nd the venous dringe exiting from the CS my differ remrkbly resulting in vrious ngiomorphologies of the fistuls. The originl clinicl presenttion chrcterized by Dndy (1937), s the tris of exophthlmos, chemosis nd bruit is cused by single defect in the wll of the internl crotid rtery during its course through the cvernous segment (C5 C4-portion). Depending on the size of this ter, smller or lrger AV shunt volume develops within short period of time. This type of AV fistul occurs either fter trum (cr ccident, blunt injury etc.) or develops spontneously fter rupture of cvernous crotid neurysm or preexisting wekness of the rteril wll (Ehlers Dnlos Type IV, Fibromusculr dysplsi). It is therefore clled direct CSF or CCF (cvernous crotid fistul). Although widely ignored in the recent literture, Wolff nd Schmidt (1939) mde the first effort to identify vrious forms of CSFs. Bsed on their own observtions in three ptients with pulsting exophthlmos (mostly posttrumtic CCFs) undergoing cerebrl ngiogrphy, s well s existing ngiogrphic descriptions in the literture, they were ble to identify four min types of venous dringe: nterior vi the SOV, posterior to the IPS, superficil corticl to the vein of Trolrd nd deep corticl vi the lterl mesencephlic nd bsl vein of Rosenthl (Fig. 4.1). If one considers the limited knowledge of cerebrl ngiogrphy only 5 yers fter its first introduction by Moniz (1927), this detiled nlysis is quite remrkble. It ws Prkinson (1965) in his erly work on trumtic crotid cvernous fistuls who mde the distinction between direct nd indirect fistuls. He considered the first type single fistul resulting from ter in the wll of the crotid nd the second type resulting from ter cross one of the smll durl brnches within the cvernous sinus. A few yers lter, Newton nd Hoyt (1970) subdivided DCSFs into two types. The first ws chrcterized by shunt djcent to or within the wll of the CS nd the second by shunt involving more distnt durl sinuses tht communicte with the CS. Ptients with n indirect rteriovenous shunt involving the CS my present with similr, but usully less drmtic nd progressive symptoms. In some ptients the signs nd symptoms, generlly considered chrcteristic of CSFs, re conspicuously bsent (Newton nd Hoyt 1970). Becuse the AV shunting develops between the network of the smll durl rteries of the ICA nd ECA or their brnches nd the CS, they re properly nmed durl cvernous sinus fistuls (DCSFs). Djindjin nd Merlnd (1973) reported on six cses of fistuls between the ECA nd the CS nd clssified them into three types: (1) fistule tht develop fter ineffective trpping or emboliztion of direct CCF; (2) fistuls tht re supplied by both ICA nd ECA brnches, or (3) true ECA-CS fistuls. Whether Type 1 cn be considered seprte fistul remins questionble becuse it represents the residul or secondry supply of n incompletely occluded direct high-flow AV shunt s described by Hyes (1963). In ddition, Peeters nd Kroeger (1979) suggested clssifiction of CSFs into direct, indirect or durl fistuls (Figs. 4.2 nd 4.3). The uthors studied 19 ptients nd found 9 indirect nd 10 direct fistuls of three different types: (1) direct internl crotid cvernous sinus fistul, (2) durl internl

66 4.1 Antomic Clssifiction 55 Fig. 4.1 d. Clssifiction of CCFs (Zbl Neurochir, 1939). The first ngiogrphic clssifiction of CSFs by Wolff nd Schmid. Bsed on ngiogrphic observtions in three ptients with pulsting exophthlmus nd reported cses in the literture t tht time, they identified four min types of venous dringe. Type A, nterior dringe vi the SOV; Type B, posterior dringe vi the IPS nd SPS; Type C, corticl dringe vi the superior nstomotic vein of Trolrd; nd Type D, deep venous dringe vi the perimesencephlic vein nd bsl vein of Rosenthl. (Note: the perimesencephlic vein s such ws not identified by the uthors, who discussed n unknown vein insted.) Fig Type A fistul (direct, trumtic), in principle identicl to the spontneous Type A fter Brrow. The wll of the crotid rtery hs defect nd blood shunts directly into the cvernous sinus (sterices) drining vi the SOV (short double rrow), the IOV (single rrow) nd the IPS (double rrow). Note tht scculr structures re sometimes visible in trumtic cses; they my represent venous outpouchings nd cn mimic cvernous neurysms Fig Type D (indirect, durl) fistul. The wll of the crotid rtery is intct nd the AV shunt is indirectly supplied by either few or network of multiple smll durl brnches (rrowheds) rising from the APA, AMM, MMA nd distl IMA nd drining from the CS (sterisk) vi the SOV (short double rrow) nd the IPS (double rrow) s well s multiple corticl veins (rrows). For Type B fistul nd Type C fistul see Figs d nd 8.19 b, respectively)

67 56 4 Clssifiction of Cvernous Sinus Fistuls (CSFs) nd Durl Arteriovenous Fistuls (DAVFs) crotid cvernous sinus fistul nd (3) durl externl crotid cvernous sinus fistul (Tble 4.2). Fermnd (1982) nd Moret et l. (1978) clssified the indirect fistuls further into two types: Type I fistuls, fed by complex network of feeders whose systemtiztion is impossible nd Type II fistuls in which ll the feeders cn be precisely identified. Picrd et l. (1983) differentited fst-flow nd slow-flow fistuls, emphsizing tht the ltter with multiple feeders ctully represent the true DCSFs. Fst-flow fistuls, exhibited single communiction. Since they were not durl fistuls, they did not require the sme tretment s trumtic CCF, regrdless if they occurred spontneously or due to ruptured cvernous neurysm. Bsed on these ntomicl differences, Brrow et l. (1985) developed more detiled clssifiction for spontneous cvernous sinus fistuls. This schemt, until recently ws the minsty of clssifiction, llowing differentition of fistuls ccording to the type of rteril supply (Fig. 4.4). He defined Type A fistuls s direct fistuls s described in preceding prgrphs. Type B D fistuls depict vrious fistuls, depending on whether the smll durl rteries rise from the ICA, ECA or both. Thus, these Type B D fistuls re clled durl cvernous sinus fistuls (DCSFs). Type B fistuls re solely supplied by ICA-brnches (ILT, MHT, Fig. 7.45) nd Type C fistuls only by the durl brnches of the ECA (MMA, AMA, APA, IMA, Fig. 7.44). Type D fistuls re supplied by both territories. This clssifiction provided prognostic vlue nd hd some impct on therpeutic decision mking. Type A fistuls re usully treted by trnsrteril pproch using detchble blloons. Type B D fistuls, on the other hnd, re effectively treted by trnsrteril or trnsvenous emboliztion, or combintion therein. Posttrumtic CCFs, not included in Brrow s clssifiction, represent morphologiclly, hemodynmiclly nd with regrd to their tretment options, Type A fistul. De Keizer (2003) emphsized the following dditionl fistul types: () presenttion of clinicl signs Type A Type C Type B Type D Fig Clssifiction of spontneous CSFs fter Brrow (J Neurosurg, 1985). Type A, direct fistul through defect in the wll of the ICA (high -flow) Type B, DCSF, indirect fistul supplied by ECA-feeder (low-flow) Type C, DCSF, indirect fistul supplied by ICA-feeder (low-flow) Type D, DCSF, indirect fistul supplied by ECA nd ICA-feeder (low-flow) Type A fistul develops either spontneously or due to ruptur of n intrcvernous ICA neurysm. They cn be ntomiclly nd hemodynmiclly identicl to trumtic direct fistuls. Indirect fistuls re lso referred to s durl cvernous sinus fistuls (DCSFs), becuse they re supplied by durl brnches of ECA nd ICA. The most frequent type is the Type D fistul (90%), often supplied by numerous brnches from both territories, sometimes bilterlly. This clssifiction does not tke into ccount the venous dringe nd does not differentite between uni- nd bilterl supply fistuls. With the incresing use of trnsvenous occlusions for tretment of DCSFs, this clssifiction is tody of less importnce, lthough still widely used. Type B or D fistuls, in the pst usully considered difficult to tret lesions (by trnsrteril emboliztion), cn be trnsvenously occluded with the sme success rte s Type C fistuls. The ltter lthough rre, were thought to be esy to tret lesions, becuse ECA feeders re less difficult to rech with microctheter

68 4.1 Antomic Clssifiction 57 Tble 4.2. Clssifictions of CSF nd (CCF nd DCSF) Author Type Type of rteril supply Wolff nd Schmidt (1939) 1 Anterior into SOV 2 Posterior into IPS 3 Into superficil corticl veins 4 Into deep pil veins Prkinson (1965) 1 Direct fistul: ter in the crotid wll 2 Indirect fistul: ter cross MHT or ILT brnch Newton (1970) 1 Durl supply djcent or within wll of the CS 2 Durl supply distnt to the CS Djindjin (1973) 1 Persistent durl AV shunt fter trpping or emboliztion of CCF 2 Supply from ICA nd ECA (mixed DCSF) 3 Supply from ECA only Peeters (1979) 1 Direct internl crotid cvernous sinus fistuls 2 Durl internl crotid cvernous sinus fistuls 3 Durl externl crotid cvernous sinus fistul Moret (1982) I By complex network of feeders (cnnot be systemtized) II Feeder identifible Picrd (1983) I Fst flow (single communiction) II Slow flow (multiple feeders, true durl fistuls ) Brrows (1985) Spontneous crotid cvernous sinus fistuls A Direct fistul from ICA (non-trumtic) B ECA supply only C ICA supply only D ECA nd ICA supply Lrson (1995) 1 Direct (trumtic) 2 Direct (rupture of intrcvernous neurysm) 3 Indirect (ICA nd ECA supply) 4 Combined direct nd indirect chrcteristics Tomsick (1997) Brrows Types A D D1 Unilterl ICA nd ECA supply D2 Bilterl ICA nd ECA supply Brci-Solrio (2000) Brrows Types A D T Trumtic Type A (AT) 1 Supply ipsilterl 2 Supply bilterl Stiebel-Klish (2002) DCSFs (venous dringe) 1 Flow reversl into nterior CS nd SOV 2 Ophthlmic vein thrombosis 2b Stgnnt ophthlmic vein flow 3 IPS 4 SPS Suh (2005) DCSFs (ngiogrphic pttern) 1 Prolifertive 2 Restrictive 3 Lte Restrictive Stomi (2005) 1 Anterior nd posterior dringe open 2 Posterior dringe closed, posterior open 3 Anterior nd posterior dringe route closed This clssifiction from Moret is mentioned in the pper of Fermnd (1982)

69 58 4 Clssifiction of Cvernous Sinus Fistuls (CSFs) nd Durl Arteriovenous Fistuls (DAVFs) on the contrlterl side, (b) connection of primitive trigeminl rtery; lthough rre, nd introrbitl connections between the ophthlmic rtery nd ophthlmic vein or its brnches. The ltter re in some series not considered seprtely nd my cuse similr symptoms, thus mimicking cvernous sinus durl rteriovenous mlformtion (Hun- Bron et l. 2000). As observed by Hun-Bron et l. (2000), even high resolution MRI my fil nd show n enlrged SOV without bnormlity of the CS. Only superselective ngiogrphy my llow identifiction of these smll slow-flow introrbitl AV shunts. Deguchi et l. (2005) recently reported purely introrbitl rteriovenous fistul tht ws successfully embolized using trnsvenous pproch vi fcil nd ngulr vein. I hve observed the cse of young mn, who presented with symptoms typicl for DCSF, cused by smll AV fistul of the introrbitl SOV (Fig. 4.5). Brrow s clssifiction (Brrow et l. 1985) specificlly proposed for spontneous lesions is bsed only on the rteril ngiorchitecture nd does not reflect the venous dringe pttern. It llows differentition with regrd to technicl difficulties nticipted during trnsrteril ctheteriztion, which ws the most common endovsculr pproch in the 1980s. Under those spects, Type C lesions were usully considered esier to tret becuse ctheteriztion of ECA feeders ws techniclly less difficult nd injecting liquid embolic gents ws cliniclly less risky. Types B nd D fistuls were, on the other hnd, seen s more chllenging lesions (nd still re by some uthors), nd re often considered difficult to tret lesions or intrctble fistuls. Picrd et l. (1987) stted: The slow flow fistuls with multiple pedicles re true durl fistuls. Their tretment is lwys limited to the emboliztion of the externl crotid brnches; emboliztion of the internl crotid rtery brnches should never be ttempted. Therefore, it is importnt to clssify these fistuls ccording to the respective prticiption of the internl nd of the externl crotid rtery, even if in most cses both re involved. In the er of trnsrteril emboliztions, the mjority of Type D fistuls hve been prtilly occluded or were completely cured only when djunctive more ggressive techniques or complementry tretment options such s microneurosurgery or rdiosurgery were pplied. Becuse of the incresing experience nd technicl dvncement in using trnsvenous techniques in the 1990s, the technicl chllenges of trnsrteril emboliztions re menwhile of little importnce. Thus, the type of rteril supply (B, C or D) hs only minor impct on whether or not to perform trnsvenous occlusion. Tody, the pttern of venous dringe, either nteriorly vi ophthlmic veins, or posteriorly vi petrosl sinuses, is certinly of greter significnce becuse these drining veins cn be used s endovsculr routes to the CS. Some fistuls re locted only on one side, but re supplied by bilterl feeders from the ICA nd the ECA. Becuse of the usully ssocited higher flow, these fistuls hve been considered by Tomsick (1997) nd Brci-Slorio et l. (2000) s more complex nd more difficult to tret nd were clssified s Type D1 nd Type D2 fistuls. However, not only the bilterl fistulous communiction is of significnce for the size of the AV-shunt, but lso the cpcity of the drining veins. Furthermore, in my experience it is more importnt to decide whether or not uni- or bilterl AV-shunt is present: the ltter cse occurs less frequently, but my be overlooked nd cn result in n incomplete occlusion. It is certinly of mjor importnce for plnning nd performing trnsvenous occlusion to decide whether to occlude the CS on one or on both sides. Whether or not bilterl supply Fig Pr-cvernous introrbitl AVF. A 35-yer-old mle presenting with double vision nd eye redness. ICA injection, lterl view shows modertely enlrged OA tht suplies smll AV shunt (sterisk) t the proximl segment of the SOV (double rrow) with recurrent durl brnch (rrow). This fistul is in fct not true DCSF, but n introrbitl communiction between OA nd SOV

70 4.1 Antomic Clssifiction 59 is present ppers less crucil thn if true bilterl AV shunt is present. Both these constelltions cn esily be mistken due to insufficient ngiogrphic imge qulity. In order to void occluding the wrong side, fistuls drining vi the contrlterl CS nd SOV should be recognized before occluding the sinus. Some fistuls, despite bilterl supply, cn be ngiogrphiclly low-flow fistuls, when the venous outflow is restricted due to thrombosed SOV or IPS. Whether or not corticl or leptomeningel venous dringe is present, constitutes n importnt fctor for clinicl decision-mking. Even though DCSFs with corticl dringe seem to bleed less frequently thn DAVF in generl (30%), bsed on the sme concept, they re often ggressively treted to prevent neurologicl deficits or hemorrhgic complictions. In order to block such venous exit nd to minimize the risk of procedure-relted intrcrnil bleeding, precise locliztion of its origin is crucil. Stiebel-Klish et l. (2002) hve studied the venous dringe pttern nd differentited into four types. 1. Venous outflow into nterior CS nd SOV ws present in 77/85 ptients. In this group corticl veins were present in 25%, IPS in 21% nd SPS in 12%. 2. Abnorml ophthlmic venous flow with ophthlmic vein thrombosis ws seen in 11/85 mong which corticl veins were found in 73%, IPS in 27% nd SPS in 27% of the cses. 3. IPS dringe ws seen in 22 cses with 41% of corticl dringe, 18% SPS dringe nd prspinl veins (4.5%). 4. SPS dringe ws seen in 11 cses with corticl dringe in ll of them (100%). Suh et l. (2005) hve recently suggested ngiogrphic differentition of DCSFs into three types: 1. The Prolifertive type (PT): Numerous rteril feeder to the CS (network) Lrge AV shunt with rpid filling of CS, fferent nd efferent veins Both CSs completely filled nd bulging into the sinus wll 2. The Restrictive type (RT): Less rteril feeders thn PT, ech identifible Oblitertion of flow in IPS, incresed flow in SOV nd corticl veins Less AV shunt thn in PT CS mrgins less well defined (loss of norml contour) 3. The Lte Restrictive type (LRT): Few rteril feeders With sluggish retrogrde venous flow Constrictive chnges of the veins CS stsis The uthors were ble to correlte presented symptoms in 58 ptients with these three dringe ptterns. Ptients with PT fistul would mostly present with cvernous pttern such s ptosis, diplopi, nisocori nd ophthlmoplegi cused by CN deficits. Ptients with RT fistul presented with cvernous symptom pttern or with n orbitl (chemosis, exophthlmos, periorbitl pin, eyelid swelling) or oculr pttern (decresed vision, incresed IOP, severe oculr pin, glucom nd retinl hemorrhge). Ptients with fistul of the LRT presented mostly with oculr pttern. A cerebrl pttern with infrction in the bsl gngli or brinstem cused by reflux into corticl veins ws seen in 5% of the ptients with RT nd LRT fistuls. Although this pproch seems ppeling, the study showed progression from one type to nother in only 7/58 ptients (12%). Angiogrms could only be compred in 11 ptients nd mny (unknown number) ptients were embolized either by the trnsrteril or trnsvenous pproch. Therefore, chnges of the venous dringe pttern in these ptients cnnot be simply relted to the nturl course of the disese. In 4/11 ptients no chnge from RT to LRT ws documented. Only some ptients with the LRT presented with serious cerebrl complictions. In my opinion, the results leve severl unnswered questions, including whether ptient with RT or LRT requires endovsculr tretment. A similr, but more simple pproch to the problem hs been proposed by Stomi et l. (2005). Bsed on 65 ptients, seen over period of 29 yers, the uthors stged the progression of the disese into three phses bsed on the venous dringe pttern. Stge 1 fistuls drin predominntly posteriorly vi the IPS nd to lesser degree nteriorly, producing minly tinnitus. Stge 2 fistuls re chrcterized by closed (thrombosed) IPS nd PP redirecting the flow into the SOV nd the IOV nd to lesser degree into the Sylvin vein. In this stge the ptients present not with bruit but with incresed ophthlmologicl symptoms. Stge 3 is defined s fistul tht drins exclusively into either the Sylvin vein or the SPS due to the occlusion of initilly nterior nd posterior drining veins. In this study, reltively smll number of ptients, 11/65 (17%) demonstrted indeed chronologicl chnge of the venous dringe pttern. One ptient (9%) developed corticl reflux. Becuse hlf of the ptients underwent trnsrteril emboliztion, the observed chnges do not llow referring

71 60 4 Clssifiction of Cvernous Sinus Fistuls (CSFs) nd Durl Arteriovenous Fistuls (DAVFs) to the nturl history of DCSFs either, even though ll 11 cses showed chnge in one direction: from 1 to 2 or 3 ssocited with reduction of drining venous chnnels. A reltively smll number of four ptients were ctegorized s Stge 3 fistuls with corticl venous dringe ll of which were directed nteriorly towrds the Sylvin vein. Only single cse showed the development of this type of dringe during ngiogrphic FU studies. Interestingly, none of the stge 3 cses showed leptomeningel venous dringe towrds the mesencephlic nd cerebellr veins even though the SPS remined ptent. Thus, the progression of these chnges seems not lwys predictble nd the vlue of the stging for future tretment strtegy is uncertin. ICA MMA 4.2 Etiologic Clssifiction According to their etiology, CSFs cn minly be clssified into spontneous nd trumtic fistuls. Most trumtic fistuls re ntomiclly Type A fistuls with direct communiction between ICA nd CS. However, in some cses, ruptured durl rtery my cuse the rteriovenous shunting into the CS leding to identicl symptoms (Fig. 4.6). A durl rtery my lso contribute to persisting direct AV shunt (Fig. 4.7), or become the only remining supply of previously treted CCF, e.g. fter ineffective trpping procedure s described by Hyes (1963). The gret mjority of indirect CSFs represent durl rteriovenous shunting lesions nd correspond to Type B D fistuls s clssified by Brrow. It becomes obvious tht, in some cses, etiologicl nd ntomicl clssifiction my overlp (see more bout etiology in Chp. 5). 4.3 Hemodynmic Clssifiction Hemodynmics hve long been used s mens to chrcterize AV shunting lesions with regrd to their clinicl prognosis. Lcking correct dt on pressure nd flow in these fistuls, rteriogrms hve often been used to estimte when shunt is low-flow or high-flow. Hyes (1958) ws the first in Fig Trumtic Type C fistul: Motorcycle ccident 15 yers previously. Externl crotid rteriogrm shows fistulous comuniction between the MMA nd the CS tht results in high-flow AV shunt, drining only nteriorly into the SOV (short double rrow). Note: This fistul could lso be considered trumtic AV-fistul of the MMA ttempting to identify different types bsed on the AV-shunting flow. He identified fistuls drining ll blood from the ICA into the CS nd those drining only prt of it to the venous side. He clssified them ccordingly s high- nd low-flow fistuls. Experience hs shown tht most Type A fistuls re highflow fistuls, the mjority of which re represented by posttrumtic fistuls (76%). Some high-flow fistuls my lso be supplied by meningel brnches (Phelps et l. 1982). Usully, (indirect) Types B D fistuls re ssocited less with AV-shunting flow thn (direct) Type A fistuls nd re clled lowflow fistuls. However, the clinicl signs my pper similr during protrcted course of the disese to the ones cused by Type A fistuls except for the pulsting exophthlmos nd the udible bruit (see Chp. 6). They frequently consist of red-eye syndrome, which is chrcterized by dilted, tortuous episclerl veins, elevted introculr pressure nd often less prominent exophthlmos (Phelps et l. 1982). The term low-flow fistul is to some degree confusing becuse it implies mild clinicl course. Some DCSFs, however, despite low-flow

72 4.3 Hemodynmic Clssifiction 61 b c Fig. 4.7 c. Type B or Type D fistul? Filling of the nterior comprtment of the CS (sterisk) through the nteromedil rmus (thin rrow) of the ILT tht courses through the suprorbitl fissure. b The sme fistulous connection (sterisk, thin rrow) filled by the rtery of the formen rotundum (thick rrow) through its connection with the nterolterl rmus of the ILT. This exmple shows nother limittion of Brrow s clssifiction besides the lcking considertion of venous dringe. From n endovsculr point of view, clssifying into Type B or D fistul might be of pure cdemic interest. The ipsilterl IPS nd CS re opcified, widely open nd cn be ccessed esily. The ngiogrphic seprtion between the nterior comprtment involved in the fistul nd the remining comprtments drining the cerbrl venous blood flow is most likely not ntomic but rther pthophysiologic or hemodynmic due to intrcvernous thrombosis. c Arterio-venogrm: Moving the msk into the erly rteril phse llows to better identify tht the fistulous comprtment (blck sterisk) is indeed imbedded (lthough ngiogrphiclly not communicting) within the nterior CS (white sterices) condition nd smll AV-shunt volume, my cuse considerbly elevted intrvenous pressure resulting in severe ophthlmologicl symptoms. This venous hypertension is often cused by venous outflow restriction usully due to stenosis or thrombosis of the CS or the SOV. Relible dt on intrcvernous pressure nd flow in DCSFs is lcking which mkes proper hemodynmic clssifiction difficult (see lso Chp. 10). Nornes (1972) studied the hemodynmic spects of five crotid cvernous fistuls, four trumtic nd one occurring spontneously. He performed intropertive blood flow mesurements nd ws ble to document the mount of fistul stel rnging from 90 to 975 ml nd stted tht the rtio reverse flow/forwrd flow is ssumed to give n indiction of the collterl cpcity of the cerebrl vsculture nd of the tolernce to occlusion of the ICA by trp ligtion. There ws no ttempt to clssify fistuls ccording to their hemodynmics nd no durl CSF ws included. Brssel (1983) imed to clssify direct CCFs with regrd to their hemodynmic effects on the cerebrl circultion. Bsed on their ngiogrphic ppernce, he divided ptients into three clsses: smll, moderte nd lrge. While smll fistuls cuse no reduction of rteril pressure or flow in the ipsilterl intrcrnil circultion, moderte fistuls do, nd consequently stel some supply from the cerebrl circultion vi the circle of Willis. Lrge CCFs shunt completely into the CS without remining ntegrde crotid flow distl to the fistul site.

73 62 4 Clssifiction of Cvernous Sinus Fistuls (CSFs) nd Durl Arteriovenous Fistuls (DAVFs) He concluded tht the ltter group requires tretment, while moderte nd smll fistuls my not. This clssifiction hs rther limited vlue for clinicl prctice, becuse mny ptients with moderte or smll AV shunts my still need tretment becuse they suffer from non-cerebrl symptoms. In ddition, DCSFs re not considered. Much lter Lin et l. (1994) pplied duplex crotid sonogrphy criteri such s flow volume nd resistnce index (RI) for clssifiction of CSFs (see lso Sect ). The uthors were ble to seprte three groups of fistuls: (1) smll RI nd incresed flow volume in the ICA: direct (Type A) CCF; (2) norml RI nd flow volume in the ICA nd ECA: durl brnch of ICA-cvernous sinus fistuls (Type B); (3) smll RI with or without incresed flow volume in the ECA: durl brnch of ECA-cvernous sinus fistuls (Type C) or durl brnches of ICA- nd ECA-cvernous sinus fistuls (Type D). This pproch hs not found wider cceptnce. Some durl CSFs my recruit lrge number of feeding pedicles cusing lrge AV shunting volume nd my hve n ngiogrphic ppernce tht resembles n AVM. They re therefore erroneously clled high-flow fistuls. Becuse quntittive dt on flow re lcking so fr, only direct AVF should be nmed s such. Bsed on the vrious spects discussed bove, truly consequent clssifiction of DCSFs with or without etiologicl spects would require incorporting the rteril nd venous ptterns s well s hemodynmic prmeters. Such n pproch, however, would necessrily result in confusingly lrge number of different types of fistuls without significnt impct on prognosis or therpy. Although proper clssifiction for DCSFs is of prime importnce, it seems for the time being n im difficult to ccomplish. Becuse these fistuls represent reltively infrequent disese, it my be prcticl to simplify or not corticl dringe is present, whether the AV-shunt is locted uni- or bilterl, nd which trnsvenous route is ccessible. Tody the mjority of DCSFs re treted by trnsvenous occlusion techniques nd trnsrteril emboliztion is minly reserved for Type A fistuls, regrdless of their etiology spontneous or trumtic. From n endovsculr therpy point of view, the differentition of B D fistuls ppers no longer very useful nd is more of cdemic interest. While it hs little prognostic vlue, for the purpose of endovsculr strtegy, the old clssifiction in direct nd indirect fistuls still seems suitble. References Agid R, et l. (2009) Mngement strtegies for nterior crnil foss (ethmoidl) durl rteriovenous fistuls with n emphsis on endovsculr tretment. J Neurosurg 110(1): Aminoff MJ (1973) Vsculr nomlies in the intrcrnil dur mter. Brin 96: Awd IA, et l. (1990) Intrcrnil durl rteriovenous mlformtions: fctors predisposing to n ggressive neurologicl course. J Neurosurg 72(6): Brci-Slorio, J.L, Brci JA, Soler F (2000). Rdiosurgery for Crotid-Cvernous Fistuls. In: The Cvernous Sinus. E. M.B. nd O. Al-Mefty EMB, Al-Mefty O (eds). 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J Neurosurg 37: Prkinson D (1965) A surgicl pproch to the cvernous portion of the crotid rtery. Antomicl studies nd cse report. J Neurosurg 23: Prkinson D (1987) Crotid cvernous fistul, history nd ntomy. In: Dolenc VV (ed) The cvernous sinus: multidisciplinry pproch to vsculr nd tumorous lesions. Springer, Berlin Heidelberg New York, pp 3 29 Peeters FL, Kroger R (1979) Durl nd direct cvernous sinus fistuls. AJR Am J Roentgenol 132: Pereir, VM, et l. (2008) Pthomechnisms of symptomtic developmentl venous nomlies. Stroke 39(12): Phtouros CC, Meyers PM, Dowd CF, Hlbch VV, Mlek AM, Higshid RT (2000) Crotid rtery cvernous fistuls. Neurosurg Clin N Am 11:67 84, viii Phelps CD, Thompson HS, Ossoinig KC (1982) The dignosis nd prognosis of typicl crotid-cvernous fistul (redeyed shunt syndrome). Am J Ophthlmol 93: Picrd L, Rolnd J, Brcrd S, Lepoire J, Montut J (1983) Spontneous durl fistuls: clssifiction, dignosis, endovsculr tretment. Springer, Berlin Heidelberg New York Picrd L, Brcrd S, Moret J, Per A, Gicobbe H, Rolnd J (1987) Spontneous durl rteriovenous fistuls. Sem Intervent Rdiol 4: Srm D, ter Brugge K (2003) Mngement of intrcrnil durl rteriovenous shunts in dults. Eur J Rdiol 46: Stomi J, Stoh K, Mtsubr S, Nkjim N, Nghiro S (2005) Angiogrphic chnges in venous dringe of cvernous sinus durl rteriovenous fistule fter pllitive trnsrteril emboliztion or observtionl mngement: proposed stge clssifiction. Neurosurgery 56: ; discussion Stiebel-Klish H, Setton A, Nimii Y, Klish Y, Hrtmn J, Hun Br-On R, Berenstein A, Kupersmith MJ (2002) Cvernous sinus durl rteriovenous mlformtions: ptterns of venous dringe re relted to clinicl signs nd symptoms. Ophthlmology 109: Suh DC, Lee JH, Kim SJ, Chung SJ, Choi CG, Kim HJ, Kim CJ, Kook M, Ahn HS, Kwon SU, Kim JS (2005) New concept in cvernous sinus durl rteriovenous fistul: correltion with presenting symptom nd venous dringe ptterns. Stroke 36: Tomsick TA (1997) Typ B,C, & D CCF: Etiology, prevlence & nturl history. In: Tomsick TA (ed) Crotid cvernous fistul. Digitl Eductionl Publishing, pp Tsi LK, Jeng JS, Liu HM, Wng HJ, Yip PK (2004) Intrcrnil durl rteriovenous fistuls with or without cerebrl sinus thrombosis: nlysis of 69 ptients. J Neurol Neurosurg Psychitr 75: Willinsky, R, et l. (1990) Angiogrphy in the investigtion of spinl durl rteriovenous fistul. A protocol with ppliction of the venous phse. Neurordiology32(2): Wolff H, Schmid B (1939) Ds Arteriogrmm des pulsierenden Exophthlmus. Zbl Neurochir 4: , Yoo K, Krisht AF (2000) Etiology nd clssifiction of cvernous-crotid fistuls. In: Eisenberg MB, Al Mefty O (eds): The cvernous sinus. Lippincott Willims & Willims, Phildelphi, pp

75 Etiology, Prevlence nd Nturl History of 5 Durl Cvernous Sinus Fistuls (DCSFs) CONTENTS Introduction Etiology nd Pthogenesis of Type A Fistuls Etiology nd Pthogenesis of Type B D Fistuls Pregnncy Hormonl Fctors Thrombosis Venous Hypertension Trum Emboliztion Congenitl Other Potentil Fctors Vrious Prevlence Nturl History 78 Introduction References 79 Although the clinicl phenomenon of the pulsting exophthlmos hs been known since Benjmin Trvers (1811) observtion, the discussion bout its underlying pthophysiologicl substrte remined controversil for long time. He ssumed erly on tht the pthologicl ntomy of the pulsting exophthlmos would be crotid-cvernous fistul, while other reports in the nineteenth century on introrbitl neurysms, cusing similr signs nd symptoms, interpreted those s the min cuse. It ws mostly the English school tht ssumed n introrbitl pthology s underlying mechnism, wheres in Frnce the cvernous sinus ws considered the true source of the pulsting exophthlmos. This ws to lrge extent due to the populr work of Nelton (1876), physicin of Npoleon nd Gribldi, who ws ble to demonstrte post mortem direct rteriovenous communiction between crotid rtery nd cvernous sinus in two ptients with pulsting exophthlmos fter trum (see lso Chp. 2). Inspired by this, Brtholow (1872) published more clinicl observtions on pulsting exophthlmos. But it ws not until Rivington (1875) nd Sttler (1880) presented their extensive monogrphs tht the ntomicl concept of (direct) crotid cvernous fistul found broder cceptnce. The term pulsting exophthlmos ws nonetheless used throughout the following 70 yers (Locke 1924; Sttler 1920; Sugr nd Meyer 1940; Wolff nd Schmid 1939; Dndy 1937; Hmby nd Grdner 1933; Nolnd nd Tylor) until it ws eventully replced by crotid cvernous fistul (Potter 1954; Echols nd Jckson 1959; Hyes 1958; Wlker nd Allegre 1956; Prkinson 1967; Hmby 1966). Although indirect fistuls frequently my cuse symptoms similr to direct fistuls, they represent in terms of etiology nd pthogenesis entirely different lesions. Reports on pulsting or non-pulsting exophthlmos in the pre-ngiogrphy er did not differentite between indirect nd direct fistuls. Even fter introduction of cerebrl ngiogrphy by Moniz (1927) t the beginning of the lst century, it took decdes until dignostic rteriogrms in qulity llowing for detiled nlysis of ngiomorphology becme vilble. Only when the fine, minute network of durl rteries could be ngiogrphiclly visulized, did seprting Type A from Types B D fistuls become possible (Cstigne et l. 1966b; Lie 1968; Newton nd Hoyt 1970). Thus, it cn be ssumed tht becuse of the lcking suitble imging tools such s selective externl nd internl crotid rteriogrphy, in mny of the historic series some spontneous or idiopthic fistuls were in fct durl cvernous sinus rteriovenous fistuls. Newton nd Hoyt (1970) described nd chrcterized clinicl, etiologicl nd ngiogrphic fetures of durl rteriovenous shunts in the CS region.

76 66 5 Etiology, Prevlence nd Nturl History of Durl Cvernous Sinus Fistuls (DCSFs) 5.1 Etiology nd Pthogenesis of Type A Fistuls Direct communictions between ICA nd CS cn be considered Type A fistuls, regrdless of their etiology. The clinicl picture of spontneous fistul (true Type A fistuls ccording to Brrows clssifiction) is usully indistinguishble from trumtic CCF. Since very erly on, it ws believed tht n inherent wekness of the intrcvernous portion of the ICA is predisposing fctor to the formtion of CSF. Delen (1870) found tht if the crotid rtery is cnnulted nd liquid is injected with force, the vessel will rupture within the CS: Aussi, en injectnt le système crotidien, von-nous constter que l crotide interne se rompt fcilement dns le sinus si l on pousse un peu fortment l injection. Sur un sujet uquel nous vions lié les deux vertebrles et l crotide primitive droite en poussnt pr l crotide primitive gunche une injection solidifible, nous vons obtenu l rupture de l crotide interne dns le sinus cvereux. L mtière à injection pénétrnt dns le sinus pss dns l veine opthlmique et les veines de l fce, relisnt insi, sur le cdver, les conditions ntomiques de l névrysme rtérioveineux. In some erly series, fistuls of trumtic origin represent 69% 77% of ll CSFs (Locke 1924; Sttler 1920) nd usully develop following severe hed trum with shrp or blunt hed injuries. In the pst, they were seen most frequently in men, being more often involved in wrs, nd industril or trffic ccidents. The modern environment with high prevlence of utomobile ccidents my hve ersed this gender difference (Hmby 1966; Debrun et l. 1988b; Vinuel et l. 1984). On the other hnd, improved hed protection for motorcycle riders seem to hve decresed the number of trumtic CCFs. Only lrge popultions riding bicycles nd still being exposed to frequent severe hed trum in some res of the world, like South-Est Asi, my explin the reltively high rte of trumtic CCFs there. A chrcteristic morphologic feture of trumtic CCFs is ter of the crotid wll llowing high-flow rteriovenous shunt to develop directly nd rpidly. Ptients often present with drmtic ophthlmic symptoms developing within few dys nd usully require emergency tretment. In some cses, however, dely of severl months my occur before symptoms, such s bruit or n exophthlmos, become evident. The size of the ter cn vry from 1 to 5 mm nd it my occur s single or multiple lcertion or in some cses s complete trnssection. Bilterl Type A fistuls, lthough rre, my occur nd hve been observed even mong the erliest reported cses ( Sttler 1930). They hve less fvorble prognosis nd my present with delyed clinicl deteriortion (Ambler et l. 1978). Angiogrphiclly, Type A fistuls show rpid AV shunting with venous dringe into efferent nd fferent veins, often significnt corticl or leptomeningel dringe, nd sometimes ssocited with complete rteril stel. In some cses, the trumtic rupture of n intrcvernous brnch of the ICA or durl brnch of the ICA cn cuse Type A fistul tht my present with only little rteriovenous shunting (Obrdor et l. 1974; Prkinson 1973). Under rre circumstnces trumtic CSFs occur due to rupture of trigeminl rtery (Berger nd Hosobuchi 1984; Kerber nd Mnke 1983; Debrun et l. 1988; Flndroy et l. 1987; Guglielmi et l. 1990; Tokung et l. 2004). Whether unreclled microtrum my be n etiologic fctor for spontneous CCFs is uncertin (Tomsick 1997). The etiology of spontneous CSFs is more difficult to scertin (Hmby 1966). Spontneous rupture of n intrcvernous neurysm cn cuse Brrow s Type A fistul tht my result in high-flow rteriovenous shunt, cliniclly nd ngiogrphiclly indistinguishble from trumtic direct fistul (Brrow et l. 1985). Dndy (1937) reported on n 18-yer-old mle who complined bout progressive exophthlmos over 6 yers with no history of n injury nd he considered congenitl intrcvernous neurysm being the only possible cuse. Locke (1924) observed in his utopsy series 7 trumtic nd 33 spontneous cses. Fromm et l. (1967) demonstrted ngiogrphiclly the existence of scculr neurysm of the cvernous crotid rtery (C4-C5-segment) in ptient who spontneously developed direct CCF. Tpts (1950) found in his series true communiction between ICA nd CS in only 50% of cses. Debrun et l. (1988b) reported 5 spontneous Type A fistuls in 132 ptients, mong whom 3 hd ruptured cvernous neurysm, 1 developed fter pregnncy nd nother ws seen in 5-yer-old child. Tki et l. (1994) sw 2 out of 44 ptients with spontneous direct shunts without ny clinicl ngiogrphic evidence of congenitl disorder, nd thus considered them cused by rupture of n infrclinoid neurysm.

77 5.1 Etiology nd Pthogenesis of Type A Fistuls 67 Nonetheless, the true incidence of Type A fistuls due to ruptured cvernous sinus neurysm is difficult to ssess becuse smll neurysms cn be obscured ngiogrphiclly or mimicked by rpidly filled nd outpouched CS (Tokung et l. 2004). Imge cquisition using high frme rte my help to identify n neurysm s documented in some reports (vn Rooij et l. 2006). To wht degree true ssocition between intrcvernous neurysms nd spontneous CCFs exists, is controversil. Only few reports on cses of CCFs, developing on the bsis of previously known cvernous neurysms, exist (Brrow et l. 1985; Lesoin et l. 1984; Horton et l. 1991). Among Brrow s 14 cses (Brrow et l. 1985) ws 51-yer-old womn presenting with lrge bilterl intrcvernous neurysms, of which one ws treted using grdul occlusion with Crutchfield clmp. The ptient developed contrlterl Type A fistul following crotid occlusion. Vinuel et l. (1984) reported on two such cses in group of 20 ptients. Klisch et l. (2003) more recently reported on series of 17 ptients with CSFs, mong whom one presented with ruptured cvernous crotid neurysm. Kobyshi et l. (2003) treted six cses of direct CCF cused by n intrcvernous neurysm. Overll, intrcvernous neurysms ccount for 1.9% 9.0% of intrcrnil neurysms nd 6% 9% of them re complicted by CCF (Linskey et l. 1990, 1991; Polin et l. 1996). vn Rooij et l. (2006) reported on 10 ptients with ruptured cvernous sinus neurysms cusing direct CCF. However, bsed on the figures shown in their pper, only one out of three (Fig. 3 there) shows convincingly enough chrcteristic scculr structure in the very erly rteril phse, before complete filling of the fistul. The sme my be true for Klisch s cse (Fig. 4 there) (Klisch et l. 2003). The true nture of ny neurysm-like shped structure within the CS remins necessrily uncertin nd some of these cses my insted represent n rteril pseudoneurysm or venous pouch. Such skepticism seems justified becuse, ccording to some studies, the incidence of spontneous direct CCFs bsed on intrcvernous neurysm is rther low (Kupersmith et l. 1992; Ingw 1991). Tomsick (1997), despite lrge number of cvernous neurysms nd lrge number of spontneous CCFs in the institution, did not observe single cse where known cvernous neurysm hd subsequently cused CCF. And surprisingly, in some lrge series like the one of Higshid et l. (1989) reporting on group of 234 direct CCFs, no spontneous fistul due to ruptured neurysm ws noted. Autopsy proven cses like the one of Rosso et l. (1999), who reported recently on postmortem exmintion of direct CCF, re rre. The uthors demonstrted smoothedged wll defect tht ws completely occluded by the detchble blloon. Microscopy t this site showed thin-wlled neurysm with deficient internl elstic lmin nd musculris medi. Non-trumtic Type A fistuls my lso occur in ssocition with connective tissue disorders such s Ehlers Dnlos Type IV (Dny et l. 1986; Desl et l. 2005; Chumn et l. 2002; Purdy 2002; Mitsuhshi et l. 2004; Hlbch et l. 1990; Debrun et l. 1996; Knner et l. 2000), fibromusculr dysplsi ( Sttler 1920; Tki et l. 1994; Zimmermn et l. 1977), or osteogenesis imperfect (Okmur et l. 1995; de Cmpos et l. 1982), pseudoxnthom elsticum (Koh et l. 1999), neurofibromtosis (Lsjunis nd Berenstein 1987), hemorrhgic telengiectsi (von Rd nd Tornow 1975) nd hereditry choriocrcinom (Chen et l. 1993), lymphoid grnulomtosis treted with cytotoxic therpy (Rosenthl nd Rowe 1987), syphilitic rteritis (Sugr nd Meyer 1940), nd fungl rteritis (Sff et l. 1989). Hypertension leding to wekness of the rteril wll hs been suggested s cuse erly on (Wolff nd Schmid 1939). Rupture of microneurysms tht develop due to dilttion nd thinning of the crotid vessel wll cuse most likely CSFs fistuls in these ptients. 5.2 Etiology nd Pthogenesis of Type B D Fistuls Due to initilly limited ntomic informtion obtined by ngiogrphy, DCSFs hve been recognized reltively lte s seprte entity (Lie 1968, Cstigne 1966). For long time, pulstion of the eyebll ws described chrcteristic for CCFs but ws ctully found in only one-third of the ptients (Hmby 1966). It ppers obvious tht some of the remining two-thirds with non-pulsting exophthlmos were likely cused by DCSFs. Although in 1961 Lie (1968) observed such cse tht hd in retrospective DCSF, he ws unble to identify exctly the fistul type due to poor imge qulity nd lck of subtrction technique t tht time. Even

78 68 5 Etiology, Prevlence nd Nturl History of Durl Cvernous Sinus Fistuls (DCSFs) in the work of Plestine et l. (1981), who 20 yers lter reported on 74 CSF ptients, nothing is mentioned with regrd to ngiorchitecture of the fistuls. This is somehow surprising, s Cstigne et l. (1966) hd lredy noted vrious ntomic chrcteristics of the rteril supply in DCSFs. They lso emphsized the need for selective ECA injections. A few yers lter, Newton nd Hoyt (1968) undertook their detiled study of clinicl symptomtology nd ngiorchitecture in 11 ptients with durl rteriovenous fistuls of the CS. They described precisely the smll durl rteries s feeding pedicles of the fistuls nd explined the development of these fistuls with ters in the wlls of these smll rteries or rupture, possibly cused by distension of the vessel wll in predisposed or disesed vessel wlls or by direct trum. Although number of studies investigted etiology nd pthogenesis of DCSFs, the exct mechnisms of their development re still unknown. Vrious fctors nd predispositions seem to contribute, some of them becuse of their similr pthogenetic role in DAVFs. Although most older series necessrily mix cuses for direct (mostly pulsting) nd indirect (commonly non-pulsting) AV shunts, some of them my lso pply to DCSFs Pregnncy Pregnncy nd childbirth hve long been known predisposing fctors for CSFs since their descriptions by Dndy (1937) nd others (Wlker nd Allegre 1956; Stttler 1920, 1930). Sttler (1880), in his first review of cses reported up to 1880, hd lredy collected 32 idiopthic cses, mong which 23 were women, 6 of whom were pregnnt,while 1 developed the fistul during delivery. Six of these women hd been repetedly pregnnt nd five hd been in lte stge of pregnncy. In ll cses the symptoms developed rther quickly. Sttler lso described cses tht occurred under certin circumstnces during physicl stress such s coughing, t pssing stool, stenuous work or severe vomiting: A womn returned from wlk nd ws bending over to tke her shoes off when the process developed. (uthor s trnsltion). Mny yers lter he found tht 28% of women hd developed their idiopthic exophthlmos (17/61) during pregnncy or within the first weeks fter delivery (Sttler 1920, 1930). Wlker nd Allegre (1956) found pregnncy n importnt precipitting fctor for bout 25% 30 % of the fflicted women, who would develop the fistul during the ltter hlf of the pregnncy or during delivery. More recent sttistics show tht only 28% of the fistuls occur during puerperium, wheres 54% develop during menopuse (Tomsick 1997b). Physicl stress during delivery my cuse rupture of smll durl vessels nd development of DCSFs. In the lrgest series published so fr, the onset of symptoms ws ssocited in 6% of cses with pregnncy (Meyers et l. 2002). Due to lck of imging, it remins uncler how mny ptients in these erly series were suffering from durl AV shunts Hormonl Fctors Tnguchi et l. (1971) confirmed Newton s observtions of the predisposition in women not only during but lso fter pregnncy. The higher incidence of DCSFs in women during menopuse nd in men over 50 yers of ge lso supports the theory of ter of n rteriosclerotic ltered vessel wll. On the other hnd, it indictes tht hormonl fctors my contribute nd possibly represent n importnt etiologicl fctor for DCSFs occurring more frequently in elderly women. The exct underlying mechnisms remin uncler. Endogenous estrogen is responsible for inhibiting the progression of rteriosclerosis in women, while exogenous estrogen my promote thrombosis. It is possible tht lck of endogenous estrogen ffects the integrity of the durl micro shunt regultion nd cn promote the development of mcro shunts (Suzuki nd Komtsu 1981). Interestingly, estrogen injections into the ECA for emboliztion of DAVFs nd meningeoms resulted in totl disppernce of the nidus in six out of eight ptients (Suzuki nd Komtsu 1981). This could be due to either ggregtion of blood pltelets, dmge of smooth muscle cells nd fibroblsts or ctivtion of extrinsic nd intrinsic clotting cscde. Infusion of the estrogen compound into mesenteric rteries of rts cused locl spherocytosis nd severe rpid degenertion of endothelil cells, followed by injury to the underlying muscle cells nd fibroblsts ( Shimizu et l. 1987). The concept tht oestrogen hs direct ngiogenic effect nd my stimulte fibroblst growth fctor tht is thought to be one of the inducing fctors of durl AVF ws tested by Terd et l. (1998).

79 5.2 Etiology nd Pthogenesis of Type B D Fistuls 69 The uthors dministered oestrogen to rts in dose similr to tht used in women in the postmenopusl period. However, they could not prove tht oestrogen in fct induces durl AVFs with higher incidence Thrombosis It ws Tnguchi et l. (1971) who recognized erly on the potentil of the minute vsculr durl network when recting to thrombosing fistul between ICA nd CS. They suggested fresh thrombus s initil stimulus for building fistul, concept widely ccepted tody. Tht thrombosis in sinuses involved by DCSFs occurs, ws observed by Voigt nd coworkers nd ws reported by Brismr nd Brismr (1976) who documented the presence of thrombus in six ptients using orbitl phlebogrphy. The thrombus ws considered secondry to the fistul nd not s source or trigger of the developing AV-shunt. Seeger et l. (1980) reported on six ptients with DCSFs, who showed thrombosed CS in their follow-up ngiogrms. I hve observed similr phenomen in two ptients: one ptient showed spontneous thrombosis of durl AVF involving the inferior petrosl sinus dignostic rteriogrm (Cse Illustrtion in Sect. 9.1). In nother ptient, I noticed chnge in the venous dringe within 3 weeks (Fig. 5.1), due to ongoing thrombosis in the CS, possibly ggrvted by the initil dignostic ngiogrphy. In third ptient with sigmoid sinus DAVF, dignostic rteriogrm showing prtilly stenosed sinus due to thrombus nd ws followed by nother within less thn 24 h which demonstrted complete occlusion of the AV shunt (Fig. 5.2). Rther little ttention hs been pid for long time to possible cusl reltionship between thrombus in the CS nd the development of CSF, which is somewht surprising when one considers tht severl relted observtions were mde in the nineteenth century. Sttler (1880) reviewed utopsies of severl cses in which no rteril disese, but enlrgement nd occlusion of the cvernous sinus nd the circulr sinus nd the superior ophthlmic vein by clotted blood ws found (uthors trnsltion). A similr cse ws observed by Hulke (1860), who wrote: the thrombosis of the cvernous sinus nd the ophthlmic vein t its entrnce hs been the cuse of pulsting exophthlmos. Sttler (1880), however, disgreed nd ws convinced it would be impossible tht thrombus in the CS is cpble to produce the group of symptoms of pulsting exophthlmos (uthor s trnsltion). Lter on, (Sttler 1920, 1930) provided more detiled report on nine cses mong which four were spontneous fistuls without evidence for ruptured ICA. He, however, criticlly stted tht those crotid rteries might hve not been fully exmined. Kerber nd Newton (1973) studied the vsculriztion of the dur mter nd observed rteriovenous AV-shunts djcent to the superior sgittl nd lterl sinus. In n ttempt to recnlize thrombosed sinus, functionl enlrgement of these shunts would occur. Houser et l. (1979) suggested tht DCSF might develop subsequently to thrombus in the CS tht induces n rteriolr neoformtion nd represents the trigger for the development of n rteriovenous shunt. Most uthors shre this opinion tody. Although ngiogrphiclly sometimes difficult to identify with certinty, thrombus in the CS hs been observed by some uthors in up to 85% of the cses (Theudin et l. 2006). Chudhry et l. (1982) considered thrombosis or thrombophlebitis of the recipient venous system the primry event in the formtion of four ptients with DAVF in the sigmoid, lterl nd superior sgittl sinus. Mironov (1994) hs provided the lrgest body of work on this subject so fr. He studied extensively the pthogenesis in 96 DAVFs in ll loctions nd found overll concomitnt phlebothrombus in more thn 50%, while thrombus ws seen in 62% of the 29 DCSFs. He discusses the chronologicl reltion between phlebothrombosis nd DAVFs, for which not enough ngiogrphic evidence exists. Not ll DAVFs develop on the ground of thrombosis nd not ll ptients with SVT develop DAVFs. Only few cses provide ngiogrphic evidence of thrombosed sinus preceding fistul (Houser et l. 1979; Chudhry et l. 1982; Pierot et l. 1993) with n intervl between 6 months nd 9 yers. It is ssumed tht thrombus undergoes orgniztion nd recnliztion, which triggers the opening of ltent preexisting AV communictions. When fistul shunts into the sinus, it will increse the venous pressure nd open more AV shunts, by some uthors being considered vicious cycle (Nishijim et l. 1992). Mironov suggested disturbnce of the myogen utoregultion of the physiologicl micro AV-shunts of the prsinusl nd bsl, probbly cused by phlebothrombosis. He lso discussed the reltionship between the size of n AV shunt nd the pressure grdient in the venous recipient. According to his concept, fistuls with smll shunt volume would

80 70 5 Etiology, Prevlence nd Nturl History of Durl Cvernous Sinus Fistuls (DCSFs) b c d Fig. 5.1 d. Dynmics in DCSF: Ongoing thrombosis in the CS reroutes the venous dringe nteriorly towrds the SOV demonstrting the dynmic process of the AV shunting in DCSF. b Initil dignostic ngiogrm, 26th August 1999, right ECA injection, AP nd lterl views: Filling of durl AV shunt t the right CS supplied by MMA, AMA nd IMA brnches. Dringe predominntly vi the right SPPS (rrow) into prcvernous sinus (thin rrow); Sylvin vein not involved; no IPS opcified. c d On the 15th September (21 dys lter), the ptient ws rescheduld for tretment. The repeted DSA in lmost identicl projections shows lrge filling defect within the right CS, indicting ongoing thrombosis, possibly triggered by the initil rteriogrm. The thrombus is not esily identifible in lterl views, lthough some of the feeding brnches from the IMA nd MMA pper less prominent nd seem to hve regressed (smll rrows). Note tht the SOV is menwhile opcified, showing chnge in the venous dringe pttern nd explining the slight ggrvtion of the ptient s symptoms within these 3 weeks. Note lso tht the left ECA supply fills lmost exclusively the right SOV, but not the SSPS, indicting not only bilterl AV shunt, but lso (ngiogrphic) comprtmentliztion (inset). (For tretment, see Cse Report IV) occur in venous recipients with low pressure, such s t the level of the skull bse, e.g. the CS s lowflow fistuls. Tsi et l. (2004) studied more recently 69 ptients with DAVF in different loctions nd found in 39% ssocited cerebrl sinus thrombosis. Thrombosis ws found lwys in the sinuses round to the fistul in the sinuses downstrem to the fistuls which supports the two min hypothesis, tht (1) venous outflow obstruction (by thrombus) my cuse opening of physiologic rteriovenous shunts or (2) venous hypertension cuses ischemi nd ngiogenesis (see below). Third, the theory tht thrombosis my be cused or ugmented by the turbulent fistul flow s suggested

81 5.2 Etiology nd Pthogenesis of Type B D Fistuls 71 b BA ICA Fig. 5.2,b. Dynmics in DAVF: Spontneous thrombosis of sigmoid sinus DAVF in less thn 24 h observed in 56-yer-old femle presenting with bruit nd hedches in Jnury Left APA injection, lterl view shows durl AV shunt (sterisk) involving the jugulr bulb nd the sigmoid sinus. Note the stenoses nd the restricted outflow (thick rrows), proximl in the IJV, nd distl in the sigmoid sinus, indicting ongoing thrombosis. b Control injection before endovsculr tretment the next morning no longer shows AV shunting due to thrombosis of the involved sinus segment, probbly triggered by the ngiogrphy on the previous fternoon. Note the filling of the vidin rtery (single rrow) vi phryngel rteries, communicting with the IMA, nd the retrogrde opcifiction of MMA brnches (double rrows). Due to chnged hemodynmics, the smll extr-intrcrnil nstomses become better visible: Crotid brnch of the ICA filling the verticl crotid segment (short rrow), clivl brnches (thin rrows) nstomosing with the C5 segment (TMH). Fint blush of the crotid siphon (ICA) nd bsilr rtery (BA) by some uthors (Chudhry et l. 1982; Nishijim et l. 1992; Lwton et l. 1997; Lsjunis et l. 1986) is supported s well. Ares of low flow velocity or flow stgntion found in sinuses where two prts of flow nterogrde cerebrl blood flow nd retrogrde fistul flow meet, could crete such hemodynmic condition. Although this potentil mechnism is not well studied, it my ply role s trigger for sinus stenosis. It is corroborted by observtion of cquired stenoses due to longstnding AV shunt flow seen in non-durl AV fistuls (see Fig. 6.2). A number of more recent studies focusing on the role of the cogultion system my dd vluble insights in the etiology of DAVFs (Krus et l. 1998, 2000; Gerlch et l. 2003; Singh et l. 2001). Krus et l. (1998) reported resistnce to ctivted protein C (APCR) in three out of seven ptients with DAVFs (non-cs loctions). APCR hs been shown to be cuse of fmilil thrombophili nd considered the most common genetic risk fctor for venous thrombosis. It lso impirs the recnliztion of sinus thrombosis, nd thus my further contribute to the development of DAVF. In lrger group of ptients, the sme uthors found evidence tht the incidence of sinus thrombosis, underlying DAVFs, is higher in ptients with FV Leiden (Krus et l. 1986). Singh et l. (2001) reported on 30-yer-old womn with thrombosis nd DAVF of the sigmoid sinus who lso hd prothrombin gene muttion. Wenderoth nd Phtouros (2003) ccidentlly discovered durl rteriovenous fistul in 58-yer-old ptient with APCR, concluding tht these ptients hve n initil predisposition to durl sinus thrombosis nd thus to pthophysiologicl conditions, possibly leding to the development of DAVF. Gerlch et l. (2003) recently studied 15 ptients with DAVFs (one cse of DCSF) nd found surprisingly high incidence (33%) of genetic thrombophilic bnormlities (muttion of prothrombin gene in 26% nd FV Leiden muttion in 6.7%). Although the number of ptients in this study is reltively smll, there is incresing evidence tht genetic thrombophilic bnormlities my contribute to the development of DAVFs. These results need to be confirmed by studies on lrger groups of ptients, but they support the theory of n inherent ssocition between DAVFs (nd DCSFs) nd sinus thrombosis. To wht degree thrombotic chnges trigger the formtion of durl rteriovenous shunt my further depend on geogrphic nd ethnic differences. It ws shown in recent survey in Jpn (Stomi 2008), tht durl sinus thrombosis might ply less importnt role thn generlly ssumed.

82 72 5 Etiology, Prevlence nd Nturl History of Durl Cvernous Sinus Fistuls (DCSFs) Venous Hypertension The concept of venous hypertension, being nother key fctor in the etiology of DAVFs, is encourged by results of niml experiments conducted by Terd et l. (1994), who demonstrted tht incresed venous pressure cn cuse newly cquired AVFs, even in bsence of thrombosis. It cn be suspected tht similr mechnism my lso ply role in humns (Phtouros et l. 2000). Kusuk et l. (2001) recently reported the development of DCSF remotely from previously thrombosed lterl sinus tht developed nother DAVF, emphsizing the role of venous hypertension. The uthors conclude tht the thrombosis cused venous hypertension not only within the superior sgittl nd right trnsverse/sigmoid sinus, but lso in the CS, cusing here micro AV shunts within the dur mter to open nd eventully to trigger the DCSFs. This is n interesting interprettion, but rises some questions. First, there ws no direct evidence tht the CS ws in fct exposed to n incresed venous pressure. Second, one would probbly expect nother fistul to develop in the neighborhood of the thrombosed lterl sinus where most likely the venous pressure ws elevted. The hemodynmic effects ssocited with durl rteriovenous shunts in the sigmoid/trnsverse sinus re my be more complex thn ssumed nd our knowledge on this subject is still limited. The role of venous hypertension s etiologicl fctor is ressured by nother observtion. A possible cusl reltionship between hypertension nd rteriovenous shunts t the CS hs lredy been ssumed by Potter (1954) s well s by Echols nd Jckson (1959), who suggested tht creting hypotension might be beneficil for cusing spontneous thrombosis of CCF. There re necdotl reports tht symptoms nd signs cused by CSF hve disppered fter ir trvel (Debrun et l. 1988; Kupersmith 1988). I hve seen one ptient who noticed his bruit, cused the first time by sigmoid DAVF immeditely fter lnding in n irplne. Another more recent ptient with DCSF reported drstic increse in eye swelling nd redness following 3-h flight. Chnges of tmospheric pressure seem to interfere with pressure in the cerebrl venous system. They my hve bidirectionl effect on the rteriovenous shunt flow, cusing either n increse, or decrese with spontneous occlusion. Ornuqe et l. (2003) hve successfully pplied this concept to tret ptients with DAVFs nd DCSFs using controlled hypotension (see lso Sect. 9.3). Lwton et l. (1997) were ble to demonstrte cusl reltionship between venous hypertension nd ngiogenic ctivity nd DAVF formtion. The uthors suggested tht venous hypertension is induced by venous outflow obstruction due to thrombus nd my initite the pthogenesis of DAVF. Venous hypertension cn cuse ischemi nd tissue hypoxi tht my stimulte ngiogenesis. This berrnt ngiogenic ctivity of durl vessels could led to rteriovenous shunting nd formtion of DAVF. The subsequent rteriliztion will increse venous pressure nd outflow obstruction nd thereby crete the vicious cycle mentioned bove tht my enlrge the AV shunt nd ggrvte DAVF into progressive lesion Trum Trum, lthough reported in some necdotl cses (Newton nd Hoyt 1970), is probbly less likely cuse of DCSF (Tomsick 1997b). Sttler (1920) hd doubts tht minor trum cn indeed cuse pulsting exophthlmos nd if so only if the vessel is disesed lredy or in cse of pregnncy (uthor s trnsltion). Tomsick (1997b) observed two ptients with spontneous fistul fter blunt hed trum, nd one ptient with Type D fistul following rhizotomy. He emphsized the development of DCSFs following severe hed trum mkes it difficult to define the exct etiology. The erly series of Hlbch et l. (1987) contined 1 ptient while lter, the sme group reported on 234 trumtic crotid nd vertebrl rtery lesions in which they observed 7 indirect fistuls. Berenstein et l. (1986) reported on 11 ptients with DCSFs of which 1 ws trumtic fistul supplied by the MMA. Jcobson et l. (1996) described in detil two cses in which ruptured AMA solely supplied CSF. True trumtic indirect fistuls represent possibly specific entity of DCSFs since their ngiorchitecture with single rtery to single vein or sinus pttern is distinctly different from the usul Type D fistul with complex network of numerous feeders emptying into the CS. This typicl ngiorchitecture, on the other hnd, cn hrdly be explined by trum. Revsculriztion of

83 5.2 Etiology nd Pthogenesis of Type B D Fistuls 73 thrombus (or more thn one) in the CS with subsequent neovsculriztion nd opening of multiple micro AV-shunts will most likely led to network of feeding vessels. Trum nd rupture will most certinly ffect only one (or few) of them, but will not crete network of vessels. A cse of trumtic DCSF following crniectomy hs been reported (Wtnbe et l. 1984). Fields et l. (2000) described 46-yer-old ptient who fter sustining gunshot wound to his fce developed CSF supplied by durl ICA brnches tht completely disppered 11 dys fter dignostic ngiogrphy. Some itrogenic CS fistuls my pper s single rtery- CS shunts. Aminoff (1973) considered the close ntomicl reltionship between durl rteries nd veins predisposition for the development of n AVF following hed trum. Moreover, the bsence of trum in the history would not exclude the possibility of minor hed trums cusing n AVF in prticulr in children nd young dults s cuse of DAVF. Otherwise, mny cses of DAVFs hve no history of trum nd, even if they do, then there is usully n intervl of severl weeks or months before symptoms of n AV shunt pper. Thus, direct trum seems unlikely to be mjor cuse for the development of DCSFs (Chudhry et l. 1982). Tomsick (1997b) gve n overview tht reveled pproximtely 3% of DCSFs re relted to trum. He observed n symptomtic DCSF during dignostic ngiogrphy for other indictions, including one CCF on the contrlterl side nd concludes tht mjor hed trum cn cuse DCSF. None of the ptients with DAVFs or DCSFs in my own mteril could clerly be relted to relevnt trum. Despite justified skepticism on the role of trum in the etiology of DAVFs nd lck of sufficient proof, it should be considered tht miniml trum might be very difficult to evlute s mny ptients will not recll minor events tht could hve resulted in the formtion of n bnorml AV shunt. Itrogenic vessel injury during endovsculr procedures my led to AV shunts involving the CS nd cn be cused by trnssphenoidl surgery of pituitry denoms (Tpts 1982; Bvinzski et l. 1997). I hve seen three itrogenic direct CCFs in my prctice, two ptients fter hypophysectomy nd one ptient who underwent septoplsty nd presented with high-flow CCF (Fig. 5.3). Ctheteriztion of cvernous durl ICA brnches for emboliztion of meningeoms my cuse rupture nd led to n indirect CSF s well (Brr et l. 1995). Figure 5.4 illustrtes such cse in which microctheter mnipultion into the mrginl tentoril rtery resulted in extrvstion tht fortuntely resolved without clinicl consequences Emboliztion The de novo development of DAVF in sigmoid or trnsverse sinus in ssocition with emboliztion of DCSF hs been reported severl times nd is explined by trnsvenous ctheter mnipultions with subsequent injury or by venous turbulence inititing thrombus nd recnliztion s trigger ( Nkgw et l. 1992; Ymshit et l. 1993; Mkiuchi et l. 1998; Kubot et l. 1999; Kwiguchi et l. 1999). Gupt et l. (2005) recently reported cse where 4 months fter trnsrteril emboliztion of DCSF new AV shunt t the ipsilterl sigmoid sinus developed. Strngely enough, the opposite order of events, nmely development of durl CSF following emboliztion of DAVF in other loction, hs to my knowledge not been reported Congenitl Although Lie (1968) proposed congenitl origin in some of the spontneous CSFs, this etiology remins specultive. DAVFs in generl, nd DCSFs in prticulr re rre in childhood, nd if they occur represent probbly seprte entity (Bigln et l. 1981; Ymmoto et l. 1995). Bigln et l. (1981) observed 7-week-old infnt with non-trumtic fistul between the externl crotid rtery nd the cvernous sinus. Konishi et l. (1990) reported cse of 2-month-old boy with congenitl fistul of the durl crotid-cvernous sinus. Skolnick nd McDonnell (2000) recently reported on 9-yerold boy who presented with proptosis, conjunctivl congestion nd decresed vision cused by spontneous durl cvernous sinus fistul. Among other resons, the fct tht the cvernous sinus is not fully developed t birth nd only prtilly prticiptes in the cerebrl venous dringe my explin why typicl durl CSF, s encountered in the dult popultion, is unlikely to occur in the peditric ge group.

84 74 5 Etiology, Prevlence nd Nturl History of Durl Cvernous Sinus Fistuls (DCSFs) b c d Fig. 5.3 d. Itrogenic direct CCF following septoplsty in 57-yer-old ptient. This ptient ws referred with exophthlmos, diplopi nd eye-redness for 4 weeks following surgery to correct septl devition in August b Direct fistul of the left CS (sterisk) with dringe into the right CS, both SOVs (short rrows), both bsl veins of Rosenthl (thin rrow) nd leptomeningel veins of the posterior foss. The fistulous opening ws too smll to be pssed with detchble blloon. Thus, trnsvenous emboliztion ws performed using the right IPS (double rrow). Tight pcking with GDC s resulted in complete occlusion of the AV shunt tht remined stble over the next 5 yers (c d FU in 2001). The ptient recovered completely Other Potentil Fctors Recently, the role of bsic fibroblst growth fctor (bfgf) nd vsculr endothelil growth fctor (VEGF) hs been studied (Terd et l. 1996; Shin et l. 2003). bfgf, considered powerful ngiogenic growth stimultor ssocited with the endothelil cell, ws found with strong immunorectivity in sinuses of ptients with DAVFs (Mlek et l. 2000). Urnishi et l. (1999) exmined histologiclly durl AVFs tht were surgiclly resected in nine ptients nd found tht the thick wll of the durl sinus stined strongly

85 5.2 Etiology nd Pthogenesis of Type B D Fistuls 75 b c Fig. 5.4 c. Itrogenic DCSF due to microctheter mnipultion during emboliztion of tentoril meningeom in June b The ruptured brnch of the MHT fills the posterior CS (sterisk) nd the IPS (double rrow). c The fistulous communiction disppered fter 10 min nd the ptient woke up without sequele. Arrow: mrginl tentoril rtery (Bernsconi nd Cssinri), double rrow: inferior petrosl sinus. Inset: Tentoril meningeom for bfgf, minly in the subendothelil lyer nd medi. VEGF ws expressed in the endothelium of the sinus in ll nine cses indicting tht ngiogenetic growth fctors my ply role in the pthogenesis of DAVFs. This ngiogenetic process could be ssocited with loss of venulr surfce properties nd contribute to venous thrombosis (Srm nd ter Brugge 2003). Lwton et l. (1997) were ble to prove tht ngiogenic ctivity mesured by the rbbit corne ssy ws significntly greter in nimls with venous hypertension, suggesting tht the venous hypertension nd sinus thrombosis my lter the blnce of prongiogenic nd ntingiogenic substnces (Folkmn 1995) Vrious The higher incidence of DCSFs in women during menopuse nd in men over 50 yers indictes potentil role of vessel wll wekening over time due to rteriosclerotic chnges. Arteril hypertension nd dibetes re considered predisposing fctors s well, but correltive dt re lcking so fr.

86 76 5 Etiology, Prevlence nd Nturl History of Durl Cvernous Sinus Fistuls (DCSFs) In summry, despite mjor dvnces in dignosis nd mngement, the etiology nd pthogenesis of DAVFs nd DCSFs remins mtter of controversy nd is fr from being fully understood. The development of durl rteriovenous shunts must be seen in close reltion with thrombotic processes nd venous hypertension in the cerebrl sinuses. Therefore, the term venous diseses ppers suitble. To wht extend other fctors trigger or contribute to the development of durl AV shunts, remins to be investigted by further experimentl nd clinicl studies. Better understnding of mechnisms of moleculr pthogenesis in the development of durl AVFs might id in the estblishment of new therpeutic mesures for this unique vsculr disese. 5.3 Prevlence Epidemiologic dt regrding the overll incidence of both DAVFs s well s of DCSFs re limited. Lrge utopsy series hve found 46 AVMs mong 3200 brin tumors (1.4%) (Olivecron nd Riives 1948). Popultion-bsed dt showed n incidence of 1.84 per 100,000 person yers during ; the incidence of symptomtic cses ws 1.22 per 100,000 person yers (Brown et l. 1996). The detection rte in more recent study ws 0.29 per 100,000 dults (Stomi 2008). According to older literture, DAVF comprise 10% 15% of ll intrcrnil rteriovenous mlformtions. Most recent uthors refer to the work of Newton nd Cronvist (1969) who reported on totl of 129 ptients mong whom 94 hd pure pil, 20 hd mixed pil-durl nd 15 (12%) hd pure durl supply. One recent review on nturl history of intrcrnil vsculr mlformtion does not provide new numbers on incidence nd prevlence of DAVFs (Brown et l. 2005). Most ptients with DAVFs re more thn 40 yers of ge (82%) nd women (71%) (Mironov 1995). Becuse similr dignostic nd therpeutic mesures pply to ll durl fistuls, DCSFs nd DAVF cn be considered s one epidemiologic group of disese. For both, the ssocition with menopuse (54%) nd puerperium (18%) nd lower incidence in men is typicl (Newton nd Hoyt 1970; Tomsick 1997b; Toy et l. 1981). In metnlysis published by Awd et l. (1990), with n dditionl 17 cses, the incidence of AVFs in the CS mong ll DAVFs ws 11.9%, wheres the reltionship of ggressive to non-ggressive cses ws 1:6.5 (Tble 5.1). In his review of 322 cses, published up to 1920, Sttler (1920, 1930) found frequency of trumtic vs idiopthic fistuls of 3:1. As elucidted bove, this distribution chnged towrds the end of the twentieth century, nd trumtic CSFs were less frequently seen in the western world. This trend is likely due to improved sfety stndrds for trffic vehicles, in prticulr motorcycles nd bicycles. Even lrge cities in Asi with high percentge of motorcycle nd bicycle riders hve decresed incidence, s recently reported from the Queen Elizbeth Hospitl in Hong Kong, where mong 80 ptients with CSF, 76 (95%) were spontneous nd only 4 (5%) were of trumtic origin (Cheng 2006). Among ll CSFs, pproximtely 10% 15% ccount for indirect or durl fistuls (Brcio-Slorio et l. 2000) nd Type D is by fr the most frequent (Tomsick 1997b). The true prevlence of DCSFs is difficult to ssess due to the fct tht mny ptients present with mild symptoms, my undergo spontneous resolution nd re never dignosed. According to series of severl mjor endovsculr centers, Type B D DCSFs occur five times more frequently thn Type A fistuls (Tomsick 1997b). Another difficulty in obtining ccurte numbers lies in the fct tht mny DCSFs ptients re studied s different groups, either included in DAVFs, CCFs or evluted s seprte entity. The series of Cognrd et l. (1995) contined 205 cses of DAVF of which 33 were DCSF (16%). Klisch et l. (2003) reported on 17 CSFs, including 11 DCSFs. Stomi et l. (2002) reported on 117 cses of benign DAVF (without corticl venous dringe) with DCSF representing the lrgest group (42.7%). Tsi et l. (2004) investigted 69 ptients with DAVFs mong whom 30% involved Tble 5.1. Frequency of DCSFs in lrge series of DAVFs References DAVFs CS Awd et l (11.9%) Cognrd et l (16%) Mironov (30.2%) Stomi et l b (42.7%) Tsi (30%) Mlek et l (34%) Chung et l (57%) Ling (69.1%) 17 cses nd metnlysis b Cses of benign DAVF

87 5.3 Prevlence 77 the CS. Chung et l. (2002) found the CS s the most common (57%) loction for the DAVF in 60 ptients. Cheng et l. (2003) reported on 27 ptients who were ssessed bsed on Cognrd s clssifiction. Tomsick (1997b) observed Type B D fistuls in 68% of ll spontneous CSF nd discusses the influence of the referring prxis. Tble 5.2 provides n overview on published series of spontneous, trumtic nd durl fistuls involving the CS. It revels tht the distribution of vrious fistul types differs considerbly mong the groups. While Andoh et l. (1991) reports n incidence of Type D fistuls of 25% nd Liu et l. (2001) noted 54%, this type is observed by Vinuel et l. (1984) nd Tkhsi nd Nkno (1980) in 100%. As discussed bove, dignostic qulity of cerebrl ngiogrphy s mjor tool for identifying, clssifying nd understnding etiology nd nturl history of CSFs hs significntly improved over the lst 25 yers. This must be considered when looking t dt in older series using ngiogrphic ssessments. Tody, high-resolution DSA provides more morphologicl informtion thn ws vilble in the pst nd helps to further develop theories nd new etiologicl concepts. Thus, it hppened probbly not completely by chnce tht in severl recent studies, ctegoriztion of DCSFs s groups with different rteril supply (Types B D) hs no longer been pplied (see Tble 5.2) (Meyers et l. 2002; Cheng et l. 2003; Stiebel-Klish et l. 2002). Tble 5.2. Prevlence of DCSFs relted to either trum or spontneous occurrence. Modified fter Tomsick (1997b) References Yers Types B D (s) Types B D (t) Type B Type C Type D Newton Tniguchi Tkhshi Seegher Hlbch Vinuel Kupersmith Brrow Debrun Andoh Kurt Sski ? Kinugs (C1/C2) 19 (D1/D2) Tki Tomsick (D1/D2) Liu ? Meyer NP NP NP NP Cheng Stiebel-Klish NP NP NP NP Klisch Suh N/A N/A 23 PT N/A 23 RT 8 Wkhloo N/A 4 2, C2 = 1 Theudin N/A NP NP Kim NP NP NP N/A 12 LRT D2 = bilterl C2 = bilterl NP = not provided According to Cognrd s clssifiction? = Not identified by uthors

88 78 5 Etiology, Prevlence nd Nturl History of Durl Cvernous Sinus Fistuls (DCSFs) Nturl History DCSFs, in generl, re referred to s benign or non-ggressive fistuls becuse of their tendency to occlude spontneously, usully cused by CS thrombosis. Although these spontneous occlusions seem to occur more frequently thn in other AV shunting lesions, they cn be ccompnied by serious clinicl deteriortions. Neither such course nor neurologicl deficit cused by venous ischemi or intrcrnil hemorrhge, both rre events, cn in fct be considered benign. But even though such clinicl course my be progressive or in some cses fulminnt, terms like ggressive, mlignnt or benign, widely used by neurordiologists, pper unsuitble nd rther imprecise to chrcterize durl rteriovenous shunting lesion. They should be reserved for diseses for which they were defined, i.e. tumors. As true for prevlence nd incidence of DCSFs, exct dt on nturl history do not exist, which is in prt due to the fct tht lrge number of fistuls is discovered reltively lte in their course. Furthermore cses undergoing dignostic ngiogrphy re necessrily ffected by the ngiogrphic procedure itself s contrst injection cn ccelerte thrombosis of the CS nd spontneous occlusion (Newton nd Hoyt 1970; Seeger et l. 1980; Voigt et l. 1971; Phelps et l. 1982). In some recent series the nturl course is dditionlly influenced by prticulte rteril emboliztion (Stomi et l. 2005; Suh et l. 2005). The number of reported spontneous occlusions reported in the literture my lie between 11% nd 90% (Vinuel et l. 1984; Kupersmith et l. 1988) nd is on verge 35% ccording to Tomsick (1997b). When looking t rtes of spontneous occlusion fter ngiogrphy one hs to consider the number of ptients reported. For exmple, frequently quoted, Phelps et l. (1982) observed 43% (in Meyers et l. 2002) occlusion rte in 3 out of 7 ptients undergoing ngiogrphy in group of 19 ptients with typicl signs of crotid cvernous fistul! This rte is lower in lrger series. Only one cse in my own mteril developed spontneous occlusion, ptient with n IPS fistul, drining vi the CS nd cusing diplopi (see lso Sect. 9.1 nd Cse illustrtion XII). Throughout the process of spontneous occlusion the progress of thrombosis in the CS nd its effects on the venous dringe is uncertin. In mny ptients regression of ophthlmologicl symptoms is preceded by n excerbtion of ophthlmologicl symptoms. CS thrombosis, spontneous or triggered by emboliztion, my involve the ophthlmic veins nd cn cuse significnt deteriortion of symptoms due to sudden increse of venous pressure (Seeger et l. 1980). This phenomenon hs been described by Sergott et l. (1987) s prdoxicl worsening. In some cses even vision loss my occur (Sergott et l. 1987; Binchi- Mrsoli et l. 1996; Suzuki et l. 1989; Knudtzon 1950; Miki et l. 1988). Becuse it my ccentute intrinsic thrombotic effects on the centrl retinl vein, n emboliztion procedure in ptient with worsening of symptoms might be contrindicted (Tomsick 1997b). If incresed introculr pressure occurs, dditionl nticogultion my be dvisble. Subcutneous use of low-moleculr heprin hs improved clinicl signs in four ptients s reported by Binchi-Mrsoli et l. (1996). Suh et l. (2005) hve retrospectively studied the evolution of fistuls over men follow-up period of 23 months. They found tht seven (30%) of their ptients, ngiogrphiclly clssified s prolifertive type (PT), showed chronologicl progression to the lte restrictive type (LRT). Unfortuntely, it is not cler from their description how mny of these ptients underwent tretment by emboliztion which mkes it presumptuous to pply the results to the nturl history. This is similrly true for the study of Stomi et l. (2005) who ctegorized ptients ccording to their chnges in venous dringe but included embolized ptients s well. As for DAVFs, presence of leptomeningel dringe is min indictor for risk ssessment nd decision-mking in ptients with DCSFs by most uthors. The number for corticl or leptomeningel reflux vries from 10% to 31% (Tble 5.3). Nevertheless, the ssocited risk of intrcrnil hemorrhge (round 2%) seems, however, reltively low compred to ptients with DAVFs in other loctions, especilly t the tentoril sinus or in the nterior crnil foss (Agid 2009). For exmple, none of the DCSF ptients in the study in Cognrd s series (Cognrd et l. 1995) showed n ggressive course nd in nother lrge study no cse with such behvior ws found (Awd et l. 1990). Consequently, corticl or leptomeningel dringe in DCSFs must not necessrily be seen n indictor of progressive or mlignnt course or nture, s in DAVFs. Yet tody, most opertors will probbly gree to tret these fistuls s erly s possible even though they my pose lesser risk thn direct CCFs with corticl dringe.

89 5.3 Prevlence 79 Tble 5.3. Frequency of corticl/leptomeningel venous dringe nd ssocited hemorrhge Authors DCSFs C/LVD Hemorrhge Hlbch et l (10%) Awd et l n/ n/ Cognrd et l (12%) 0 Tomsick (16%) 0 Stomi et l b (26.1%) 1 (1.5%) Theudin et l (18.5%) 0 Stiebel-Klish (26%) 2 (2.2%) et l Meyers et l (31%) 2 (1.5%) 17 cses nd metnlysis In summry, lcking ccurte dt, only limited knowledge on prevlence nd nturl history in ptients hrboring DCSFs exist. Lrger studies with long-term follow-up of ptients, not undergoing ny sort of tretment including mnul compression therpy, will be needed to obtin relible informtion on the nturl course of the disese. The term benign fistul, referring to generlly milder clinicl course, does not pper suitble nd should be reserved for diseses of tht biologicl nture. References b Cses of benign DAVF Agid R, et l. (2009) Mngement strtegies for nterior crnil foss (ethmoidl) durl rteriovenous fistuls with n emphsis on endovsculr tretment. J Neurosurg. 110(1): Ambler MW, Moon AC, Sturner WQ (1978) Bilterl crotid-cvernous fistule of mixed types with unusul rdiologicl nd neuropthologicl findings. J Neurosurg 48: Aminoff MJ (1973) Vsculr nomlies in the intrcrnil dur mter. Brin 96: Andoh T, Nkshim T, Arki Y, Ski N, Ymd H, Kgw Y, Hirt T, Tnbe Y, Tkd M (1991) Spontneous crotid-cvernous sinus fistul; nlysis of 16 cses. No Shinkei Gek 19: Awd IA, et l. (1990) Intrcrnil durl rteriovenous mlformtions: fctors predisposing to n ggressive neurologicl course. J Neurosurg 72: Brcio-Slorio JL, Brci JA, Soler FM (2000) Rdiosurgery for crotid-cvernous fistuls. In: Eisenberg MB, Al Mefty O (eds) The cvernous sinus. Lippincott Willims & Willims, Phildelphi, pp Brr JD, Mthis JM, Horton JA (1995) Itrogenic crotid-cvernous fistul occurring fter emboliztion of cvernous sinus meningiom. AJNR Am J Neurordiol 16: Brrow DL, Spector RH, Brun IF, Lndmn JA, Tindll SC, Tindll GT (1985) Clssifiction nd tretment of spontneous crotid-cvernous sinus fistuls. J Neurosurg 62: Brtholow R (1872) Aneurysms of the rteries of the bse of the brin-their symptomtology dignosis nd tretment. Ann J Med Sci 64: Bvinzski G, Killer M, Knosp E, Ferrz-Leite H, Gruber A, Richling B (1997) Flse neurysms of the intrcvernous crotid rtery report of 7 cses. Act Neurochir (Wien) 139:37 43 Berenstein A, Scott J, Choi IS, Persky M (1986) Percutneous emboliztion of rteriovenous fistuls of the externl crotid rtery. AJNR Am J Neurordiol 7: Berger MS, Hosobuchi Y (1984) Cvernous sinus fistul cused by intrcvernous rupture of persistent primitive trigeminl rtery. Cse report. J Neurosurg 61: Binchi-Mrsoli S, Righi C, Cisc P (1996) Low dose heprin therpy for durl cvernous sinus fistuls. Neurordiology Suppl:15 Bigln AW, Png D, Shuckett EP, Kerber C (1981) Externl crotid-cvernous fistul in n infnt. Am J Ophthlmol 91: Brismr G, Brismr J (1976) Spontneous crotid-cvernous fistuls: phlebogrphic ppernce nd reltion to thrombosis. Act Rdiol Dign (Stockh) 17: Brown RD Jr, Wiebers DO, Torner JC, O Fllon WM (1996) Incidence nd prevlence of intrcrnil vsculr mlformtions in Olmsted County, Minnesot, 1965 to Neurology 46: Brown RD Jr, Flemming KD, Meyer FB, Cloft HJ, Pollock BE, Link ML (2005) Nturl history, evlution, nd mngement of intrcrnil vsculr mlformtions. Myo Clin Proc 80: Cstigne P, Blncrd P, Lplne D, Djindjn R, Sorto M (1966) Spontneous rteriovenous communiction between the externl crotid nd the cvernous sinus. Bull Soc Ophtlmol Fr 66:47 49 Cstigne P, Lplne D, Djindjin R, Bories J, Augustin P (1966b) Spontneous rteriovenous communiction between the externl crotid nd the cvernous sinus. Rev Neurol (Pris) 114:5 14 Chudhry MY, Schdev VP, Cho SH, Weitzner I Jr, Puljic S, Hung YP (1982) Durl rteriovenous mlformtion of the mjor venous sinuses: n cquired lesion. AJNR Am J Neurordiol 3:13 19 Chen MN, Nkzw S, Hori M (1993) A cse of primry intrcrnil choriocrcinom with crotid-cvernous fistul. No Shinkei Gek 21: Cheng K (2006) Neuro-endovsculr therpy of crotid cvernous fistul. Hong Kong Med Diry 11 Cheng KM, Chn CM, Cheung YL (2003) Trnsvenous embolistion of durl crotid-cvernous fistuls by multiple venous routes: series of 27 cses. Act Neurochir 145:17 29 Chumn H, Trobe JD, Petty EM, Schwrze U, Pepin M, Byers PH, Deveikis JP (2002) Spontneous direct crotid-cvernous fistul in Ehlers-Dnlos syndrome type IV: two cse reports nd review of the literture. J Neuroophthlmol 22:75 81 Chung SJ, Kim JS, Kim JC, Lee SK, Kwon SU, Lee MC, Suh DC (2002) Intrcrnil durl rteriovenous fistuls: nlysis of 60 ptients. Cerebrovsc Dis 13:79 88 Cognrd C, Gobin YP, Pierot L, Billy AL, Houdrt E, Cs-

90 80 5 Etiology, Prevlence nd Nturl History of Durl Cvernous Sinus Fistuls (DCSFs) sco A, Chirs J, Merlnd JJ (1995) Cerebrl durl rteriovenous fistuls: clinicl nd ngiogrphic correltion with revised clssifiction of venous dringe. Rdiology 194: Dndy W (1937) Crotid-cvernous neurysms (pulsting exophthlmos). Zentrlbl Neurochir 2: Dny F, Frysse A, Priollet P, Brutus P, Bokor J, Ctnzno G, Bernrd P, Christides C, Beylot C (1986) Dysmorphic syndrome nd vsculr dysplsi: n typicl form of type IV Ehlers-Dnlos syndrome. J Ml Vsc 11: de Cmpos JM, Ferro MO, Burzco JA, Boixdos JR (1982) Spontneous crotid-cvernous fistul in osteogenesis imperfect. J Neurosurg 56: Debrun GM, Dvis KR, Nut HJ, Heros RE, Ahn HS (1988) Tretment of crotid cvernous fistule or cvernous neurysms ssocited with persistent trigeminl rtery: report of three cses. AJNR Am J Neurordiol 9: Debrun GM, Vinuel F, Fox AJ, Dvis KR, Ahn HS (1988b) Indictions for tretment nd clssifiction of 132 crotidcvernous fistuls. 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93 References 83 Terd T, Tsuur M, Komi N, Higshid RT, Hlbch VV, Dowd CF, Wilson CB, Hieshim GB (1996) The role of ngiogenic fctor bfgf in the development of durl AVFs. Act Neurochir (Wien) 138: Terd T, Higshid RT, Hlbch VV, Dowd CF, Hieshim GB (1998) The effect of oestrogen on the development of rteriovenous fistule induced by venous hypertension in rts. Act Neurochir (Wien) 140:82 86 Theudin M, et l. (2006) Dignosis nd tretment of durl crotid-cvernous fistule: consecutive series of 27 ptients. J Neurol Neurosurg Psychitry Tokung K, Sugiu K, Kmed M, Ski K, Tersk K, Higshi T, Dte I (2004) Persistent primitive trigeminl rtery-cvernous sinus fistul with intrcerebrl hemorrhge: endovsculr tretment using detchble coils in trnsrteril double-ctheter technique. Cse report nd review of the literture. J Neurosurg 101: Tomsick TA (1997) Typ A (direct) CCF: etiology, prevlence nd nturl history. In: Tomsick TA (ed) Crotid cvernous fistul. Digitl Eductionl Publishing, Phildelphi, pp Tomsick TA (1997) Typ B,C, nd D CCF: etiology, prevlence nd nturl history. In: Tomsick TA (ed) Crotid cvernous fistul. Digitl Eductionl Publishing, Phildelphi, pp Toy S, Shiobr R, Izumi J, Shinomiy Y, Shig H, Kimur C (1981) Spontneous crotid-cvernous fistul during pregnncy or in the postprtum stge. Report of two cses. J Neurosurg 54: Trvers B (1811) A cse of neurysm by nstomosis in the orbit, cured by the ligture of the common crotid rtery. Med Chir Tr 2:1 16 Tsi LK, et l. (2004) Intrcrnil durl rteriovenous fistuls with or without cerebrl sinus thrombosis: nlysis of 69 ptients. J Neurol Neurosurg Psychitry 75: Urnishi R, Nkse H, Skki T (1999) Expression of ngiogenic growth fctors in durl rteriovenous fistul. J Neurosurg 91: vn Rooij WJ, Sluzewski M, Beute GN (2006) Ruptured cvernous sinus neurysms cusing crotid cvernous fistul: incidence, clinicl presenttion, tretment, nd outcome. AJNR Am J Neurordiol 27: Vinuel F, Fox AJ, Debrun GM, Peerless SJ, Drke CG (1984) Spontneous crotid-cvernous fistuls: clinicl, rdiologicl, nd therpeutic considertions. Experience with 20 cses. J Neurosurg 60: Voigt K, Suer M, Dichgns J (1971) Spontneous occlusion of bilterl croticocvernous fistul studied by seril ngiogrphy. Neurordiology 2: von Rd M, Tornow K (1975) Spontneous crotid cvernous fistul in cse of hereditry hemorrhgic telngiectsi (Osler-Rendu) (uthor s trnsl). J Neurol 209: Wlker AE, Allegre GF (1956) Crotid-cvernous fistuls. Surgery 39: Wtnbe A, Tkhr Y, Ibuchi Y, Mizukmi K (1984) Two cses of durl rteriovenous mlformtion occurring fter intrcrnil surgery. Neurordiology 26: Wenderoth JD, Phtouros CC (2003) Incidentl discovery of durl rteriovenous fistul in ptient with ctivted protein C resistnce. AJNR Am J Neurordiol 24: Wolff H, Schmid B (1939) Ds Arteriogrmm des pulsierenden Exophthlmus. Zbl Neurochir 4: , Ymmoto T, Asi K, Lin YW, Suzuki K, Oht S, Oht K, Ymmoto M, Ichiok H (1995) Spontneous resolution of symptoms in n infnt with congenitl durl croticocvernous fistul. Neurordiology 37: Ymshit K, Tki W, Nkhr I, Nishi S, Sdto A, Kikuchi H (1993) Development of sigmoid durl rteriovenous fistuls fter trnsvenous emboliztion of cvernous durl rteriovenous fistuls. AJNR Am J Neurordiol 14: Zimmermn R, Leeds NE, Nidich TP (1977) Crotid-cvernous fistul ssocited with intrcrnil fibromusculr dysplsi. Rdiology 122:

94 Neuro-Ophthlmology in 6 Durl Cvernous Sinus Fistuls (DCSFs) CONTENTS Introduction Extrorbitl Oculr Symptoms Orbitl Pin Crnil Nerve Deficits nd Ophthlmoplegi Orbitl Symptoms Exophthlmos Conjunctivl Engorgement nd Chemosis Retinl Hemorrhge Cornel Dmge Orbitl Bruit Puls-synchronous Pulstion of the Eyelid nd Bulb Secondry Glucom nd Visul Loss Other nd Neurologicl Symptoms Differentil Dignosis 92 Introduction References 94 Hemodynmic dysregultion in AV-shunting lesions of the CS led in 80% of cses to elevted pressure in peri- nd introrbitl veins. This results in interstitil edem nd incresed cliber of the orbitl veins nd the CS, which cuses in turn mechnicl compression nd ischemi. Spectrum nd progression of resulting neuroophthlmologicl deficits in ptients with DCSF re determined by individul hemodynmics nd ngiorchitecture of the fistuls dringe (Tble 6.1). Most DCSFs drin vi the superior ophthlmic vein into the ngulr, suprorbitl nd fcil veins. Such nterior dringe is usully ssocited with more impressive clinicl symptoms. It my cuse ipsilterl CN deficits nd dmge to orbitl orgns. The posterior dringe vi IPS or SPS insted rrely cuses ophthlmologic symptoms, but my be re- sponsible for oculomotor deficits due to ischemic, or less frequently, mechnicl disturbnce of CN functions. Fistuls with posterior dringe my lso cuse trigeminl neuropthy or fcil nerve presis (Eggenberger 2000; Rizzo 1982). Assocition with locl thrombosis of the CS or IPS is often found. It my led to rpid deteriortion cused by cute elevtion of the intrvenous nd introculr pressures nd cn be the reson for slow recovery lter on. Bi- or contrlterl symptoms occur in 10% of the ptients with DCSFs nd cn be due to thrombotic occlusions of ipsilterl drining veins nd involvement of intrcvernous nd /or bsilr sinuses s well s of the contrlterl CS in the fistuls dringe. It remins uncler whether venous thrombosis, often seen on ngiogrms, hs developed secondrily on the bsis of hemodynmic turbulences, or s residuum of the initil thrombotic processes considered triggering fctor in the pthogenesis of DCSFs (Grove 1984). Complictions nd unfvorble long-term outcomes in the nturl course of the disese minly involve the eye. The spontneous occlusion rte of DCSFs in the literture my lie between 11% nd 90%, depending on observtions mde by different uthors (Vinuel et l. 1984, Keltner et l., 1987b, Kupersmith 1988). Interestingly enough, severl uthors hve reported disppernce of the AV shunting following dignostic ngiogrphy (Grove 1984; Keltner et l. 1987b; Phelps et l. 1982; Voigt et l. 1971). Thus, spontneous cure seems possible nd should be considered in therpeutic decision-mking. The clssicl Dndy s trid seen in Type A or trumtic CCFs (Fig. 6.1) chemosis, exophthlmos nd bruit is rrely observed in ptients with durl CSF. Becuse of the usully chronic, cliniclly milder nd vriform mnifesttion of symptoms, the dignosis of DCSF cn be less strightforwrd nd my not llow stndrd procedurl regimen. If n individul ptient presents with nonspecific symptoms, the clinicl picture my provide initil

95 86 6 Neuro-Ophthlmology in Durl Cvernous Sinus Fistuls (DCSFs) Furthermore, hemodynmic turbulences in the fistul leding to pinful irrittion of the meninges cn increse during physicl exercise or elevted blood pressure. Extroculr or extrorbitl symptoms beside hedches re rre. Anecdotl reports of life-thretening epistxis or intrcerebrl hemorrhges in cse of leptomeningel venous dringe (Keltner et l. 1987) or ischemic brin stem infrcts fter sinus venous thrombosis (Uchino et l. 1997) nd typicl trigeminl neurlgi (Ott et l. 1993) exist. Fistulous connections in the posterior CS my cuse mechnicl compression of the Gsserin gnglion or vsculr stel phenomen ffecting in prticulr the first or second division, resulting into neurlgi nd dysesthesi (Mdsen 1970; Plestine et l. 1981). They cn initilly occur isolted nd thus my esily be mistken for idiopthic trigeminl neurlgi (Ott et l. 1993; Rizzo et l. 1982). Ptients with periorbitl cephlgi nd ccompny c b d Fig. 6.1 d. Clssic clinicl presenttion of trumtic direct CCF tht is often cute nd fulminnt, but cn be delyed for severl dys or weeks, in some cses for severl months. A 24-yer-old mn fter cr ccident seen in July b Severe exophthlmos nd chemosis of the right eye, ssocited with udible bruit. c The rteriogrm shows mssively enlrged CS (sterisks) with posterior bulging into the crnil cvity nd dominnt nterior dringe into significntly enlrged SOV. No intrcrnil stel. Note tht such severe chemosis is rre in DCSFs (see Fig. 6.4) d Fistul occlusion with one detchble blloon signs pointing towrds etiology, hemodynmics nd prognosis. Therefore, knowledge nd creful nlysis of the neuroophthlmologicl symptoms re required for differentil dignosis, effective, individul, risk- nd prognosis-oriented use of dignostic nd therpeutic mesures in ptients with DCSFs. 6.1 Extrorbitl Oculr Symptoms Orbitl Pin Persistent frontl or periorbitl cephlgi of vrying intensity, often strting cutely, is frequent initil symptom in ptients with DCSF. These hedches re usully cused by locl thrombosis within the CS or the SOV.

96 6.1 Extrorbitl Oculr Symptoms 87 b c Fig. 6.2 c. Aggrvted chemosis in low-flow AV fistul., 88-yer-old ptient with diplopi due to 6th nerve plsy since 08/98. She presented with incresing proptosis nd ggrvted chemosis in 02/99. b, Left ICA injection AP view shows low-flow fistul drining into the right SOV (rrow). c, This vessel turned out to be prtilly occluded by lrge intrluminl thrombus s reveled by superselective injection into the CS during the tretment session (rrows). Venous outflow obstruction ssocited with low-flow AV shunt leds to venous hypertension nd my cuse drmtic symptoms (see lso Chp. 10) ing ophthlmoplegi, initilly misdignosed s suffering from migrine, cluster hedches, Tolos-Hunt syndrome or intrcrnil neurysms, hve been described s well (Hwke et l. 1989; Brzis et l. 1994; Komorsky 1988) Crnil Nerve Deficits nd Ophthlmoplegi Unilterl ophthlmoplegi cn be seen in c. 50% of the ptients with DCSFs (Miychi et l. 1993) nd often represents the first objective symptom. It usully becomes mnifest fter weeks or months following development of fistul. The elevted introrbitl venous pressure leds to progressive swelling of the oculr muscles nd to reduced contrctility nd limited motility of the eye bulb. On the other hnd, dilted vessels nd vsculr stel phenomen result in mechnicl nd ischemic oculomotor nerve dmge. Due to their course through the CS, the sixth CN is most frequently (46% 85%), the third CN less frequently (36%) nd usully in cses with posterior dringe, nd the fourth CN (11%) is rrely involved (Kupersmith et l. 1986, 1988). In cses of nterior dringe, the ophthlmoplegi is often ccompnied by other orbitl symptoms such s exophthlmos nd chemosis. In contrst, fistuls with posterior dringe vi the IPS my be the cuse for isolted ophthlmoplegi nd should be included in the differentil dignoses of intrcrnil neoplsms, cvernous neurysms nd meningitis (Acierno et l. 1995). Although diplopi in these ptients is usully reversible, it requires intensive neuro-ophthlmologicl cre nd erly intervention. In some cses of longstnding AV shunting into the CS, diplopi my become permnent. 6.2 Orbitl Symptoms Severl uthors postulte nterior dringe or disturbnce of the nterior venous outflow from the

97 88 6 Neuro-Ophthlmology in Durl Cvernous Sinus Fistuls (DCSFs) Tble 6.1. Incidence of frequent signs nd symptoms in DCSFs in recent series (Meyers et l. 2002; Stiebel-Klish et l. 2002; de Keizer 2003; Theudin et l. 2006; Suh et l. 2005) Meyers et l. (2002) % of 135 Stiebel-Klish et l. (2002) % of 85 de Keizer (2003) % of 68 Kim (2006) % of 65 Theudin et l. (2006) % of 27 Suh et l. (2005) 58 Conjunctivl injection Chemosis Propotosis Diplopi Bruit b 27 Retroorbitl pin Elevted IOP Decresed visul cuity Retinl hemorrhge De Keizer (2003) differentited in 68 spontneous (durl, orbitl nd direct) nd 33 trumtic (direct nd durl) fistuls b Subjective bruit in 24 nd objective in 7 ptients Suh et l. (2005): divided 58 ptients into four min symptom pttern Orbitl pttern (chemosis, exophthlmos,pin,eyelid swelling): 53% Cvernous pttern (ptosis,diplopi, nisocori, ophthlmoplegi): 71% Oculr pttern (decresed vision, IOC > 20 mm Hg oculr pin, glucom, retinl hemorrhge): 64% Cerebrl pttern (seizures, hemorrhge): 5% CS s precondition for the presence of orbitl symptoms in ptients with DCSFs, lthough its ngiogrphic ppernce my be mimicked by n ntomicl vrint or be cused by thrombotic occlusion of the IPS (Grove 1984; Hoops et l. 1997) Exophthlmos As result of the chroniclly elevted introrbitl venous pressure prolpse of the orbitl soft tissue my develop, which is commonly less prominent thn in ptients with direct CCFs. In most cses it is less thn 5 mm nd cn initilly be overlooked. The most precise method of mesurement is to exmine the ptient in reclining hed position using the Hertel Exophthlmometer. A mssive exophthlmos, rrely observed in cses of DCSF, cn led to chemosis, lid swelling, lgophthlmos nd cornel dmge. If n cute incresing exophthlmos, ssocited with pin nd lid swelling occurs nd is followed by spontneous improvement, thrombosis of the SOV or the CS should be considered Conjunctivl Engorgement nd Chemosis Typiclly, rteriliztion of conjunctivl veins is ssocited with other ophthlmologic mnifesttions, prticulrly with exophthlmos, nd cn be found in 82% 100% of ptients (Phelps et l. 1982; Plestine et l. 1981; Kupersmith et l. 1988) with introrbitl symptoms. It my led to dilttions nd tortuosities of conjunctivl veins, which re often the cuse for misdignosis such s inflmmtory conjunctivitis. However, the conjunctivl injection in ptients with CSF is, different from llergic, virl or bcteril conjunctivitis, chrcterized by brightred, corkscrew veins. Except for cses of secondry infections, it usully occurs without inflmmtory secretion. These veins re clled specific epibulbr loops by De Keizer (2003) nd represent the most superficil lyer, developing due to opening of smll cpillry connections t the outside of the orbit (Fig. 6.3). Enlrged connections on the eyebll develop between the recurrent conjunctivl ciliry veins nd posterior conjunctivl veins (specific limbl loops) (de Keizer 1979, 2003).

98 6.2 Orbitl Symptoms 89 c b Fig. 6.3 c. Conjunctivl injection (eye redness) in DCSF. Symptoms in DCSFs cn be similir to tht of direct CCF ptients, but re usully milder nd develop less drmticlly. A bruit is infrequently reported. This 86-yer-old womn presented in Mrch 2002 with mild exophthlmos, eye redness nd 6th crnil nerve plsy (). The close-up shows the typicl corkscrew diltion of epibulbr veins, which cn be considered pthognomonic for DCSFs (b). The type of venous dringe (nterior or posterior) my determine the clinicl presenttion. This fistul s dringe involves nteriorly the SOV (rrow), nd posteriorly the SPS (dotted rrow), the nterior pontomesencephlic vein, bsl vein of Rosenthl nd leptomeningel veins of the posterior foss (c). Only some of the ptients with retrogrde corticl or leptomeningel venous dringe present with venous ischemi or hemorrhge. In most cses, the clinicl symptoms re purely opththlmologicl. Tretment ws performed using TVO (see Chp. 8, Fig. 8.4) Conjunctivl chemosis is defined s n edem of the scler nd occurs in 25% 90% (Plestine et l. 1981; Kupersmith et l. 1988; Vinuel et l. 1984) of the cses, ccompnying conjunctivl injection in ptients with DCSFs. In prticulr, when exophthlmos occurs, it my cuse significnt prolpse of the conjunctiv with lgophthlmos nd trophic dmges of the corne (Figs. 6.4, 6.4). Chemosis my occur before proptosis nd is invribly limited to the inferior plpebrl conjunctiv (Miller 1998) Retinl Hemorrhge In severe cses of venous diltion nd elevted intrvenous pressure, optic disc swelling nd retinl hemorrhges, cused by venous stsis nd impired retinl blood flow, with secondry ischemi or hypoxi, cn occur (Fig. 6.5) (see Cse Report I). These intrretinl hemorrhges cn be both, flme-shped (locted in the nerve fiber lyer) nd punctute (locted in the outer retinl lyers) (Miller 1998) nd cn be ssocited with centrl retinl vein occlusion (Kupersmith et l. 1996). De Keizer (2003) s well s Stiebel-Klish et l. (2002) found them in up 18% of their ptients Cornel Dmge Dehydrtion of the corne, usully pinful, cused by lgophthlmos, is the min cuse for corne irrittions in ptients with DCSFs. Therpeutic mngement of this exposure; kertopthy my be complicted by dysfunction of the fcil nerve with

99 90 6 Neuro-Ophthlmology in Durl Cvernous Sinus Fistuls (DCSFs) Fig Severe chemosis in misdignosed DCSF. Despite repeted imging studies including MRI nd CT, the correct dignosis in this 73-yer-old womn ws delyed for more thn 4 months. During this period, her differentil dignoses included conjunctivitis, mxillo-fcil tumor nd orbitl phlegmone. She underwent extrctions of nine (!) teeth, held responsible for her infectious process. The ptient presented with retroorbitl pin, exophthlmos, swelling nd redness of her right eye with significnt chemosis nd visul loss (IOP 23 mmhg) when she ws dmitted in July Such chemosis with rterilized conjunctivl nd episclerl veins my be indistinguishble from tht of direct CCF (see lso Cse Report III). Fig Retinl hemorrhges in DCSF. This 56-yer-old womn presented in October 2000 with bilterl proptosis nd eye-redness fter not being correctly dignosed for more thn 22 months. The fundoscopy shows venous diltion nd significnt opticl nerve swelling. Extensive flme-shped (superficil) nd some punctute (deep) intrretinl hemorrhges, probbly cused by centrl retinl vein occlusion. (see lso Cse Report I) presis of the orbiculr oculi muscle nd reduced lcrimtion. Progressive dmges of the cornel epithelium cn be initilly pinless but my led to locl infections, cornel bltions nd ulcertions nd beside locl hydrtion nd ntibiosis often temporry trsorrhphy (eyelids prtilly sewn together) becomes necessry. Due to vriform clinicl mnifesttions, considering differentil dignoses is essentil for optimizing therpy of ptients with prolpsed reddish eye bulb. In generl, bulb-compressing methods should be voided to minimize the risk of subconjunctivl bleedings in cse of n underlying rteriovenous fistul. Differentil dignoses include minly thyroid relted orbitopthy (most common cuse of unilterl nd bilterl proptosis in dults), neoplstic diseses (lymphom, primry nd secondry CNS tumors, in children prticulrly rhbdoid tumors nd srcoms), llergic rections, inflmmtions (virl/bcteril conjunctivitis, myositis), vsculr nd pseudotumors of the orbit s well s introrbitl bleedings (post-trumtic, prneoplstic) Orbitl Bruit The bruit (French word for noise) over the temporl bone or the orbit cn be subjective nd/or objective (usculttory) nd is usully synchronous with the hertbet. It my pper s buzzing, swishing or roring nd represents one clssicl symptom of high-flow CCFs. It is found in only 25% of ptients with DCSFs, minly in cses with posterior dringe (Hlbch et l. 1987). The bruit develops due to rteriovenous turbulences within the CS, which my rech the inner er orgn vi sound trnsmission through the skull. It my increse due to spontneous occlusion of fistul feeder or during physicl exercise or increse of blood pressure. Otherwise, the bruit my decrese or completely dispper following thrombosis, spontneous occlusion of fistul feeder or during mnul compression of the crotid rtery in the neck. Bruit, commonly considered benign symptom, cn become gret source of nnoynce preventing some ptients from sleeping (Mdsen 1970). I hve observed ptient with DAVF of the sigmoid sinus who ws unble to crry on his profession due to the distress. However, such severe discomfort cused by bruit is rre in DCSFs. A bruit my lso not be reported becuse it is not very intense, the ptient hs got used to

100 6.3 Other Neurologicl Symptoms 91 it or my not ssocite it with oculr symptoms (Miller 2007). Comprble (udible) bruits in the crniofcil region cn be found in ptients with rteriosclerotic stenoses of the crotid siphon, subclvin rtery or vsculr tumors. A congenitl or posttrumtic hypo- or plsi of the sphenoid bone my simulte the bruit of CCF through trnsmission of brin pulstions to the orbit. A bruit my lso be herd in rre cses of rised ICP or meningeoms (Miller 1998) Pulse-synchronous Pulstion of the Eyelid nd Bulb Birch-Hirschfeld (1930) ws the first to describe pulse-synchronous movements of the eye lid nd bulb in ptients with CCFs. They develop due to the rteriliztions of introrbitl veins, lso involve the bulb, nd re visible or cn be found by plption in bout 5% 20% of ptients with DCSF. Bilterl ssessment of oculr pulse mplitudes (OPA) using the pneumotonometer or Goldmnn tonometer differs in 93% of ptients with CCF by >1.6 mm, nd is relible non-invsive method for identifying CCF (Golnik nd Kulwin 1997; Golnik nd Miller 1992) Secondry Glucom nd Visul Loss After mnifesttion of orbitl symptoms, the chronic elevted introrbitl venous pressure my led in 20% of ptients with DCSF to blockge of Schlemm s cnl. This my subsequently led to gonioscopiclly detectble elevted introculr pressure (secondry glucom), retinl ischemi nd usully reversible loss of visul cuity. Mny uthors postulte tht the elevted episclerl venous pressure is responsible for secondry glucom, rrely for ppill edem, retinl bltions, centrl vein thrombosis nd hemorrhgic retinopthy (2%) (Phelps et l. 1982; Brke et l. 1991; Jorgensen nd Gutthoff 1985). In 11% of the ptients bilterl symptoms re found. In generl, the clinicl severity of visul loss correltes more with the venous dringe pttern thn with the volume nd flow velocity of AV shunt. Meyers et l. (2002) found diminished visul cuity is found in up to 31%. In ptients with rpid visul loss, neuropthy of the optic nerve nd occlusion of the superior ophthlmic vein re found, respectively, while distl stenosis merely contributes to clinicl improvement (Hlbch et l. 1992). Ptients with DCSF nd fulminnt glucomtous loss of vision need, beside endovsculr therpy, temporry locl β-blocker or Dimox (Acetzolmide) ppliction, or even more invsive ophthlmologic intervention (cnthotomy, gonioplsty) (Fiore et l. 1990). It cnnot be emphsized enough tht in ptients with unilterl glucom, DCSF must lwys be included in the differentil dignosis. Stiebel-Klish et l. (2002) hve provided the most detiled description of frequency of signs nd symptoms in lrger group (85) of ptients so fr. The uthors differentited crnil neuropthy ccordingly into sixth (34%), third (19%), fourth (5%) nerve plsies, fifth nerve dysfunction (2%) nd fcil nerve presis (1%). They found reltively frequent optic neuropthy (31%), vertigo in 5%, intrcerebrl hemorrhge in 2%, nd corticl venous infrct in 1% of their ptients. 6.3 Other nd Neurologicl Symptoms Intrcrnil hemorrhge cused by CSFs hs lredy been reported to occur by Sttler (1930), De Schweinitz nd Hollowy (1908) nd others in bout 3% of cses, most of which, however being cused by CSFs of trumtic origin. Hrding et l. (1984) reported on two ptients with DCSF, who experienced spontneous intrcerebrl hemorrhge within 18 months fter onset of their symptoms. Skum et l. (2006) reported recently cse where the hemorrhge developed contrlterl to the fistul side, mimicking hypertensive putminl bleeding. Kuwym et l. (1998) described ptient who presented with frontl subcorticl hemorrhge nd Nkhr et l. (1996) reported nother who developed temporo-prietl hemtom due to ipsilterl fistul. Although corticl or leptomeningel dringe cn be found in 31% of the ptients, intrcerebrl hemorrhge seem to occur less frequently thn in DAVF, in only 1.5% (Meyers et l. 2002). In generl, centrl nervous system symptoms or dysfunction re less frequent nd hve been observed in lrger ptient groups in only 7/85 cses with vertigo

101 92 6 Neuro-Ophthlmology in Durl Cvernous Sinus Fistuls (DCSFs) (5%), intrcerebrl hemorrhge (2%) nd corticl venous infrct (1%) (Stiebel et l. 2002). Cerebrl or cerebellr symptoms cn be cused by venous outflow restriction or venous hypertension. They re rre events nd occur less frequently thn in DAVFs nd re only observed, if corticl venous dringe is present. Iwski et l. (2006) hve recently reported DCSF complicted by pontine venous congestion. The uthors observed isolted sixth nerve plsy in 71-yer-old womn tht ws cused by brin stem edem due to n AV shunt with exclusive venous posterior dringe into SPS, cerebellr corticl veins nd inferior vermin vein. Only few more of such cses hve been reported so fr (Uchino et l. 1997; Kurt et l. 1999; Ki et l. 2004; Tkhshi et l. 1999), showing tht in fct corticl venous dringe is not lwys the cuse, but often leptomeningel retrogrde venous dringe of the AV shunt insted (Dvies et l. 1997). An unusul clinicl presenttion is posterior ischemic optic neuropthy (PION) s recently suggested by Hshimoto et l. (2005), who observed ptient with sudden unilterl vision loss fter n oculr motor disturbnce nd pulstile tinnitus. The rteriogrm reveled fistul which ws in prt supplied by recurrent meningel brnch rising from the ophthlmic rtery possibly cusing rteril stel. Other rre complictions of DCSF include mculr exudtive retinl detchment (Grg et l. 2006), bnorml choroidl circultion (Klein et l. 1978), myelopthy (Ohnishi et l. 2003), nd fcil nerve presis (Moster et l. 1988). 6.4 Differentil Dignosis Durl CSFs presenting with mild nd slowly progressing symptoms re often misdignosed in their erly stge (Miller 2007). Considering DCSF in the differentil dignoses of inflmmtory nd other orbitl diseses cn be crucil. In recent study, Stiebel-Klish et l. (2002) were ble to demonstrte good correltion between venous dringe pttern nd clinicl signs. Dringe into the nterior CS nd SOV ws well correlted with orbitl congestion, elevted IOP nd optic neuropthy. Dringe into the IPS ws well correlted with third nerve plsy, dringe into the SPS llowed prediction for CNS symptoms. In series only 10 ptients with bilterl signs hd true bilterl AV shunts. While four ptients hd bilterl eye signs without evidence of dringe into the SOVs, three hd unilterl congestion with bilterl shunts drining into both SOV. Iked et l. (2005) reported recently on ptient with prominent nterior dringe who presented with bsent orbito-oculr signs. By contrst, cses with dominnt posterior dringe my present not with the typicl white-eyed cvernous shunt s described by Acierno et l. (1995). I hve seen ptients tht, despite exclusive posterior dringe, presented with eye-redness (Fig. 6.3). The ngiogrphic demonstrtion of either nterior or posterior dringe lone will not lwys or not completely explin the symptoms (Fig. 6.6). Eqully or even more importnt is probbly the ssocited venous pressure. This pressure my, especilly in low-flow fistuls, not be significntly elevted nd only increses if venous outflow restriction strts to occur. Vrious pthologies should be considered differentil dignoses in clinicl prctice, including conjunctivitis, thyroid orbitopthy, orbitl pseudotumor, myositis, orbitl cellulites, episcleritis, meningeom or Tolos-Hunt syndrome nd llergic rections. (Brzis 1994; Grove 1984; Miller 2007; Newton 1970; Oestreicher 1995; Phelps 1982; Procope 1994). Pulsting exophthlmos cn lso be cused by sphenoid dysplsi in neurofibromtosis (Recklinghusen) or fter neurosurgicl removl of the orbitl roof. Dilted episclerl nd conjunctivl loops cn be observed in CS thrombosis, venous mlformtions, brin AVMs or DAVFs (see Figs. 7.48, 7.49). Other cuses include Grves disese (Lore et l. 2003), idiopthic elevted episclerl pressure, orbitl vein vrition, superior ven cv syndrome, pulmonry hypertension (Akdumn et l. 1996), scleritis with vortex vein blockge nd mlignnt endocrine exophthlmos (de Keizer 2003). If the fistul is smll nd thus not detectble by CT or MRI, ptient cn be misdignosed, leding not only to distress but lso to indequte therpeutic mesures nd flse tretment effects (Figs. 6.4 nd 6.7). Theudin et l. (2006) reported just recently dely in dignosis of up to 22 months; similr to my own experience (Fig. 6.5., Cse Report I), underlining the dignostic dilemm in ptients with smll AV-shunts, not detectble by CT or MRI. Selective ctheter ngiogrphy is indispensble in these cses nd cn be performed by n experienced neurordiologist with very low morbidity (Willinsky et l. 2003).

102 6.4 Differentil Dignosis 93 b c d e Fig. 6.6 e. White-eyed cvernous shunt. This 75-yerold womn presented in Jnury 2003 with just miniml diltions nd tortuosities of some episclerl veins ( b). Fundoscopy shows only minor diltion of retinl veins (c d). In mny, but not ll cses, the type of dringe (nterior or posterior) my explin the clinicl presenttion. This fistul drins posteriorly, but only into the SPS nd connected supr- nd infrtentoril leptomeningel veins (dotted rrows), not into the IPS (e). Like the ptient in Fig. 6.3., this womn hd no neurologicl deficits or other clinicl signs tht would indicte such dringe pttern. Fistul occlusion ws chieved using TVO (see lso Figs. 7.45, 8.3., 10.4)

103 94 6 Neuro-Ophthlmology in Durl Cvernous Sinus Fistuls (DCSFs) b Fig. 6.7, b. Overlooked DCSF in ptient with eye-redness. Eye-redness nd chemosis with diltion, tortuosity nd chrcteristic corksrew ppernce of episclerl veins in 54-yer-old ptient who presented fter being incorrectly dignosed for more thn 3 months. Differentil dignoses included endocrine ophthlmopthy, inflmmtion, immunopthy nd orbitl tumor/lymphom nd pseudotumor cerebri, for which she ws treted with corticosteroids nd eventully even underwent biopsy. The ptient ws seen by four different specilists: Endocrinologist, rheumtologist, neurologist nd n ophthlmologist. She underwent negtive CT scn nd MRI exm before the definite dignosis could be mde using cerebrl ngiogrm. b shows tiny AV shunt (sterisk), supplied by the ILT with sluggish flow in the SOV tht is not notbly enlrged (rrow) nd ppers prtilly occluded, nd thus my not pper prominent on CT or MRI (Courtesy: R. Klucznik, Houston). This nd other similr cses (Cse Reports I nd III), with significnt delys in correct dignosis nd proper tretment, still occur, emphsizing the key role of intrrteril DSA for timely dignosis in ptients with smll AV shunts, not detectble by MRI or CT In summry, the signs nd symptoms in ptients with low-flow DCSF re in principle similr to those with direct high-flow CCF, but milder nd less progressive. In the initil stge of the disese, nonspecific signs such s retro orbitl hedches, mild conjunctivl injection or isolted diplopi my occur. Consequently, the disese my be overlooked nd cn be mistken s endocrine orbitopthy, conjunctivitis or oculr myositis. Neglecting DCSF in the clinicl differentil dignoses my cuse progression of the disese with serious deteriortion of the ptient s symptoms including the risk of complete vision loss. References Acierno MD, Trobe JD, Cornblth WT, Gebrski SS (1995) Pinful oculomotor plsy cused by posterior-drining durl crotid cvernous fistuls. Arch Ophthlmol 113: Akdumn L, Del Priore LV, Kpln HJ, Meredith T (1996) Uvel effusion syndrome ssocited with primry pulmonry hypertension nd vomiting. Am J Ophthlmol 121: Brke RM, Yoshizumi MO, Hepler RS, Kruss HR, Jbour BA (1991) Spontneous durl crotid-cvernous fistul with centrl retinl vein occlusion nd iris neovsculriztion. Ann Ophthlmol 23:11 17 Benndorf, G, et l. (1999) Occlusion of the inferior petrosl sinus (IPS) for endovsculr tretment of longstnding trumtic crotid cvernous sinus fistul (CCF). In Annul Meeting, Europen Society of Neurordiology. Vienn. Birch-Hirschfeld A (1930) Kurzes Hndbuch der Ophthlmologie, vol 1. Springer, Berlin Heidelberg New York Brzis PW, et l. (1994) Low flow durl rteriovenous shunt: nother cuse of sinister Tolos-Hunt syndrome. Hedche 34: Dvies M, Sleh J, Ter Brugge K, Willinsky R, Wllce MC (1997) The nturl history nd mngement of intrcrnil durl rteriovenous fistule. Intervent Neurordiol 3 de Keizer RJ (1979) Spontneous crotico-cvernous fistuls. The importnce of the typicl limbl vsculr loops for the dignosis, the recognition of glucom nd the uses of conservtive therpy in this condition. Doc Ophthlmol 46: de Keizer R (2003) Crotid-cvernous nd orbitl rteriovenous fistuls: oculr fetures, dignostic nd hemody-

104 References 95 nmic considertions in reltion to visul impirment nd morbidity. Orbit 22: De Schweinitz G, Hollowy T (1908) Pulsting exophthlmos: its etiology, symptomtology, pthogenesis nd tretment. Sunders, Phildelphi Eggenberger, E, et l. (200) A bruitl hedche nd double vision. Surv Ophthlmol. 45(2): Fiore PM, Ltin MA, Shingleton BJ, Rizzo JF, Ebert E, Bellows AR (1990) The durl shunt syndrome. I. Mngement of glucom [see comments]. Ophthlmology 97:56 62 Grg SJ, Regillo CD, Aggrwl S, Bilyk JR, Svino PJ (2006) Mculr exudtive retinl detchment in ptient with durl cvernous sinus fistul. Arch Ophthlmol 124: Golnik C, Kulwin D (1997) CCF: neuro-ophthlmolgic findings. In: Tomsick TA (ed) Crotid cvernous fistul. Digitl Eductionl Publishing, Phildelphi, pp Golnik KC, Miller NR (1992) Dignosis of cvernous sinus rteriovenous fistul by mesurement of oculr pulse mplitude [see comments]. Ophthlmology 99: Grove AS Jr (1984) The durl shunt syndrome. Pthophysiology nd clinicl course. 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Willims nd Wilkins, Bltimore, pp Miller, NR (2007) Dignosis nd mngement of durl crotid-cvernous sinus fistuls. Neurosurg Focus. 23(5): p. E13 Miychi S, Negoro M, Hnd T, Sugit K (1993) Durl crotid cvernous sinus fistul presenting s isolted oculomotor nerve plsy. Surg Neurol 39: Moster ML, Sergott RC, Grossmn RI (1988) Durl crotidcvernous sinus vsculr mlformtion with fcil nerve presis. Cn J Ophthlmol 23:27 29 Nkhr I, Tki W, Muro K, Ohkt N, Mtsumoto K, Isk F, Kikuchi H (1996) Endovsculr tretment of cvernous durl AVFs (spontneous CCFs): results in 50

105 96 6 Neuro-Ophthlmology in Durl Cvernous Sinus Fistuls (DCSFs) ptients. In: Tki W, Picrd L, Kikuchi H (eds) Advnces in interventionl neurordiology. Elsevier Science, Amsterdm, pp Newton TH, Hoyt WF (1970) Durl rteriovenous shunts in the region of the cvernous sinus. Neurordiology, :71 81 Oestreicher, JH, Frueh BR (1995) Crotid-cvernous fistul mimicking Grves eye disese. Ophthl Plst Reconstr Surg,. 11(4): Ohnishi H, Deguchi J, Ymd M, Kuroiw T (2003) Cvernous rteriovenous fistul presenting myelopthy: cse report. No Shinkei Gek 31: Ott D, Bien S, Krszni L (1993) Emboliztion of tentoril durl rterio-venous fistul presenting s typicl trigeminl neurlgi. Hedche 33: Plestine AG, Younge BR, Piepgrs DG (1981) Visul prognosis in crotid-cvernous fistul. Arch Ophthlmol 99: Phelps CD, Thompson HS, Ossoinig KC (1982) The dignosis nd prognosis of typicl crotid-cvernous fistul (redeyed shunt syndrome). Am J Ophthlmol 93: Procope, JA, et l. (1994) Durl cvernous sinus fistul: n unusul presenttion. J Ntl Med Assoc, (5): Rizzo M, Bosch EP, Gross CE (1982) Trigeminl sensory neuropthy due to durl externl crotid cvernous sinus fistul. Neurology 32:89 91 Skum I, Tkhshi S, Tomur N, Kinouchi H (2006) Durl rteriovenous fistuls of the cvernous sinus with onset of intrcerebrl hemorrhge mimicking hypertensive putminl hemorrhge. Act Neurochir (Wien) 148: Sttler H (1930) Pulsierender Exophthlmus. In: Grefe A, Semisch T (eds) Hndbuch der Gesmten Augenheilkunde. Springer, Berlin Heidelberg New York, pp Stiebel-Klish H, Setton A, Nimii Y, Klish Y, Hrtmn J, Hun Br-On R, Berenstein A, Kupersmith MJ (2002) Cvernous sinus durl rteriovenous mlformtions: ptterns of venous dringe re relted to clinicl signs nd symptoms. Ophthlmology 109: Suh DC, et l. (2005) New concept in cvernous sinus durl rteriovenous fistul: correltion with presenting symptom nd venous dringe ptterns. Stroke 36: Tkhshi S, Tomur N, Wtri J, Mizoi K, Mnbe H (1999) Durl rteriovenous fistul of the cvernous sinus with venous congestion of the brin stem: report of two cses. AJNR Am J Neurordiol 20: Theudin M, Sint-Murice JP, Chpot R, Vhedi K, Mzighi M, Vignl C, Sliou G, Stpf C, Bousser MG, Houdrt E (2006) Dignosis nd tretment of durl crotid-cvernous fistule: consecutive series of 27 ptients. J Neurol Neurosurg Psychitry Uchino A, Kto A, Kurod Y, Shimokw S, Kudo S (1997) Pontine venous congestion cused by durl crotid-cvernous fistul: report of two cses. Eur Rdiol 7: Vinuel F, Fox AJ, Debrun GM, Peerless SJ, Drke CG (1984) Spontneous crotid-cvernous fistuls: clinicl, rdiologicl, nd therpeutic considertions. Experience with 20 cses. J Neurosurg 60: Voigt K, Suer M, Dichgns J (1971) Spontneous occlusion of bilterl croticocvernous fistul studied by seril ngiogrphy. Neurordiology 2:

106 Rdiologicl Dignosis of DCSFs 7 CONTENTS 7.1 Non-invsive Imging Techniques CT nd MRI Doppler nd Crotid Duplex Sonogrphy Intr-rteril Digitl Subtrction Angiogrphy (DSA) Introduction Technique Angiogrphic Protocol for DCSFs Angiogrphic Antomy of the Cvernous Sinus Flt Detector Technology in Neurongiogrphy Rottionl Angiogrphy nd 3D-DSA Dul Volume Technique (DVT) Angiogrphic Computed Tomogrphy (ACT), DynCT (Siemens), C-rm Flt Detector CT (FD-CT), Flt Pnel CT (FP-CT) or Cone Bem CT Imge Post-Processing D Studies of the Cvernous Sinus Region 148 References Non-invsive Imging Techniques CT nd MRI (Figs ) The clinicl presenttion of ptients with chrcteristic neuroophthlmic symptoms does not usully require invsive imging techniques, nd correct dignosis cn be mde with certinty using CT or MRI techniques. As outlined in the previous chpter, non-invsive imging my fil in ptients with low-flow AV shunts, nd ptient with typicl symptoms my be misdignosed over time. If there is clinicl suspicion of n inflmmtory or tumorous process in the orbitl or peri-orbitl region, computed tomogrphy (CT) or mgnetic resonnce imging (MRI) is commonly performed. In the cse of DCSF, the imge my show dilted or thrombosed vein, indicting n underlying vsculr pthology. An exophthlmos or proptosis cn often be dignosed using routine CT scn (Figs. 7.1 nd 7.2). However, only when the AV shunting volume is lrge enough will the CS become visible s n enhncing spce occupying lesion. Ohtsuk nd Hshimoto (1999) performed seril enhnced computed tomogrphy (DE-CT) scnning of the cvernous sinus nd provided direct evidence of pthologicl shunting from the crotid rtery to the cvernous sinus. By scnning seril xil imges round the sell turcic t intervls of 3 s the uthors were ble to obtin n erly filling of the CS in direct (two) s well s in indirect (five) fistuls. This technique ws lso found to be useful in reveling CS thrombosis by non-filling even in lte venous phses. A differentition between Types A B nd C fistuls ws possible, bsed on the delyed stining of the CS when only ECA brnches supplied the fistul.

107 98 7 Rdiologicl Dignosis of DCSFs b c Fig. 7.1 c. Computed tomogrphy findings in two ptients with DCSFs. Significnt exophthlmos nd enlrged SOV (b) on the right side. c Enhncement of the right (not notbly enlrged) SOV fter contrst dministrtion, indicting n AV shunt t the CS in nother ptient. Note: In cses with posterior dringe, CT of the orbit my pper norml b c d Fig. 7.2 d. Computed tomogrphy in ptient with DCSF.,b Axil views shows mild exophthlmos nd n enlrged SOV on the left side. c,d Coronl nd sgittl reformtted views show the enlrged SOV behind the eyebll. (Courtesy: A. Biondi, Pris)

108 7.1 Non-invsive Imging Techniques 99 A focl bulging or diffuse distension of the CS on contrst enhnced CTs hs been detected in 50% 64% of the cses (Ahmdi et l. 1983; Uchino et l. 1992). The SOV cn be enlrged on the fistul site in 86% 100% on post-contrst CTs nd in 75% 100% on spin echo MR imges (Ahmdi et l. 1983; Uchino et l. 1992; Hirbuki et l. 1992; Komiym et l. 1990; Elster et l. 1991). If corticl venous dringe is present, those enlrged veins my become visible on xil CT. Wtnbe et l. (1990) described cse of Type D fistul with considerbly enlrged veins visulized on contrst enhnced CT. In ddition, SPECT ws performed showing reduced regionl cerebrl perfusion in this re, cused by elevted trnsvenous pressure. D Angelo et l. (1988) described cse with Type A fistul nd dilted veins in the temporl region, which were identified s Sylvin veins. Teng et l. (1991) reported brin stem edem in two ptients with Type D fistuls tht becme visible on CT fter occlusion of the norml venous dringe, probbly triggering the development of corticl venous dringe. In both cses complete disppernce of the edem ws documented. Uchini et l. (1997) reported two ptients with pontine venous congestion due to DCSFs. Brin stem edem due to DCSFs hs lso been described by Tkhshi et l. (1999) in two ptients, in which fter occlusion of the fistul, reversl of the edem on MRI becme evident. Edemtous chnges due to venous hypertension cn lso be seen in both hemispheres being minly limited to the white mtter, like vsogenic edem ( Cornelius 1997). When n intrcrnil hemorrhge occurs s compliction of CCF, CT is the preferred dignostic tool (D Angelo et l. 1988). This type of compliction is minly seen in Type A fistuls, but only rrely observed in DCSFs (Stoh et l. 2001), even though corticl venous dringe is reltively frequently present (see Tble 5.3). In most cses the bleeding occurs djcent to the dilted veins. MRI is superior to CT in showing discrete chnges due to AV shunts of the CS, becuse it my demonstrte not only the enlrged SOV but lso miniml proptosis nd extroculr thickening of the muscles. Sto et l. (1997) recently reported on flow voids shown in eight of 10 ptients with Type D fistuls using spin echo sequences. In prticulr MRA cn be helpful in low-flow conditions, s often present in DCSFs. MRI findings cn be discrete on MIPs nd rw dt (source imges) cn be useful for nlysis (Cornelius 1997; Acierno et l. 1995; Chen JC et l. 1992; Dietz et l. 1994) (Figs. 7.3, 7.6 nd 7.8). MRA, s well s conventionl spin echo sequences, cn demonstrte bnorml corticl dringe (Chen JC et l. 1992), s seen in Figure 7.5b nd 7.8. Under certin circumstnces MRA is prticulrly helpful for erly detection of CCF with posterior dringe (Fig. 7.8), lso clled white eyed shunt syndrome (Acierno et l. 1995). These ptients present with hedches nd pinful oculopresis but without orbito-oculr congestion, which my esily led to wrong dignosis of, e.g. Tolos Hunt syndrome or pinful ophthlmoplegi. Conventionl spin echo sequences cn be norml in these ptients; however, MRA would be dignostic in showing bnorml vessels rising from the posterior CS (Figs. 7.5 nd 7.6). The CS itself cn become visible even due to norml venous flow cusing flow voids in TOF-MRAs s hs been observed by Cornelius (1997) in eight of 50 ptients. Severl uthors hve described flow voids within the CS on spin echo MR imges in ptients with CSFs (Uchino et l. 1992; Hirbuki et l. 1992; Komiym et l. 1990). Hiri et l. (1998) sw flow voids in the CS less frequently in their ptients nd found in 3% flse positive results, emphsizing the difficulty to differentite norml venous flow in the CS from bnorml flow voids cused by n AV shunt bsed on spin echo MR imges. Another MR finding is the flow-void of the intercvernous sinus in contrst enhnced T1-weighted imges (Figs. 7.3, 7.4, 7.6, 7.7). Sergott et l. (1987) recommended MRI s the initil exm in ptients with known rteriovenous shunts nd clinicl deteriortion. The uthors observed three ptients with prdoxicl worsening of clinicl symptoms due to thrombosis of the SOV, which ws reveled by incresed signl intensity in T1-weighted imges. According to Cornelius (1997), intr-rteril DSA is not required in ptients with clinicl worsening if MRI shows thrombosed SOV nd n improvement under conservtive mngement. On the contrry, Goldberg et l. (1996) observed tht MRI led to dignosis in only 5 of 10 ptients (50%). Schuknecht et l. (1998) were ble to show tht high-resolution contrst enhnced CT nd MRI exm cn demonstrte not only thrombi within the CS, but lso in its tributries such s the SOV, the SPPS nd even the IPS. MRI my help to differentite CSF from mlignnt tumors, vsculitic processes nd intrcrnil AV mlformtions. If thrombosis occurs, the signl ppers s white hyperdense spot on the T1-

109 100 7 Rdiologicl Dignosis of DCSFs b c d e f Fig. 7.3 f. MRI in ptient with DCSF nd posterior dringe. T1-weighted coronl imge shows flow voids in the left CS. b TOF MRA shows both CSs nd the posterior ICS in MIP views; no nterior dringe towrds SOV is pprent. c e MRA source imges revel bright signl spots within the posterior CS on both sides nd within the ICS (rrows). f DSA, right ICA shows intense opcifiction of the posterior CS (sterisk) nd dominnt posterior dringe vi the IPS (double rrow). (Courtesy: G. Gl, Odense)

110 7.1 Non-invsive Imging Techniques 101 b c d e Fig. 7.4 e. MRI in two ptients with DCSF. Axil TSE T2- weighted imge showing flow void in the left CS (rrow). b Axil IR T1-imge showing the enlrged SOV (rrow). c ICA injection lterl shows the AV shunt t the posterior CS drining nteriorly in the SOV (rrow). d T2-weighted coronl imge shows numerous flow voids in the left CS. e DSA, right ICA injection fills the ICS nd the left CS (sterisks) with dringe into corticl veins (rrow) nd the IPS/IJV. (Courtesy: A. Biondi, Pris)

111 102 7 Rdiologicl Dignosis of DCSFs b c d Fig. 7.5 d. MRA (TOF) in two ptients with DCSFs. Axil view of ptient with bilterl symptoms: incresed signl intensity in the left posterior CS (sterisk) indicting smll low-flow fistul without cler demonstrtion of drining vein (Courtesy: R. Prsche, Neuruppin). b Axil view of ptient with left-sided symptoms: Lrge AV shunt cusing incresed signl intensity (due to higher flow) in the nterior CS (sterisk), the SOV (rrow) nd the superficil middle cerebrl vein (corticl dringe, short rrow) (Courtesy: B. Snder, Berlin). c If the AV shunt itself is not evident, MRA my show indirect signs, such s retrogrde filling of the left sigmoid sinus, s in this ptient cused by stenosis t the level of the JB. d DSA shows filling of left CS, SOV, IPS nd sigmoid sinus (rrows) (Courtesy: Dr. G. Gl, Odense) weighted imge (de Keizer 2003). The visuliztion of drining veins my require using both phse contrst techniques (3D PC MRA) for demonstrting the dilted SOV nd ssocited reflux, nd 3D TOF MRA for demonstrting the IPS (Ikw et l. 1996). If the SOV is not the drining vein it my not be demonstrted with 3D PC MRA. The IPS is usully shown better on TOF MRA, becuse it runs in superior inferior direction cusing stronger time of flight effects. Hiri et l. (1998) hve compred the vlue of fst imging with stedy stte precession (FISP) to

112 7.1 Non-invsive Imging Techniques 103 b c d e Fig. 7.6 e. MRI nd MRA in two ptients with DCSFs. T1-weighted imge, coronl plne shows dilted vessel within the right CS (rrow) with flow voids, indicting AV-shunting. b Source imge of the MRA (TOF) shows higher signl intensity (rrows) djcent to the right ICA. c e MRA in vrious projections revels the cvernous sinus AV shunt, involving both CSs (sterisks) s well s the intercvernous sinus (rrows). Note: The exct type of the fistul (Type A D), detils of the rteril ngiorchitecture or venous dringe pttern cn often not be evluted. (Courtesy: A. Cmpi, Miln) contrst enhnced CT nd spin echo MR imging nd found it superior in the dignosis of CCF. Their group of 17 ptients included 14 DCSFs in which hyperintensity of the CS ws noted in most cses (11/14). In DCSFs with very slow flow this hyperintensity cn be missed, leding to flse negtive results nd necessitting creful serch for other findings relted to the venous dringe. In highly vsculrized DCSFs, multiple hyperintensive curviliner structures or spots djcent to or within the CS were seen, likely corresponding to durl feeders. Becuse these findings were not observed in direct

113 104 7 Rdiologicl Dignosis of DCSFs b c d e Fig. 7.7 e. MRI/MRA in DCSF. c T2-weighted imges. Axil plne through the CS: Vgue flow void on the left side (sterisk). b Coronl view through the mid orbit shows flow void cused by the SOV (rrow). c Mild exophthlmos nd slightly enlrged SOV ( rrow). d Axil TOF revels the fistul t the left CS drining into the SOV (inset: oblique view). e DSA with simultneous rteril nd venous injection for better understnding of the ntomy confirms DCSF (sterisks), drining into the SOV (rrow). Note, there is no posterior dringe (inset), even though both IPSs re widely open s demonstrted by the jugulr phlebogrm. (Courtesy: A. Biondi, Pris)

114 7.1 Non-invsive Imging Techniques 105 b Fig. 7.8,b. MRI/MRA in DCSF with nterior nd posterior (leptomeningel) dringe. MRA TOF A, bnorml signl in the left CS (sterisk) nd scrcely in the left SOV (rrow) s well s posterior to the CS, indicting possible leptomeningel venous dringe (short rrow), which ws confirmed by DSA. b MRA source imge revels the bnorml signl in the left CS (sterisks). (Courtesy: U. Schweiger, Berlin) CCFs, they my help to differentite direct from indirect fistuls. 3D FISP imges showed posterior venous dringe, but were not helpful in detecting corticl dringe, which is n importnt detil not to be missed. Becuse relying on MRI nd 3D TOF MIP imges lone my led to underdignosis of indirect CSFs, MRA source imges become quite vluble (Figs. 7.3, 7.6, 7.8 nd 7.9). Tsi YF et l. (2004) recently encountered dilemms in reviewing MRI flow voids nd identified them in only five of eight ptients with DCSFs. The uthors detected n engorged CS only in four cses nd swollen extroculr muscles in none. Flow rtifcts resulting from pulstion of the cvernous ICA my corrupt CS detils nd CSF pulstion my result in flow voids in the prepontine cistern mimicking enlrged bnorml vessels. Air in the sphenoid sinus my cuse susceptibility rtifcts or prtil volume effects. Becuse MIP reconstruction my cuse vsculr distortion they need to be reviewed crefully. Relince on MIPs lone my led to misdignosis in 50% of the cses. On the other hnd, reding of the source imges of 3D-TOF MRA llowed the correct dignosis in ll eight cses. Therefore, in order not to overlook smll AV shunts, creful evluting of MRA source imges should be included in ll doubtful cses. Nevertheless, even with improved technology, MRI nd MRA cnnot replce high qulity DSA for differentiting direct Type A nd indirect Type D fistuls with certinty (Tsi YF et l. 2004). Dignostic sensitivity of MRI cn be enhnced with contrst nd mgnetiztion prepred rpid grdient echo sequences (MP-RAGE), llowing for better ssessment of retrogrde venous dringe thn T1-weighted SE imging (Kitjim et l. 2005). Kwon et l. (2005b) found direct fistul visuliztion in 75% 86% of the 27 DAVF (11 DCSFs), lthough the reviewers were not blinded to ngiogrphy in this study. The uthors suggest looking for ny suspicious flow void cluster round durl sinus. Chen CC et l. (2005) hve recently compred the utility of CTA nd MRA source imges in the dignostic of 53 direct CCFs. They found CTA s useful s DSA nd superior to MRA in ccurtely loclizing the fistulous connection, in prticulr when it ws locted in segment four ccording to Debruns clssifiction (Debrun et l. 1981). Finlly, CT nd MRI cn be used to rule out complictions of EVT, for documenttion of coil msses or liquid embolic gents within the CS, or to detect residul/recurrent AV shunting (Figs. 7.9 nd 7.10).

115 106 7 Rdiologicl Dignosis of DCSFs b Fig. 7.9,b. MRA source imges in DCSF pre nd post emboliztion. Contrst enhnced T1-weighted imges pre- nd post trnsvenous occlusion of bilterl DCSF. The drk res in b correspond to the bright res in both CSs in (sterisks), confirming tht pltinum coils hve been pcked in the previously AV shunting comprtments. There is no signl bnormlity tht would indicte residul shunt, suggesting tht MRA could be used for non-invsive FU. Note, however, tht miniml AV shunt cn be missed; to rule out smll residul fistul with certinty, intr-rteril DSA remins indispensble. (Courtesy: G. Gl, Odense) b Fig. 7.10,b. Computed tomogrphy in two DCSF ptients post emboliztion. Post-emboliztion CT revels the positioning of the coils within the right CS. b Follow-up CT fter ptient presented with trnsient deficit fter TVO. The smll hyperdense re in the left frontl lobe (rrow) is likely due to repeted contrst injections during endovsculr tretment. Both indictions for cross-sectionl imging hve become clinicl pplictions for DynCT (see below) Doppler nd Crotid Duplex Sonogrphy (Fig. 7.11) Ultr-sonogrphy represents cost-effective, noninvsive method for the study of introrbitl hemodynmic prmeters in ptients with CSFs. However, ultr-sonogrphy is not suitble to visulize the complex venous ntomy of the cvernous sinus, or to rule out corticl or leptomeningel venous dringe (Belden et l. 1995; Flhrty et l. 1991); therefore, its vlue for tretment plnning is limited.

116 7.1 Non-invsive Imging Techniques 107 b c Fig c. Color-Doppler ultrsound in DCSF: Flow reversl in the superior ophthlmic vein. A 39 yer-old mn with durl AV shunt t the posterior CS cusing orbitl signs on the right side before emboliztion. Arteriovenous flow directed towrds the probe, depth 4.5 cm. Totl flow: 234 ml/min. b Mesurement of the inferior ophthlmic vein lso shows rterio-venous shunting flow, directed towrds the probe. c Superior ophthlmic vein fter successful emboliztion: Normliztion of blood flow direction (Courtesy: R. de Keizer, Leiden)

117 108 7 Rdiologicl Dignosis of DCSFs In some studies, Doppler sonogrphy hs shown vlue for dignosis nd follow-up in ptients with CSF (Belden et l. 1995; Flhrty et l. 1991; Erickson et l. 1989; Munk et l. 1992). It llows for the ssessment of direction nd velocity of blood flow, s well s the differentition of typicl venous flow pttern from n rterilized vein with chrcteristic bi-phsic flow (Erickson et l. 1989). De Keizer (1986) performed Doppler flow velocity mesurements in 35 ptients with direct nd indirect fistuls (14 trumtic, 21 spontneous) nd found specific flow pttern in 100% of direct nd in 80% of the indirect communictions. He recorded his mesurements s hemtotchogrms (HTGs) nd found normliztion of flow pttern in ptients with direct fistuls fter emboliztion. In more recent rticle (de Keizer 2003) he points to the difficulty of seprting the rteril flow in the suprtrochler rtery from the bnorml rterilized flow velocities nd pttern in the ophthlmic veins. When the flow velocity in the ICA ws dditionlly found to be bnorml, direct fistul could be identified in ll cses. Color Doppler methods re helpful in differentiting rteril from venous flow. De Keizer lso recommends the use of Doppler mesurements for monitoring conservtive tretment using mnul compression. Lin et l. (1994) suggested the ppliction of duplex crotid sonogrphy (DCS) for hemodynmic clssifiction of CSFs (see Chp. 4) with specil emphsis on the resistnce index (RI) nd the flow volume, bsed on 14 cses. The uthors suggest the use of sonogrphy for screening nd follow-up becuse DCS cnnot ccurtely identify Type B fistuls nd is limited in its differentition between Types C nd D fistuls for which ngiogrphy remins indispensble. Chiou et l. (1998) were ble to verify the complete oblitertion of crotid cvernous fistuls in 13 ptients (10 posttrumtic nd three spontneous) with color Doppler ultrsonogrphy. The uthors found spiculted wveform with turbulent flow pttern in most of their direct (Type A) fistuls, while ptients with indirect fistuls showed low-resistive rteril pulstile pttern. In these six ptients with DCSFs, rdiosurgery ws performed nd cure ws documented by sonogrphy, resonble pproch when repeted ngiogrphy cn be spred. Nevertheless, for finl documenttion of ntomicl cure, ngiogrphy ws performed. Arning et l. (2006) studied 17 ptients with DAVFs nd were ble to detect AV shunting lesions in 100% if the ECA ws exmined. In contrst to brin AVMs tht re detectble only in cses with lrge shunt volume, most DAVFs cn be dignosed becuse of their supply by ECA brnches tht lose their chrcteristic flow pttern s resistnce vessels. Assessment of venous dringe pttern, however, is difficult if not impossible nd DSA s n initil dignostic tool remins necessry. Tsi LK et l. (2005) studied similr series of ptients with DAVFs, one group undergoing endovsculr tretment nd nother undergoing only clinicl nd sonogrphic follow-up. The uthors found good correltion between the increse of the RI nd the effectiveness of the tretment in DAVF locted t mjor sinuses, but not t the CS. This finding ws in greement with previous study of the sme group (Tsi LK et l. 2004) in which the sensitivity of using the ECA-RI ws only 54% for cvernous sinus fistuls while it ws 86% for non-cvernous sinus AVFs. This discrepncy is likely explined by the reltively smll AV shunting volume in most DCSFs thus hving rther little impct on the flow in the ECA. Therefore, it ppers dvisble to combine DCS with Doppler flow imging of the superior ophthlmic veins (Chen YW et l. 2000). In ptients with solely posterior dringe, it might be impossible to depict n bnorml Doppler flow pttern in the SOV, thus leving ngiogrphy s lst resort for correct dignosis. In summry, modern cross-sectionl imging such s CT nd MRI provide correct dignosis in mny cses or cn t lest rise the suspicion of CSF. The combintion of trnscrnil nd trnsorbitl Doppler sonogrphy nd crotid duplex sonogrphy further increses the sensitivity of non-invsive imging. Sonogrphy provides informtion on blood flow, while CTA nd MRI help to delinete the ngiomorphology of the rteriovenous communiction. The venous dringe pttern, representing the min morphologicl feture of DCSF, cusing minly the clinicl symptoms nd being often the potentil endovsculr pproch, cn often not be imged to stisfctory degree. Low-flow fistuls with smll or prtilly thrombosed SOVs my be completely missed. Therefore, not only ptients with uncler symptoms, but ll ptients with DCSFs should eventully undergo n i.. (intr-rteril) DSA t lest once during the course of the disese, regrdless of the plnned therpeutic mngement.

118 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) Intr-rteril Digitl Subtrction Angiogrphy (DSA) Introduction It ws Egs Moniz, Portuguese neurologist, who on July 7, 1927, presented revolutionry dignostic method to the Societé de Neurologie de Pris (Moniz 1927). His L Encephlogrphie rterielle: son importnce dns l locliztion des tumeurs cerebrles ws the birth of novel wy to detect intrcrnil tumors by injecting contrst into the surgiclly exposed cervicl crotid rtery. By 1931, Moniz ws ble to perform 180 rteriogrms in this wy nd described rteril, cpillry nd venous ngiogrphic phses 3 yers lter.. Cerebrl ngiogrphy ws further developed by numerous investigtors mong which Loehr, Lim, Bergstrnd, Olivecrno, Toennis, Tkhshi, Wolff nd Kryenbuehl (Loehr 1933; Lim 1935; Bergstrnd et l. 1936; Wolff nd Schltenbrnd 1939; Kryenbuehl 1941) provided significnt contributions. Dyes (1938) suggested bi-plne rdiogrphy. In 1944 Holm emphsized the dvntges of cinerdiogrphy nd sequentil imging becme possible thnks to Erik Lysholm (Lysholm 1931). The surgicl exposure of the crotid rtery initilly used ws soon followed by percutneous techniques in 1936 nd 1940 ( Tkhshi 1940). To replce Thorotrst, iodide contrst medi were introduced in 1939 nd were continuously improved over the following yers. Interestingly enough, lthough rteriogrphy ws rpidly ccepted in Europe, it ws not until the studies by List et l. (1945) nd Ecker (1951) ppered tht this dignostic technique gined similr cceptnce in the United Sttes (Kryenbühl nd Ysrgil 1979). The introduction of Seldinger s technique ( Seldinger 1953) simplified nd fcilitted further the use of percutneous needle plcement nd ctheteriztion techniques. Development of subtrction techniques ( Ziesdses des Plntes 1934, 1935, 1961) nd ngiogrphic mgnifiction (Leeds et l. 1968; Wende nd Schindler 1970) were technicl milestones in the history of modern cerebrl ngiogrphy. These were complemented by new computerized technology, digitl subtrction ngiogrphy (DSA) tht ws employed in the lte 1970s to re-explore intrvenous (i.v.) ngiogrphy. At the RSNA in 1980, three compnies introduced DSA equipment nd by 1983 more thn 30 mnufcturers sold X-ry systems cpble of digitl subtrction. Becuse of its limittions, i.v. DSA ws soon replced by intr-rteril (i..) DSA tht permitted the use of smller ctheters nd smller mounts of iodine contrst nd hs persisted until tody. The introduction of computerized tomogrphy (CT) in 1973 represented the beginning of new er in dignostic neurordiology cross-sectionl imging of soft tissue structures such s the brin prenchym nd the ventriculr system (so-clled low-contrst imging). Numerous erly predictions stted tht, becuse of its invsiveness nd the ssocited compliction rte, cerebrl ngiogrphy ws soon going to dispper from the spectrum of dignostic tools. However, due to remrkble technologicl improvements nd reduced risks, it not only persisted over the lst 20 yers, it even went through renissnce in the 1990s when mnufcturers strted investing in its further development. No longer just s dignostic modlity, but more miniml invsive mens to gin ccess for endovsculr techniques, it becme one of the key tools for modern imge-guided therpy in interventionl crdiology nd rdiology. High-resolution intr-rteril DSA currently plys mjor role in the mngement of ptients with vsculr diseses of the CNS nd other res of the humn body Technique Due to the lrge mount of ntomic, pthologic nd functionl informtion provided, i.. DSA hs remined the gold stndrd for dignostic nd tretment plnning of intrcrnil AV-shunting lesions. As emphsized in the previous section, the use of i.. DSA is essentil for the timely dignosis of smll, low-flow AV shunts. Modern ngiogrphic systems relibly provide high qulity visuliztion of the smllest vsculture in the CS region, fcilitted by the use of biplne cquisitions, nd not ffected by rtifcts. Frme rtes up to 15 frmes re currently possible, llowing temporl resolution not obtinble with CT or MRI. This resolution is of prticulr importnce in high-flow durl or direct AV shunts when identifying the dominnt supply or the exct site of the fistul (Figs nd 7.49) is crucil.

119 110 7 Rdiologicl Dignosis of DCSFs High-resolution monitors in the control room s well s in the ngiosuite re required. Intr-rteril ngiogrms re obtined using 4-(uthor s preference) or 5-F dignostic ctheters, usully with HH1 configurtion, llowing for esy nd fst ccess to the ICA nd ECA brnches. Simultneous cquisitions in AP nd lterl projections re stndrd requirements for stte-of-the-rt cerebrl ngiogrphy. Selective ctheteriztions of ll supplying territories re mndtory; overview injections from the CCA re not cceptble. In order to obtin complete informtion on the cerebrl vsculture nd not only in the region of interest, lrge fields of view, in ddition to mgnified projections, re dvisble. Attention should be pid s to whether proximl suprortic vessels re tortuous or disesed. To exclude n AV shunt, dignostic ngiogrm cn be performed without nesthesi in outptients. Furthermore, ngiogrphy is helpful to rule out other cuses of tinnitus such s fibromusculr dyplsi (Arning nd Grzysk 2004), dissections ( Pelkonen et l. 2004), or crotid siphon stenoses (Hrtung et l. 2004). The risks of stroke ssocited with cerebrl ngiogrphy re not trivil but hve decresed with time nd experience, with improvement of equipment nd dvncement of endovsculr tools (Hnkey et l. 1990; Dion et l. 1987; Fisher et l. 1985; Kerber et l. 1978; Anonymous 1995). Recent studies revel rte of 1.3% 2.3% for ll neurologicl complictions nd 0.4% 0.5% for permnent neurologicl deficits (Leffers nd Wgner 2000; Willinsky et l. 2003). Willinsky et l. (2003) reported on 2899 consecutive cerebrl DSAs prospectively studied. Among 39 complictions (1.3%), 20 were trnsient (0.7%), 5 (0.2%) were reversible nd 14 (0.5%) were permnent. The uthors lso found tht neurologicl complictions were significntly more frequent in ptients over 55 yers of ge, in ptients with cerebrovsculr disese nd when the fluoroscopic time ws longer thn 10 min. The ltter fct reflects n importnt spect, which is experience. The influence of ctheter technique, opertor experience nd procedure time hs been reported (McIvor et l. 1987) nd is, in my opinion, key fctor in reducing complictions. The choice of ctheter size for plcement in cerebrl vessels plys nother importnt role. During my clinicl INR prctice t Chrité, I used 4-F dignostic ctheters lmost exclusively between 1993 nd 2003 (Cordis SUPER TORQUE, ). In pproximtely 6000 ptients (c. 25,000 30,000 selective injections) dignostic cerebrl ngiogrms permnent neurologicl deficit ws observed in three ptients. More thn 90% of these dignostic cerebrl ngiogrms were performed without the id of guidewire, even in older ptients. This becme possible only fter the introduction of brided ctheter mteril in It is my strong belief tht reducing guidewire mnipultions in generl helps to lower the compliction rte. Ptients with DCSFs re often elderly, nd my present with dvnced rteriosclerotic disese. Becuse no lrge guiding ctheter is being plced in the internl crotid rtery, the length of n endovsculr tretment session, s risk fctor in this ge group, plys minor role when performing trnsvenous occlusions. Usully only 4-F dignostic ctheter needs to be plced in the ECA for rod mpping or control injections throughout the procedure. Overll, in experienced hnds, the compliction rte of cerebrl ngiogrphy, locl nd neurologicl, is below the numbers reported in the 1980s nd justifies its use whenever cliniclly necessry Angiogrphic Protocol for DCSFs As for brin AVMs, complete visuliztion of the intrcrnil vsculture is importnt, including the ipsind contrlterl ECA territories (Figs ). The rteriogrm should provide the following ngiomorphologicl informtion: Locliztion of the fistul site (Figs ) Differentition between indirect nd direct fistul (Figs ) Demonstrtion of the entire rteril supply, in prticulr in cses with DCSF with visuliztion of so-clled dngerous nstomoses Visuliztion of the CS nd its tributries with possible stenosis, thrombosis, ectsis, drining veins such s IPS, SOV, ICS etc. Identifiction of fresh thrombus in the CS or IPS Identifiction of risk fctors such s CS vrices, intercvernous pseudoneurysms or corticl dringe Identifiction of trum signs such s dissection or trnssection Identifiction of disesed, stenosed or tortuous suprortic rteries Preexisting disese such s FMD or Ehlers-Dnlos Visuliztion of collterl circultion with djcent territories such s crotid bifurction Debrun (1995) suggested the following protocol for dignostic work-up in CCFs:

120 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 111 Selective bilterl injection of the ICA Selective injection of the scending phryngel rtery, bilterl (if necessry through microctheter) Selective injection of the internl mxillry rtery Picrd et l. (1987) lso emphsized the need for n exhustive ngiogrphic work-up for visuliztion of ll potentil feeders: the cvernous brnches of ILT nd MHT, the jugulr, hypoglossl nd crotid rmi rising from the APA, IMA brnches (cvernous brnch of MMA nd AMA s well s rtery of the formen rotundum) nd recurrent deep ophthlmic rtery. A durl rteriovenous fistul of the CS cn be uni- or bilterl nd often involves multiple feeders of both ICA nd ECA territories, lso in cses where the shunt is unilterl (Debrun 1995). Becuse current tretment is performed in most centers using trnsvenous routes, precise nlysis of the venous ntomy hs become crucil for successful procedure. The im of ngiogrphy in ptients with Type B D fistuls is to determine the exct loction of the rteriovenous communiction, nd to decide which comprtment of the CS is involved: nterior or posterior (or both), left or right (Figs nd 7.49). To identify precisely the site of the fistul, selective injection of ICA nd ECA brnches is necessry. The differentition between uni- nd bilterl fistuls should be done in mgnified AP projections (Fig. 7.38). Enlrged durl brnches of the cvernous ICA should not be mistken for n opcified CS. This differentition is sometimes difficult nd probbly the reson why the incidence of bilterl fistuls ppers overestimted in clinicl prctice s well s in the literture (Ernst nd Tomsick 1997). Durl rteries cn cross the midline to supply n AV shunt t the contrlterl CS, occsionlly even when there is no significnt ipsilterl supply. In fct, in some cses, the shunt is neither right nor left, but my involve the ICS (Fig. 7.40). For identifiction of ECA feeders, lterl nd AP projections should be used, nd sometimes AP views t vrious ngultions re helpful. When performing the dignostic work-up, one should be wre of the fct tht durl fistul involving the IPS is usully supplied by APA brnches nd drins often times first into the CS, then into the SOV nd thus cn cuse very similr clinicl picture (Fig. 7.46). Lsjunis et l. (1987) hve recommended performing selective ctheteriztions using even microctheter if necessry, to visulize the smllest pedicles nd to identify the so-clled dngerous nstomoses (Lsjunis 1984). These nstomoses re not per se dngerous nd consist minly of the AFR, AMA nd MMA with connections to ILT, nd the APA connected to ICA nd TMH. They re prcticlly lwys present, but cn be obscured on ngiogrms due to their smll size, poor imge qulity nd individul hemodynmics (see Sect ). Being wre of their existence is indispensble for voiding ischemic complictions during trnsrteril emboliztions in ECA brnches, the only dnger being ignoring or neglecting norml vsculr ntomy in the CS region Angiogrphic Antomy of the Cvernous Sinus Arteril Antomy: Figs Venous Antomy: Figs CSF Antomy: Figs In some cses of high-flow durl AV shunts, n extensive network of smll durl rteries cn be found, of which only some cn be identified nd most re not lrge enough to be ctheterized with currently vilble devices. The ngiogrphic ntomy of the venous system in the region is influenced by hemodynmics of the cerebrl circultion nd of the AV shunt, selectivity of injections (rteril or venous), nd thrombotic processes in the CS, SOV or IPS. In my own prctice the following work-up hs usully been performed: Selective bilterl ICA injections, AP nd lterl Selective bilterl ECA injections, AP nd lterl (including APA) Selective VA injections, AP nd lterl Selective phlebogrphy, AP nd lterl (t different levels below the jugulr bulb) Selective injection into the ECA herein mens tht overflow of contrst into the ICA territory is voided. The pressure during mnul injection should be pproprite to the difference in vsculr resistnce between both vsculr beds nd distl ctheter plcement into the IMA is usully helpful. It is importnt tht ICA injections re obtined in mgnified views nd cudl ngultions (Wters view). These projections llow for esier differentition between uni- nd bilterl fistuls, s well s (Text continues on p. 122)

121 112 7 Rdiologicl Dignosis of DCSFs b Fig. 7.12,b. A 30-yer-old ptient with left temporl AVM. Selective left ECA injection, AP () nd lterl (b) view. The rteriovenous shunt leds to incresed flow in the ICA nd to enlrged externl-internl crotid nstomoses. From the MMA rises recurrent meningel brnch, which fills the OA (thick rrow). The AFR (rrow) rises from the pterygopltine rtery nd runs in AP projection crnilly nd medilly through the formen rotundum (cnlis rotundus) to connect with the ILT ( sterisk). The AMA (double rrow) reches the ILT with meningel brnch through the formen ovle. Other nstomoses/brnches: Anterior deep temporl rtery (short rrows), SPA brnches to the ethmoidl rteries of the OA, (dotted rrow), pterygovginl rtery ( rrowhed). (Modified from Benndorf, 2002) b Fig. 7.13,b. A 5-yer-old boy with right temporl DAVF (double rrowheds). Selective ECA injection, AP () nd lterl (b) view. The min supply of the rteriovenous shunt is provided by n enlrged MMA (thick rrow). Clerly visible is the AMA (rrow), pssing with brnch through the formen ovle nd filling the ILT (sterisk) vi its posteromedil rmus (double rrow). This vessel my run in AP projection in medilly convex turn

122 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 113 b Fig. 7.14,b. A 33-yer-old ptient with direct CSF (Type A), 2 yers fter cr ccident. Selective left ECA injection, lterl projection. The ILT (sterisk) is enlrged due to the high-flow AV shunt nd displced posteriorly. It fills vi the AFR (rrow), which hs developed network of multiple smll durl vessels t the level of the formen rotundum, nd vi meningel brnch of the AMA (double rrow) tht directly origintes from the IMA. The frontl brnch of the MMA is hypoplstic, however fills the ophthlmic rtery (thick rrow) retrogrdely. The non-subtrcted imge (b) shows the typicl projection of the ILT origin onto the center of the sell b MMA Fig. 7.15,b. A 65-yer-old ptient with bilterl DAVFs of the sigmoid sinus. Selective ECA injection, left lterl () nd AP (b) projection. The enlrged AFR (rrow) fills vi the ILT (sterisk), the rmus superior (mrginl tentoril rtery, Bernsconi), which courses posteriorly nd lterlly towrds the AV-shunt. In AP projection, the formen rotundum (rrowheds) nd the rtery (rrow) coursing through, cn be identified on non-subtrcted imge (b). The enlrged rtery of the pterygoid cnl (vidin rtery, thick short rrow), courses posteriorly nd communictes with brnches of the MMA nd the APA nd ICA. The AMA (double rrow) gives rise to smll brnch tht seems lso to connect with the ILT

123 114 7 Rdiologicl Dignosis of DCSFs b MC MC Fig. 7.16,b. The sme ptient s in Fig Superselective injections in the rtery of the formen rotundum () nd in the ccessory meningel rtery (b) through microctheter (MC), lterl projections. The AFR (rrow) psses through the formen rotundum nd is connected with the nterolterl rmus of the ILT (sterisk). From the connection with the ILT courses smll brnch (triple rrow) to the MMA nd reches its petrous brnch (thick rrow) distl to the formen spinosum. b The AMA (double rrow) rises in this cse directly from the internl mxillry rtery giving off brnch to the formen ovle, which connects to the ILT s well s to the the petrous brnch of the MMA (thick rrow). (Double rrowheds, mrginl tentoril, or medil tentoril rtery with typicl undulting course (my lso rise from the MHT or in rre cses from the AMA nd MMA (Benndorf 2008) b Fig. 7.17,b. Selective ECA injection, AP () nd lterl (b) projections. In this cse, the crotid siphon fills not only vi the retrogrdely opcified ophthlmic rtery (thick rrow), but lso vi the ILT ( sterisk) which receives blood from the AFR (rrow) tht often shows typicl irregulr zig-zg course in lterl view. In AP view, this segment of the vessel usully projects s dot with irregulr contours (rrow), which is lwys centered in the formen rotundum (see inset)

124 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 115 b Fig. 7.18,b. Selective ICA injection, lterl view subtrcted () nd non-subtrcted (b) views. Antomic vrint of the ophthlmic rtery. A lrge dorsl ophthlmic rtery (thick rrow), from the C4 segment (intrcvernous OA, or recurrent OA) nd very smll ventrl ophthlmic rtery (double rrow) re visible. The former runs s the nteromedil brnch of the ILT (sterisk) through the suprorbitl fissure. The ltter is, despite its smller size, quite importnt becuse it usully supplies the centrl retinl rtery. Note the choroidl blush from djcent ciliry rteries in the lte phse (rrowhed, inset). Note: The tiny brnch (rrow) coursing posteriorly to rech the formen spinosum is the posterolterl brnch of the ILT) b Fig. 7.19,b. Sme ptient, selective left ECA injection, AP () nd lterl (b) views. The MMA hs lrge ophthlmic brnch, which retrogrdely supplies lso the dorsl ophthlmic rtery (thick rrow). In the erly rteril phse this vessel, however, is initilly filled by the ILT ( sterisk), which on the other hnd is supplied by very smll AFR (double rrow) tht connects with the very sme, bove-mentioned posterolterl brnch (rrow). This exmple demonstrtes the usefulness to look for the AFR in AP (inset), when the lterl view shows only fint opcifiction. Short rrow: pterygovginl rtery well visible with its typicl downwrd turn

125 116 7 Rdiologicl Dignosis of DCSFs b Fig. 7.20,b. Selective right ECA injection, AP () nd lterl (b) views. The AFR (rrow) rises from distl internl mxillry rtery in the pterygopltine foss, coursing crnilly, medilly nd posteriorly to rech the ILT (sterisk), which in this cse is only fintly opcified nd ppers dditionlly supplied by the posterolterl brnch (double rrows) of the MMA (thick rrow). Note: Beside the fct tht these brnches re not enlrged due to n AV shunt, the sptil resolution of the II system used here, reches its limits to disply the minute rteril communictions (see lso Fig. 7.55). Short rrow: pteryvginl rtery with its chrcteristic downwrd turn b Fig Sme projections s in Fig. 7.20, non-subtrcted views. The AFR psses through the formen rotundum nd cretes the typicl bove-mentioned irregulr dot (rrow), projecting exctly onto this formen (rrowheds). In some cses, the AFR is esier identifible in AP thn in the clssic lterl view, where other nstomotic chnnels my tke similr course. In lterl view, the ILT reches the C4 segment of the ICA pproximtely t the middle of the sell (sterisk), usully locted between the superficil temporl rtery (double rrow) nd the MMA (thick rrow)

126 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 117 b c d Fig d. Left ECA injection in AP () nd lterl (b) projections. Demonstrtion of nother importnt nstomosis between ECA nd ICA: After pssing the formen spinosum, the MMA (thick rrow) gives off smll brnch (rrow) tht trvels medilly nd crnilly to connect with the posterolterl rmus of the ILT (sterisk). In non-subtrcted AP view (inset) this vessel does not project onto the formen rotundum. The corresponding ICA injections in AP nd lterl projection in c nd d show the sme rmus tht runs from the ILT (sterisk) cudlly nd lterlly, leding to fint opcifiction of the MMA (thick rrow) vi its connection t the level of the formen spinosum

127 118 b c ICA d ICA e f ICA ICA AMA AMA Fig f. Communiction between C5-segment of the ICA nd scending phryngel rtery (APA)., b ICA injection, lterl nd oblique projections: Demonstrtion of the brnches of the MHT (sterisk): The mrginl tentoril rtery (Bernsconi) with its chrcteristic undulting course scends posteriorly (double rrowheds). Additionl brnches: Inferior hypophysel rtery contributing to the blush of the posterior lobe of the pituitry glnd (thick rrow), rteries to the clivus (double rrow) from the dorsl meningel rtery, nstomosing with brnches of the neuromeningel rmus of the APA. Dotted rrow: Recurrent rtery of the formen lcerum, triple rrow: Cpsulr rtery fter Mc Connell. c f Superselective filling of the APA, AP nd lterl view erly rteril phse (c,d), lterl view mid nd lte rteril phse (e, f). d f re identicl projections to : Anstomosing brnches between neuromeningel rmus (double short rrow) nd ICA: Lterl clivl rtery (double rrow) from the jugulr rmus. Further identifible is the crotid brnch (dotted rrow), rising from the superior phryngel rtery (rrow) nd connecting with the recurrent rtery of the formen lcerum tht rises from the C5 portion. These nstomoses provide retrogrde filling of the MHT (sterisk), which explins why hypophysel blush my be seen in some selective APA injections. Thick rrow: Proximl internl mxillry rtery opcified vi n nterior brnch of the superior phryngel rtery to the Eustchin tube (white sterisk) nd AMA. (Modified from Benndorf 2002)

128 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 119 b Fig. 7.24,b. Right ICA venogrm AP () nd lterl view (b). A lrge SMCV (rrow) drins vi the SPPS (thick rrow) into the nterior CS (sterisk). The CS drins into the inferior petrosl sinus of norml cliber (double rrows) nd the pterygoid plexus (lrge rrowhed). The ltter connects with the CS vi venous plexus in the formen ovle (rrowhed), which lies in AP projection medil to the IPS. Note the white line (dotted rrow), seprting the CS from lterl structure tht my correspond to lterocvernous sinus (LCS) b ICA ICA Fig. 7.25,b. Right ICA venogrm, lterl view. The SMCV (rrow) drins into the SPPS (thick rrow), which before opening into the CS (sterisks) is joined by the uncl vein (short rrow), smll CS tributry, often neglected in textbooks. No filling of the pterygoid plexus is seen. The CS my (ngiogrphiclly) vry in shpe nd size (see lso Fig. 7.34). In () there is lrge nterior CS, nd smller posterior CS, s opposed to (b), where the posterior CS ppers the min comprtment. The vriform shpe of the CS depends to some degree on the size nd course of the ICA within the CS, s cn be pprecited when using sequentil subtrction technique (insets). Good visuliztion of the IPS in both cses (double rrows)

129 120 7 Rdiologicl Dignosis of DCSFs b c d LCS Fig d. Right ICA venogrms, AP () nd lterl view (b). The right cvernous sinus (sterisk) fills vi the SMCV nd SPPS. It lso drins vi the intercvernous sinus (double rrowheds) to the contrlterl side nd vi the left IPS (short rrow = prs horizontlis, long rrow = prs verticlis). Becuse of superposition with the venous plexus of the formen ovle (FO-plexus rrowhed) nd the PP (lrge rrowhed) in AP projection, the ipsilterl IPS (double rrow) is difficult to identify. Both venous structures cn be better distinguished when more cudl ngultion, creting less foreshortening nd projecting the FO-plexus crnil nd lterl to the IPS, is used. These ntomicl reltionships lso become clerer when sequentil subtrctions re used (b,d). Dotted rrow: Seprting CS nd lterocvernous sinus (LCS).

130 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 121 b ICA c Fig c. Right ICA venogrm, lterl (, b) nd AP (c) projection. The SMCV (rrow) runs s lrge vein, drining severl smller vessels, towrds the SPPS (thick rrow), which is joined by the uncl vein (short rrow), before reching the CS (sterisks). From here, the blood drins vi the IPS (double rrows) into the IJV, vi the SPS (short double rrows) into the trnsverse sinus, nd vi smll emissry vein (smll rrowhed) into the pterygoid plexus (lrge rrowhed). In the subtrction, the ICA mrks filling defect seprting the nteroinferior nd posterosuperior CS, s seen in the sequentil subtrction (b). Note tht in () the contours, outlining the non-opcified ICA lumen (ICA), belong to the internl crotid rtery venous plexus (ICAVP) of Rektorzik (double short rrows). (See lso Figs ) b Fig. 7.28,b. Right ICA venogrm AP () nd lterl (b) view with shifted msk (sequentil subtrction). The Sylvin veins nd the SPPS (thick rrow) do not rech the CS, but drin more lterlly into prcvernous sinus (PCS, rrow), tht empties vi lrge emissry vein (rrowheds) into the pterygoid plexus (lrge rrowhed)

131 122 7 Rdiologicl Dignosis of DCSFs for better identifiction of the IPS, which my be opcified fintly during the lte venous phse when filled vi the norml venous dringe of the brin (Fig. 7.26). It is widespred misconception tht n IPS tht is filled during the erly rteril phse, or not ngiogrphiclly involved in the fistul s dringe, must be considered either thrombosed or otherwise completely blocked (Fig. 7.29). In ctul fct, fistul tht hemodynmiclly (or visibly) does not communicte with the IPS my be very well connected ntomiclly nd could be pproched by endovsculr mens (Fig. 7.42). Thus, creful nlysis of the lte venous phse in crotid nd vertebrl ngiogrms in both plnes is dvisble (Cse report III, Figs nd 7.42). In my experience, the IPS is more frequently ptent thn ssumed nd, in contrst to the erly sttement from Theron (1972), my lso well opcify in ngiogrms of the posterior foss (Figs nd 7.30). As discussed in Sect. 4.1, rteril ngiorchitecture hs been the min bsis for clssifying spontneous CS fistuls from surgeon s point of view (Brrow et l. 1985). The rteril ngiogrphic fetures re nonetheless of minor importnce for plnning trnsvenous emboliztions. The differentition between Types B D nd Type A fistuls, or between direct nd indirect (durl) fistuls, is of little or no relevnce for occlusion of fistulous CS comprtments. More importntly, the rdiologic work-up of CSFs must include detiled nlysis of the venous ngiomorphology. Such nlysis requires knowledge nd good understnding of the norml ntomy of the CS, its tributries nd its drining veins (Figs ). The min tributries of the nterior CS re the superior ophthlmic veins nd the inferior ophthlmic veins, usully not visulized following ICA injections. Becuse the SOV usully drins the nsl mucos, this vein is frequently seen on norml ECA rteriogrms. In cses of hyperemic nsl mucos, s seen under certin physiologicl conditions (e.g. in young girls during menstrution, this vein cn pper surprisingly erly nd be intensely opcified, so tht the inexperienced opertor my suspect n AV shunt. The connection of the vein with the CS nd its dringe vi the IPS becomes visible in lterl views, while in AP view the typicl omeg configurtion (Figs ) cn be seen. Other importnt tributries re the superficil middle cerebrl vein (SMCV Figs ) nd the sphenoprietl sinus (SPPS, Fig. 7.27). The SMCV drins either directly or vi the SPPS into the CS. Frequently this vein my lso bypss the CS nd drin into lterocvernous sinus (Sn Milln Ruiz et l. 1999) or directly into the formen ovle plexus nd the extrcrnil PP, fct tht hs found little ttention in the older literture. A minor tributry of the CS is the uncl vein. This smll vein, best seen in lterl views coursing from postero-superior to ntero-inferior, drins either directly or together with the SPPS into the CS (Figs. 7.25, 7.27, 7.32). Ech tributry my chnge its function nd turn into drining vein when the venous pressure increses due to n AV shunt. Such chnges gin prticulr importnce in cses of high-flow lesions, but re less often seen in Type B D fistuls s compred to direct CCF. However, when performing trnsvenous emboliztion, corticl or leptomeningel dringe must not be missed. If coils re incorrectly plced, pressure increse with subsequent intrcrnil hemorrhge my result. The SOV is most frequently opcified in retrogrde fshion nd fills the ngulr vein, the fcil vein nd eventully the IJV. In cse of lrge AV shunt volume, the ngulr vein my lso opcify the contrlterl fcil vein nd the frontl vein. The cliber of these veins cn be crucil for tretment plnning, s re elongtions, thrombi or stenoses, nd other fctors tht could possibly compromise ctheter nvigtion. In some cses, the suprorbitl vein fills the medil temporl vein (Figs. 7.31, 7.32 nd 7.91), which cn be mistken in lterl views s SOV, but cn lso serve s n pproch to the CS (Agid et l. 2004; Kzekw et l. 2003). Incresed blood volume my be directed to the contrlterl CS nd from there to the contrlterl SOV nd IPS. The AV shunt my lso drin vi the PP. Often the venous dringe will use the IPS, which my be opcified on both sides. The visuliztion of this sinus is of importnce, becuse of its ntomicl proximity nd reltively stright course, mking it the venous ccess route of choice. The opcifiction of the CS s well s its drining veins in DSA my vry significntly, depending on ntomicl nd hemodynmic fctors (Fig. 7.35). Thrombotic processes within the CS or the IPS further influence their ppernce in n ngiogrm obtined by rteril contrst injection. Tht is lso why some of the most ccurte descriptions of the CS nd the SOV ntomy cn be found in reports on orbitl phlebogrphy from the 1960s 1970s, where contrst medium ws directly injected into frontl vein nd forced into the orbitl venous system by the use of tourniquet (Lombrdi nd Psserini 1967; Cly et l. 1972; Brismr nd Brismr 1976; Brismr et l. 1976).

132 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 123 b c d Fig d. Right () nd left (b) ICA venogrm, sme ptient, lterl views. The CS (sterisk) is not well visulized becuse most of the venous blood from the Sylvin fissure drins vi superior nd inferior nstomotic veins (rrows), nd through prcvernous sinus (short rrow). Accordingly, the IPS is just fintly opcified (double rrows), which does not necessrily men tht this sinus is thrombosed or occluded. c, d Venogrm of the posterior circultion of the sme ptient in AP (c) nd lterl (d) projection. The right, nd less clerly the left IPS re opcified (double rrows). Their contrst filling is provided by both superior petrosl sinuses (short rrows), tht rech the posterior CS (sterisk) nd drin blood from the posterior foss. The right nd left cvernous sinus re joined by the posterior intercvernous sinus (rrowheds). Note tht the prcvernous sinus, filled in b, is lso fintly opcified (short dotted rrow). This exmple demonstrtes tht the ssumption tht non-opcified IPS must be occluded nd thus cnnot be pssed with microctheter is erroneous. Hemodynmic fctors, type of injection nd possible thrombus formtion influence the ngiogrphic ppernce of venous collector tht receives blood from different tributries of both hemipsheres, the posterior circultion nd the ECA territory. Arrow: Lterl mesencephlic vein; dotted rrow: Stem of petrosl vein

133 124 7 Rdiologicl Dignosis of DCSFs b Fig. 7.30,b. CS visuliztion in venogrm of the posterior circultion AP () nd lterl (b) projection. The right nd left (double rrows) IPS re opcified vi the posterior CS (sterisk) tht receives blood from both SPSs (short rrows) nd from drining veins of the posterior foss (). Note tht the mirror vessel running prllel to the IPS in (b) is the inferior petroclivl vein (IPCV, rrowheds) tht lies t the inferior surfce of the petroclivl fissure (see Figs ). Arrow: Anterior pontomesencephlic vein, dotted rrow: Stem of petrosl vein If the SOV is scrcely opcified or not visible t ll due to thrombosis of the vein or of the CS, selective phlebogrm of the CS my help to clrify the sitution nd explin drmtic clinicl symptoms in some smll AV shunts. If n IPS does not fill t ll during n ngiogrm erly or lte, it my indeed be thrombosed. It is believed tht recent thrombosis of the IPS is the cuse for their clinicl deteriortion by incresing the venous pressure nd rerouting the venous dringe nteriorly. This condition is otherwise not necessrily reson to use n lterntive pproch, becuse recently thrombosed IPS is usully not very difficult to pss with microctheter. Trnsvenous phlebogrms of the IJV/IJB region ply mjor role in the dignostic work-up of DCSFs, more so when trnsvenous pproch is plnned. Becuse the IPS is the most common pproch, its precise ntomicl reltionship to the IJV is of key interest. Intrvenous injections of contrst often provide more relible informtion on the ntomy in this region thn intr-rteril DSAs nd re vluble when decide whether or not the IPS pproch would be fesible (Fig. 7.41). In some cses simultneous rteril nd venous injections cn further fcilitte ntomic understnding (Fig. 7.7) Such sitution is demonstrted in cse where the IPS ws missed on intr-rteril injections due to locl thrombosis (Cse report III). Yet, the phlebogrm showed widely open IPS through which microctheter could esily be nvigted. Even in cses of complete thrombosis of the IPS, often tiny residul structure is seen s sort of stump (Cse report I, III). Becuse this venous stump or notch cn be mde more or less visible, the phlebogrm should be repeted using different ctheter positions, including injections below the jugulr bulb. These will help to opcify the IPS in cses of deep termintion or berrnt IPS (Fig. 7.37). Section discusses the vrints of entry of the IPS into the IJV in more detil, of which I hve observed five cses. When such n extrcrnilly locted connection between IPS nd IJV is present, it my be used for trnsvenous ctheteriztions of the CS s well (Benndorf nd Cmpi 2001). In cses where ngiogrphic ntomy remins difficult to red, sequentil subtrction (msk shifting into the rteril phse) is simple nd useful technique. Plcing the msk within the erly rteril phse provides white (ICA) nd blck (CS) contrst imges displying the ntomicl reltionship between portions of the CS, its fferent nd efferent veins nd the cvernous ICA (Figs ). (Text continues on p. 139)

134 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 125 S I b S I S II S II S III S III SR IR S I S II S III MTV c d RMV RMV Fig d. Dringe of ECA territories in two ptients.,b Right nd left ECA venogrm, AP nd lterl views (inset): Intense mucosl blush (double rrowheds) opcifying both SOVs (rrows) nd the right fcil vein (thick rrow). On the left side there is opcifiction of the CS (sterisk), which receives blood from the right CS (superimposed by nsl mucos) nd drins vi the IPS (double rrows) into the internl jugulr vein, s seen in lterl view (inset). The vrious segments of the SOV (typicl Omeg shpe) re better demonstrted in Wters view (b) tht lso demonstrtes the nsl rcde (rrowhed) nd the ngulr vein (double rrowhed), joining the fcil vein (thick rrow). The ltter cn be differentited from the posteriorly locted junction of SOV nd CS. Note the three segments of the SOV (I III), nd its superior (SR, long rrow) nd inferior (IR, short rrow) root. Recognizing this ntomic disposition my become helpful for plnning TVO through the fcil vein nd SOV. The superior root is more commonly used (Biondi et l. 2003). c, d Right nd left ECA-venogrm in AP nd lterl projection (insets): Intense blush of the nsl mucos (double rrowhed), which in this cse drins only prtilly vi the right SOV (rrow), but minly vi ngulr veins into the fcil veins (double rrowheds nd thick rrow respectively), nd vi suprorbitl veins (short double rrows) into the middle temporl veins (MTVs, insets, short rrows, The MTVs run in lterl projections prllel to the SOVs posteriorly nd then turn cudlly to join the retromndibulr veins (RMVs). The CS (sterisk) nd the IPS (double rrows) re fintly opcified on the right, but not on the left side. Insets: The MTV often hs distinctive shrp turn when coursing round the zygom (rrows)

135 126 7 Rdiologicl Dignosis of DCSFs b c PCS SPS Fig c. Right ECA venogrm AP () nd lterl view (b). Right ICA venogrm lterl (c). The intensely opcified nsl mucos drins vi the SOV (rrow) nd the IOV (thin short rrow) into the CS (sterisk), which connects with the IPS (double rrows), s well s with the pterygoid plexus (lrge rrowhed) vi smll emissry vein (smll rrowhed). There is significnt component drining vi the middle temporl vein (short double rrow) into the retromndibulr vein (dotted rrow). The right corticl veins (c) drin mostly vi lrge vein of Lbbé (short rrows), while the CS minly drins lrge uncl vein (rrow). PCS: Prcvernous sinus, SPS: Superior petrosl sinus. Note the typicl Omeg shpe of the SOV in AP projection () b Fig. 7.33,b. ICA nd ECA dringe of the cvernous sinus. Right common crotid venogrm AP () nd lterl view (b). The lte venous phse shows the dringe from the cvum nsi nd the right cerebrum, both opcifying the CS. SOV: (short rrow) nd its entry into the CS (sterisk). The sphenoprietl sinus (thick rrow), which brings blood from the Sylvin veins (rrow), is lso visible. The cvernous sinus drins minly into the IPS (double rrows) nd the internl jugulr vein

136 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 127 b ICA c d Fig d. Cvernous sinus opcifiction vi ECA (,b) nd ICA injection (c,d) AP nd lterl. The SOV (rrow) drins into the CS (sterisk) nd IPS (double rrow), while the SMCV drins (rrow), in ddition, into the pterygoid plexus. Angiogrphic ppernce of the CS nd its drining veins depends on the injected territory nd on locl hemodynmics. A single territory ngiogrm my only prtilly visulize the venous ntomy nd should not be used s the only dignostic exm to ssess the venous ntomy. The filling defect in b is not thrombus in the CS, but cused by the ICA lumen (d)

137 128 7 Rdiologicl Dignosis of DCSFs b SPS c d SPS Fig d. Unusul (ntomic) disposition of the cvernous sinus/inferior petrosl sinus. ICA injection AP () nd lterl (b). Sequentil subtrctions (c,d). The posterior CS (sterisk) is filled exclusively vi lrge uncl vein tht tkes n unusul posterior turn (short rrow) nd drins into the IPS (double rrow). The nterior CS is not opcified. Corticl dringe from the Sylvin territory is directed towrds prcvernous sinus (rrow) tht empties into the emissry vein of the formen ovle (thick rrow) nd reches the pterygoid plexus. This ngiogrphic ppernce of n isolted posterior CS my in fct not represent the true ntomy

138 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 129 b c d e f Fig f. Vrint ngiogrphic ppernce of cvernous sinus ntomy. Six norml ngiogrms with sequentil subtrctions llow for relting rteril (white) nd venous (blck) structures to ech other.,b Common configurtions, dringe vi PCS into PP (c), Unusul nterior loop of SMCV nd SSPS (d). e,f Interestingly, the SOV my serve not s tributry, but s drining vein of the CS. This cn be norml finding, seen in some ICA injections under physiologicl condition. It does not lwys indicte pthologicl condition, such s n AV shunt or elevted intrcrnil pressure s ssumed in the pst. In clinicl prctice, cerebrl ngiogrms show numerous vritions nd no ngiogrm will look like nother. Importnt is to understnd the bsic ntomic dispositions

139 130 7 Rdiologicl Dignosis of DCSFs b c d e Fig e. The bbernt IPS. The bbernt IPS or deep termintion cn esily be overlooked on stndrd projections (, b). The oblique projection in c fcilittes understnding of this ntomic disposition nd reveles tht the IPS (double rrows) courses prllel to the IJV for severl centimeters, before ctully joining it. Recognizing this ntomic dis position my sve vluble time during EVT. This ntomic vrint hs been successfully used s n pproch for trnsvenous ccess to the CS in two cses (d, Benndorf et l. 2001). Arrow in c, ctheter in ICA. e Illustrtion of norml nd deep termintion of the IPS

140 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 131 b c d Fig e. A 70-yer-old ptient, with exophthlmos nd chemosis of 4 months stnding.. Selective ICA injection on the right () nd CCA filling on the left (b), lterl projection: Bilterl opcifiction of the posterior CS ( sterisk) vi enlrged brnches of the MHT ( rrowheds). c In the venous phse slow opcifiction of the entire CS ( sterisks), which drins primrily vi the IOV (double rrows) nd secondrily vi the SOV (rrow). Both ophthlmic veins, in prticulr the SOV, seem to be prtilly thrombosed nd connect vi bridging (psidl) veins (thin rrow). There is no evidence of corticl venous dringe. The missing opcifiction of the IPS indictes tht the fistul does not drin posteriorly. This does not necessrily men the IPS is indeed completely occluded nd not ccessible for trnsvenous emboliztion. d,e Selective bilterl internl crotid rteriogrms, AP projection (sme ptient): The filling of the CS (sterisk) in the erly rteril phse on the right (), s well s on left (b) becomes more evident indicting true bilterl fistul (rrowheds: tiny feeder rising from both, the right nd left MHT) e

141 132 7 Rdiologicl Dignosis of DCSFs b c d Fig d. Indirect supply of DCSFs by ICA-OA collterls., b Not only TMH nd ILT prticipte in the supply of DCSFs. Minute brnches, such s this single recurrent meningel brnch rising from the ophthlmic rtery, my contribute. c, d In this reltively high-flow durl AV shunt, the min supply from the ICA is not provided by ILT or TMH, but comes from multiple recurrent meningel nd posterior ethmoidl rteries (rrows) of the enlrged ophthlmic rtery. This type of rteril supply is not fully covered by Brrows clssifiction

142 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 133 b c Fig c. Type-D fistul. A 77-yer-old ptient with diplopi nd chemosis on the right side. Selective injection of the right ECA () nd the left ICA (b) in AP view: Opcifiction of the intercvernous sinus (rrowhed) nd the right CS (sterisks) by tiny durl rteries of the IMA: the AFR (thin rrow), the vidin rtery (thin double rrow), nd petrosl brnches of the MMA (short rrows). Furthermore, supply by brnches of the MHT (thin rrows) of the contrlterl ICA. Note tht some of the AV shunting is ctully locted in the midline (rrowhed) nd tht different fistulous comprtments of the CS re opcified in nd b, communicting vi stenotic segment (long rrow in b). c Right ECA lterl view: The right CS (sterisks) drins the AV shunt initilly into the IOV (double rrows) nd secondrily in the SOV (not shown). Posterior dringe into the IPS is not visible. The AFR (singulr thin rrow) nd the vidin rtery (double thin rrows) re shown. The brnches supplied by midline nstomoses in (triple rrowhed), re difficult to identify: Limittion of 2D-DSA. MMA MMA AMA AMA b Fig. 7.41,b. A 78-yer-old ptient with exophthlmos, chemosis nd diplopi. Selective ECA injection from the left side in Wters () nd lterl (b) views. Opcifiction of the right CS (sterisk) by multiple feeding pedicles from the contrlterl ECA, forming network (thin rrows) nd converging to smll chnnel (rrow) tht connects with the right side. b The AFR (singulr thin rrow), brnches from the AMA (thin double rrow) nd the MMA (thin triple rrows) cn be identified better in lterl view. Insets: lter phses. (Modified from Benndorf 2002)

143 134 7 Rdiologicl Dignosis of DCSFs b c d JB Fig d. A 71-yer-old ptient with left-sided conjunctivl injection cused by right CS AV shunt. Right ICA injection AP () nd lterl (b) views: Opcifiction of the right CS from brnches of the right MHT. Dringe to the contrlterl side vi the intercvernous sinus to the left CS. From here, only miniml filling of the presumbly prtilly occcluded left SOV (thin rrow) is seen. Significnt dringe vi leptomeningel nd deep veins (short double rrow: bsl vein of Rosenthl), s well s corticl veins (rrow) of the left hemisphere is evident. The tpered IPS ppernce usully indictes recent thrombosis, nd thus does often not prevent ctheteriztion. c, d Sme ptient. Right ICA venogrm, lterl (c): In the lte venous phse, neither the CS (sterisks) nor the IPS is opcified. Phlebogrm of the right IJV, lterl (d): The 6-F guiding ctheter (thick rrow) should be positioned below the jugulr bulb (JB) so tht vessels entering the IJV t lower level re less likely missed. Often only then, the irregulr lumen of the thrombosed IPS (double rrow) becomes more or less visible s short stump. Chnging position of the ctheter nd repeted injections my help to identify n berrnt IPS (Fig. 7.37). This ptient ws successfully treted using this IPS s venous route (Benndorf et l. 2000)

144 b 135 Fig. 7.43,b. Posterior leptomeningel nd deep venous dringe in DCSF., b When fistul involves minly the posterior CS (sterisks), the dringe my not use nterior tributries. In such cse, the IPS is most likely thrombosed nd the only remining exit is the SPS (double rrow) subsequently emptying into the nterior pontomesencephlic vein (short thin rrow), the lterl mesencephlic vein (thin rrow), the bsl vein of Rosenthl (rrow) nd cerebellr veins (thin double rrows). This ptient presented without neurologicl deficit, s do the mjority of ptients with DCSFs nd cortcil dringe. The term leptomeningel venous dringe my be more suitble description for this dringe pttern thn just corticl venous dringe b c TMH ICS Fig c. Complex DCSF. Selective injections into the APA re very useful for pre-tretment evlution. They often revel better the ngiorchitecture thn globl ECA/ICA injections s there is little superimposition by norml vsculr territories. Right APA injections, lterl view, reveling the different types of venous dringe: nterior (SOV), corticl (Sylvin veins, rrow), deep (bsl vein of Rosenthl, double rrow) nd posterior (cerebellr veins, dotted rrow). Corticl venous dringe into Sylvin rises ususlly from the lterl CS, while posterior leptomeningel nd deep venous dringe commonly rises from the posterior CS nd SPS. Such disposition my become importnt when selective coil pcking is performed. Blocking the flow into the SOV will increse the flow nd pressure in the other venous exits. Coil pcking should idelly be performed so tht blockge of the corticl nd deep venous dringe is ssured during the procedure (see Chp. 8). Lrge rrow: Vertebrl rtery, short rrow: Odontoid rch, rrowhed: Pterygovginl rtery, nstomosis with the internl mxillry territory vi brnches to the Eustchin tube (sterisk). b Mgnified view shows the rteril supply from clivl brnches (rrows) of the neuromeningel trunk (thick rrow), crotid brnch from the superior phryngel rtery (long dotted rrow) nd n dditionl nterior nstomosis (short rrow). c Filling of clivl brnches of the right (rrows) nd left (dotted rrows) side through midline nstomoses. There is some erly filling of the right CS (lrge rrow), before drining vi the ICS towrds to the left side, indicting smll AV fistul on this side, while the min AV shunting involves the left CS (sme ptient s in Fig. 7.44c,d)

145 136 7 Rdiologicl Dignosis of DCSFs b Fig. 7.45,b. Introrbitl, prcvernous DAVF. ICA injection, lterl view. The AV shunt (rrow) is ctully locted t the level of the superior orbitl fissure (SOF), supplied by the sme recurrent meningel brnch s in Fig. 7.44, but shunting directly into the proximl SOV. b Superselective injection fter nvigtion of microctheter into the CS nd IOV (thick rrow, inset). The SOV is thrombosed in its distl portion nd fills retrogrdely from the IOV vi bridging (psidl) vein. No ngiogrphic evidence for communiction between SOV nd CS. Not true DCSF, lthough ngiogrphic ppernce is like, nd cusing similir symptoms (see Figs nd 7.72). Arrowheds: Frontl MMA brnch. b Fig. 7.46,b. Two non-cvernous DAVFs cusing symptoms tht mimic DCSF. APA injection, lterl view: DAVF of the IPS (thick rrow), supplied by clivl brnches of the neuromeningel division nd drining into the CS (sterisk) nd SOV (double rrow), cusing diplopi due to 4th nd 6th nerve plsy. b VA injection, lterl view: Tentoril DAVF t the vein of Glen (thick rrow) with nterior dringe vi the bsl vein of Rosenthl (rrow) nd CS (sterisk) into both SOVs (double rrow) cusing exophthlmos nd visul deficit (Benndorf et l. 2003) b Fig. 7.47,b. Mxillo-fcil AVF drining into the SOV, CS nd IPS. The min dringe of this AVF is directly vi the enlrged nd tortuous right fcil vein (thick rrow), ngulr veins (rrows) filling both SOVs (short rrows) nd the CSs (sterisks) nd from here into the left IPS (double rrow). Note the filling of suprorbitl nd frontl veins (short thin rrows) nd the middle temporl vein (dotted rrow)

146 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 137 b B c d Fig d. True high-flow CSF. A 28-yer-old ptient, presented fter cr ccident 2 yers previously, Type-A fistul. ICA rteriogrm, lterl: In contrst to DCSFs, in direct fistuls, frequently multiple ecttic corticl veins develop due to complete rteril stel tht is cused by the high flow: No opcifiction of intrcrnil rteries in the ipsilterl hemisphere. Note, the venous neurysm (rrow) is, ccording to current ngiomorphologic criteri, risk fctor for bleeding. Thin double rrows: Superior ophthlmic vein, which is only to minor extent involved in the dringe. Even in so-clled high-flow DCSFs, such extensive corticl venous dringe is not observed. ICA rteriogrm (6 frmes/second) erly rteril phse, AP (b) nd lterl (c): The lrge defect in the crotid wll ( rrowheds) is only recognizble in AP projection nd despite the higher frme rte (6 frmes/second) not clerly visible in lterl view, due to the very rpid filling of the enlrged CS (sterisks). Significnt enlrgement of the IPS (double rrow), which hs gined cliber, equl to the tht of the ICA (I). d Huber s mneuver: Vertebrl rteriogrm, lterl view. Under mnul compression of the crotid rtery nd simultneous contrst injection into the vertebrl rtery, the loction of the wll defect between C4- nd C5-segment (rrowheds) cn redily be identified. Its exct size nd orienttion (medil or lterl) remins difficult to evlute: Limittion of 2D-DSA. (During EVT of direct CSF, detchble blloon cn be nvigted through the crotid ter nd is inflted until occlusion of the fistul is documented. In some cses, such blloons my deflte fter detchment nd the fistul reopens (ovl filling defect B in )

147 138 7 Rdiologicl Dignosis of DCSFs b c d e f g h Fig h. Vlue of high frme rtes for high-flow AV fistuls (here direct CCF)., b Stndrd 2D-DSA (3 frmes/ second) shows high-flow CSF with n uncler fistul site. c h Higher temporl resolution with 15 frmes/second shows the fistul site better. Note tht the erliest filled structure (sterisk) hs scculr, neurysm-like ppernce, which is most likely cused by venous outpouching of the CS. This CCF ws cused by motorbot ccident, not by ruptured cvernous crotid neurysm

148 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) Flt Detector Technology in Neurongiogrphy (Figs ) Flt detector (FD) technology ws introduced in the erly 1990s, lmost 100 yers fter the discovery of X-rys by Conrd Roentgen. Initil medicl pplictions were for thorx nd skeletl X-rys, followed by digitl mmmogrphy. Although it hs only recently become possible to implement FDs in ngiogrphic systems, imge intensifier systems re rpidly being replced in crdic, generl nd neurongiogrphy. The min reson for this development is clerly improved imge qulity of 2D DSA imging combined with other technologicl dvntges. The currently dominting FD technology is bsed on n indirect X-ry conversion process, using cesium iodide (CsI) scintilltor nd n morphous silicon ctive pixel mtrix (Figs ). Cesium iodide cn be grown s needle-shped crystls mesuring 5 10 μm in dimeter, nd ensuring tht light reches the photodiode with only little sctter, while limiting its lterl diffusion. This scintilltor lso hs very good X-ry bsorption properties (Sphn 2005). The detector used in the system described here is 30 cm 43 cm (A-plne) nd 20 cm 20 cm (Bplne) with pixel size of nd μm respectively. This pixel size is optimized for high resolution required in dignostic rdiology nd provides squre mtrix of bout 9 million pixels on the lrge detector. FDs re smller nd dd less weight to the C-rm llowing for fster rottions, providing more stbility nd fewer distortions. While imge intensifier systems (II systems) used multi-step conversion from X-ry bems into digitl imge signl tht cuses increse of noise, this conversion is only two-step process in FD technology (Figs nd 7.52). Resulting dvntges over II systems re more imge formts, less geometric distortion, more homogenous exposure, excellent corse contrst nd high X-ry sensitivity. One of the most importnt fetures of FDs is their wide dynmic rnge (14 bit vs 12 bit in II systems). It llows one to cover wider dose rnge without risking wrong exposure, which is prticulrly dvntgeous in ngiogrphy where broder dose rnges need to be covered from low levels of bout 10 ngy to much higher system dose levels of bout 5 μgy (Sphn 2005). The high dynmic rnge provides better contrst resolution (16,384 different grey scle vlues vilble per pixel s opposed to 4096) llowing for soft tissue imging using DynCT (see below). In ddition, n increse in imge mtrix from (1 k) to (2 k), llows for better identifiction of smll rteries such s durl brnches of the cvernous portion of the ICA. Figure 7.55 shows n exmple tht demonstrtes the improved imge qulity of 2D- DSA Rottionl Angiogrphy nd 3D-DSA (Figs. 7.58, 7.68 nd ) Erly experimentl work on rottionl ngiogrphy ws performed in the 1970s nd ws quickly followed by clinicl pplictions (Cornelis et l. 1972; Voigt et l. 1975). Thron nd Voigt (1983) were ble to demonstrte tht cerebrl rottionl ngiogrphy dded vlue to the existing ngiotomogrphy or mgnifiction ngiogrphy (Wende nd Schindler 1970), techniques used t tht time to improve visuliztion of cerebrl neurysms nd AVMs. The uthors used 70-mm cmer, mounted onto C-rm tht cquired one imge every 5 during 5 6 s sweep. Although digitl subtrction ws not possible t tht time, the set up provided reltively good visuliztion of size, shpe nd orienttion of cerebrl neurysms. The next step ws the development of the so-clled 3D-morphometer by French investigtors (Heutot et l. 1998). The morphometer consisted of CT gntry in which two X-ry tubes plus the imge intensifier systems were integrted. This rther complex technology provided ngiogrphic 3 D imges of neurysms nd AVMs of stisfctory qulity. Three-dimensionl imges of intrcrnil vsculture cquired with rotting C-rm were presented for the first time by Picrd et l. (1997). Since then, continuous improvement of 3D imging using rotting C-rms hs resulted in widely ccepted clinicl use of 3D ngiogrphy in neuroendovsculr tretment. Becuse the min focus in 3D ngiogrphy ws initilly on dignosis of cerebrl neurysms nd AVMs, bony structures djcent to this vsculture compromised its visuliztion nd were commonly removed during the imge post processing (Siemens systems). Advntges of simultneous reconstructions of osseous nd vsculr structures hve been demonstrted only to limited degree ( Gilloud et l. 2004), but re obvious in res with complex r- (Text continues on p. 142)

149 140 7 Rdiologicl Dignosis of DCSFs Imge Intensifier Input screen Photo cthode Electron optic Output screen Light optic Video cmer X-Rys Imge signl Scintiltor Conversion of x-rys into light Low Energy Electrons High Energy Electrons Fig Conversion process in n trditionl imge intensifier (II) uses multiple steps to convert n X-ry bem into digitl imge signl (X-rys-light-electrons-light-electrons). This cn cuse incresed bckground noise, while the sptil resolution is determined by the cmer s resolution, which is uslly 1K ( ) Photo Diode Conversion of the light photons into electric chrges Red out Mtrix (TFT) Fig As opposed to imge intensifiers, the FD uses only two-step (indirect conversion) conversion process (X-ryslight-electric signl) X-Rys CSL-Scintiltor -Si Pixel mtrix Fig C-rm mounted imge intensifier of its lst genertion. This system hs been used for rottionl ngiogrphy nd 3D-DSA since its introduction in the lte 1990s (see exmples in Figs , 7.81, 7.83, 7.85) Fig New technologies employ flt detector (FD) with fst-imging cpbility. The FD used in Siemens nd Philips systems is bsed on Cesium Iodide (CsI), combined with n morphous silicon ctive mtrix rry. This provides excellent quntum efficiency nd good resolution due to the needle-shped or pillr-like crystl structure, limiting the lterl light diffusion. The mtrix cn be incresed to 2K ( ), lthough monitor systems for full 2K resolution re not commercilly vilbe yet. As n lterntive, new medicl grde displys (56 inch, pixel) re recently vilble

150 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 141 b Fig The CsI crystls cn be grown in needle-shped form, mesuring 5 10 μm. The size of one detector element (154 μm) defines (mong other fctors) the sptil resolution in n cquisition mode without binning (pixel binning). In order to reduce the size of dt sets to be reconstructed, progrms with vrious binning modes (1 1, 2 2 detector elements), fster rottions nd fewer projections re used Fig Flt detector used in the current biplne systems Axiom Artis dba. Are: cm Pixel size: μm (detector element size) Frme rtes: Up to 30 fps (monoplne), 15 fps biplne. No binning: 7.5 fps Binning: 10 ngy 3.5 μgy Anlog to digitl conversion: 14 bit. Fig. 7.55,b. 2D-DSA demonstrting the improved imge qulity of cerebrl ngiogrms using recent FD technology. Two rteriogrms of the left ICA, sme views in the sme ptient. June 2004, b August Although the overll filling of the ICA in ppers better, opcifiction of the cvernous crotid brnches, MHT (short rrow) nd ILT (rrow), s well s of the mndibulr rtery (thin rrow) is clerly superior (not only shrper, but lso revels more brnches). Note tht in theory, the sptil resolution of n II system using the mximum zoom formt is 6 LP/mm, while the FD reches pproximtely 3 LP/mm. Vrious other fctors including size of focl spot (0.3 mm), dynmic rnge (14 vs. 12 bit) nd improved imge post processing lso ply role.

151 142 7 Rdiologicl Dignosis of DCSFs rngement of these components such s the skull bse. For quite some time, investigtors hve been focusing on improving ntomic understnding using colored plstic csting of vessels in cdver specimens s the min tool to investigte the minute nd vsculr ntomy imbedded in bony cnls nd formin, especilly in the middle crnil foss nd prsellr region (Lsjunis 1984; Sn Milln Ruiz 1999; Prkinson 1963, 1984; Rhoton et l. 1979, 1984). The use of 3D vsculr imging of the cvernous sinus nd its relted structures employing 3D ngiogrphy nd modern reconstruction techniques, documented in the literture only scrcely (Nishio et l. 2004; Lsjunis et l. 2001, Mitsuhshi et l. 2007; Hiu et l. 2009), hs been of mjor interest for the uthor ( Benndorf 2002). Initil results of such 3D reconstructions (Figs , 7.81 nd ), using non-subtrcted rottionl ngiogrphy, obtined with n II system (Neurostr, Siemens), which lredy encourging. Using simultneous reconstructions of the osseous nd vsculr structures, it hs been possible to visulize smll durl rteries nd their course through skull bse formin. One cn follow the rtery of the formen rotundum through its formen nd cnl, the course of the AMA through the formen ovle or nstomotic vessels through the formen lcerum. Using this type of ngiogrphic 3D imging, identifiction of rteries feeding DCSF becomes esier nd is possible without using trditionl 2D lndmrks ( Benndorf 2002, 2008; Hiu et l. 2009). Commonly, high-contrst tissue like bone cuses reconstruction rtifcts. Due to isotropic resolution of cone-bem reconstructions, s used in 3D-DSA, these rtifcts become of minor importnce (Fhrig et l. 1997). Ech voxel hs the sme size in the X, Y nd Z directions, leding to reduction of the nonliner prtil volume effect. Visuliztion of vessels nd bone in mximum intensity projections (MIPs) is possible becuse vsculr density is incresed up to 8000 HU, compred to HU, typicl for bone. The combintion of high contrst nd sptil resolution provides higher ccurcy in displying ntomic detils, not obtinble using other imging technology, including modern multi-slice CT scnners. Using n II system (C-rm mounted imge intensifier), el Sheik et l. (2001) showed tht highresolution multiplnr reconstructions of osseous spongios cn be obtined (rottionl osteogrphy). The usefulness of 3D reconstructions fter cervicl myelogrms, reveling the reltionship between contrst-filled thecl sc nd osteophytes of the cervicl spine hs lso been demonstrted (Kufeld et l. 2003). Imge qulity of ngiogrphic 3D reconstructions hs been significntly improved by the recent introduction of FD technology, s demonstrted in exmples displying smll vessel reconstruction using the older II system (Neurostr, Siemens Medicl) nd the ltest FD system (Axiom Artis, dba, Siemens Medicl) in Fig The current sptil resolution of the FD is defined by the size of its detector elements: 154 μm providing 0.13 mm which correspond to 4.1 Lp/mm (Nyquist frequency) in the unbinnend mode, while the mximum resolution of Somtom 64 is 0.36 mm, corresponding to 1.4 LP / mm. The vilble computing power cn use 20-s rottion with 543 projections only when two detector elements re combined (binned), which doubles the pixel size from 154 to 308 μm. Specil progrms, cpble of using the full detector resolution, providing so-clled 2 k dtsets, re currently under evlution. Nonetheless, visibility of vsculr nd bony detils is superior to conventionl (II bsed) 3D-DSA or CT/CTA. This high sptil resolution is of importnce for imging smll rteries such s the durl brnches of the cvernous ICA nd its communictions with the ECA. The rtery of formen rotundum for exmple hs norml dimeter of pproximtely 135 μm (Lng 1979). Different from the rteril network within nd round the CS, the venous ntomy ppered to be of rther minor interest in the medicl literture (Brun nd Tournde 1977; Brun et l. 1976). Hence, veins nd sinuses in the middle crnil foss nd the CS region nd its communictions with the extrcrnil veins nd venous plexus of the skull bse re neither well studied nor fully understood. More recently, severl investigtors hve pointed out the importnce of these veins for ntomy nd physiology (Gilloud et l. 2000; Schreiber et l. 2003; Doepp et l. 2004; Tkhshi et l. 2005) of the cerebrl blood circultion. Angiogrphic computed tomogrphy (ACT, see below)-bsed MIPs, obtined using intrvenous or intr-rteril contrst injections, cn revel even the smllest veins nd sinuses in this re (Benndorf 2002; Nishio 2004; Mitsuhshi 2007). Becuse of its superior sptil nd temporl resolution, intr-rteril DSA is the imging tool of choice for cerebrl vsculr lesions, primrily for rteriovenous shunting diseses. Anlysis of rdiology dt is mostly done by reding 2D imges. Interventionl neurordiologists nd other endovsculr neurosur-

152 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 143 geons re forced to mentlly combine multiple views to crete 3D model, dding further complexity to wht is lredy difficult tsk. This complex mentl process constitutes universl problem to be solved not only for surgeons using 2D informtion when operting vsculr lesions in 3D spce, but lso for the endovsculr opertor forced to use similr mentl processing to trnslte 2D imges. In order to monitor nd control the ctheter positions nd the delivery of embolic devices nd gents in complex vsculr structure such s the CS, complete understnding of 3D ntomy cn become crucil. Four min steps compose 3D ngiogrphy ( Siemens system): Dt cquisition: (Fig. 7.59) C-rm rottions between 5 nd 20 s cquiring imges in projection mtrix of (1 k) or (2 k) Dt trnsfer: The dt re trnsferred using fst 1:1 connection (100 Mb/s) Dt reconstruction: Using the modified cone bem method of Feldkmp, xil slice imges re clculted in mtrix of or Dt processing: Imge post processing is performed using dedicted softwre pckge (Syngo DynCT, Leonrdo, Siemens) in VRTs (In Spce) or s MPRs or MIPs for cross-sectionl imging The cquisition is obtined using rotting C-rm with vrious progrm settings, depending on the clinicl nd dignostic question. A stndrd 3D-DSA is performed using 5- or 10-s rottions, obtining opcified 130 or 273 projections. After positioning the ptient s hed in both plnes into the iso-center, the C-rm moves to two predetermined positions before strting the ctul rottion. A 3D-DSA consists of n initil msk run nd second filling run. Projections re cquired in identicl positions of the C-rm nd ngle triggered, llowing for precise projection-ngle determintion for the reconstruction. The totl ngle per rottion is typiclly 200 or 220. During the filling run, contrst medium is injected either mnully or using power injector ccording to the length of rottion. A typicl injection protocol for 10-s rottion is 2.5 cc/s, totl of 28 cc. Imges re then trnsferred to worksttion for post processing nd utomticlly reconstructed using either or mtrix, either s subtrcted or non-subtrcted dtset. Secondry reconstructions cn be performed using vrious mtrix sizes, kernels nd volumes of interest (VOIs). The steps necessry to obtin 3D reconstruction my slightly differ mong vrious mnufcturers. The Allur system from Philips for exmple cquires usully filling run only. For subtrcted reconstruction, the msk run hs to be performed seprtely s second step which cuses dely nd possible dditionl motion rtifcts Dul Volume Technique (DVT) (Fig. 7.60) Current softwre for ngiogrphic systems (Axiom Artis dba, Siemens Medicl Solutions) llows for seprtely reconstructing the two components of DSA rottionl ngiogrm. The msk run is reconstructed to provide informtion on rdiopque objects within the projection field like bony structures, coils, clips or stents. Then, second reconstruction is performed using the subtrcted filling run, which contins only informtion on vsculr structures filled during the rottion. In 5-s rottion minly rteries will be filleds, but during 10-s rottion cerebrl veins will lso be filled. Both volumes cn be loded nd interctively displyed on the Leonrdo (Siemens Medicl Solution) worksttion. The user cn freely choose the extent to which ech volume is shown. This tool, initilly developed to better seprte coils or clips from residul or recnlized neurysms, llows excellent imging of the vsculr-osseous reltionships in complex ntomicl regions. DVT permits visuliztion of vsculr nd osseous ntomy simultneously nd llows for identifying their precise ntomicl reltionship without interfering with ech other. DVT is further helpful for demonstrting coil mss, clip or stent in reltion to n neurysm neck. Consequently, neurysm regrowth, misclipping nd stent mlpposition cn be detected more esily thn on 2D-DSA imges Angiogrphic Computed Tomogrphy (ACT), DynCT (Siemens), C-rm Flt Detector CT (FD-CT), Flt Pnel CT (FP-CT) or Cone Bem CT (Figs. 7.58, 7.59, ) The integrtion of flt detectors into rotting C-rms in combintion with higher number of projections per rottion hs led to nother new modlity tht llows cross-sectionl CT-like imges to be obtined. The use of multiplnr reconstructions, bsed on rottionl ngiogrms or rdiogrms, is per se not new (Text continues on p. 146)

153 144 7 Rdiologicl Dignosis of DCSFs b Fig Biplne FD system with lrge FD (30 40 cm) for the A-plne nd smll FD (20 20) for the B-plne (or two lrge FDs re currently lso vilble). Reconstructions bsed on rottionl ngiogrms (rdiogrms) llow crosssectionl (CT-like) imging tht provides soft tissue visuliztion (DynCT) superior to tht obtinble with imge intensifier systems (The Methodist Hospitl Houston) c Fig Cone bem CT is bsed on the mesurement of n entire volume (cone) in one single orbit, obtined with either imge intensifier or flt pnel detector systems (from SUETENS 2002) Fig c. The rotting C-rm is cpble of quiring up to 543 projections (20 s, increment 0.4) tht re mostly used to reconstruct 3D dtset within less thn minute. This 3D volume cn be viewed in cross sectionl imging mode either s multiplnr reconstructions (MPRs), mximum intensity projections (MIPs) or in volume rendering technique (VRT)

154 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 145 b Fig DynCT (543 projections in 20 s, bone norml ), 1 mm MPR. Intrprocedurl bleeding tht occurred during AVM emboliztion. Note, lthough the contrst resolution is lower thn in conventionl CT, hyperdensities cused by intrprenchyml blood (here mixed with some contrst fter vessel perfortion) become visible. In this cse, no significnt mss effect nd no hydrocephlus ws noted so tht the procedure could be continued c Fig Dul volume technique (DVT). Both rottionl sweeps re used for reconstructing two seprte dt sets. The filling run provides the vsculr ntomy, while the msk contins the relevnt bckground informtion, usully consisting of high-contrst objects, in this cse the pltinum coils. Both volumes re simultneously viewed, while the mount of informtion being displyed cn be freely chosen Fig DynCT (543 projections in 20 sec, bone shrp ) 20 mm MIP, shows postemboliztion cst of Onyx fter endovsculr occlusion of DAVF of the cribiforme plte (inset). The resolution of high-contrst objects, such s cst of liquid embolic mteril is more detiled thn chievble by digitl rdiogrphy or conventionl CT. This dt set ws obtined immeditely postemboliztion, while the ptient ws still under generl nesthesi on the ngio tble

155 146 7 Rdiologicl Dignosis of DCSFs nd hs been reported in number of clinicl pplictions. Its clinicl use, however, hs been limited to multiplnr reformtted imges for visuliztion of high-contrst objects, such s contrst filled vessels or bony structures (3D myelogrm, etc.). In the pst, cross-sectionl imging in neurordiology hs been minly performed using conventionl CT nd MRI. The use of rotting C-rms in the ngiogrphic suite ws initilly focused on 3D imging of intrcrnil vsculr lesions such s neurysms nd AVMs, employing minly volumerendering techniques. Conventionl CT or X-ry computed tomogrphy produces cross-sectionl imges, bsed on X-ry ttenution. The word tomogrphy stems from the Greek words tomos (slice) nd grphe (write). Angiogrphic computed tomogrphy (ACT, when contrst is used to opcify vsculr structures) or DynCT (Siemens) is similr cross-sectionl imging modlity bsed on the cone bem lgorithm from Feldkmp, while in conventionl CT prllel bem geo metry, or in recent CT genertions fn bem geometry, is usully employed. The terms flt detector CT, flt pnel CT or C-rm CT re menwhile synonymously used for wht is techniclly cone bem CT. The cone bem CT technology using C-rms ws pioneered by Siemens but is menwhile implemented by other vendors too (X-perCT by Philips). The description of technologicl detils in the following chpter is bsed on the use of the first instlled ngiogrphic bi-plne FD system t the Methodist Hospitl Houston, Texs. This system hs been improved nd continuously upgrded since September The cone bem reconstruction lgorithm is the bsis for rottionl 3D ngiogrphy, where the 3D dtsets re reconstructed from series of 2D projections. The method is derived from the stndrd fn bem formul. The density in one voxel is obtined s the sum of contributions from ll projections through the voxel while the ngle between two projections (increment) nd the distnce between the X-ry tube nd the detector re tken into ccount (Suetens 2002). With the introduction of FD technology into modern ngiogrphic systems, cross sectionl imging in the ngiogrphic suite hs gined importnce providing so-clled low-contrst imging. In prticulr, the improved contrst resolution due to the wider dynmic rnge (see bove) currently llows for soft tissue imging in the ngiogrphic room. Using 20-s single rottion cquiring 543 non-subtrcted projections (Axiom Artis, Siemens), contrst resolution of < 10 HU cn be chieved, llowing for visulizing low contrst structures such s grey nd white mtter nd the delinetion of brin tissue from the intrcrnil ventriculr system. In other words, this cross-sectionl imging llows for visuliztion of hyperdense intrcrnil lesions such s subrchnoid hemorrhge (SAH), hemtom cused by vessel perfortion during n endovsculr tretment or developing hydrocephlus. Erly recognition nd monitoring of intrprocedurl complictions during neuroendovsculr tretment hs become possible nd is of gret vlue for interventionl neurordiologists nd endovsculr neurosurgeons (Hern et l. 2006). DynCT contributes to the overll sfety of EVT (Figs nd 7.62), which is of prticulr importnce for the often time-consuming trnsvenous emboliztions of DAVFs or DCSFs Imge Post-Processing (Figs ) Vrious softwre tools for post-processing nd nlyzing ngiogrphic volume dt sets re in use tody. Similr to CTA, they employ dedicted disply modes, such s MPRs, MIPs or VRTs. Becuse not every interventionl neurordiologist or endovsculr neurosurgeon my be fmilir with 3D imging some of the bsic principles re explined in the following. 1. MPR: Multiplnr Reformtting technique to disply up to three orthogonl cut plnes using the verged voxel vlues long one bem, which cn be correlted with true 3D imge. 2. MIP: Mximum Intensity Projection technique to disply up to three orthogonl cut plnes using the mximum voxel vlues long one bem. 3. VRT: Volume-Rendering Technique technique to visulize the structure of volume dtsets. 4. SSD: Surfce Shded Disply technique to visulize surfce tht corresponds to n isovlue in the dtset. 1. Multiplnr Reformtting (MPR) is one of the oldest visuliztion techniques used for viewing 3D medicl imges, providing the possibility for n rbitrrily positioned nd oriented 2D plne to be plced in 3D dt set so tht the projection of the dt on tht plne my be viewed. In modern softwre pckges there re usully three plnes simultneously shown, ech of them corresponding to one of the mjor xes, sgittl, coronl nd xil (Fig. 7.63). This llows for precise orienttion nd locliztion of ny object in the 3D dt set. The

156 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 147 Sgittl MPR b Coronl MPR Suprclinoid ICA CS ICA CS Infrclinoid ICA CS CS c Axil MPR d MPR CS ICA CS e MIP ICA CS ICA Fig e. Cross-sectionl imging in the ngiogrphic room. c 1-mm Multiplnr reformtted imges (MPRs) of DynCT (20 s, 543 projections, 20% of 300 mg Iodine, 2 cc/s, 40 cc) showing the CS region. Views re vilble in three orthogonl plnes (sgittl, frontl nd xil) tht cn be tilted nd rotted into ny desired ngle. Curved reformtted imges re lso possible. Note the clcifictions of the crotid wll on both sides (rrows). d e 5-mm MPR nd 5-mm MIP: Incresing thickness of the MPR cuses blurring of the object contours while the MIP remins reltively shrp, llowing for delinetion of the ICA, surrounding bone nd clcifictions

157 148 7 Rdiologicl Dignosis of DCSFs thickness of the plnes cn be djusted to the size of the object (e.g. vessel dimeter) nd they cn be moved long relevnt xis to provide the desired view. Curved reformtions in ny direction re possible nd extremely useful to provide views inside the lumen of curved ntomic structure (vessels, osseous cnls etc.). 2. Mximum Intensity Projection (MIP) is volumetric imging technique tht uses only the brightest voxel (hence mximum intensity) long one bem to be projected into picture (Fig. 7.64). Objects with high density, such s contrst filled vessels, clcifictions nd metl structures such s clips, stents nd coils, pper brighter while the surrounding tissue remins drk grey. MIPs cn visulize very smll structures with high density; however, they do not provide depth perception. Thus, the viewer cnnot decide if bright structure is in front or in the bckground of the volume he is looking t. To improve the visuliztion, bckground structures suppressed by pproprite windowing, whereby n effect of semi-trnsprency cn be obtined. The volumes cn be interctively rotted nd three orthogonl plnes cn be simultneously visulized. The lck of depth informtion hs to be considered when viewing thick MIP imges (10 40 mm), becuse it my exggerte certin findings nd more importntly, obscure others (Rybicki et l. 2006). MIPs re visully ppeling nd provide superior detection of detils in high contrst objects such s very smll vessels or stents. For dignostic purposes or mesurements, MIPs re of limited vlue nd should be used prudently. 3. Volume Rendering Technique (VRT), probbly the most widely pplied method for disply of threedimensionl structures, uses men vlue corresponding to vlue of bsorption s seen in n X- ry imge (Figs nd 7.66). A volume rendered imge represents the complete dtset nd does not rely on surfce informtion. All voxels re rendered with specificlly ssigned opcity (vn Ooijen nd Irwn 2006). In contrst, in n MIP obtined out of 256 slices of mtrix, less thn 1% of the originl informtion is used. In other words, the originl informtion is reduced to bout 1%. Tody, VRT hs replced SSD in most softwre pckges on grphic worksttions. In VRT, the identifiction of different tissue types is bsed on grey or colorgrey vlue rendering, in which the visible prt cn be modified using so-clled histogrm (trnsfer function). Since ll informtion is stored, the rnge of visible objects cn be interctively modified by chnging this window nd only structures relevnt to the viewer, e.g. blood vessels, coils nd clips, re mde visible. This trnsfer function is dvntgeous s compred to SSD, where fter ech chnge of the threshold new clcultion is required. The dditionl use of colors llows for better seprtion of certin structures such s vessels nd coils or clips. A volume rendered imge is intrinsiclly not 3D imge, but opcity nd brightness cn be modified so tht the surfce of dtset becomes more-dimensionl ppernce. Using specific cutting tool nd six vrible clip plnes, further editing is possible. Bones or overlying vessel loops cn be eliminted so tht only relevnt structures in the volume of interest (VOI) re left. Semitrnsprent imges my be of prticulr vlue when visulizing AVMs or cerebrl neurysms. For vsculr lesions t the skull bse or prsellr region, it is generlly of interest to visulize djcent osseous structures s well. For this purpose, both non-subtrcted projections nd pproprite windowing llows for the disply of bony structures in more or less rdiolucent (X-ry like) or trnsprent fshion. This fcilittes identifying not only the vsculture of the skull bse, but lso its ctul reltionship with imbedding osseous structures D Studies of the Cvernous Sinus Region (Figs ) In the pst, both rdiologists nd mnufcturers hve given little ttention to the imging of norml vsculr ntomy using 3D ngiogrphy. Angiogrphic visuliztion of smll rteries, such s durl brnches of the cvernous ICA, hs been limited by the sptil resolution nd the trditionl 2D disply of stndrd DSA. Thus, 3D imging of smll rteries nd veins in complex ntomicl regions would hve significnt vlue s it contributes to our understnding of both norml vsculr ntomy s well s pthologicl lesions. The following section focuses prticulrly on 3D imging of the cvernous sinus region using rottionl ngiogrphy nd rdiogrphy. Of mjor interest for the uthor ws: (1) the visuliztion of smll nstomosing brnches between ECA nd ICA territories when coursing through the skull bse, middle crnil foss nd the cvernous sinus nd (2) the detiled venous r-

158 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) mm MIP ICA PCS b IMA High opcity b 5 mm MIP c Low opcity (trnsprent) c 20 mm MIP Bone mode Fig c. Mximum intensity projections (MIPs) of crotid syphon in different thicknesses. With incresing thickness of the MIP, more objects, or n entire vessel segment become visibile, while other informtion, such s the non-clcified lumen my become obscured by overly with djcent osseuous structures. There is no depth perception (c) Fig c. Volume rendering techniques (VRTs) of the sme dtset in three different viewing modes using sgittl clip plnes. Mnipultions of the trnsfer function, opcity nd brightness permit choosing disply setting tht provides the most relevnt informtion. Incresing opcity () llows visuliztion of vessels closer to the viewer, such s the prcvernous sinus (PCS) nd the internl mxillry rtery (IMA). b The trnsprent mode is useful for identifying vsculr loops. Clcifictions of the ICA wll my be obscured nd re better seen in c. Note lso the difference in depth perception between the VRTs nd the MIPs in Fig. 7.64c

159 150 7 Rdiologicl Dignosis of DCSFs b c d Pyrmid Clivus e Clivus Fig e. DynCT of dry skull, VRTs (B in trnsprent mode resembling skull X-ry): 20 s rottion, 543 projections, Excellent visuliztion of the skull in opque (), nd trnsprent (b resembling skull X-ry) mode. Bony lndmrks nd formin of the middle crnil foss cn be redily identified t the inner surfce (c, d). e View onto the outer surfce of the centrl skull bse. Compre to Fig Formen ovle 2 Formen spinosum 3 Formen rotundum (cnlis rotundis) 4 Formen mgnum 5 Superior orbitl fissure 6 Crotid cnl 7 Jugulr formen 8 Petroclivl fissure 9 Formen lcerum 10 Anterior clinoid process 11 Posterior clinoid process

160 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 151 MPR b MPR 3 c 6 MPR Fig c. DynCT of dry skull: 0.2 mm multiplnr reconstructions (MPRs) (20 s rottion, 543 projections, ). Visuliztion of the osseus skull bse in orthogonl plnes, usully sgittl, coronl nd xil (oblique nd curved MPRs re possible). Osseous formin, cnls nd fissures cn be simultneously visulized in three orthogonl plnes, here showing two xil (, b) nd one coronl plne(s) (c). Note in coronl plne, the most common lignment of the three posterior formen of the pterygopltine foss crrying ech n rtery tht my serve s nstomosis between ECA nd ICA territory: The formen rotundum most lterl nd superior, the vidin cnl in the middle, nd the pterygovginl cnl most medil nd inferior (Rumboldt et l. 2002). In this exmple the pterygovginl cnl is incomplete (semicnl). Also visible is the SOF tht is not directly connected to the PPF, but indirectly vi the orbit nd IOF. 1 Formen ovle 2 Formen spinosum 3 Formen rotundum (cnlis rotundis) 4 Formen mgnum 5 Superior orbitl fissure 6 Crotid cnl 7 Vidin cnl 8 Petroclivl fissure 9 Pterygovginl cnl (semicnl) chitecture of the CS nd its connections with the IPS/IJV junction. The mjority of smll durl rteries tht rise from the ECA enter the intrcrnium through skull bse formin, nd re often not sufficiently visulized on stndrd 2D-DSA. Although their topogrphy hs been studied extensively by vrious investigtors (Prkinson 1984; Rhoton et l. 1984; Lsjunis et l. 2001; Lng 1979; Hcein-Bey et l. 2002), these vessels re not lwys esy to identify due to their smll size nd overlying bony structures. Likewise, the venous ntomy in this region is to dte not fully understood due to limited cpcity of 2D-DSA to visulize peculir nd complex venous structures. The origin nd course of durl brnches involved in the complex ngiorchitecture of the cvernous sinus hs been the topic of numerous ntomiclrdiologicl studies. Superselective rteriogrphy of ECA brnches hs been introduced by Cstigne et l. (1966), Newton nd Hoyt (1968) nd others (Djindjin nd Merlnd 1978). The extensive work of Djindjin nd Merlnd (1978) hs invlubly contributed to our understnding of crnio-fcil vsculr ntomy nd still remins unmtched to this dte. Lsjunis nd Berenstein (1987) nd (Text continues on p. 156)

161 152 7 Rdiologicl Dignosis of DCSFs b c d SOV ILT e f ICA ICA CN3 CN6 Fig f. Cvernous sinus ntomy. DSA (prior) nd ACT (fter) in ptient undergoing intrcrnil stenting. Note the opcifiction of the mrginl tentoril rtery (short rrow) nd the clivl brnch rising from the MHT (long rrow). b Erly filling of the CS (sequentil subtrction). c f Simultneous opcifiction of the ICA (bright) nd the CS tht drins into the IPS (double rrow) nd IPCV (rrowheds). c Lterl, d oblique view. e Posterior view tht shows the course of the clivl rtery (rrow) crossing the IPS (double rrow). f Delinetion of the oculomotor nd the bducens nerves within the opcified CS. CN3 is the most cephled nerve, while CN6 is considered the only nerve lying in fct within the CS. ACT hs very low temporl resolution s compred with DSA, but provides vluble three dimensionl ntomic informtion

162 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 153 b Petrous pex ICA ICA ICA Petrous pex Clivus Fig. 7.69,b. Visuliztion of crnil nerves in the CS using ACT (10 s 3D-DSA, Axiom Artis). The xil () nd coronl (b) MIPs (0.2 mm) show n indirect opcifiction of the CS (sterisks), delineting its vsculr nd neurl contents. The course of the bducens nerve (CN6) through Dorellos cnl between the petrous pex nd the clivus becomes well visible. The bowshped Dorello s cnl, through which the bducens nerve courses before reching the cvernous sinus, is locted inside venous confluence, which occupies the spce between the durl leves of the petroclivl re. The bducens nerve perfortes the dur, courses below the petrosphenoidl ligment of Gruber nd reches the lterl wll of the intrcvernous ICA b c VRT ICA Formen ovle Formen ovle d ICA Formen ovle MIP Formen ovle Fig d. Angiorchitecture of ECA/ ICA nstomoses using 3D-DSA. Right ECA rteriogrm in AP () nd lterl (b) views. Arteriovenous shunt, supplied by the MMA with n dditionlly enlrged AMA (short rrow) giving rise to brnch tht courses crnilly to rech the ILT (rrow). 3D Rottionl ngiogrphy with Neurostr (nonsubtrcted, 8 s rottion, 80 projections), lterl view. c The VRT shows the AMA (short rrow) nd its connection to the posteromedil brnch of the ILT (rrow). In xil view (MIP), the relted osseous structure (d) is visulized nd the course of the nstomosing brnch (rrow) through the formen ovle (dotted circle) cn be seen. (Modified from Benndorf 2002)

163 154 7 Rdiologicl Dignosis of DCSFs b c d Formen spinosum Formen ovle MMA e Formen ovle MMA Formen spinosum Fig e. Angiorchitecture of DCSF using 3D-DSA. Right ECA rteriogrm AP () nd lterl (b): Very smll rteriovenous shunt t the right CS (sterisk), exclusively supplied by brnches of the right ECA: Durl brnch of the AMA (thin rrow) nd rmus recurrens (rrow), rising from the MMA just fter pssing the formen spinosum (dotted circle). The CS nd the SOV (short double rrow) re incompletely filled, indicting prtil thrombosis. c e 3D Rottionl DSA (non-subtrcted, 8 s rottion, 80 projections, Neurostr) VRTs: The recurrent brnch of the MMA (rrow) courses in n nteriorly convex curve first medilly nd then posteriorly (e). The trnsprent mode in AP view (c) revels ccurtely the ngiorchitecture; some smll pedicles re better recognizble thn on DSA. e, f Axil views onto the middle crnil foss, demonstrting the course of this recurrent rmus (rrows), nd showing the AMA (thin rrow) tht enters the intrcrnium through the formen ovle (dotted circle). Asterisk: CS, double white rrow: IOV. (Modified from Benndorf 2002).

164 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 155 b c d e f g Fig g. Locliztion of fistul site using ACT. Left ICA rteriogrm lterl rteril () nd venous phse (b): A smll AV fistul (sterisks) of the proximl SOV (short rrows), supplied by single pedicle of the meningel rmus recurrens of the OA (rrow) tht my mimic DCSF (see introrbitl AV shunt in Figs nd 7.45). The venous phse shows tht the fistul site is ctully remote from the CS (BW sterisk) nd superimposed onto the SPPS (rrowheds). 3-Rottionl Angiogrphy (ACT with Neurostr, 14 s rottion, 132 projections). Thick MIPs, AP lterl (c) nd AP (d) views: Visuliztion of the AV fistul with feeding pedicle rising from the OA nd drining vein in good qulity, providing ntomicl informtion equl to the DSA. e f Coronl nd sgittl, thin MPRs llow for locliztion of the fistul site (rrow) t the level of the superior orbitl fissure (SOF). g In the VRTs, the course of the supplying feeder (rrow) rising from the OA nd trveling lterl to the fistul point (rrowheds) is seen either from crnil (left) or from cudl (right). (Modified from Benndorf 2002)

165 156 7 Rdiologicl Dignosis of DCSFs Lsjunis (1984) followed this trdition in the lte 1980s, using meticulously superselective rteriogrphs, obtined by mnul subtrction techniques nd providing n imge qulity superior to tht of DSA t tht time. Digitl subtrction ngiogrphy, developed in the lte 1970s, but remrkbly improved in the 1980s nd 1990s, menwhile llows for visulizing in greter detil rteril nd venous ntomy in one of the most complex ntomic regions of the humn body. But even this high-resolution DSA, the current gold stndrd for vsculr imging, is limited in its cpcity to demonstrte relibly nd understndbly peculir vsculr ntomy. Thus, not only in the pst but to dte, ngiogrphic studies hve frequently been verified nd complemented by detiled ntomic dissections (Lsjunis nd Berenstein 1987; Lsjunis 1984; Prkinson 1963, 1984; Rhoton et l. 1979). Colored csts of cdver vessels, obtined by injecting liquid plstics, were used to study detiled rteril nd venous ntomy (Rhoton et l. 1984; Sn Milln Ruiz et l. 2002; Mrtins et l. 2005; Rhoton 2000). On the other hnd, improved modern X-ry technology, using high resolution 3D DSA nd contrst enhnced DynCT, is cpble of displying 3D vsculr nd osseous ntomy in the CS region in surprising qulity, fcilitting remrkbly rdio-ntomicl studies of this fine vsculr network in vivo ( Benndorf et l. 2000; Hiu et l. 2009). The usefulness of 3D ngiogrphy for endovsculr tretment of direct crotid cvernous fistuls hs been recently reported by Kwon et l. (2005) who found 2D-DSA less relible in detecting remining pseudoneurysm fter blloon detchment. 2D- DSA indicted the possibility of trction-induced instbility or intr-rteril blloon position, but 3D volume rendered imges were superior in terms of dignostic informtion. Cut plnes, perpendiculr to the vessel xis, provided orthogonl views, which permitted better identifiction of residul fistul. Becuse detchble blloon is contrst filled, s is the prent vessel, both structures cn be mde visible in their precise reltionship. This type of imging cn become very helpful, fter incomplete or subtotl blloon occlusion The exct site of CCF cn ngiogrphiclly be identified performing Huber s or Mehringer s mneuver (Huber 1976; Mehringer et l. 1982); however, its exct orienttion nd size within the crotid wll cn remin difficult to evlute due to unopcified blood nd lrge AV shunt volume, even when using higher frme rtes such s 15 f/s (Fig. 7.43). VRTs my fcilitte the identifiction of such fistul site; however, its mesured size depends on the threshold tht is subjectively chosen by the viewer, problem tht is not solved by most current 3D softwre. Ishid et l. (2003) described CCF, identified by 3D-DSA using VRT nd virtul endoscopic view. The ntomicl orienttion of the fistul ws esily understood. Yet its dimensions were not found mesurble in precise mnner nd the blloon size could not be selected bsed on the 3D-DSA. Both studies indicte n incresing use of 3D-DSA for evlution of crotid cvernous fistuls. MPRs or MIPs obtined by either DSA or ACT (contrst enhnced DynCT) re in this regrd superior to VRT s they llow more precise nlysis of the vessel morphology. Less dependnt on subjective thresholding, the site of the crotid ter cn Fig h. Angiorchitecture of DCSF t the right CS using ACT. Right ECA injection AP nd lterl view (, b). The AV shunt is locted t the right CS nd supplied by severl durl brnches rising from the AMA (thin rrow), MMA (long thick rrow) nd distl IMA (short thick rrow). c h 3D-DSA (non-subtrcted, 14 s rottion, 132 projections, mnul injection, Neurostr). This exmple shows the course of durl brnches supplying the AV shunt t the CS (sterisk); MIPs in coronl (left) nd sgittl (right) plne. The AMA (long rrow) courses verticlly nd psses the formen ovle to rech the intrcrnil durl network supplying CS AV shunt (c,d). The AFR (short thick rrows) courses posteriorly nd verticlly through the cnlis rotundis nd connects to the sme network (e,f). The ACT further llows for identifying precisely the fistul site (sterisk, see lso Figs. 7.72, 7.78). It is interesting to observe tht n dditionl brnch ppers to rise from the sphenopltine portion of the IMA, nd courses similrly, but ctully superior to the AFR, posteriorly to rech the AV shunt (rrowheds). This smll rtery psses pprently through both the inferior nd superior orbitl fissures nd is lso demonstrted in Figs Insets: showing the pssge of corresponding brnches through the formen ovle (xil view in e), nd through the formen rotundum (coronl view in f). Additionlly seen in g is the contribution from petrosl brnches of the MMA (long thick rrows), s well s from clivl brnches of the APA (dotted rrows). Asterisk: CS AV shunt, PPF: Pterygopltine foss

166 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 157 b c d e f PPF g h MMA MMA APA

167 158 7 Rdiologicl Dignosis of DCSFs be esier identified; size mesurements for potentil use of detchble blloons becomes more relible. In ddition, ccompnying osseous lesions, such s frctures of the temporl bone, cn be detected. For precise locliztion of durl CSF, ACT cn be helpful too, s shown in n erlier study by the uthor using previous II technology (Figs. 7.71, 7.72). Recently, the vlue of 3D-DSA hs beens further enhnced by DVT, which specificlly improves the understnding of ntomicl rrngements between rteries or veins nd their surrounding osseous structures. As shown in Figures 7.75 nd 7.77, DVT llows for correlting the course of ny vessel within its osseous neighborhood, long bony sutures nd cnls s well s through formin in the skull bse. The reconstruction mtrix is usully , providing voxel size of pproximtely 0.1 mm. Contrst ppliction is performed using 4- or 5-F dignostic ctheter, plced for stndrd ngiogrphy in the ECA or ICA. In some cses, superselective injection using microctheter, plced within n ECA brnch, the IPS or the CS itself, is useful nd mkes obtining opcifiction of selectively chosen territory possible. The dvntge of DVT compred to ntomic dissections is tht tissue cn be virtully cut in slices without destroying it. The 3D volume remins entirely intct nd such computerized grphic dissection cn be repeted nd reproduced s often s necessry. Also, the vessels hve their physiologic dimensions s they re filled with blood under certin pressure. MPRs simultneously disply three different cut plnes, fcilitting the orienttion in spce for the opertor enormously. Curved reformtting mode llows for reconstruction long irregulr nd curved ntomic structures such s cnls or vessel courses. Figures show how the identifiction of smll rteries feeding n rteriovenous shunt is fcilitted. In cses where numerous feeders or network of rteries supplies DCSF, 2D-DSA my not lwys provide sufficient informtion to fully understnd the vsculr topogrphy. Despite extensive descriptions in textbooks using dissections nd complex illustrtions, mentl trnsltion of 2D imges into 3D ntomy remins difficult tsk, even for the experienced ngiogrpher. Figures show tht in some cses of dominnt ECA supply of cvernous AV shunt, the vessel referred to s rtery of the formen rotundum represents in fct not single vessel but my insted consist of two or more smll brnches, pssing through the cnlis rotundis. This observtion hs not been reported in the literture. Furthermore, in mny rteriogrms, the AP projection shows bundle of vessels coursing posteriorly tht re difficult or impossible to distinguish from ech other, even when using lterl views. Figure 7.76 is n exmple of DAVF t the sigmoid sinus with this type of complex rteril supply from the internl mxillry rtery. By using mm MPRs, 3D imging of the osseous skull bse in excellent qulity is possible nd llows depiction of fine detils such s formin nd cnls tht provide the pssge for nerves nd vessels, s well s for spred of infectious or tumorous diseses. Every single brnch of the rteril network, e.g. the MMA, AMA, the vidin rtery, nd the rtery (-ies) of the formen rotundum, cn be relted to their surrounding osseous structure, llowing for precise identifiction. These thin slice MPRs re simultneously displyed in three perpendiculr plnes, so tht ech vessel cn be followed long its ntomicl course in every possible projection, not obtinble on 2D- DSA. For exmple, orthogonl views of formin nd cnls, nd the simultneous disply of corresponding sgittl nd xil projections, re prticulrly helpful in imge nlysis. Due to their trditionlly nlogous use in most ntomicl textbooks, xil plnes for disply of vsculr ntomy re didcticlly useful. In cses where n rtery tkes n irregulr or oblique course, curved reformtted imges cn be used ccordingly. This technique, fcilitting understnding of norml vsculr ntomy, my lso be used to detect rteries nd veins (see below) tht hve been widely ignored in textbooks. For exmple, Figs nd show n rtery tht ppers quite similr to the rtery of the formen rotundum, rising from the distl internl mxillry rtery nd coursing posteriorly nd crnilly. This rtery is pprently very smll since it ws noticeble only in cses with AV shunts t the CS. It lies superior to the AFR on lterl DSA views nd courses through the IOF nd then through the SOF to rech the CS. DynCT nd DVT show more clerly the topogrphy of this distl IMA brnch tht represents possibly n dditionl ECA/ ICA nstomosis. The distl portion of the IMA, the pterygopltine rtery, s described in the current literture, divides into three posterior brnches from lterl to medil (in AP views): the AFR, the vidin rtery nd the pterygovginl rtery (Lsjunis et l. 2001; Hrnsberger et l. 2006; Osborn 1980). An dditionl brnch coursing bove the AFR crni- (Text continues on p. 163)

168 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 159 b c d PPF e Fig e. ACT (contrst enhnced DynCT) using FD technology: 20 s, 20% contrst (300 mg), 2 cc/s, 40 cc, 543 projections, Axiom Artis.,b ECA injection lterl nd AP views show meningiom, supplied by enlrged brnches of the MMA, AMA nd IMA. c e ACT in 2 mm MIPs showing the cnl nd its vsculr (rteril) contents, c in sgittl, d in xil nd e in coronl plnes. It revels the course of the rtery of the formen rotundum through the cnlis rotundis. This rtery is often not just single vessel, but my consist of 2 3 brnches tht follow this route nd my resemble smll network (see lso Figs 7.75, 7.76)

169 160 7 Rdiologicl Dignosis of DCSFs SOV b PPF c d SOV SOV AMA in FV FO MMA in FS AMA MMA Fig d. Trumtic (D)CSF. DSA, ACT nd DVT (10 s 3D-DSA, Axiom Artis). Externl crotid rteriogrm, lterl view, shows fistulous communiction between the AMA (thick rrow) nd the CS. The AV shunt hs lso recruited two distl brnches of the sphenopltine rtery, tking the course of the rtery of the formen rotundum (smll rrow: MMA). b 0.4 mm MPR, sgittl view through the pterygopltine foss revels both brnches pssing through the cnlis rotundus (inset: Axil view), thus could be clled rteries of the formen rotundum. c (DVT) nd d (0.2 mm MIPs) revel tht the AMA (rrow) does not ctully course through the formen ovle s observed in the mjority of the cses (78%), but through the sphenoidl emissry formen (formen of Veslius) tht lies directly medil nd nterior. PPF: Pterygopltine foss, sterisk: CS, SOV: Superior ophthlmic vein, FO: formen ovle, FS: Formen spinosum, FV: Formen venosum. Double rrow: Dringe into pontomedullry nd spinl veins. Fig h. 3D ngiorchitecture of DAVF of the trnsverse sinus, supplied by multiple feeders, rising from the IMA, AMA nd MMA., b Left ECA injection AP nd lterl. The network of enlrged feeding pedicles is hrd to evlute in AP projection, while in lterl view this seems t lest prtilly possible. Three mjor feeding pedicles pper to rise from the sphenopltine rtery. 1) Superior: Artery trveling through the IOF nd SOF (thin rrows); 2) Middle: Artery(ies) of the formen rotundum (2 vessels, short thick rrows). 3) Inferior: Vidin rtery (double rrows). The brnch coursing through the IOF nd SOF ppers to rise distl from the AFR nd courses in lterl view lmost prllel, but bove the AFR. The vidin rtery is the most medil nd most distl brnch of ll involved feeders. c h ACT (c,d) bsed on non-subtrcted reconstruction of 10 s 3D-DSA (273 projections, Axiom Artis). 20 mm MIPs show the origin nd course of the vessels in their reltion to the osseous structures: through the cnlis rotundis (e); Through the vidin cnl reching the crotid (f); Through the IOF nd SOF (g). Insets: Corresponding xil views. Coronl view (h) showing the three min feeding vessels, while coursing through the corresponding bony formin in orthogonl projection. The pterygovginl rtery tht usully lies medil to the vidin rtery nd psses through the pterygovginl cnl is not shown here (see Fig. 7.80j) SPA: Sphenopltine rtery in the pterygopltine foss

170 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 161 b c MMA d SPA SPA AMA Formen ovle MMA e f Sphenoid sinus g SOF h SOF IOF SOF

171 162 7 Rdiologicl Dignosis of DCSFs b SPA AMA c d SPA Fig d. DAVF of the trnsverse sinus, supplied by multiple feeding vessels, rising from the IMA, AMA nd MMA (sme cse s in Fig. 7.76). DVT bsed on 10 s 3D-DSA. Oblique view from posterior. b Lterl view using sgittl clip plnes. c,d PA nd AP view using coronl clip plnes. Although providing lower sptil resolution, DVTs enble one to pprecite better the three-dimensionl osseous-vsculr reltionships shown in Fig Thin rrow: The brnch pssing first through the IOF nd then through the SOF, rising more distlly thn the AFR nd courses in lterl view lmost prllel to the AFR. Short thick rrows: Artery(ies) of the formen rotundum. Double rrow: Vidin rtery s the most inferior rtery in lterl view (pterygovginl rtery not shown). SPA: Sphenopltine rtery

172 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 163 lly through the suprorbitl fissure, s seen by the uthor in severl cses, hs not been reported so fr (see Fig. 7.79). Referring to the osseous structures, which this vessel courses, the nme rtery of the orbitl fissures could be suggested (Fig. 7.79b). It is not cler whether this brnch nstomoses with rmus from the ILT or nother (durl) rtery from the ICA. Becuse it ws only seen in AV shunting lesions of the CS region, more detiled studies re necessry to further vlidte this ntomic disposition. Such n observtion confirms the enormous vlue of high-resolution cross-sectionl nd 3D imging of neurovsculr ntomy. Figure 7.80 shows the indirect supply of long stnding CCF fter ineffective ligtion. In this cse the fistul s supply on the right side comes directly from the suprclinoid ICA. On the left side, however, the ECA ppers to be significnt contributor s well. While the DSA does not give certinty regrding the topogrphy of the dominnt feeder, the MIPs redily show the loction of this specific vsculr segment: in xil views, this rtery projects exctly onto the course of the crotid cnl in the petrous bone, identifying this vessel s revsculrized segment of the petrous ICA. This informtion is difficult to obtin using 2D-DSA only nd, if possible, is likely to require meticulous knowledge of this specific ntomic re. Relting rteries to their corresponding osseous structures in the skull bse provides new level of ccurcy in imging minute rteril ngiorchitecture. Hiu et l. (2009) recently described the efficcy of DynCT digitl ngiogrphy in detecting the fistul sites in durl AV shunts. Thus, currently obtinble ngiogrphic 3D dtsets not only contin more relevnt ntomic informtion. Another mjor benefit is tht this informtion is mde vilble in more didctive mnner s it fcilittes understnding of sptil reltionships between complex structures of different origin. Knowledge of neurordiologicl nd surgicl ntomy remins essentil for lerning endovsculr (nd surgicl) procedures. The mjor drwbck of 3D-DSA nd DynCT, when compred to ntomic dissections, is their limittion to disply non-rdiopque structures. Soft nd ft tissue, nd thus neurl structures in the CS region, cnnot be directly visulized using X-rys, but re well delineted when the CS is opcified. Similr to the dvnced 3D imging of rteril network involving the CS, venous ntomy of the skull bse nd the middle crnil foss cn be studied using modern 3D-DSA nd ACT. Three dimensionl visuliztion of venous ntomy cn in principle be obtined using two different pproches. The first pproch is 3D venogrphy: s prt of reconstruction obtined on the bsis of n rteril injection s is usully performed for stndrd 3D- DSA. During 10-s rottionl ngiogrm, prt of the venous circultion is opcified nd reconstructed. Becuse of contrst dilution nd fewer projections contining contrst-filled veins per rottion, venous structures re usully not sufficiently opcified nd frequently superimposed by rteries dominting the dtset. To reverse this rteril dominnce in the dt set, the rteril injection is strted during the lst seconds of the bck sweep, before the filling run begins. Thus, fewer projections with rteril thn with venous filling re reconstructed nd veins will consequently dominte in the reconstructed 3D volume. This technique, clled 3D rottionl venogrphy, hs been successfully used by the uthor ( Benndorf 2002) using conventionl (II) C-rm system nd is shown in Fig Nishio et l. (2004) hve reported on the vlue of 3D rottionl venogrphy using C-rm mounted flt detector system. The uthors obtined venogrms, bsed on rottionl DSA, using delyed third rottion (filling run) with intervls between 5.5 nd 9 s. The mount of contrst injected ws 13.9 ml (2.2 ml/s) in the CCA nd 12 ml (2.3 ml) in the ICA. It is concluded tht neurosurgeons will benefit from better understnding of venous ntomy in the posterior foss nd the skull bse when choosing surgicl pproches to vsculr or neoplstic lesions in this region. Figure 7.82 shows rottionl venogrphy using the currently vilble FD modlity tht illustrtes the improved visuliztion of venous ntomy by isolting the venous prt of the circultion from the rottionl ngiogrm. The following setting ws used in the cse shown: 5-s rottion, 5-s dely of contrst injection, 2 cc/s injection rte, 10 cc totl volume. Employing projection mtrix, exclusive visuliztion of the venous circultion in the injected cerebrl territory is possible with sptil resolution superior to CTA nd MRA. Other dvntges re tht overlys with rteril structures or vessels from territories other thn the injected ones do not compromise the dignostic informtion. Mitsuhshi et l. (2007) recently reported the successful ppliction of this technique for evlution of inferior petrosl sinus ntomy nd developed new clssifiction bsed on their results. The second pproch is 3D phlebogrphy. Another wy of obtining high qulity 3D dtsets of (Text continues on p. 167)

173 164 7 Rdiologicl Dignosis of DCSFs b c d e Fig o. Angiorchitecture of complex DCSF using ACT., b Right ECA injection AP nd lterl, showing durl AV shunt involving the right CS. Arteril supply is provided by n extensive network of durl brnches rising from the IMA, MMA, AMA nd APA. The venous dringe involves the bilterl CS (sterisk), both SOVs (rrows) nd midline structure, whose ntomic reltionship cnnot be redily determined (B/W sterisk). There is lso fint communiction (dotted rrow) with the left IJV (thick rrow). c e ECA-ACT (20 s, 2% contrst, 40 cc, 543 projections, Axiom Artis) of the left ECA territory shows the extensive rteril network, providing the rteril supply rising lso

174 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 165 f g AOF CS AOF AOF CS AFR ViA SPA MMA SPA h i SOV CS VP VP APA SPA from the APA (clivl brnches). Three orthogonl MIPs provide good overview showing the feeding rteries in bright contrst nd the venous dringe in drk grey. The window level is chosen to disply the osseous bckground s well. In prticulr, xil views like in e cn become very helpful in understnding complex vsculr ntomy. (The cse ws treted successfully by the uthor nd Dr. A. Biondi in 2008 t Pitié-Slpêtrière Hospitl, Pris). f k Higher resolution secondry reconstruction bsed on the sme rottion revels more detils. Min feeding pedicles cn be better seprted, especilly when the thickness of the MIPs is chnged. Arrow: Recurrent meningel MMA brnch. Double rrow: Petrosl MMA brnches. h demonstrtes the more nterior supply from the sphenopltine rtery (SPA) with the rtery of the formen rotundum (AFR), vidin rtery (ViA) nd rteries coursing through the orbitl fissures (AOF). Choosing more posterior plne, the coronl view in i revels enlrged clivl brnches (rrow) of the APA nd petrosl MMA brnches (double rrow) tht seem to supply minly the venous pouch in the midline (VP). Continued on next pge

175 166 7 Rdiologicl Dignosis of DCSFs SOV j k SOF AOF AOF SOV CS l m ICA CS ICS VP CS CS ICA CS n o S VP SOV S VP CS Fig o continued. j k This cse is the third observtion of the forementioned dditionl posterior communiction between the SPA nd the CS re (see Figs. 7.73, ). The brnch initilly tkes verticl course through the inferior orbitl fissure nd courses then horizontlly through the superior orbitl fissure (SOF). Accordingly, Artery of the orbitl fissures (AOF, dotted rrow) my be suggested (see lso Fig. 7.79). This vessel, lso visible in f nd g, is not mentioned in textbooks, nd ws observed by the uthor in cses with AV shunts, cusing enlrgment of ECA-ICA nstomotic brnches. l m The CS ppers to be clerly seprted from the venous pouch nd communictes only vi smll chnnel on the fr left side (yellow rrow). The min fistulous connection is lso visible (red rrow). n This sgittl (midline) plne demonstrtes tht the venous pouch (VP) is not locted on the superior surfce of the clivus nd thus, less likely represents the bsilr plexus, s initilly ssumed. It ppers to be locted inside the bone, delineting the sphenoid sinus (S, inset). o shows further detils on the venous ngiorchitecture tht re impossible to gin from 2D-DSA. The SOV crries n neurysm like pouch (rrow); before pssing the SOF, second, smller venous neurysm (dotted rrow) is seen t the corticl vein tht leves the posterior CS nd courses in prllel to the SOV before turning lterl to rech the temporl cortex

176 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 167 Fig Trditionl description of terminl posterior brnches of the internl mxillry rtery: The rtery of the formen rotundum (superior g 26 ), the vidin rtery (middle, g 17 ) nd the pterygo-vginl rtery (inferior, g 16 ). (From Lsjunis et l. 2001) the intrcrnil venous circultion is to directly inject contrst into veins or sinuses during rottionl DSA. By eliminting rteries completely from the dt set, this technique llows for retrogrde (ginst the physiologicl blood flow) opcifiction of venous structures including veins nd sinuses, obscured during regulr contrst pssge fter rteril injection. The result my, depending on the selectivity of ctheter plcement nd the communictions between djcent veins, show extensive visuliztion of veins nd sinuses not obtinble through rteril injections. Employing DVT, direct comprison of venous structures to the imbedding osseous ntomy in multiplnr cross-sectionl imges is possible. This revels 3D informtion on venous ntomy in much greter detil thn tht vilble by other clinicl imging modlities, including DSA, so fr. In prctice, for the CS region 5-F ctheter is plced t the level of the jugulr bulb or into the IPS (during smpling) to perform either unilterl or even bilterl injections during 10-s DSA or even 20-s ACT (contrst enhnced DynCT). Reconstructions re performed in nonsubtrcted mode, or re obtined s DVT (DSA). Figures demonstrte how this method remrkbly fcilittes the perception of the complex venous ntomy in the CS region. The connections between CS, the ICS nd drining veins such s the IPS nd emissry veins, pssing through the formin, re displyed. Simultneous bilterl injections of contrst provide extensive opcifiction of venous structures, commonly not visulized on stndrd 2D-DSA. Three dimensionl phlebogrphy using ACT nd DVT llows for the most ccurte identifiction of veins nd sinuses embedded in their djcent osseous structures. Venous structures usully obscured on 2D- DSAs by dense bony structures, such s the internl crotid rtery venous plexus (Rektorzik), or 3 4 Fig Additionl posterior brnch (1), rising from the distl sphenopltine rtery nd coursing through the inferior nd superior orbitl fissures to rech the CS. This course ws identified using ACTs nd DVT in three cses. While the AFR (2) often hs posteriorly scending oblique course, this rtery of the orbitl fissures (AOF) courses more verticlly first, nd then turns horizontlly to rech the CS region. 3: Vidin rtery, 4: Pterygovginl rtery. : SOF, b: IOF, c: Formen rotundum, d: Vidin cnl, e: Pterygovginl cnl (Text continues on p. 171) c b 1 2 d e

177 168 7 Rdiologicl Dignosis of DCSFs OA b ILT c d OA SPA SPA e f ICA AMA MMA g h SPA ILT OA ICA OA MMA SPA ILT MMA AMA ICA AMA i j OA PPF AMA

178 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 169 k l ICA ICA m n SOV o SOV Fig o. ACT of CS ntomy. Long-stnding (20 yers) right trumtic CCF, ineffectively treted by crotid ligtion., b Right ECA lterl nd RAO projections: Recruitment of vrious ipsilterl ECA collterls: Artery of the formen rotundum (smll rrow), vidin rtery (double rrow), nd collterl brnches by the scending phryngel rtery (APA, triple rrows). Note the retrogrde filling of the ophthlmic rtery (OA) vi n nstomosing brnch pssing through the inferior orbitl fissure (IOF, dotted rrow). c k ECA-ACT (20 s rottion, 50% contrst, 2 cc/s, 40 cc, 543 projections): MIPs revel filling of the residul ICA lumen (rrow) vi brnches of the APA (vs vsorum), s well s the vidin rtery. The ILT does not directly contribute, but fills the OA vi its nteromedil brnch. c The AFR connecting with the nterolterl brnch of the ILT, nd the nstomosis with the OA ( dotted rrow in c d). e f The vidin rtery sgittl nd xil (double rrows), while f lso depicts the pterygovginl rtery (long rrow, lso in ), s tiny vessel in the pteryovginl cnl. c The postero-lterl brnch ( rrowhed) of the ILT, filled by the MMA. g h 30 mm MIPs lterl nd xil, provide overviews of the complex network. i Sgittl 2 mm MIP shows the AMA nd its contributing pedicle through the formen ovle, not clerly visible on DSA (inset: Frontl view, short rrow: AFR). Regulr rrows in e h: Pointing to the residul ICA lumen (ICA). Triple rrow in g APA brnches reconstituting the ICA lumen. J is 0.2 mm frontl view (MPR), slightly posterior to the pterygopltine foss showing three posterior brnches of the sphenopltine rtery (SPA) from medil to lterl: Pterygovginl rtery (long rrow), vidin rtery (double rrow) nd rtery of the formen rotundum (short rrow). The vidin cnl nd the smller pterygovginl cnl my lie very closely together with only subtle demrction in between (in this cses the AOF ws not visulized). k Oblique 2 mm sgittl MIP long the course of the crotid cnl (inset xil view) showing the residul/reconstituted ICA lumen (ICA, rrow) tht my involve some vs vsorum. Asterisk: Cvernous sinus, PPF: Pteryopltine foss, OA: Ophthlmic rtery, SPA: Sphenopltine rtery. l, m Right ICA erly nd lte phse: Rpid retrogrde filling of the AV shunt I (sterisks), minly drining posteriorly into the IPS (double rrows). n, o Interestingly, the ACT revels tht the SOV is ctully not completely occluded in its proximl segment, s could be ssumed from the DSA (dotted rrows). It clerly fills lso segment inside the orbit, nd even smll vein coursing behind the eyebll (yellow rrows)

179 170 7 Rdiologicl Dignosis of DCSFs b Formen ovle Formen ovle c MIP d MIP ICA Fig d. 3D ngiorchitecture of norml CS dringe. Right ICA venogrm AP (), lterl (b). Norml dringe vi the Sylvin vein (rrows) tht courses prllel to the sphenoprietl sinus (thick rrow) nd receives the uncl vein (short rrow), just before entering the CS (sterisk). From here, the dringe uses venous emissrium (rrowhed), which courses through the formen ovle to rech lrge pterygoid plexus (lrge rrowhed). The IPS (double rrow) is only in AP projection visible. c,d Venous 3D Rottionl venogrphy. MIPs, obtined by mnul contrst injection during the msk run (non-subtrcted, 14 s rottion, 132 projections, Neurostr). Intense opcifiction of the Sylvin veins nd SPPS. Prticulrly well visible is the uncl vein (c, shorter rrow). ICA: The remining contrst in the ICA superimposes prtilly onto the venous structures. (Modified from Benndorf 2002)

180 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 171 the inferior petroclivl vein (IPCV) become visible nd cn be followed long their course through the petrous pyrmid nd long the petroclivl suture, respectively. Rektorzik s plexus, for exmple, is poorly described in the rdiologicl literture nd scrcely mentioned in older textbooks. This internl crotid rtery venous plexus (ICAVP) ws originlly described by Rektorzik in 1858 nd ws observed by Knott (1882) nd some other uthors (Sn Milln Ruiz et l. 2002; Hike 1902; Knecht 1937; Pullus et l. 1977; Aubin et l. 1974; Lng nd Weigel 1983). In mny recent textbooks on venous ntomy of the skull bse, s well s in numerous ngiogrphic studies of the CS ntomy, the ICAVP is more or less neglected (Theron 1972; Brun et l. 1976; Tkhshi et l. 2005; Shiu et l. 1968; Miller et l. 1993). This my in prt be due to the fct tht more recent imging studies on the venous ntomy in this re were focused on structures tht represent potentil trnsvenous pproches to the CS for endovsculr occlusion of CSFs. The ICAVP is certinly too smll in cliber for such endevor; however, its ppernce on ngiogrms nd crosssectionl imging modlities should be known to everybody involved in dignosis or tretment of rteriovenous shunting lesions in this re. DVT imging precisely revels the origin, the course of the ICAVP through the crotid cnl s well s its connection with the IPS/IJV junction. The plexus leves the CS between the emissry vein (plexus) of the formen ovle nd the IPCV tht rises from the CS just nterior to the IPS (Figs ). This cretes n pprently consistent configurtion of four mjor veins leving the lterl nd lteroposterior CS: 1. Formen ovle plexus 2. Internl crotid rtery plexus (Rektorzik s, ICAVP) 3. Inferior petroclivl vein (IPCV) 4. Inferior petrosl sinus (IPS) (1) The emissry vein coursing through the formen ovle (formen ovle plexus) often ppers reltively smll when it leves the CS but my increse in size on its wys through the formen nd usully connects to lrge pterygoid plexus. This vein ws used s trnsvenous pproch for occlusion of DCSF by Jhn et l. (1998). (2) After emerging from the CS, Rektorzik s plexus initilly ppers more like continuous venous lining within the osseous petrous cnl, covering the wll of the crotid rtery with very thin inner lumen. Following the course of the ICA, it trvels more lterlly thn the other efferent veins nd ppers to trnsform into plexiform rrngement of smll veins. In this study mteril, this plexus-like structure ended somewhere in the crotid cnl nd ws not directly connected with the IPS, but gve rise to smll vein tht curved medilly to rech the IPS/ IJV junction (Figs. 7.86, 7.89 nd 7.91). The course nd rchitecture of the ICAVP cnnot be pprecited on DSA, where in AP views it is often times superimposed or mistken s the emissry vein to the formen ovle. In lterl projections only the portion covering the nterior nd posterior wll of the ICA, where the X-ry bem psses tngentilly through its contrst filled lumen, the plexus is somehow recognizble (Figs. 7.27, , 7.86, 7.89). In orthogonl views obtinble only using high resolution 3D dt sets, the fct tht the plexus does not cover the entire crotid rtery circumference, becomes evident by figure C ppernce. (3) The IPCV rose between Rektorzik s plexus nd coursed lmost in prllel to the IPS, posterolterlly nd cudlly towrds the IPS/IJV junction. This vein indeed cretes mirror imge of the IPS s described by Rhoton (2000) nd lies so close to the IPS tht it cn esily be mistken on 2D-DSA for doubled IPS or plexus type of IPS. As reveled by DVT, this vein lies on the externl surfce of the lower skull bse, is connected to the formen lcerum plexus (FLP), which ws not lwys identifible, nd trvels long the petroclivl suture to rech the IPS/IJV junction, where it my drin directly or vi the ACC into the IJV. It is interesting to note tht the prtilly extrcrnil course of the IPCV is mentioned by severl uthors, but it is to my knowledge not reported through which osseous opening this vein courses. Bsed on the mteril studied it cn be ssumed tht the formen lcerum serves s n exit for the IPCV, but more detiled studies re required to confirm this topogrphy. No report on the use of this vein s trnsvenous ccess to the CS exists in the literture, but bsed on ntomicl course nd size of this vein, such n pproch could be fesible in selected cses. Due to difficulties distinguishing between IPCV nd IPS bsed on DSA only, n ccidentl use of the former insted of the ltter is possible, especilly in cse of poor ngiogrphic imge qulity. (4) The IPS is the best known efferent vein of the CS nd its ntomy hs been studied extensively minly becuse of its frequent use for petrosl sinus smpling. This sinus hs lso been incresingly (Text continues on p. 179)

181 172 7 Rdiologicl Dignosis of DCSFs b c d e f g h i ICA ICA j Formen ovle Fig j. 3D ngiorchitecture of norml CS dringe. Left ICA venogrm AP (), lterl (b). Norml dringe vi group of Sylvin veins (rrows) tht course towrds the sphenoid wing (thick rrow). Insted of connecting with the CS, they use minly the emissry vein of the formen ovle to rech the pterygoid plexus (sterisk). c f 3D rottionl venogrphy using FD technology (VRT). The first (c) nd lst (d) projections of 5-s rottion demonstrte tht lmost exclusively venous structures re filled (subtrcted, 5-s rottion, 8 s dely, 18 cc/s). e, f Reconstructions of the subtrcted filling run ( , kernel: bone norml), showing the excellent visuliztion of venous circultion in the left hemisphere, including smll corticl veins. Although losing the higher temporl resolution of single DSA imge, the 3D-DSA dt set shows the entire venous territory t once, fcilitting the understnding of n rchitecture tht is more complex thn perceivble from the 2D-DSA. g j Vrious views llow one to visully seprte the different venous components t the bse of the brin. g i The rrngement of these veins from lterl (g), medil (h) nd AP (i). The Sylvin veins drin into lrge confluens tht courses long the floor of the middle crnil foss. This confluens divides into three seprte veins before converging gin t the level of the formen ovle, which is best seen in view from crnil nd posterior in DVT (j). ICA: Some residul contrst opcifies the proximl ICA s well

182 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 173 b R JB R R JB l p PVP d c JB l p PVP l JB JB e R JB Fig e. 3D ngiorchitecture of the CS in DCSF (Cse report III). Phlebogrphy vi 6-F guiding ctheter in the left IJV, AP () nd lterl views (b): Both CSs (sterisks) nd IPSs ( double rrows) re intensely opcified. Visuliztion of the SPS ( rrows) s well s of the left SOV. No opcifiction of fistulous connection on the right side. Additionl visuliztion of the inferior petro-clivl vein (IPCV) on ech side (rrowheds) nd of the internl crotid rtery venous plexus (ICAVP, Rektorzik, R). The ltter is difficult to identify on stndrd projections due to its thin, plexus-like structure, lining the wll of the crotid cnl. 3D rottionl phlebogrphy with Neurostr (non-subtrcted 14 s rottion, 132 projections), VRT (c,d) nd MIP (e), seen from nterior (c), posterior (d) nd cudl (e): Opcifiction of both CSs with demonstrtion of its prtil trbeculr ( septted ) structure. Visuliztion of both IPSs nd the left SOV. The osseus bckground fcilittes the ntomicl orienttion. The IPCV is best seen in c nd e seemingly crossing the course of the IPS from medil to lterl. e The more lterl course of the internl crotid plexus (Rektorzik). d The nterior condylr confluens (ACC, lrge sterisk) medil to the jugulr bulb (JB). Note the connections with nterior (), lterl (l) nd posterior (p) condylr veins. (Modified from Benndorf 2002)

183 174 7 Rdiologicl Dignosis of DCSFs b PP PP c d FLP R i l coils i PP coils e f R R R PP Fig f. Venogrm (,b obtined by rteril injection) nd phlebogrm (c f obtined by venous injection) of ptient with DAVF of the sigmoid sinus drining retrogrdely into the IPS, CS nd SOV due to IJV stenosis. As seen in this exmple, the ICAVP (Rektorzik) is usully not visible in the venous phse of crotid ngiogrm, lthough the filling defect (if present) cused by the intrcvernous ICA, my indicte its loction. Due to higher pressure when injecting contrst directly into the IPS or IJV, the phlebogrm my revel more rchitecturl detils of the cvernous sinus nd its communictions. It shows the pil veins, commonly involved in the dringe of CSF: SMCV (dshed rrow), uncinte vein (short dshed rrow), lterl mesencephlic vein (dotted rrow), cerebellr vein (short dotted rrow). The IPCV (rrowheds) nd the ICAVP (short rrows outlining its contours) re better visible in lter phses (e,f). In this cse, more prominent vein, coursing through the formen ovle (thick rrow), is seen in AP view lterl to the ICAVP. This exmple further underlines how unrelible venogrm for the visuliztion of the venous ntomy my be: No IPS is seen in,b but is clerly filled bilterlly in c f! B/W sterisk: CS, white sterisk: ACC, : Anterior condylr vein, l: Lterl condylr vein, i: Internl jugulr vein, FLP: Formen lcerum plexus. Insets: DAVF of the sigmoid sinus

184 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 175 b R R I PP PP l c d R i R PP PP I Formen mgnum e I Formen mgnum R PP Fig e. 3D phlebogrm in VRT nd MIPs, dt set obtined using n imge intensifier system (Neurostr). c The volume rendered mode shows the reltionship of the three min veins leving the CS: IPS (double rrow), Rektorzik plexus (R), nd fr lterl, the plexus to the formen ovle (thick rrow). The IPCV (rrowheds) is not well shown, but becomes pprent in the two MIPs (d, e). Note tht the qulity of the reconstruction using older technology is inferior to tht of current FD systems, which provide higher sptil nd better contrst resolution nd llow for esier identifiction of visulized sinuses nd veins (see exmples below). d, e ACT, mnul contrst injection (nondiluted), obtined using 14 s rottion, 132 projections (Neurostr, post-processed with Leonrdo). B/W sterisk: CS, white sterisk: ACC, : Anterior condylr vein, l: Lterl condylr vein, i: Internl jugulr vein, PP: Pterygoid plexus

185 176 7 Rdiologicl Dignosis of DCSFs b R R l I I I c d R e f R R

186 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 177 g h ICAVP R R i j R ICAVP R Fig f. Venous Antomy of the CS/IPS/IJV.,b IJ Phlebogrphy, AP nd lterl, obtined during IPS smpling. c f 3D Phlebogrphy using ACT (20 s, 2% contrst, 40 cc, 543 projections, Axiom Artis), MIPs. c View from posterior nd bove, d f lterl views in 5 mm sections from medil to lterl. Note tht on lterl DSA views, the ICAVP is visible only s nterior nd posterior contours of the filling defect cused by the ICA lumen. On DSA the IPS cn usully be identified, while IPCV nd Rektorziks plexus re usully difficult to recognize. Viewing MIPs from posterior llows for better understnding. The IPCV crosses the IPS from medil to lterl nd hs similr course (mirror imge ccording to Ktsut et l. 1997). c shows reltively consistent rrngement from medil to lterl: IPS-IPCV-ICAVP tht cn be pplied to ngiogrms, lthough often not redily perceivble there. B/W sterisk: CS, double rrows: IPS, rrowhed: IPCV, thick rrow: Emissry vein to the PP (formen ovle plexus), white or blck sterisk: ACC, : Anterior condylr vein, l: Lterl condylr vein, R: Internl crotid rtery venous plexus (ICAVP, Rektorzik, outlined by the short rrows in b). g j. 3D-phlebogrphy using ACT (20 s, 2% contrst, 40 cc, 543 projections, Axiom Artis). Bilterl IPS injections provide better filling of the veins, llowing for more complete imge nlysis nd better seprtion between IPS, IPCV nd ICAVP. g is 5 mm MIP tht shows the sme filling defect s b. h,i revel the rther plexiform structure of the ICAVP tht is impossible to perceive using 2D-DSA. j is n oblique lmost view orthogonl view, demonstrting the thin venous lining between the unopcified ICA nd the osseous crotid cnl tht cretes n incomplete circle or figure c. B/W sterisk: CS, double rrows: IPS, rrowhed: IPCV, regulr rrow: Emissry vein to the PP (formen ovle plexus), thick short rrow: PP, white sterisk: ACC, : Anterior condylr vein, l: Lterl condylr vein, R: Internl crotid rtery venous plexus (ICAVP, Rektorzik)

187 178 7 Rdiologicl Dignosis of DCSFs b IJV l I I R l I I c d R R l PP PP l e f PP R l l l I I I I Fig f. Venous ntomy of the CS/IPS/IJV. 3D phlebogrphy (10 s 3D-DSA, Axiom Artis) in different views from nterior (), lterl (d), right posterior (b), right nterior (c) nd crnio-cudl (e). f DVT from RAO (Sme cse s Fig. 7.87). The three-dimensionl ngiorchitecture cn be better perceived using these VRTs in DVT. The IPCV nd Rektorziks plexus cn be redily differentited. The IPCV mkes shrp zig-zg turn (rrow) to communicte with the IPS, before entering the ACC. The course of Rektorzik s plexus is best seen in e, where it psses more lterl thn IPS nd IPCV towrds the crotid cnl in the petrous bone. It ppers to become thinner on its wy nd drins vi smll curved vein tht is only visible on MIPs (long thin rrow in 7.86c) into the IJV. f shows the reltionship of the venous rchitecture to the posterior skull bse (oblique view from posterior. Double yellow rrows in c: Left IPS. B/W sterisk: CS, double rrows: IPS, rrowheds: IPCV, thick short rrow: Emissry vein to the PP (formen ovle plexus), white sterisk: ACC, : Anterior condylr vein, l: Lterl condylr vein, R: Internl crotid rtery venous plexus (ICAVP, Rektorzik), I: Anterior internl vertebrl venous plexus. PP: Pterygoid plexus

188 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 179 used s trnsvenous pproch for endovsculr occlusion of CSFs tht will be covered in detil in the next chpter. One interesting spect of my observtions is the fct tht the IPS nd the IPCV my be connected through severl smll bridging veins (Figs nd 7.90), which my ply role in cses of sinus thrombosis, nd which could lso resemble the plexiform type of the IPS/IJV junction tht hs been described in the literture (Shiu et l. 1968; Miller et l. 1993). Mitsuhshi et l. (2007) hve clssified the IPS-IJV junction into six types bsed on 3D rottionl venogrms. They found tht the IPS drins directly into the JB in only 1.2%, while most frequent ws the dringe either into the upper IJV (34.9%), or into the lower IJV (37.3%). These results emphsize the vlue of 3D-DSA imging for understnding venous ntomy. The ACC ws demonstrted in ll 3D phlebogrms. This venous collector, originlly described by Trolrd (1868), ws recently rediscovered by Sn Milln Ruiz et l. (2002). The uthors performed high qulity rdiogrms on cdver csts nd correlted their findings with MRA studies. It ws found tht the ACC connected with six min venous structures: inferior petrosl sinus (IPS), internl crotid venous plexus (ICAVP, Rektorzik), nterior condylr vein (ACV), lterl condylr vein (LCV), prevertebrl venous plexus (PVP), nd internl jugulr vein (IJV). This is to some extent different from my observtions, where Rektorzik s plexus did not seem directly connected to the ACC, but rther through smll medilly curved vein, reching the IPS, which my correspond to the medil petrosl vein. The IPCV ws not included s tributry of the ACC by Sn Milln Ruiz et l. (2002), but s connecting vein between ICAVP nd ACC. In contrst the IPCV ws found in our mteril to be connected to the ACC, independently from Rektorzik s sinus. The illustrtion of Sn Milln Ruiz et l. (2002) showed the IPCV (clled there inferior petro-occipitl vein) rising from Rektorzik s plexus which is not confirmed by this study where both veins ppered to rise seprtely from the CS: the IPCV coursing minly extrcrnilly to rech the ACC, while the ICAVP trvels intrcrnilly through the petrous pyrmid. Tkhshi et l. (2005) recently studied the ntomy of the crniocervicl junction round the suboccipitl sinus using MRI nd lso described six tributries to the ACC, including the IPCV, but neglecting the ICAVP. Figure 7.92 summrizes some of the observtions in the IPS/ACC/IJV junction so fr, dding the IPCV to the tributries of the ACC. More detiled studies re on-going nd will dd knowledge of the topogrphy of the venous ntomy in the prsellr nd skull bse region. For successful ctheter nvigtion into the CS, knowledge of the possible ntomicl rrngements nd communictions between IPS, IJV nd ACC is essentil, since vritions from norml ntomy re not seldom nd 2D ngiogrms my look confusing, especilly when locl thrombosis is present or the IPS hs to be pproched through the ACC. Bilterl retrogrde injections through dignostic ngiogrphic ctheters within ech IPS nd 3D reconstructions using DynCT nd DVT provide n extensive three-dimensionl visuliztion of the venous ntomy t the posterior skull bse nd of the CS, including its fferent nd efferent veins. This technique provides ntomic informtion, not obtinble by 2D-DSA or other cross-sectionl imging modlities such s CTA or MRA, nd is without doubt regrding its topogrphic ccurcy nd imge qulity comprble to colored plstic csts in cdver vessels. Advnced three-dimensionl imging using modern rottionl ngiogrphic techniques bsed on high-resolution FD technology enhnces not only our existing knowledge of venous ntomy in the prsellr region nd the skull bse. It lso llows the study of smll venous structures nd their communictions, trditionlly obscured on ngiogrms nd thus poorly described or even neglected in the literture. In summry, modern bi-plne DSA bsed on FDtechnology llows for precise nd detiled visuliztion of ll relevnt rteril nd venous structures. It remins the gold stndrd for timely nd correct dignosis s well s for optimizing tretment plnning in ptients with DCSFs. The combintion of 2D-DSA with novel 3D imging tools such s ACT nd DV technique, s well s 3D venogrphy nd 3D phlebogrphy provides ntomicl informtion in qulity tht sets new stndrd for vsculr imging in this re, llowing for computerized grphic dissection of vsculr structures within their osseous embedment. Beside high-qulity ngiogrphic equipment nd sufficient trining, profound knowledge of the vsculr ntomy, including its ngiogrphic ppernce, remins the essentil bsis for successful nd sfe endovsculr occlusion of CSFs.

189 180 7 Rdiologicl Dignosis of DCSFs b PVP l IJV PVP I IJV l l c d R ICA R PVP IJV IJV l e f R ICA R ICA IJV l PP IJV PVP Fig f. Venous ntomy CS/IPS/IJV. f IJ phlebogrphy, left ( c) nd right (d f) side AP nd lterl, obtined during IPS smpling. On the left side, both the IPS (double rrow) nd the IPCV (double rrowhed) cn be identified, while on the right side, there is dominnt filling of the IPCV (triple rrowhed). Its course is very similir nd could be esily mistken for tht of the IPS. The right IPCV drins directly into the IJV, but is lso connected to the ACC vi severl smll veins (d,e). The left IPCV crosses in AP projection the IPS to rech the ACC (,b). Cross-flow through the ICS fills the IPS on the left (double rrow in d,e) nd the IPCV on the right (triple rrow in,b). The right IPS is vguely identifible in e (dotted double rrow), but uncler in f due to superimposition with the left IPS (cross-flow filled). c nd f show longitudinl filling defect, corresponding to the scending course of the C5 ICA segment tht is outlined by Rektorziks venous plexus (R, smll rrows). The very thin lumen of this plexus is clerly seen only in tngentil projections of its nterior nd posterior prt (see lso inset in f). Note in b smll curved intrpetrosl vein, connecting the ICAVP nd the ACC (long thin rrow). B/W sterisk: CS, double rrows: IPS, rrowheds: IPCV, white sterisk: ACC, : Anterior condylr vein, l: Lterl condylr vein, R: Internl crotid rtery venous plexus (ICAVP, Rektorzik), I: Anterior internl vertebrl venous plexus. PP: Pterygoid plexus

190 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 181 b l e IPS l IJV I IPCV I IJV PP l c I d I Fig k. Venous ntomy CS/IPS/IJV (compre to Fig. 7.89). 3D phlebogrphy (10 s 3D-DSA, Axiom Artis), right side, AP () nd PA (b) lterl (c) nd medil (d) view, obtined during IPS smpling using bilterl injections (sgittl clip plne used to exclude the left side). The IPCV (double rrowheds), nd especilly the IPS (double rrow), lthough only in frgments possibly due due to thrombosis, re visulized. The IPCV, which ppers enlrged nd plexiform shows severl bridging veins to the pterygoid plexus (thick rrow), s well s to the IPS (c, d rrows in e) nd courses inferiorly, in prllel to the IPS (c, d). This detiled ntomy cnnot be perceived from the 2D-DSA. The IPS ppernce could esily be interpreted s plexiform Type III (Fig d nd e) type fter SHIU et l. (1968, see Fig. 3.11). B/W sterisks: CS, double rrows: IPS, rrowhed: IPCV, thick short rrow: Emissry vein to the PP (formen ovle plexus), white sterisks: ACC, : Anterior condylr vein, l: Lterl condylr vein, I: Anterior internl vertebrl venous plexus, PP: Pterygoid plexus, R: Internl crotid rtery venous plexus (ICAVP, Rektorzik) Continued on next pge

191 182 7 Rdiologicl Dignosis of DCSFs f g l PVP I I h i BP BP I I MS IJV I MS j k FLP PP PVP MS IJV IJV PP PVP i i Fig k. Continued. f k Venous ntomy IPS/IJV in DVT obtined by bilterl 3D phlebogrphy (10 s 3-D DSA, Axiom Artis). f,g DVT, two volumes seprtely reconstructed bsed on identicl projections of msk (f) nd filling run (g). h,i View t the posterior skull bse from bove nd oblique. j View t the centrl skull bse from below. k Crnio-cudl view onto the formen mgnum, clivus nd sell. The venous structure seen in h nd i represents the right IPS (red double rrowhed), s it courses long the inner (superior) surfce of the petroclivl fissure to rech the ACC nd IJV, while the IPCV is not shown. The sinus is not redily identifible on high-resolution DSA (compre with 7.89d,e) tht shows predominntly the IPCV. The rther plexiform structure of the vein cn, on the other hnd, redily be identified in j, where it courses long the outer (inferior) surfce of the petroclivl fissure (triple rrowheds). There re numerous smll connections between the IPCV nd the PP, the PVP s well s the FLP. B/W sterisk: CS, double rrows: IPS, rrowhed: IPCV, white sterisk: ACC, : Anterior condylr vein, l: Lterl condylr vein, R: Internl crotid rtery venous plexus (ICAVP, Rektorzik). BP: Bsilr plexus, MS: Mrginl sinus, PVP: Prevertebrl plexus, IJV: Internl jugulr vein, PP: Pterygoid plexus, FLP: Formen lcerum plexus, i: Anterior internl vertebrl venous plexus

192 7.2 Intr-rteril Digitl Subtrction Angiogrphy (DSA) 183 b MTV SOV SOV PP R R PP ICA RMV PP EJV Fig. 7.91,b. Venous ntomy of the IPS/IJV junction. ACT () using 20 s rottion, 20%, 2 cc/s, 40 cc, nd DVT (b), obtined by 10 s 3D-DSA (simultneous bilterl IPS injections). Thick MIP (20 mm) reveling the extensive opcifiction of both CSs, including some of its efferent nd fferent veins. Note the bilterl ICAVP nd the medilly coursing intrpetrosl veins (long rrows). The rrngment of the four min drining veins leving the lterl nd posterolterl CS: - Emmissry vein to the formen ovle (formen ovle plexus, FOP) - Internl crotid rtery venous plexus (ICAVP, Rektorzik) - Inferior petroclivl vein (IPCV) - Inferior petrosl sinus (IPS) ws found consistently in six ptients undergoing 3D phlebogrphy during petrosl sinus smpling (see lso Fig. 7.92). b DVT showing the connection between superior ophthlmic vein nd middle temporl vein tht drins into the retromndibulr vein nd the externl jugulr vein (EJV). Asterisk: CS, double rrows: IPS, rrowhed: IPCV, R: Internl crotid rtery venous plexus (ICAVP, Rektorzik). MTV: Middle temporl vein, RMV: Retromndibulr vein, EJV: Externl jugulr vein, PP: Pterygoid plexus, SOV: Superior ophthlmic vein Drining (efferent) veins of the posterolterl CS 1 Anterior intercvernous sinus (ICS) 2 Posterior intercvernous sinus 3 Cvernous sinus (CS) 4 Vein of formen ovle 5 Internl crotid rtery venous plexus (ICAVP, Rektorzik) 6 Inferior petroclivl vein (IPCV) 7 Inferior pterosl sinus (IPS) 8 Internl jugulr vein (IJV) 8 Jugulr bulb (JB) 9 Anterior condylr confluens (ACC) 10 Lterl condylr vein (LCV) 11 Anterior condylr vein (ACV) 12 Prevertebrl vein 13 Bridging (petrosl) vein Fig Artistic drwing of the posterolterl cvernous sinus nd its min drining veins, bsed on observtions using 2D-DSA, 3D phlebogrms (view from posterior). The ICAVP (Rektorzik) nd the inferior petroclivl vein (IPCV), often not redily identifible on ngiogrms, re illustrted. The IPCV courses lmost in prllel to the IPS, yet slightly lterl nd inferior long the outer (inferior) petroclivl fissure (suture) nd hs no verticl nd horizontl portion. This vein is not well described in texbooks; in some it is clled petrooccipitl sinus (relting to petrooccipitl suture ) or inferior petrooccipitl vein fter Trolrd. It mkes in its distl portion medil turn, while the IPS courses lterlly. Rektorzik s sinus ppers rther like plexus, surrounding the crotid wll nd my be difficult to identify on ngiogrms, nd thus hs been widely neglected in the ngiogrphic literture. It is nmed here ICAVP nd courses more lterl thn the IPCV following the ICA into the crotid cnl nd my be connected to the IPS, ACC or IJV vi smll veins. The ICAVP my not serve s ccess route to the CS, but cn be involved in the venous dringe of trumtic or durl CSFs. The ACC lies medil nd slightly nterior to the jugulr bulb nd my communicte with the IPS, IPCV, Rektorzik s sinus, IJV, ACV, LCV nd severl smll prevertebrl veins. In one cse severl smll veins, connecting the IPCV with the PP nd the IPS, were found.

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198 Endovsculr Tretment 8 (Cse Reports I VII, Cse Illustrtions I XI) CONTENTS 8.1 Techniques of Trnsvenous Ctheteriztion Inferior Petrosl Sinus Approch Alterntive Approches to the Cvernous Sinus Trnsfemorl Superior Ophthlmic Vein Approch Trnscutneous Superior Ophthlmic Vein Approch Trnsorbitl Puncture of the Superior or Inferior Ophthlmic Vein Other Techniques Embolic Agents Polyvinyl Alcohol (PVA) nd Embospheres Stinless Steel Coils Pltinum (Non-detchble) Pushble Microcoils Detchble Pltinum Coils HydroCoils Liquid Embolic Agents: Cynocryltes (N-butyl-cynocrylte, Acrylic Glue, Histocryl TM, Trufill TM n-bca, Glubrn TM ) Onyx (Ethylene-Vinyl Alcohol Copolymer) Stents Results of Trnsvenous Emboliztions Approches (Benndorf et l. 2004) Angiogrphic nd Clinicl Outcome Cses Reports Cse Report I: Approch vi the Thrombosed IPS Cse Report II: Cross-Over Approch vi the IPS Cse Report III: Approch vi the Thrombosed Cvernous Sinus Cse Report IV: Trnsfemorl Cross-Over Approch vi the Fcil Vein nd SOV Cse Report V: Trnsophthlmic Approch (Cnnultion) vi the SOV Cse Report VI: Direct Puncture of the SOV Cse Report VII: Direct Puncture of the Sylvin Vein After Crniotomy Cse Illustrtions Discussion of Trnsvenous Occlusions Approches IPS Approches Thrombosed Cvernous Sinus Trnsfemorl Fcil Vein/Superior Ophthlmic Vein Approch Approches vi the Middle Temporl Vein or the Frontl Vein Trnsfemorl Superior Petrosl Sinus Approch Trnsfemorl Pterygoid Plexus Approch (PP Approch) Trnsfemorl Corticl Vein Approch Trnscutneous SOV Approch Trnsorbitl SOV Approch Direct Puncture of the Formen Ovle Direct Puncture of the CS vi the SOF Sylvin Vein Approch: Combined Surgicl/ Endovsculr Tretment Embolic Mterils Prticles Coils NBCA (Histocryl TM, Glubrn TM, Trufill TM ) Ethylene-Vinyl Alcohol Copolymer (Onyx TM ) Stents nd Covered Stents Antomic Results, Clinicl Outcome nd Complictions of Trnsvenous Occlusions nd Trnsrteril Emboliztions Conclusion 267 References Techniques of Trnsvenous Ctheteriztion (Figs ) Mnul techniques of ctheteriztion nd emboliztion my differ from center to center nd vry significntly between opertors. The following descriptions re bsed on the uthor s personl experiences

199 190 8 Endovsculr Tretment gined over more thn decde performing EVT in cerebrovsculr lesions. Although minly bsed on subjective mnul hndlings nd personl preferences, these ccounts my be beneficil, especilly for younger collegues. As for most endovsculr procedures in the tretment of cerebrovsculr lesions, the emboliztion of DCSFs is performed under generl nesthesi, preferbly under supervision of n experienced neuronesthesiologist. Trnsvenous ctheteriztions nd emboliztions re often lengthy procedures (2 4 h), during which the typiclly elderly ptients would hve difficulties remining still on n ngiogrphic tble. Generl nesthesi with endotrchel intubtion is therefore widely used in most centers in the world, s it lso llows for sfer monitoring nd esier mngement in cses of intrprocedurl complictions such s rupture nd hemorrhge. b Fig. 8.1, b. Endovsculr tools preferbly used by the uthor for trnsvenous ctheteriztions of the cvernous sinus: Microctheter Trcker Excel -14 (BSC, Fremont CA), brided nd hydrophilic coted, outer dimeter of the distl tip: 0.65 mm. Arrow: introduced hydrophilic micro guidewire Hedliner (Terumo, Jpn), very flexible with 1:1 torqubility, dimeter of the 45 preshped tip: 0.3 mm. It dpts well to very nrrow nd irregulr venous structures nd enbles use of the Loop Technique (b Fig. 8.3) to pss thrombosed venous segments. This very supple guidewire hs been used lterntely with the hydrophilic coted Trnsend (BSC, Fremont, CA) tht hs stiffer tip, but lso provides more support for the microctheter b Fig. 8.2,b. Terumo microguidewires with hydrophilic coting. Glidewire Gold Neuro with 8 cm tpered distl end nd 2 mm Gold tip comes in 0.011, 0.014, nd b Hedliner (MicroVention, Terumo, Jpn) hs superelstic kink-resistnt Nitinol core with 20 cm or 35 cm distl tper nd 20 mm gold tip for enhnced rdiopcity. The wire is vilble in vrious pre-shped tip configurtions, such s 45 nd 90, or s 90 /150 double ngle nd comes in sizes of 0.012, nd Although vilble for more thn 10 yers, microguidewires from Terumo re still considered superior to their competitors, minly becuse of their durbility. The Hedliner proved prticulrly useful for dvncement in nrrowed nd thrombosed sinuses (Figs. 8.3, 8.15, 8.17, 8.20, 8.31, 8.38)

200 8.1 Techniques of Trnsvenous Ctheteriztion Inferior Petrosl Sinus Approch (Cse Reports I III, Fig. 8.3) After rteril nd venous femorl punctures nd plcement of 6-F introducer sheths, n intrvenous bolus of 50 IU/kg of body weight heprin is dministered. Monitoring is routinely performed in most centers to keep the ctivted clotting time (ACT) t level of pproximtely s or 2 bove the norml level. A 4-F Hedhunter 1 ctheter (H1, Cordis, Mimi, FL) is dvnced into the ECA or ICA on the side of the dominnt fistul supply to llow for rod mpping nd control injections throughout the procedure. A 5-F or 6-F ngled guiding ctheter (e.g. Envoy, Cordis Endovsculr, Mimi Lkes, FL) is plced in the IJV on the side tht shows dominnt dringe, or tht looks most promising on the dignostic ngiogrms. The ctheters re flushed with heprinized sline (1500 IU per liter). Arteril nd venous femorl punctures my be performed uni- or bilterlly (Fig. 8.13). Alterntively, direct stick of the jugulr vein my be used, especilly in cses with thrombosed jugulr vein on the fistul side. Becuse the entire ctheteriztion system becomes shorter nd provides better support for supple microctheter, direct jugulr stick mkes microctheter nvigtion through the IPS esier. A microctheter my even be nvigted directly through 4-F or 5-F sheth when the IPS is widely open. The less ccess tools nd devices re used, the esier the technicl prt of the procedure is, including deployment of coils, glue or ctheter exchnge. The guiding ctheter should be dvnced close to the jugulr bulb for performing phlebogrms using either mnul injections (20-cc syringe), or power injector. If the ntomy looks bnorml, or the IPS ppers thrombosed or is bsent, contrst injections should be repeted from different positions nd combined with 3D phlebogrm. Chnging the position of the guide my be helpful, becuse in some cses the IPS my terminte t lower level or even outside the skull bse, nd thus will be missed on stndrd 2D ngiogrms s seen in Fig (Benndorf nd Cmpi 2001b; Clzolri 2002). If the IPS is not identifible on either side, even fter repeted phlebogrms, creful imge nlysis is crucil in order to identify the smll notch or stump tht is often the only residul filling of thrombosed sinus (Figs. 8.3, 8.4, 8.14). This should include dignostic rteril injections, prticulrly in cses in which there is no posterior dringe, or the IPS is thrombosed. In those cses, the sinus my be wide open, utilized s n efferent vein by the norml cerebrl venous dringe, nd in fct representing fesible trnsvenous pproch (Cse report III). If the IPS cn be identified, retrogrde ctheteriztion is redily performed using smll hydrophiliccoted microctheter, such s 2.6-F FsTrcker 10, (Boston Scientific, Fremont, CA). Brided, reinforced microctheters such s the 2.4-F Trcker Excel-14, 1.7-F Excelsior SL 10 (Boston Scientific, Fremont, CA) or the 3.0-F Rpid Trnsit 18 (Cordis, Endovsculr, Mimi Lkes), dvnced over 0.012, or guidewire, usully llow esier nvigtion through the IPS nd the CS, especilly in cses, where the contrlterl CS (cross-over) needs to be reched. Reinforced microctheters re more stble throughout the procedure, n importnt fctor in chieving dense coil pcking in the fistulous comprtment. They lso show fewer tendencies to kink, which is dvntgeous when using lrger coils. An experimentl study showed tht even some reinforced ctheters, such s the Rpid Trnsit 18 (Cordis Endovsculr, Mimi Lkes, FL) my develop luminl irregulrities in curved model tht my crete high resistnce for embolic gents (Kiyosue et l. 2005). Significnt friction my result when pushing fibered coils (Cse Report IV). In cses where the IPS cnnot be identified with certinty, the residul notch, if visible, should be ctheterized very gently with smll, hydrophilic guidewire. For this purpose, the hydrophiliccoted wire (Hedliner, Terumo Corportion, Tokyo, Jpn) is considered the most suitble guidewire to be use. It is highly flexible, kink-resistnt wire with super-elstic nitinol core providing 1:1 torque rtio, nd hs 20-mm gold coil t the tip for enhnced rdiopcity (Fig. 8.2). The Hedliner hs superior lubricity nd is vilble in 200 cm length in vrious pre-shped tip configurtions such s 45º, 90º, 1.5-mm J-Tip ngle nd double ngle. Some opertors my prefer the Synchro2 wire (BSC, Ntick, MA) tht is vilble s hydrophilic guidewire (externl dimeter 0.36 mm), or guidewire (externl dimeter 0.3 mm) with pre-shped pltinum-tungsten lloy tips. The proximl portion is coted with polymer (Polytetrfluoroethylene, PTFE) to enhnce trcking nd mnipultion. An lternte choice is the Mirge, hydrophilic coted guidewire (0.20 mm) mnufctured by MTI (Microtherpeutics, Irvine,

201 192 8 Endovsculr Tretment b c d?? e f g h??? i u j k l m n o p q r s t

202 8.1 Techniques of Trnsvenous Ctheteriztion 193 CA; Fig. 8.11). Another new development is the steerble guidewire Wtusi (Micrus Endovsculr Corportion, Sn Jose, CA) vilble in (0.36 mm), mde of stinless steel with hydrophilic coting of the distl 40 cm. The smllest guidewire currently vilble is the Sorcerer, (Blt Extrusion, Montmorency, Frnce), hydrophilic nitinol wire. The choice of microguidewires nd microctheters depends, to lrge degree, on personl preferences, s well s on the type nd level of cquired mnul skills. Using the microctheter, the Hedliner should be dvnced only few mm into the thrombosed IPS nd then exchnged for more stble hydrophilic coted guidewire such s the Trnsend (Boston Scientific, Fremont, CA). This guidewire provides more stbility to dvnce the microctheter, while its tip is less flexible thn tht of the Hedliner. The technique, utilized by the uthor to pss thrombosed IPS, is to lterntely nd slowly dvnce both guidewires with the help of the microctheter towrds the posterior CS. It requires ptience nd full concentrtion nd should be performed by n experienced opertor. Aggressive or forceful mnipultions my cuse rupture of the IPS. For dvncing ctheter through n irregulr, stenosed, or even occluded sinus, the following technique proved prticulrly useful: The guidewire is dvnced not with stright end, but insted with loop tht is formed within the nrrowed lumen by bending the tip. Such distl shpe cn be nvigted much more esily thn one would expect s it dpts better to n irregulr lumen thn stright guidewire. It gets less entngled in smll septi or stenotic segments of the trbeculted cvernous sinus structure. This method, lso clled the Loop Technique (Figs. 8.3, 8.15, 8.20, 8.17), published erlier, hs been used since with gret success (Benndorf et l. 2000), nd hs been dpted by others (Cheng et l. 2003). A Terumo guidewire such s the Hedliner-12 with pre-shped 90º ngle or double ngle is prticulrly esy to form. In order to void unintentionl ctheteriztion of bridging or pontine veins nd to prevent perfortion with subsequent intrcrnil hemorrhge, s reported by some uthors (Hlbch et l. 1988; Oishi et l. 1999), the simultneous use of bi-plne blnk rod mpping nd fluoroscopy of the ntive bckground re helpful. When the posterior CS is pproched through the IPS, it is usully possible to nvigte the microctheter into the nterior comprtment nd its connection with the SOV without difficulties. It is dvisble to bring the microctheter s close s possible to the SOV, preferbly in its first segment. Coil deployment should begin within the SOV pproximtely 2 3 mm before its entry into the CS. This initil coil positioning is crucil to void incresed dringe of the AV shunting into the SOV nd ophthlmologicl deteriortion. On the other hnd, coils deployed in the nterior SOV (2 nd or 3 rd segment) my cuse thrombosis nd occlusion of the entire vein with blockge of the centrl retinl venous dringe leding to serious clinicl consequences. To chieve dense pcking from the beginning, it is wise to strt with coil size tht cn be esily stbilized in n enlrged SOV (e.g. 5 mm/15 cm). Three-dimensionl or complex coils my lso be used to crete bsket tht, similr to neurysms, is subsequently filled with smller nd softer coils (Fig. 8.5). The more stble this initil pcking within the proximl SOV or t the CS-SOV junction is, the better it my be used s scffold for subsequent coils. It will llow dense pcking of the CS, without the risk of dislodging coils into the drining vein. Commonly, the coil plcement is performed from nterior to posterior to keep the ccess through the IPS free until the end of the procedure. This is useful strtegy becuse there is lwys the chnce tht coils get indvertently plced erly dur- Fig. 8.3 u. Loop Technique for ctheter nvigtion through the thrombosed IPS. Type D fistul in n 80-yer-old femle with 6 th nerve plsy nd mild red eye., b Initil DSA demonstrting n AV shunt t the posterior CS drining into SPS nd leptomeningel veins, not into the IPS. c f Bilterl lte venous phse. Note there is no filling of the IPS here. g i Jugulr phlebogrm showing the residul notch of the IPS. Note tht in (g) due to the ctheter position (too high, white rrow), the IPS is not opcified. h, i When the guiding ctheter is positioned lower (white rrow), severl communicting veins re visulized. In this cse, it is minly the inferior petroclivl vein (IPCV, rrowhed), while the IPS (double rrow) is only fintly opcified. j, k Rod Mp nd retrogrde ctheriztion of IPS using the loop techniqe (lterl view). l r Stepwise, gentle dvncement of the microctheter with lternting guidewires (Trnsend nd Hedliner). Control sinogrms (n, q) my not show ny connection to the CS or to the fistul during nvigtion through the IPS. r, s Successful nvigtion into the posterior CS nd subsequent coil pcking with complete occlusion by the end of the procedure (t). u Artist s illustrtion of the loop technique using n ngled (90 ) Hedliner tht llows dvncement of the microctheter even through nrrowed nd thrombosed IPS (Benndorf et l., 2000)

203 194 8 Endovsculr Tretment ing the procedure in the posterior CS, or even in the IPS. If this hppens, before the ctul AV shunt is occluded, complete pcking of nteriorly locted fistuls my become difficult, or the entire procedure my be jeoprdized. Losing ccess to the CS or to fistulous comprtment in cses of n incomplete occlusion cretes the risk of rerouting the dringe towrds corticl veins, cusing corticl venous hypertension nd possibly hemorrhges. In cses with dringe towrds the contrlterl side of the fistul, the microctheter should lwys be dvnced there first. Such cross-over pproch vi the intercvernous sinus llows blocking the dringe before withdrwing the ctheter nd pck- b c d e f

204 8.1 Techniques of Trnsvenous Ctheteriztion 195 ing coils t the fistul s side (Fig. 8.15, 8.17, 8.22). This helps the opertor to void leving fistulous comprtments behind tht usully require more chllenging ctheteriztions lter on. Moreover, this is crucil in cses with bilterl fistuls, especilly when the CS pproch is possible from only one side (Fig. 8.22). Filing to occlude the contrlterl fistul side first my result comprtmentliztion, nd second my complicte the mngement of the ptient. If corticl dringe is present, the most crucil prt of the procedure is to block the connection between the CS nd the corticl vein t the beginning of the occlusion, even before plcing coils into the SOV (Fig. 8.4). Only such blocking will prevent per- g h i j Fig. 8.4 j. Disconnection of leptomeningel nd corticl venous dringe with coils. Selective injection into left scending phryngel rtery. AP view demonstrtes AV shunt t the posterior CS (sterisk) drining into the SOV (double rrow) nd the SPS (rrow) cusing leptomeningel nd deep venous dringe. b e Lterl rod mps. An Excelsior SL-10 ws nvigted round the venous pouch into the SPS first. Two GDC -10 UltrSoft coils (3/8) were used to block the dringe into the SPS before pcking the CS itself. Then, the microctheter ws slightly withdrwn into the CS nd 3D-GDC (8/20) ws deployed into the venous pouch nd subsequently filled with multiple GDC Soft nd UltrSoft coils (totl of 15 coils) until complete occlusion ws documented. Note tht during the coiling of the CS, the fistul continued to drin into the SOV, whers it ws disconnected from the SPS (f). Note: In this cse, one coil could not be electrolyticlly detched nd hd to be mnully seprted from the pusher wire by clockwise rottion until it broke ( mechniclly detched, Lee et l. 2008). No dverse clinicl event occured. g, h Selective injection into the CS, lterl nd AP view shows the blocked venous dringe t the posterior CS (b/w sterisk) nd the SOV (blck sterisk) using two microctheters (dul IPS pproch, Fig. 8.31). There ws significnt corticl venous dringe left (thin rrows). i, j Lterl nd AP rod mps show the selective pcking of the lterl venous exit, blocking the corticl venous dringe. Note tht due to superimposition with the SOV in stndrd lterl nd AP views, the venous exit to the corticl veins my be difficult to perceive. Crnio-cudl ngultion of the B-plne (g,i) nd cudl-oblique working-projection of the A-plne (h, j) demonstrted better this connection (rrows) nd helped to nvigte the ctheter to the proper position for selective occlusion (yellow rrows). See lso Cse Illustrtion XI.

205 196 8 Endovsculr Tretment sistence, or even worsening by rerouting of the venous dringe tht is ssocited with incresed risk of intrcrnil hemorrhge. At the end of the procedure, occluding the IPS itself should be voided s it my be needed s n ccess route in subsequent session. Secondly, n occlusion of the IPS my, if it serves s dringe for the CS, compromise the norml cerebrl circultion of the posterior foss. Although 4-F or 5-F guiding ctheter cn be dvnced into the IPS, one should remember tht, especilly in cses with corticl venous dringe, such mneuver my elevte venous pressure sufficent enough to cuse intrcerebrl hemorrhge s recently reported (Theudin et l. 2007). The ngiogrphic endpoint of the endovsculr procedure is either subtotl or complete occlusion of the fistul. If there is miniml residul AV shunt visible, the procedure cn usully be stopped, becuse the postopertive normliztion of the ACT will further promote ongoing thrombosis within the CS. Ongoing thrombosis in the CS my be impeded by the hepriniztion of the ptient during the procedure. Subtotl occlusion is often sufficient nd will led to complete occlusion within few dys or even 24 h when the cogultion system is normlized (Cse Reports II nd IV). Whenever possible, it is dvisble to void overpcking the CS. Additionl prticulte emboliztion using PVA or Embospheres fter TVO, lthough performed by some investigtors, hs rrely been necessry in the uthor s experience. After extubtion, the ptients re usully trnsferred to post-nesthesi cre unit, nd then to norml unit. Only if dverse effects or signs of visul deteriortion re observed, will the ptient be heprinized for 48 h. Some opertors suggest reversl of systemic hepriniztion with protmine sulfte (10 mg per 1000 U) (Vinuel et l. 1997). In order to void post-procedurl complictions, the dditionl use of closure device such s Angio-Sel TM (St. Jude Medicl Inc.) is incresingly prcticed by mny opertors. Postopertive hedches due to mechnicl pressure induced by the coils cn usully be controlled with nlgesics (300 mg ASA dily). If postopertive CN plsy occurs, dditionl corticosteroids cn be dministered (e.g. Decdron 4 8 mg every 6). Every ptient should undergo n ophthlmologicl exm few dys fter the procedure. Mny times, symptoms improve during the first 24 h. The ptient my be dischrged either the next dy or fter 2 3 dys, nd is then seen for clinicl follow-up fter 3 months with t lest one ngiogrphic follow-up fter 6 9 months Alterntive Approches to the Cvernous Sinus Trnsfemorl Superior Ophthlmic Vein Approch (Cse Report IV) When the ipsi- or contrlterl IPS pproch clerly fils, trnsfemorl SOV pproch my be performed during the sme or subsequent session. This is done in the following wy: A 4-F guiding ctheter (0.038 inner lumen) is introduced into the fcil vein nd nvigted s distlly s possible. The closer the tip of this ctheter is plced to the ngulr vein, the esier the ctheteriztion of the SOV will be with microctheter. A too proximlly plced guide often requires more microctheter mnipultions in the fcil nd ngulr veins. These veins tend to become more mobile within the subcutneous soft tissue, mking dvncing the microctheter through tortuous SOV difficult. A slightly stiffer microctheter, such s the brided Rpid Trnsit 18, my be fesible. The use of trixil ctheter systems (8 F-4 F-2 F), lthough never employed by the uthor, cn lso be helpful (Suzuki et l. 2006) Trnscutneous Superior Ophthlmic Vein Approch (Cnnultion, Cse Report V) This pproch is chosen if both the IPS nd the trnsfemorl SOV pproch fil to provide ccess. Under generl nesthesi, the ptient gets prepred in the surgicl OR, or (under sterile conditions) in the ngiogrphy suite. An experienced ophthlmic surgeon, ophthlmologist or, s in the uthor s prctice, mxillo-fcil surgeon should perform n upper-lid or sub-brow cut to mobilize the ngulr vein. The vessel is then gently held with suture nd cnnulted with 20- or 21-G thin or ultr-thin wll needle (e.g. Terumo UTW 21). A smll microguidewire, preferbly wire, is crefully introduced nd nvigted into the distl SOV. The the blunt plstic cnnul is stbilized with suture until the end of the procedure. Then, the ptient my be trnsferred to the OR (if not lredy prepred in the ngiogrphy suite). A smll microctheter (Trcker-10, Excelsior SL-10) is introduced nd nvigted into the proximl SOV or CS, which is usully possible without difficulties. Some opertors suggest slightly different technique, where the microctheter is directly introduced into the vein, which hs been ligted proximlly nd

206 8.2 Embolic Agents 197 distlly with silicon vsculr loops. These loops re pssed through smll peditric feeding tubes to control the bleeding while dvncing the ctheter using two-person technique (Miller 2007). For ll SOV pproches, the pcking of coils is performed in the reverse order compred to the IPS pproch, strting t the most posterior spect of the CS nd finishing the coil pcking t the SOV CS junction. In this mnner, the coil pcking begins t the posterior or contrlterl comprtment of the CS; the disconnection between CS nd SOV is done s the lst step. At the end of the procedure, the vein is mnully compressed for few minutes before the skin is sutured Trnsorbitl Puncture of the Superior or Inferior Ophthlmic Vein (Cse Report VI) Filure of ll previously described pproches justifies more ggressive technique, in the sme or subsequent session. A bi-plne rod mp is obtined using the 4-F dignostic ctheter demonstrting the course of the vein deep in the orbit. Under sterile conditions, 21- or 22-guge needle (e.g. Terumo UTW 21 or micropuncture set) is gently dvnced long the medil wll of the orbit posterior to the globe, using bi-plne fluoroscopy. When the needle reches the deep orbit, the SOV or the IOV is crefully cnnulted nd smll microctheter (Trcker-10) is introduced. The IOV is punctured by dvncing the needle long the inferior orbitl rim (White et l. 2007). After the microctheter is dvnced into the CS, coils re deployed, s described bove. Puncturing n rterilized vein within the orbit is delicte mneuver. Stbilizing the needle is crucil while mnipulting microctheter or pushing coils into the CS. Losing this ccess cn not only jeoprdize the procedure, but my lso cuse introrbitl hemorrhge with potentil vision loss. Avoiding excessive tension on the frgile venous wll by lesser dense pcking within the SOV my be dvisble Other Techniques For lterntive trnsfemorl, trnscutneous nd trnsorbitl CS pproches, including the superior petrosl sinus (SPS), pterygoid plexus (PP), the fcil vein (FV), the middle temporl vein (MTV), the frontl vein (FV), superficil middle cerebrl vein (SMCV) nd direct puncture of the CS, see discussion below. 8.2 Embolic Agents (Figs ) To cover the wide rnge of vrious embolic mterils nd their hndling is beyond the scope of this chpter. Embolic gents of prticulr interest for trnsrteril or trnsvenous occlusions of durl CSFs will be described below Polyvinyl Alcohol (PVA) nd Embospheres PVA prticles (Contour PVA, Boston Scientific, Fremont; TruFill TM PVA, Cordis Endovsculr, Mimi Lkes, FL) hve been employed for long time in wide rnge of pplictions nd re used frequently in preopertive emboliztion of vsculrized tumors such s meningioms, glomus tumors or cpillry hemngioms (Bendszus et l. 2000; Mnelfe et l. 1976; Wright et l. 1982; Berenstein nd Greb 1982; Kerber et l. 1978). In the 1980s nd erly 1990s, PVA ws lso used for embolizing brin AVMs (Scilf nd Scotti 1985). PVA prticles cn be injected wherever liquid embolic gents re considered unsfe, nd coils re unsuitble for ntomic or hemodynmic resons (Wright et l. 1982; Kerber et l. 1978; Jck et l. 1985). The prticles re mnufctured by different vendors in size between μm nd up to μm, nd re selected bsed on the cliber of the vessel in the trgeted territory. One longstnding disdvntge of PVA hs been the fct tht these prticles not only vried in size (rnges), but lso hd n irregulr surfce cusing ggregtion, clumping nd occlusion of ctheters nd proximl vessel segments. In ddition, the prticles showed tendency to swell fter being in contrst suspension for some time nd usully hd to be replced by new mixture severl times throughout the tretment session. Newer PVA prticle types come s hydrophilic microspheres in clibrted size (Contour-SE, Boston Scientific). They re nturlly opque with more uniform size distribution, wider rnge of sizes nd come pre-hydrted in sline in prefilled syringe. Alterntively, Triscryl geltin microspheres (Embospheres, Guerbet Biomedicl, Louvres, Frnce) cn be used nd my offer some dvntges becuse they re precisely clibrted t μm nd hve fewer tendencies to ggregte (Lurent et l. 2005; Beujeux et l. 1996; Derdeyn 1997). A recent comprison hs shown tht they produce less

207 198 8 Endovsculr Tretment blood loss when used for emboliztion of meningioms, presumbly becuse of more distl penetrtion ( Bendszus et l. 2000). In generl, smller prticles re used for embolizing tumors ( μm), becuse they will better penetrte smll tumor cpillries. Becuse smll PVA prticles my migrte into the pulmonry circultion when embolizing AV shunts, lrger prticles up to 1000 μm hve to be used for TAE of DCSFs. Injection of PVA is performed under fluoroscopy fter the prticles re suspended in iodine contrst mteril ( Berenstein nd Greb 1982; Szwrc et l. 1986), the concentrtion of which should be djusted to the inner lumen of the microctheter nd to the flow in the trgeted territory. The use of prticles nd embospheres is different from injecting crylic glue into n AVM nidus. In order to llow for prticles nd embospheres to rech the desired vsculr trget, sufficient flow must be mintined within the feeding pedicle. The concentrtion of PVA in the contrst suspension is chosen depending on the size of the vessels supplying the DCSF, but should be very dilute t first to void obstruction of the microctheter. According to chnges in the locl hemodynmics tht progressively occur during injection due to incresing blockge of the vsculr bed, the concentrtion of prticles nd their size my be djusted throughout the procedure. To strt with smller nd continue with grdully incresed prticle sizes is usully most effective. For optiml visul monitoring of the embolic flux nd erly detection of reflux mgnified blnk rod mpping is strongly recommended. The more distl ctheter is plced, the smller the prticles tht should be chosen. On the other hnd, the injection of prticles smller thn μm into the MMA, IMA or AMA my led to crnil nerve plsy. A more globl injection into the IMA using lrger prticles (possible through 4-F dignostic ctheter) my show n immedite ngiogrphic chnge, but is usully ineffective to chieve long-term occlusion of n AV shunt. As n djunct for trnsvenous coil occlusion, or when the im is minly to induce flow reduction nd to promote thrombosis, such strtegy my be pproprite. The ngiogrphic endpoint should be slow ntegrde or stgnnt flow Stinless Steel Coils Coils mde of stinless steel, lso nmed Ginturco coils fter its inventor Cesre Ginturco ( Anderson et l. 1977, 1979; Brun et l. 1985; Chung et l. 1980), hve dimeter between nd inches nd hve been used for long time for emboliztions in peripherl vsculr territories. Interwoven Dcron fibers increse the thrombogenicity of the coils, which require lrger dignostic ctheter, limiting their ppliction in the neurovsculr territory. They re the forerunners of the vrious detchble nd non-detchble pltinum coils vilble tody Pltinum (Non-detchble) Pushble Microcoils (Fig. 8.6) These coils hve lso been clled free or pushble coils nd re vilble in different lengths, dimeters nd shpes (stright, helicl, flower or spirl) (Grves et l. 1990; Morse et l. 1990; Yng et l. 1988), provided by severl compnies. Some mnufcturers hve dded Dcron fibers to increse the thrombogenicity while friction is minimized for the use in smll microctheters. Constnt flushing of the microctheter with heprinized sline is required to void friction within the microctheter tht my cuse blockge nd dmge of the ctheter lumen, necessitting exchnge. Such ctheter chnge often leds to loss of distl position, lengthening of the procedure or even necessittes rescheduling for subsequent session. Thus, for pushing coils, specificlly fibered coils, it is recommended to hve little or no friction t ll, miniml wll tension nd preferbly no kinks in the ctheter. Especilly lrger coils my produce friction when dvnced through shrp vessel turn nd sometimes hve to be deployed by forceful injection of sline bolus using 2- or 5-cc syringe. This will propel the coil through the ctheter lumen, technique tht my sve time nd costs. Coil positioning cn be slightly less ccurte nd some coils my be mlpositioned by flow reversl. Thus, this technique should be pplied only with some experience in endovsculr techniques. In generl, the use of pushble coils my in some cses be ssocited with coil migrtion or recoil of the microctheter, leding to coil plcement in unwnted nd sometimes ctstrophic positions ( Hlbch et l. 1998). Pushble fibered coils hve only been used by the uthor in ddition to detchble coils, to increse the thrombogenicity of the bre pltinum coil mesh. They re lso vilble in complex configurtions, such s VortX-coils (Boston Scientific). VortX-coils nd other fibered coil

208 8.2 Embolic Agents 199 configurtions hve become vilble with detchment system s GDCs (Hlbch et l. 1998). Other mnufcturers hve developed similr devices such s the nylon fibered coils (NXT, EV3), in sizes rnging from 2 20 mm to mm, combining the dvntges of being highly thrombogenic nd controllble by similr detchment system (Henkes et l. 2004; Hung et l. 2005; Tkzw et l. 2005; Zink et l. 2004). One min dvntge of pltinum s mteril for coils is its high opcity compred to stinless steel nd its MR comptibility. Some opertors hve used this type of coil for trnsrteril emboliztion of ECA feeders ( Giouleks et l. 1997). However, while their plcement leds to proximl occlusion, resulting in shunt reduction, it seldom produces permnent oblitertion. The mjor downside of trnsrteril coil emboliztion in AV shunting lesions is tht future rteril pproches through the sme pedicle re compromised nd new tretment sessions my become jeoprdized, unless TVO is used (Fig. 8.7). Thus, proximl rteril occlusion with coils should be voided whenever possible. It hs never been considered useful by the uthor, except when trgeting selective vessel blockge to void untowrd migrtion of embolic gents vi ECA-ICA nstomoses (e.g. MMA-OA nstomoses in preopertive emboliztion of meningioms). On the other hnd, for trnsvenous occlusions of DAVFs in generl, nd for DCSFs in prticulr, even non-detchble fibered coils re quite useful nd effective in ccelerting the occlusion process due to their high thrombogenicity. However, if these coils re not properly positioned or densely enough pcked, the chnces of leving residul AV shunt in comprtmentlized CS is high Detchble Pltinum Coils (Fig. 8.5) The first detchble coil, the GDC system (Guglielmi detchble coil system), is non-fibered, soft bre pltinum coil mounted on stinless steel wire tht cn be detched by electrolysis fter plcement in the desired loction. This coil system ws primrily developed for sfer emboliztion of intrcrnil neurysms, fter it becme evident in the 1980s tht using pushble coils nd detchble blloons ws ssocited with too mny serious complictions due to their inherent limittions. Mulln (1974) ws lredy ble to induce n occlusion of CSF using n electric current pplied through copper wires surgiclly introduced into the CS. Guglielmi becme interested in niml experiments on electrothrombosis in rteries nd in 1979, ccidentlly observed, during ttempts to induce electrothrombosis in n experimentl neurysm, detchment of the electrode from the steel wire (Strother 2001). This incident ws in principle the discovery of the electrolytic detchment of pltinum coils tht ws implemented in the GDC system in 1991 nd subsequent systems. GDC hs menwhile proved highly effective in the tretment of intrcrnil neurysms nd ws FDA pproved in The use of precise detchment mechnism is importnt not only for neurysm tretment, but lso for trnsrteril or trnsvenous occlusions of AV shunting lesions. Proper positioning of the coils in distnt loction, while lwys hving the possibility to retrieve the coil, is of key importnce for sfely nd effectively performing TVOs. In fct, one of the first successful tretments using GDCs ws direct CCF, occluded by F. Vinuel in 1990 using only two coils (Guglielmi et l. 1992). GDCs re mde of soft pltinum lloy nd re usully vilble in the configurtions shown in Tble 8.1. GDC Soft coils re mde of thinner wire thn stndrd coils nd thus more plible (e.g. GDC-10 Soft coils re 38% softer thn GDC-10 stndrd coils). In order to minimize the mechnicl stress pplied to crnil nerves coursing through the CS, the use of softer coils for the CS pcking is beneficil. At the sme time denser coil mesh cn be chieved tht is similr to neurysm tretments mjor fctor for chieving complete occlusion of the fistul. The use of coils with complex or sphericl configurtion t the beginning of the coil pcking, when bsket in certin comprtment of the CS needs to be ccurtely built, is lso dvntgeous. This is the cse t the connection between the CS nd the SOV or corticl vein. Buckling nd kick-bck of Tble 8.1. Avilble lengths nd dimeters of GDCs Coil Pltinum wire Ø (inch) Outer Ø (inch) Helix Ø (mm) Length (cm) GDC GDC-18 SOFT , 3, 4, 5, GDC GDC-10 SOFT , 3, GDC-10 U-SOFT ; 2,5; 3, 4 1 8

209 200 8 Endovsculr Tretment b c d e f g h Fig. 8.5 h. Pltinum detchble coils. GDC-18 coil, used only in few cses becuse of its stiffness. b GDC-10 Soft nd UltrSoft coil (rrow, c. 50% softer thn regulr soft coils) with similr size (3 mm/4 cm). GDC-10 coils were the preferred type of coils used in the uthors erly experience, often combined with soft coils (Cse Reports I VI, Figs. 8.3, 8.4). Bre pltinum coils re vilble in different lengths nd dimeters, provided tody by vrious mnufcturers. They re less thrombogenic thn fibered coils, but usully more plible nd thus more suitble for dense pcking. The mjor dvntge of ll detchble over pushble coils is the possibility of repositioning them until optiml deployment is chieved. Softer coils cn be pcked very densely, while minimizing mechnicl pressure to the crnil nerves within the CS. c 3D coils (BSC) nd other coils with sphericl configurtion cn be useful s strter coils to block the connection between the CS nd the SOV or corticl veins s precisely s possible. This first 3D-coil genertion ws reltively stiff nd could dislodge the microctheter during detchment, leding to loss of position. d The newly developed 360 coil for the GDC system with n improved design (BSC). Severl other mnufcturers hve developed coils with complex or sphericl configurtions tht re superior to the originl 3D coil (e Complex coil, MicroVention; f MicruSphere, Micrus Endovsculr). g MicroPlex HyperSoft Coil (MicroVention). This new, highly complint coil provides remrkble pcking density. Its extremely soft proximl end minimizes movement nd deflection of the microctheter tip nd reduces buckling or kick-bck. The coil llows for more precise coil plcement or trgeted venous comprtments nd fcilittes repositioning (see Cse Illustrtion VI). HyperSoft coils re vilble in 2 6 mm 1 8 cm length. h Ultipq Coil (Micrus Endovsculr), prtillly used in Cse Illustrtion III

210 8.2 Embolic Agents 201 b c Fig. 8.6 c. Vrious fibered (pushble) coils (BSC). Simple, fibered coil, here s C-shped spirl (3/7 mm) with ttched Dcron fibers for incresed thrombogenicity. These spirl coils re comptible with Trcker-10 nd -18 nd re introduced using specil pusher or injecting sline. The min drwbck of ll pushble coils is tht they cnnot be repositioned. Furthermore, the fibers my cuse friction nd dmge to the microctheter, requiring ctheter exchnge tht is undesirble during lengthy trnsvenous occlusions. b, c Fibered coils (2/6 mm) with complex configurtion such s this VortX coil cn be dded to ccelerte thrombosis. However, becuse of their size nd stiffness, they my not lwys dpt to the smll spces nd interstices in the CS. Consequently, the ctheter my dislodge, leding to loose pcking or loss of ccess. This my result in prtil occlusion or comprtmentliztion of the CS. Fibered coils, lso vilble s detchble versions, hve been used by the uthor s djunctive device in some cses microctheter hs occsionlly been problem, especilly with the reltively stiff first 3D-GDC genertion from BSC. This hs been overcome with newer coils from other mnufcturers (Fig. 8.5d f). SR Coils (stretch resistnt coils) hve interwoven double strnds of polypropylene or other mteril tht prevent stretching nd unrveling, which is dvntgeous when long-used microctheter develops friction or even kinks nd cnnot be replced. Although coil unrveling or filure to detch during TVO re technicl complictions, their consequences re less drmtic thn during neurysm tretment. As bil out, the coil my be mechniclly detched (Lee nd Yim et l. 2008) by clockwise rottions until it breks, which does not cuse hrm, even when the rest of the bsket in the CS gets entngled (personl unpublished experience; see lso Fig. 8.4). So-clled UltrSoft coils (BSC) provide further stiffness reduction of bout 50%, llowing for pcking density of up to 55% in experimentl neurysms (Piotin et l. 2004). These coils my be deployed in extremely smll vsculr pockets (Benndorf et l. 2002). Recently, new HyperSoft coil (MicroVention Fig Ineffective trnsrteril emboliztion of IMA brnches with coils. Right ECA injection. AP view shows n AV shunt t the righ CS (sterisk) drining to the left side nd cusing corticl venous dringe. This ptient ws trnferred from nother institution fter undergoing TAE with coils (rrows). Proximl occlusion of ECA supply of n AVF with ny type of coil (or glue) usully hs little or no effect. It seriously compromises future ttempts of ctheteriztion insted nd should be voided. This ptient ws successfully mnged by TVO nd fully recovered (Benndorf et l., 2000)

211 202 8 Endovsculr Tretment Inc., Aliso Viejo, CA) with remrkble complince nd shorter, softer detchment zone hs been introduced (Fig. 8.5g). This coil llows high pcking density while minimizing microctheter movement nd proves very useful for treting DCSF (Cse Illustrtions V). Pltinum detchble coils hve been used to lrge degree in the group of ptients studied by the uthor nd only in some cses hve been combined with fibered coils. Detchble coil systems from vrious other mnufcturers re vilble tody providing equl, if not better, mechnicl properties compred to GDCs. Beside electrolysis, other detchment techniques hve been developed using mechnicl mechnism such MDS (Blt Extrusion) or DCS (Willim Cook Europe), het (Micrus Endovsculr Corportion, Sn Jose, CA) or hydrulic pressure (MicroVention, Cordis Neurovsculr) tht llow deplopment of pltinum coil in quick, sfe nd relible mnner. The dditionl coting of coils with bioctive mterils such s polyglycolic-polyctic biopolymer (PGLA) to promote fibrocellulr prolifertion nd incresed endotheliliztion is of little relevnce for occlusion of DCSFs. A fundmentlly different pproch to the problem of coil compction nd long-term occlusion of neurysms hs been the introduction of coils covered with hydrogel (HydroCoils) HydroCoils (Figs ) Among the new coil genertions with bioctive surfce coting, hydrogel, mounted on detchble pltinum coil (HydroCoil, HES,) hs ttrcted ttention for the tretment of DAVFs. The Hydro- Coil is pltinum-bsed coil with Hhydrogel coting, mteril tht expnds while in contct with wter or blood. The wter bsorption leds to swelling of the mteril which results in n increse of the coil dimeter fter being deployed in the blood circultion. This rther unique property of the HES proved dvntgeous in treting cerebrl neurysms (Kirsch et l. 2006; Goto nd Goto 1997; Nemoto et l. 1997; Hnok et l. 2006; Schuknecht et l. 1998; Mironov 1994; Hlbch et l. 1992; Stomi et l. 2005; Tpts 1982). It is currently believed tht while the HES increse their dimeter, they ctully do not over-swell, so tht dditionl mechnicl pressure will not occur. A HydroCoil 18 expnds from to 0.034, HydroCoil 14 expnds from to nd the HydroCoil 10 expnds from to The mximum reposition time of these coils is 5 min. in 0.021, or inner lumen microctheter, respectively. A new detchment system (V-Trk) utilizes thermo-mechnicl technology by sending current to heting coil t the end Hydrted b Dehydrted x 150 Mgnifiction, 25 kv, 200 μm x 500 Mgnifiction, 20 kv, 100 μm Fig. 8.8,b. Hydrogel fter contct with wter., b The porous hydrogel provides n excellent substrte for heling (neointim formtion, smooth muscle cell migrtion) becuse blood components (proteins, etc.) re bsorbed into the hydrogel during the swelling process. Hydrogel is polymeric mteril tht is cpble of swelling in wter (diffusion of wter through the polymer cusing disentnglement of polymer chins nd swelling). Intelligel technology provides ph-dependnt, controlled expnsion in response to chnges in the environment. The hydrogel preprtion is liquid rection mixture tht contins: A monomer cross-linker, polymeriztion inititor, nd porosigen. A controlled expnsion rte is imprted through the incorportion of unsturted monomers with ionizble functionl groups

212 8.2 Embolic Agents 203 b c d Fig. 8.9 d. HydroCoils. HydroCoils-10, -14, nd -18 before nd fter expnsion. HydroCoils-18 re deliverble through lumen microctheter. The outer dimeter is unexpnded nd expnded The mximum repositioning time in lumen microctheter is 5 min. b The HydroCoil-10 is pushble through lumen microctheter, hs mximum repositioning time of 10 min (Excelsior 10/18), 7 min (SL 10 & Prowler -14) nd 5 min (Prowler -10). This coil is stretch-resistnt nd expnds from to The stiffness is comprble to MicroPlex-10 coil. HydroCoils pper dvntgeous over stndrd pltinum coils for occlusion of venous chnnels, such s the CS. They increse the overll volumetric pcking, while cusing less pressure to the crnil nerves. This reduces the totl mount of coils s well s rdition exposure. c HydroCoil-14 Pltinum coil core (0.008 ) with gel covering nd pltinum overcoil. The Hydrogel polymer expnds 3 in the presence of blood to dimeter of The coil hs helicl configurtion nd cn be delivered through lumen or lrger microctheter. d HydroSoft Coils. The Hydrogel expnds beyond the pltinum wind providing up to 70% more volume fill thn 10 system coil for better volumetric filling nd mechnicl stbility. Swelling of the gel occurs to lesser degree nd reches bout Coils re vilble in 2 6 mm 1 8 cm length b Adhesive Severed Polymer Tether Coupler Fig. 8.10,b. V-Trk system. The newer detchment system consists of more flexible delivery pusher nd self-contined, disposble integrted power supply. The coil is detched vi thermo-mechnicl detchment mechnism. Het cts s ctlyst to sever polymer tether connecting the pusher to the implnt. The tether is tied nd dhesively bonded to the proximl end of the implnt. Becuse of tht only very short til () is left fter detchment. No melted polymer or prticles re relesed during the detchment nd the end of the implnt is squre, reducing risk of jmming with other coils inside the microctheter. Coils re detched in 0.75 s

213 204 8 Endovsculr Tretment of the pusher. The coil is ttched with polymer tether under tension. The het severs polymer tether, detching the coil in bout 0.75 s. The use of HES for AV shunting lesions is described only in limited number of reports to dte (Mrden et l. 2005; Morsi et l. 2004). First, the swelling of the hydrogel is idel for progressive mechnicl occlusion of venous AVF comprtment, s it will djust to the irregulr surrounding ntomy better thn bre metl coils. Such progressive swelling nd occlusion process cn help to reduce the totl number of coils needed for trnsvenous occlusion, s seen in cerebrl neurysms ( Deshies et l. 2005). Second, the softness of the gel will llow dense pcking of the coils, while reducing mechnicl pressure on intrcvernous structures nd the risk of CN deficits. It hs been shown tht the hydrogel expnsion does not contribute to ny chnge in the intrneurysml pressure (Cnton et l. 2005). Intervls of 5 10 min between control ngiogrms fter ech coil deployment (llowing for full expnsion of the gel) my lredy revel significnt reduction of the AV shunting flow, indicting progressive occlusion nd thrombosis. The swelling my increse the dimeter of the coils up to five to 11 times of stndrd pltinum coil, which llows for chieving higher volumetric pcking thn possible with bre pltinum coils while reducing the totl mount of metl. The fct tht the gel will swell fter the coil hs been deployed mkes this coil very suitble for progressive occlusion of lrger venous comprtments such s durl sinuses. Thus, the usefulness of HydroCoils for occluding DAVFs nd DCSFs is rther obvious. In Cse Illustrtion IV, effective trnsvenous occlusion using HydroCoils is demonstrted Liquid Embolic Agents: Cynocryltes (N-butyl-cynocrylte, Acrylic Glue, Histocryl, Trufill TM n-bca, Glubrn TM ) In the 1980s, NBCA (N-butyl-2-cynocrylte), initilly known s Avcryl in the US nd s Histocryl in Europe (Brun-Melsungen, Germny) replced IBCA (Isobutyl-2-cynocrylte), being the first crylic glue for medicl ppliction (Brothers et l. 1989). Histocryl is tissue dhesive tht polymerizes when in contct with ion solutions such s contrst, sline or blood, nd ws initilly developed for use in dermtology. Cynocrylte ws for long time considered the only gent cpble of cusing permnent occlusion when injected intrvsculrly. Becuse of this property, it hs been widely used in the tretment of brin AVMs nd AVFs. The experience in using glue is extensively published (Brothers et l. 1989; Duffner et l. 2002; Liu et l. 2000,b; Henkes et l. 1998; Cromwell nd Kerber 1979; Kerber et l. 1979; Gounis et l. 2002; Li et l. 2002; Sdto et l. 2000; Rffi et l. 2007; Troffkin nd Given 2007; Wkhloo et l. 2005). Its liquid form llows penetrtion into very smll vessels nd is prticulrly suitble for completely occluding n AVM nidus (Brothers et l. 1989). The polymeriztion of NBCA strts fter few seconds of contct with blood nd cn be controlled only to some degree, depending on vrious fctors such s blood flow velocity, speed of the injection, ph of the blood nd temperture of the glue. In order to increse rdiopcity for fluoroscopic control, Histocryl is mixed by most investigtors with Lipiodol (ethiodized oil), cottonseed-oil-bsed contrst gent mde by Lbortorie Guerbet (Frnce). In ddition, this mixture decelertes the polymeriztion depending on the concentrtion of lipiodol from 1 30 s (Cromwell nd Kerber 1979). Introducing the mixture with lipiodol significntly improved the hndling of glue ( Stoesslein et l. 1982). While concentrtions of pproximtely 50% (60/40) were used in erly yers, glue hs been incresingly diluted recently. For AVMs, dilutions of 20% 25% or less re menwhile preferred. A concentrtion below 15%, lthough still usble, significntly impirs the polymeriztion; its dhesive properties re reduced, nd the glue my esily migrte into the venous side of the AV shunt. Becuse of the potentil hzrdous complictions, the hndling of crylics hs to be studied nd prcticed extensively before it my be sfely nd effectively used. Prior to the emboliztion with glue, the microctheter must be flushed with 5% glucose (dextrose in wter) to void premture polymeriztion within the ctheter. Ech injection of glue must be crefully observed under fluoroscopic control in order to recognize erly even the smllest reflux into the proximl feeder nd to void gluing the ctheter to the vessel wll. Additionl use of tntlum powder in the mixture further enhnces rdiogrphic visibility. A smll reflux (up to 5 mm) my be tolerted with modern hydrophilic ctheters, depending on the concentrtion of the glue nd the loction of the microctheter, prior to removl. When glue is injected directly into sinus or venous comprtment such s the CS, gluing of

214 8.2 Embolic Agents 205 the microctheter is less of problem. It hs been found tht polymeriztion of glue my be further prolonged by dding tntlum powder. A concentrtion s low s 10% 15% hs been used by dding tungsten nd lipiodol for TAE of five complex DCSFs (Liu et l. 2000b). Gounis et l. (2002) showed tht predictbility of the emboliztion process with NBCA cn be improved by dding glcil cetic cid to the embolic mixture. Due to regultory issues in North Americ in the 1990s, the use of crylic glue ws limited in the US compred to Europe. In 2000, slightly modified version of Histocryl, Trufill TM n-bca (Cordis Neurovsculr, Mimi lkes, FL) received FDA pprovl for tretment of brin AVMs, nd showed good combintion of penetrtion nd permnence (Jordn et l. 2005). It is lso mixed with Ethiodol (Trufill TM -NBCA Cordis Neurovsculr) in vrious concentrtions from 2:1, 3:1, etc. The intrvenous injection of glue for occlusion of DCSFs hs been reported only to limited degree (Wkhloo et l. 2005) nd ws used in few cses in the studied group of ptients (Benndorf et l. 2004, see lso Fig. 8.35). A modified cynocrylte, Glubrn TM (nd Glubrn 2 TM ), ws recently introduced in Europe (Rffi et l. 2007) Onyx (Ethylene-Vinyl Alcohol Copolymer) (Fig. 8.11) Ethylene-vinyl lcohol copolymer (EVOH, Onyx, EV3, irvine CA) is newer liquid embolic gent whose min chrcteristic is its non-dhesiveness. In contrst to dhesive cryltes, Onyx is precipitting embolic gent tht minly cuses mechnicl vessel occlusion. It prevents microctheters from gluing to the vessel wll, nd thus llows significntly prolonged injection times (up to 40 min or more). It is mixed with solvent, dimethyl sulphoxide (DMSO) nd tntlum powder (Jhn et l. 2001) nd comes in redy-to-use vils for AVMs nd AVFs in three concentrtions: 6% (Onyx 18), 6.5% (Onyx 20) nd 8% (Onyx 34), dissolved in DMSO (Suzuki et l. 2006). Onyx is pre-mixed, rdiopque, injectble embolic fluid tht solidifies upon contct with queous solutions or physiologic fluids. In contrst to NBCA, this property llows for temporrily pusing the injection to prevent untowrd lekge into non-trgeted territory (Jordn et l. 2005). It forms spongy polymeric cst nd skin solidifying from the outside while continuing to flow, much like lv, in the liquid center. Thus, Onyx cn be delivered in reltively cohesive mnner. The initil description of its use for emboliztion of AV shunting lesions cme from Tki et l. (1990) nd Terd et l. (1991). The uthors reported the successful emboliztion of cerebrl AVMs with EVAL (ethylene vinyl lcohol copolymer) dissolved in DMSO. Sfety concerns emerged fter niml studies conducted by Chloupk et l. (1994), who discovered significnt ngiotoxicity of the DSMO. A reexmintion, however, showed no cute hemodynmic effects, nd no infrction or SAH t slower injections rtes of 30, 60 nd 90 s ( Chloupk et l. 1999). Subsequent studies reveled tht the min fctors influencing vsculr toxicity re the contct time with the rteril wll nd the totl volume of DMSO. It ws demonstrted tht DMSO enters the bloodstrem, is bsorbed into tissue nd metbolized to dimethyl sulfone (DMSO 2 ) nd dimethyl sulfide (DMS) (Chloupk et l. 1999). These metbolites re eliminted vi the kidneys (within 1 week; 80%), nd vi the skin or lungs, cusing grlic odor to the breth until complete elimintion: dys. Onyx received FDA pprovl for the tretment of cerebrl AVMs in 2005 nd hs been incresingly used since (Nogueir et l. 2008; Mounyer et l. 2007; Toulgot et l. 2006; Cognrd et l. 2008; Art et l. 2004). The use of Onyx hs number of technicl dvntges over cynocrylte, the most importnt of which is the possibility of prolonged injections in slow nd controlled fshion. If properly performed, it llows chieving deeper nidus penetrtions in AV shunting lesions. Whether or not long-term occlusion rtes re comprble or superior to the ones obtinble with NBCA remins to be nswered by ongoing nd future studies. Among the disdvntges of Onyx is its limittion to DMSO comptible microctheters such s the Rebr, brided, reltively stiff microctheter, the Ultrflow nd the Mrthon (Ev3, Irvine CA) both flow-guided microctheters. The Echelon is nitinol brided, over-the-wire ctheter nd is vilble s Echelon 10 (1.7F) nd Echelon 14 (1.9F). It hs preshped tip of 45 or 90 degrees nd is prticulrly useful for trnsvenous occlusions, s it lso llows plcement of coils (see Figs nd 8.34). Other comptible ctheters re the flow-guided 1.2-F 1.8- F Bltcci nd the brided Coril+ blloon ctheter (Blt Extrusion, Montmorency, Frnce). A novel

215 206 8 Endovsculr Tretment b c d e f Fig f. Onyx nd tools for its use. Onyx (EV3, Irvine CA) is pre-mixed, rdiopque, injectble embolic fluid. It consists of ethylene-vinyl lcohol copolymer (EVOH), dimethyl sulfoxide solvent (DMSO) nd micronized tntlum powder. b Ultrflow HPC (EV3, Irvine CA): Flow-guided DMSO comptible microctheter. c Mirge (EV3, Irvine CA): hydrophilic coted tpered guidewire (0.2 mm) with proximl prt of mm for dditionl support. d Blt-Sonic microctheter (Blt Extrusion, Montmorency, Frnce). This novel microctheter with detchble tip (Fusecth) mkes the use of Onyx sfer nd more efficient. The detchble distl segment llows longer reflux so tht the cst cn rech more of the vsculr bed distl to the microctheter. e, f Polymer precipittion occurs upon contct with ionic solutions. The solvent diffuses wy nd spongy polymeric cst forms. This forms skin - solidifying from the outside in. The liquid center continues to flow (like lv) concept is the recent introduction of microctheter with detchble tip (Detchble Fusecth, cm) tht llows removl of the microctheter even when entrpment occurs (Blt-Sonic). This will improve sfety nd efficcy becuse it llows penetrtion of more vsculr beds (Fig. 8.11d). Some opertors consider second drwbck the fct tht the long injections, required in some cses for complete nidus csting, my be ssocited with n incresed rdition dose. Although unlike with NBCA gluing of microctheters is not n issue, ctheter entrpment by Onyx reflux my still occur nd cn in some cses led to clinicl complictions, such s embolic infrction or cerebrl hemorrhge. Finlly, it must be mentioned tht, even though ppeling s new mteril, the use of Onyx for trnsrteril emboliztion or trnsvenous occlusions is not without potentil hzrds. Trnsient or permnent CN deficits my occur ( Elhmmdy et l. 2009; Lv et l. 2008) Stents Stenting hs been used in the recent pst for occluding direct CCFs either in combintion with coils or s single tretment (Weber et l 2001; Men et l. 2003; Ahn et l. 2003; Moron et l. 2005). In order to primrily support coil plcement or to secondrily keep coils within the CS, severl intrcrnil stents re vilble, such s the Neuroform 3 (Boston Scientific, Fremont, CA), Enterprise (Cordis Neurovsculr, Mimi Lkes, Fl), LEO (Blt Extrusion, Montmorency, Frnce) or Phros (Biotronik, Micrus Corportion, Sunnyvle, CA) nd others. However, their use for occlusion of DCSFs is, if t ll, of limited vlue, nd thus detiled description is beyond the scope of this chpter. A relevnt reduction of rteril fistul supply by plcing highporosity intrcrnil stents cnnot be expected.

216 8.2 Embolic Agents 207 The only stents with some ppliction for durl AV shunts of the CS re covered stents. Covered stents hve been used to some degree in the recent pst to sel leks in the crotid wll of direct CCFs (Archondkis et l. 2007; Nesens et l. 2006; Redekop et l. 2001; Kocer et l. 2002; Felber et l. 2004). The device being minly used is the Jostent (Abbott Vsculr Devices, Illinois), coronry stent grft tht consists of stinless steel 316L body, covered with polytetrfluoroethylene (PTFE) lyer. It is vilble in vrious sizes between 3 mm 5 mm dimeter nd 9 mm 26 mm length (Fig. 8.12). The Jostent hs been primrily developed for treting coronry neurysms nd pseudoneurysm ( Heuser et l. 1999; Gercken et l. 2002). This stent hs mrkers for ccurte plcement nd low-crimped profile, comprble to conventionl coronry stent systems. The PTFE mteril is plced between two stinless steel struts llowing for stent expnsion in one direction, while remining rigid in the other. Idelly, the fully expnded stent retins its rdil strength to resist vessel wll pressure. In order to chieve optiml pposition to the vessel wll, the stent is blloon-expnded with minimum expnsion pressure of 14 tm. Although the stent comes with tpered tip design for incresed crossbility nd enhnced trckbility nd is comptible with guidewire, the device is reltively stiff to be used in the cerebrl circultion nd its plcement requires dul ntipltelet therpy nd ASA for n indefinite period of time. Therefore, the use of such device in ptients with DCSF needs to be crefully blnced ginst the potentil risk of rteril dissection nd stent thrombosis. The development of devices dedicted for neurovsculr pplictions is under wy nd will hopefully find its introduction into clinicl prctice soon. Fig Covered stent. Jostent Coronry Stent Grft (Abbot Vsculr, IL), blloon expndble, stinless steel (316L) with n ultr thin, expndble PTFE lyer. The stent requires 7-F or 8-F guiding ctheter nd is reltively stiff device. Expnsion rnge is between mm, 9 26 mm lengths re vilble. Advncement through the tortuous intrcrnil ntomy, like the petrous or cvernous ICA, my become difficult nd is in some cses impossible b Fig. 8.13, b. Bi-plne ngiogrphic suite for neuroendovsculr tretment (Interventionl Neurordiology Suite, Ben Tub Generl Hospitl, Houston, Tx). Although personl preferences, hbits nd choices of tools nd devices my vry from center to center, generl rules to be followed re quite similr. Modern systems should hve t lest six monitors for simultneous rdiogrphic control in two projections (Left: Live fluoro, middle: Rod mp, right: Reference). Additionl monitors my be used for displying 3D dt or physiologicl monitoring. Ltest developments feture full-color, 56-inch lrge screen disply tht enbles users to select their preferred screen lyout for the plnned procedure step t tbleside. b Bilterl femorl ccess for EVT of CSF. Arteril ccess on the right, venous ccess on the left side. For control ngiogrms, plcement of 4F dignostic ctheter in the ICA or ECA is sufficient. In this cse, 6-F guiding ctheter ws used to nvigte HyperGlide blloon (EV3) into the ICA for temporry infltion during trnsvenous coilpcking of direct CCF. The venous side ws ccessed with 6-F guiding ctheter nd two rotting hemosttic vlves (Y-connectors) tht llow dul plcement of microctheters into the CS (bove). Both systems re connected to three-wy stopcock with n extension tht llows contrst injections for rod mpping or ngiogrphic controls. Continuous pressure flushings for guiding nd microctheters re mndtory to void contmintion with ir or clots. A clen nd orgnized work environment in the ngio suite (which should preferbly be mintined throughout the procedure) is key to voiding thrombembolic nd other technicl complictions. Well trined, dedicted nurses nd technicins ply n importnt role for successful tem

217 208 8 Endovsculr Tretment 8.3 Results of Trnsvenous Emboliztions (Figs , Tbles ) Approches (Benndorf et l. 2004) The inferior petrosl sinus (IPS) most frequently served s venous route in totl of 37 cses (82%), mong which 25 procedures (67.5%) resulted in successful occlusion without employing n dditionl route. In ten of these cses (27%), the IPS ws successfully ctheterized despite n pprent prtil or complete thrombosis. In three cses (8.1%), the IPS ws ccessed using direct stick of the internl jugulr vein. The fcil vein (FV)/superior ophthlmic vein (SOV) pproch ws used in 13 cses (29%). The SOV ws surgiclly cnnulted in eight, while the remining five ptients underwent trnsfemorl ctheteriztion or direct puncture. In ll 13 cses, n IPS pproch ws ttempted first nd either did not llow complete occlusion or filed entirely. In one ptient, trnsfemorl pproch nd percutneous cnnultion filed nd the ptient underwent needle puncture of the deep (intrconl) SOV. In four cses (8.8%), the superior petrosl sinus (SPS) pproch ws chosen, in three s single pproch, nd in one ptient with bilterl fistul, it ws combined with n IPS pproch. One ptient (2.2%) with primry dringe vi the frontl vein underwent percutneous puncture of the vein using n 18-G needle with subsequent complete occlusion of the fistul. Another ptient, in whom ll trnsfemorl techniques filed, underwent open surgery nd direct puncture of the Sylvin vein using n 18-G needle nd Trcker-18. The fistul showed complete occlusion fter deployment of two fibered coils. The microctheter ws successfully nvigted to the contrlterl side in 13 ptients (29%), in 11 cses (24%) using the IPS nd in two (4%) using the SOV pproch Angiogrphic nd Clinicl Outcome Tble 8.2. Summry of results chieved in the group studied (Benndorf et l. 2004) Demogrphics: 45 Ptients Femle/mle: 32/13 Age: Symptoms: red eye (20), chemosis (22), exophthlmos (29), diplopi (25), retroorbitl pin (7), visul loss (9), bruit (4), vertigo (1) Fistul type: D (41), B (3), C (1) Bilterl fistul site: 8 Corticl dringe: 9 (20%) no neurologicl deficit, no ICH Trnsvenous pproches: IPS: 37, thrombosed IPS: 10 FV/SOV: 13, trnsfemorl (4), surgicl exposure (8), direct (trnsorbitl) puncture (1) Superior petrosl sinus: 4 Frontl vein: 1 Sylvin vein: 1 Combined: 10 Cross-over: 8 Direct puncture of IJV: 3 Results: Antomic cure: 42/45 (93%), (3 finl follow-up pending) Single/multiple sessions 35/10 Initil result complete occlusion: 28 (12 subtotl) Clinicl cure: 41/45 (91%) four improved (finl follow-up pending) Complictions: Deth: 0 Mjor permnent: 0 Minor permnent: 0 Minor trnsient: 1/45 (2.2%, 6 th CN-plsy) Minor extrvstion (IPS): 1/45 (2.2%, cliniclly silent) Trnsient incresed IOP: 2/45 (4.4%) Awrded with Mgn Cum Lude, ASNR 2004 Settle In the group of ptients studied (Benndorf et l. 2004), complete occulsion of the fistul ws chieved in 42/45 ptients (93%). Finl ngiogrphic followup ws still pending in three. This result ws ccomplished in 35 cses, using single tretment session, while in ten cses two or three sessions were required to chieve complete occlusion. Complete occlusion s immedite post-emboliztion result ws documented in 30 (66%) cses, while in 12 (26.6%) subtotl or incomplete occlusion ws documented. Follow-up t 3 months confirmed complete occlusion of the AV shunt. These numbers refer to the totl number of ptients (n = 45), while the ones seen for ngiogrphic follow-up (n = 42) showed 100% ngiogrphic occlusion. Clinicl cure ws chieved in 41/45 (91%); four ptients reported improved symptoms by telephone interview, finl follow-up ws still pending. There were no procedure-relted permnent ophthlmologicl or neurologicl deficits. One ptient (2.2%) developed minor trnsient 6 th CN plsy fter coiling of the right nd left CS using IPS pproch through thrombosed sinus. She ws treted with corticosteroids for 1 week nd ws symptom free when seen for her clinicl follow-up. In one

218 8.3 Results of Trnsvenous Emboliztions 209 ptient (2.2 %), venous extrvstion ws observed during ctheteriztion of the IPS. The ptient woke up without ny clinicl sequele. In two ptients (4.4%), trnsient ggrvtion of their ophthlmologic symptoms ws seen fter trnsfemorl SOV pproch filed to provide ccess to the fistul site. Both ptients underwent more ggressive procedures (one hd surgicl SOV cnnultion, the other deep SOV puncture). Both recovered fully fter complete occlusion of the fistul. Out of this group of ptients (Benndorf et l. 2004), seven typicl venous pproches to the CS will be described in the following s Cse Reports (Figs ) illustrting technicl possibilities for gining endovsculr ccess nd ssocited problems. These cses were treted by the uthor t the Chrité, Humboldt University Berlin between 1992 nd They re complemented by Cse Illustrtions (Figs ) showing vluble lterntive pproches or techniques provided by other experienced opertors from interntionl centers Cses Reports (Figs ) Cse Report I: Approch vi the Thrombosed IPS (Figs ). A 56-yer-old ptient presented in October 2000 with n 18-month history of swollen eye, conjunctivl engorgement nd retroorbitl pin. Since Februry 1999, the ptient noticed occsionl occipitl pin combined with dizziness nd nuse for 2 3 dys s well s bilterl diplopi. After severl hospitl stys nd tretment with corticosteroids becuse of suspected endocrine orbitopthy, oculr myositis nd pseudo tumor cerebri in July 1999, DCSF ws dignosed using intrrteril DSA. By tht time, the ptient hd undergone severl CT nd MRI exm tht remined inconclusive. After n unsuccessful ttempt to embolize the fistul in nother institution, nd clinicl worsening, the ptient ws referred 14 months lter to our institution. At tht time, she hd moderte bilterl exophthlmos nd noticeble conjunctivl injection with introculr pressures of 18 mm Hg in the right eye nd 20 mm Hg in the left eye. The ptient further complined bout horizontl diplopi. Her funduscopy showed significntly elongted veins nd mssive retinl hemorrhges. Intrrteril DSA reveled smll AVF t the left posterior CS. The fistul ws exclusively supplied by ipsilterl brnches of the TMH (Type B fter Brrows) nd drined into the left SOV nd vi the ICS into the right CS nd SOV. Becuse there ws no posterior dringe identifible, phlebogrphy ws performed prior to the endovsculr procedure. Repeted intrvenous injections from different ctheter positions in the IJV reveled tiny notch in the vicinity of the jugulr bulb. Here, hydrophilic microguidewire (Trnsend-14) ws introduced few mm nd used to nvigte smll microctheter, the Trcker Excel (0.014 ). Then, more flexible guidewire, the Terumo Hedliner (0.012, 90º), whose tip ws formed into smll loop, ws dvnced. This loop ws gently dvnced within the presumbly thrombosed IPS lumen. Control ngiogrms were performed using miniml mnul injection pressure tht showed nrrowed, irregulr lumen without ny recognizble communiction to the CS or to the fistul. A bi-plne blnk rod mp llowed control of the ctheter nvigtion, while voiding indvertent entry into smll pontine veins. After reching the left posterior CS, superselective contrst injection demonstrted the fistul s dringe into both prtilly thrombosed SOVs. In this mnner, the microctheter ws dvnced through the ICS into the right CS. The occlusion of the fistul strted with plcement of detchble pltinum coils, t first with 3/8 mm (GDC-10), nd ws continued from right to left through the ICS initilly with 2 3 mm coils nd some GDC Soft coils (13 coils totl). After reltively dense pcking of the posterior venous (CS) pouch on the left side ws ccomplished, the control series indicted n occlusion of the fistul. The ptient prtilly recovered from her symptoms within the following week nd noticed complete disppernce of her diplopi 8 weeks lter. The venous congestion resolved s well nd control ngiogrm fter 5 months confirmed complete nd permnent occlusion of the fistul Cse Report II: Cross-Over Approch vi the IPS (Figs. 8.17, 8.18) In Februry 1999, 77-yer-old womn reported flickering in front of her right eye of 4 yers stnding. She lso presented with proptosis of her right eye, incresing diplopi due to 3 rd, 4 th nd 6 th CN plsy s well s chemosis nd decresed vision for (Text continues on p. 215)

219 210 8 Endovsculr Tretment b c d e f?? Fig f. Cse I: Approch through thrombosed IPS, initil DSA. Right () nd left (b) ICA injections, AP views: Smll rteriovenous shunt t the left CS (sterisk), supplied by brnches from the ipsi- nd contrlterl MHT (rrows). Left ICA injection lterl view, erly (c) nd lte (d) phse: Opcifiction of the posterior CS (sterisk). No dringe vi the IPS, which is nrrowed nd does not opcify in the lte venous phse, except for short tpered segment (double rrows). Such residul tpering my indicte more recent thrombosis tht often fcilittes ctheter nvigtion. e, f Phlebogrm of the left IJV, AP (e) nd lterl (f): No cler (?) visuliztion of the IPS (double rrows)

220 8.3 Results of Trnsvenous Emboliztions 211 b c d e f g h Fig i. Cse I: Trnsvenous occlusion. Sinogrms of the left IPS, in lterl () nd AP (b) views: Irregulr, highly stenotic lumen of the IPS due to thrombosis. A connection to the rteriovenous shunt cnnot be identified. c e Corresponding rod mps during ctheteriztion of the IPS (double rrows) in lterl (c) nd AP (d,e) views: The highly flexible Hedliner cn be nvigted even through these extremely irregulr nd nrrowed structures, when its tip is formed into loop (rrow, loop technique ). f,g Sinogrms fter reching the CS, AP nd lterl views: Demonstrtion of the fistul s dringe into the left SOV (long rrow) nd thin intercvernous sinus (rrowheds) into the right SOV (short rrow), s well s into both IOVs (thin rrows). Abrupt stgntion of contrst within both nterior SOVs (double rrow), suggesting thrombosis. h Dense coil pcking (GDC Soft) strting in the right CS, continued through the ICS to the left posterior CS (sterisk) resulted in complete occlusion (i). (Benndorf 2002) i

221 212 8 Endovsculr Tretment b c d e f g h Fig h. Cse I: A 56-yer-old womn with n t lest 18-month history of bilterl eye redness nd diplopi. Proptosis, irrittion of the conjunctiv nd the fundus. d Before emboliztion: Bilterl episclerl congestion nd hyperemi of the iris. Fundoscopy shows significnt tortuousities nd congestion of retinl veins s well s extensive retinl hemorrhges nd ppillry edem in both eyes. e h At 3 months fter emboliztion: Nerly complete resolution of the conjunctivl signs nd normliztion of the fundus (Benndorf 2002)

222 8.3 Results of Trnsvenous Emboliztions 213 b c d e f h i g Fig i. Cse II: Cross-over IPS pproch, initil DSA nd trnsvenous occlusion.,b Left ECA injection, AP nd lterl views: Arteriovenous shunt involving the ICS (white sterisks) nd right CS (b/w sterisk), which exclusively drins into the IOV (short rrow) while the SOV (rrow) is filled secondrily. Arteril feeders lso rise from the ipsilterl ECA nd ICA (Type D). Thick rrow: Fcil vein. Both IPSs re not opcified. c, d ICA injection, lte venous phse, AP view: Opcifiction of the irregulr left IPS, suggesting ongoing thrombosis. e g Rod mps, LAO views: Successful nvigtion of the microctheter from the contrlterl IPS into the right CS. Note: The prtilly thrombosed intercvernous sinus with highly stenotic segment cn be pssed using the Hedliner nd loop technique (rrows). h Sinogrm fter reching the right CS, AP view: Opcifiction of the fistul s dringe vi the IOV (short rrow), nd secondrily vi the SOV (rrow), tht ppers occluded in its posterior segment. i Non-subtrcted AP view: Coil occlusion strting in the right CS nd continued to the left side. Dense pcking on the right side, where GDC Soft coils could be deployed even in the nrrowed segment between CS nd ICS (white rrow). On the left side, VortX coils cused disdvntgeous friction in the microctheter preventing denser pcking (white double rrow).

223 214 8 Endovsculr Tretment b c d e f Fig f. Cse II: Finl result nd follow-up. Left CCA injection, AP () nd lterl (b) views: Subtotl occlusion of the fistul t the end of the procedure. Miniml residul AV shunt t the right CS (sterisk), opcifying the IOV (short rrow) nd the fcil vein (thick rrow). c,d Follow-up: Left ICA nd ECA injections AP views: The follow-up exm fter 9 months shows complete occlusion. The coils in the right CS re plced medilly nd lterlly to the internl crotid rtery. (Benndorf et l. 2000). Note: A subtotl occlusion of the AV shunt cn often be sufficient s long s the coil pcking is dense enough. Reversl of Heprin contributes to the induced thrombosis nd follow-up exms usully show complete occlusion of the fistul (see lso Fig. 8.22c). e A 77-yer-old ptient suffering from proptosis for 4 yers presented with eye redness nd chemosis, diplopi due to 3 rd, 4 th nd 6 th nerve plsy s well s decresed vision for 2 months. f At 6 months fter endovsculr therpy, complete recovery

224 8.3 Results of Trnsvenous Emboliztions months. DSA showed smll AVF on the right CS (Brrows Type B) with primry dringe vi the right inferior ophthlmic vein nd secondrily into the right superior ophthlmic vein in the nterior orbit. Posterior dringe vi the IPS ws not recognizble on either side. The lte venous phse of the left ICA injection showed fintly opcified IPS on the left side tht ws chosen for trnsvenous pproch. After plcement of 6-F guiding ctheter Trnsend-14 (0.014 ) nd Terumo-12 (Hedliner ) were used to nvigte microctheter (Trcker Excel) through the left IPS into the ipsilterl CS. Advncing Hedliner s tip, formed to loop, llowed for pssge through high-grde stenosis within the intercvernous sinus nd for entry into the right CS. Occlusion of the sinus ws strted within the right CS using GDC-10 (5 mm/15 cm) nd continued with smller nd more plible GDC Soft coils. Coil pcking ws crried out from contr- to ipsilterl nd within the highly stenotic segment. On the left side, the pcking ws completed by two VortX-18 coils, which cused suddenly incresed friction within the microctheter, thus dense pcking ws not chievble in this comprtment (totl number of coils=21). The procedure ws terminted when the ngiogrm showed subtotl occlusion with miniml residul flow in the IOV. The ptient s ophthlmoplegi improved within the few dys. Her visul cuity hd incresed t the time of dischrge, 1 week lter. She ws completely symptom free fter 6 months; control ngiogrm fter 9 months confirmed complete occlusion Cse Report III: Approch vi the Thrombosed Cvernous Sinus (Figs. 8.19, 8.20) A 73-yer-old former nurse presented in July 2000 with persistent visul problems. In her history, eye redness, retroorbitl pin nd minor exophthlmos on the right side were found over the previous 3 months. A CSF ws ssumed but ruled out by n initil MRI exm. Vrious differentil dignoses including inflmmtory (conjunctivitis, myositis, phlegmon) nd tumorous diseses of the orbit nd the djcent sinuses, were considered. In this context, the ptient underwent extrctions of nine teeth nd n orbitl biopsy. Becuse her symptoms did not improve, the ptient ws eventully trnsferred to perform DSA t our institution. On dmission, she showed exophthlmos nd considerble vision loss, s well s n increse of the introculr pressure to 32 mm Hg. The ngiogrm showed very smll low-flow fistul of the right CS tht ws exclusively supplied by the ipsilterl AMA (Brrows Type C). The venous dringe ws provided by the ipsilterl prtilly thrombosed SOV nd IOV, while the IPS did not seem involved, but showed fint opcifiction in the lte venous phse. After plcement of 6-F guide in the IJV, phlebogrm showed widely open IPS of norml cliber on both sides nd filling of the left SOV. The AV shunt in the right CS, however, ws not identifible. A microctheter (Trcker Excel) could be dvnced without difficulty into the posterior CS. Repeted control injections through the microctheter showed filling of the posterior portion of the sinus, but no communiction with the fistul site in the nterior CS. Attempts to nvigte the microctheter into the nterior comprtment remined initilly unsuccessful. After severl exchnges of guidewires, dvncing Hedliner 0.012, 90 in loop technique ws eventully successful nd the microctheter could be pushed into the previously not visulized fistulous nterior CS comprtment. Here, the connection between nterior CS nd SOV ws blocked with one GDC-10 (4 mm/10 cm) first, then the nterior CS comprtment ws pcked until complete occlusion of the fistul ws documented (totl of four GDCs). Postopertively, the ptient ws heprinized for 48 h during which her symptoms slightly improved. She reported the next dy subjectively improved vision. After 1 week, she ws dischrged nd recovered completely within 4 weeks. Her introculr pressure decresed to 10 mm Hg. In clinicl follow-up 4 months lter, the ptient s symptoms were completely resolved nd she still reported further improved vision. The ptient suffered from n unrelted minor stroke couple of months lter, nd ws reluctnt to undergo nother control ngiogrm Cse Report IV: Trnsfemorl Cross-Over Approch vi the Fcil Vein nd SOV (Figs. 8.21, 8.22) A 74-yer-old womn presented t the hospitl (July 1999) with persistent retro-orbitl pin. In ddition, she suffered from diplopi nd ptosis, underwent cupuncture without ny significnt improvement nd ws trnsferred by her neurologist for invsive vsculr dignostics. Intr-rteril (Text continues on p. 220)

225 216 8 Endovsculr Tretment b c d Fig d. Cse III: Approch through the thrombosed CS, initil DSA. Right ECA injection, lterl view erly () nd lte (b) rteril phse: Very smll rteriovenous shunt of the right CS (sterisk), supplied by brnches of the ECA, such s recurrent brnch of the MMA (rrowhed, see inset: AP view) nd tiny pedicle from the AMA (thin rrow). This fistul could not be identified on MRI or MRA. Both the SOV (rrow) nd the IOV (short rrow) pper prtilly thrombosed. No opcifiction of the IPS. Note: A rre cse of Type-C fistul. c Right ICA venogrm, AP view: Fint opcifiction of the right IPS (fistul side) in AP view (dotted double rrow). The left IPS (double rrow) is better opcified. Inset: Jugulr phlebogrm: Cler visuliztion of the right (double rrow) nd left IPS s widely open vessels. This exmple demonstrtes tht globl ngiogrm (rteriogrm nd venogrm) my be unrelible for selecting the most promising venous route to the CS. d Sinogrm, lterl view: After successful pssge of the right IPS (double rrow), selective contrst injection into the posterior CS (sterisk) shows initilly no connection with the fistulous comprtment in the nterior CS. This cn be due to ntomic comprtmentliztion, or more frequently thrombosis

226 8.3 Results of Trnsvenous Emboliztions 217 b c d e f Fig f. Cse III: Trnsvenous occlusion nd finl result. Rod mp, lterl view: As demonstrted for the IPS, the loop technique is helpful in nvigting guidewire (Hedliner ) through non-opcified (thus likely thrombosed) CS. b Sinogrm, lterl view: When the nterior CS comprtment (sterisk) is reched, the AV fistul becomes visible, drining into both ophthlmic veins. c Rod mps, lterl view: Plcement of the coils in the nterior CS proximl to the veins, disconnecting the nterior dringe from the AV shunt. d Control ngiogrm of the right CCA, lterl view: Complete occlusion of the rteriovenous fistul t the end of the procedure. e A 73-yer-old ptient with exophthlmos, eye redness, ggrvted chemosis nd decresed vision for severl months. Vrious differentil dignoses included tumorl lesion (requiring biopsy) nd phlegmon (leding to nine teeth extrctions). f Complete recovery within 4 months fter occlusion of the fistul. (Benndorf 2002)

227 218 8 Endovsculr Tretment b c d Fig d. Cse IV: Trnsfemorl SOV pproch, initil DSA. Right ECA injection, AP view: Arteriovenous shunt of the right lterl CS (sterisks), which drins minly into the right SPPS (short rrow). The rteril supply is provided by network of smll brnches, including the AFR (thin rrow) nd brnches of MMA (triple rrow). No opcifiction of the right SOV. Note lso the recurrent middle meningel brnch (dotted rrows). b Left ECA injection, AP view: Lrge rteriovenous shunt t the left CS (white sterisk) with filling of the intercvernous sinus (rrowheds) nd the right CS (b/w sterisk), which drins vi the SOV (rrow). Rre cse of true bilterl fistul. Lrge rrow: Right fcil vein. c Right ECA injection, lterl view: Some of the smll brnches supplying the fistul on the right side such s the AFR (thin rrow), the AMA (thin double rrow) nd the MMA (short thin double rrow) re better identifible. The dominnt venous dringe of the right CS fistul into the SPPS (short rrow) nd vi prcvernous sinus (short double rrow) is seen. d Left ECA injection, lterl view: The enlrged right SOV (rrow) serves s sole drining vein of the left-sided rteriovenous shunt. Well visible is lso the nrrowing of the ophthlmic vein while pssing through the suprorbitl fissure (short rrow). It is interesting to note tht AV both shunts pper to utilize different dringe routes

228 8.3 Results of Trnsvenous Emboliztions 219 b c d e f Fig f. Cse IV: Trnsvenous occlusion nd finl result. Sinogrm, lterl view: Trnsfemorl plcement of microctheter (short rrow) vi the right IJV, fcil vein nd right SOV (rrow) into the left CS (cross-over pproch). Decresed AV-shunting flow fter plcing the first coils (sterisks). b Non-subtrcted view, in AP view: Pcking of coils from the left CS nd continued to the right side. Note: Pushing of some dditionl VortX coils through the tortuous ntomy cused considerble friction in the microctheter, preventing denser pcking of the ICS. c Right ECA injection, AP view: Subtotl occlusion of the rteriovenous fistul t the end of the procedure with miniml residul AV-shunting flow in the CS (sterisk) nd SPPS (short rrow). d Right ECA injection, AP view: Complete occlusion of the fistul during the follow-up exm 4.5 months lter. e A 74-yer-old ptient with exophthlmos, ptosis, chemosis nd diplopi. f Miniml residul conjunctivl veins, otherwise resolved symptoms t follow-up (Benndorf 2002)

229 220 8 Endovsculr Tretment DSA reveled n rteriovenous shunt t the right nd left CS (true bilterl DCSF) drining into the right SOV nd sphenoprietl sinus. An initil ttempt to occlude the fistul remined unsuccessful, becuse the IPS could not be ctheterized nd the microctheter ws mneuverble only into the bsilr plexus from which pssge into the CS ws not possible. In the second session, 4-F guiding ctheter ws plced in the right fcil vein nd then dvnced until just proximl to the ngulr vein. Ctheteriztion of the SOV from here ws not possible t first, becuse different microctheters (Trcker Excel, Trcker-18 nd Trcker-10) did not hve sufficient mechnicl support to overcome tortuousities in the nterior SOV. Chnging to stiffer ctheter type (Rpid Trnsit18) finlly llowed for ccessing the CS nd nvigting through the intercvernous sinus into the contrlterl CS. Deployment of GDC-18 coils, strting with 4 mm/10 cm, nd some VortX18-coils resulted in prtil shunt reduction. Becuse the VortX coils exhibited noticeble friction, the occlusion ws continued with GDCs-10. Despite severl efforts, dvncing the microctheter to the connection between CS nd sphenoprietl sinus ws not possible due to the stiffness of the microctheter. Therefore, coil pcking ws continued in the ICS. A subtotl occlusion with stgnting flow in the SOV ws noted t the end of the procedure. The ptient recovered well within the next couple of dys. She initilly developed postopertive swelling of the upper eyelid nd complined bout incresed pin. The diplopi improved fter 4 dys, nd her vision improved from 0.4 to 0.8 within 5 dys. The ptient ws dischrged under low moleculr heprin (1 week) nd 300 mg spirin dily for 4 weeks. After 5.5 months, ngiogrphic control documented complete occlusion of the fistul while the symptoms hd completely resolved Cse Report V: Trnsophthlmic Approch (Cnnultion) vi the SOV (Figs. 8.23, 8.24) A 56-yer-old mle (summer 1997), presented with exophthlmos, eye redness nd history of diplopi for 5 dys. His IOP ws elevted to 33 mm Hg. His rteriogrm showed smll durl AVF on the right CS tht primrily drined into the right SOV. Mnul compression therpy ws the initil therpeutic pproch, but filed to show effect fter 7 weeks. The ptient experienced slight progression of his symptoms. In order to occlude the fistul endovsculrly, trnsvenous IPS pproch to the right CS ws chosen. However, it ws impossible to dvnce the microctheter through n pprently occluded sinus or through the tortuous SOV. 48 following the unsuccessful ctheteriztions, the ptient developed n incresed IOP nd decresed vision. Therefore, in subsequent session, the SOV ws surgiclly exposed nd punctured with 21-G needle (Terumo). This llowed the introduction of Trcker-10 tht reched the CS within few minutes without difficulties. The fistulous comprtment of the CS ws densely pcked with GDCs, which resulted in immedite occlusion of the fistul. After removl of ctheter nd cnnul, the vein ws mnully compressed for bout 20 min. The upper lid cut ws closed with suture. No postopertive bleeding or other complictions were observed. A control ngiogrm fter 5 min showed stble result. The ptient fully recovered nd ws ble to pursue his work s lyout designer in mjor newspper. An ngiogrphic control 18 months lter confirmed stble occlusion of the fistul Cse Report VI: Direct Puncture of the SOV (Figs. 8.25, 8.26) A 59-yer-old techer (November 1996), reported n incresing bruit, diplopi, eye redness nd prominence of his left eye. His cerebrl ngiogrm reveled smll rteriovenous fistul t the suprorbitl fissure tht ws supplied by meningel brnches of the ipsilterl ophthlmic nd mxillry rteries nd drined exclusively into the ipsilterl SOV. Trnsrteril emboliztion of the feeding pedicles from the ECA resulted in only prtil occlusion. Becuse the symptoms of the ptient did not improve, trnsvenous pproches were performed in Februry Due to thrombosis of the IPS, nd high-grde stenosis of the SOV, dvncing microctheter into the CS ws impossible by trnsfemorl techniques. The ptient s condition deteriorted significntly over the following 48 h with secondry glucom nd elevted uncontrollble IOPs of up to 76 mm Hg. Thus, the SOV ws surgiclly exposed nd cnnulted with 21-G venicth (Terumo). Unfortuntely, the ctheter nvigtion remined unsuccessful due to the highly stenotic SOV. Concerning the criticl sitution of the ptient, direct puncture of the deep SOV posterior to the eyebll, ws considered s (Text continues on p. 225)

230 8.3 Results of Trnsvenous Emboliztions 221 b c d f Fig f. Cse V: Trnsophthlmic SOV pproch, initil DSA nd trnsvenous occlusion. Right nd b left ICA injection AP views: Arteriovenous shunt t the right CS (sterisks) with supply from right nd left MHT (dotted rrow). (Inset: Additionl supply from right ECA). Dringe vi SOV (rrow) only, no opcifiction of the IPS. c Sinogrm AP nd lterl (d): After exposure of the SOV by incision of the upper eye lid, cnnultion using 21-G needle (Terumo) nd gentle nvigtion of microctheter (Trcker-10) through this vein, which is possible despite thrombosis in its nterior segment (double rrow). A previous ttempt to ctheterize the SOV by trnsfemorl pproch filed due to significnt tortuousities of the SOV nd the ngulr vein. e Rod mp lterl: Plcement of the first coil in the right CS. f ICA injection, non-subtrcted AP view: Coils re densely pcked in CS, conforming to the medil nd inferior crotid wll

231 222 8 Endovsculr Tretment b c d Fig d. Cse V: Finl result nd follow-up. Arteriogrm of the left ICA () AP nd ECA (inset). The control ngiogrms reveled complete occlusion of the rteriovenous fistul t the end of the procedure. b Bilterl ICA rteriogrm confirmed this result 18 months lter (Benndorf et l. 2000b). c Before trnsvenous occlusion: A 56-yer-old ptient with mild exophthlmos, eye redness nd diplopi due to 6 th nerve plsy. This ptient initilly underwent mnul compression therpy for 7 weeks tht filed to show n effect. d At 3 months fter tretment: Normliztion of the introculr pressure nd resolution of the clinicl symptoms, llowing the ptient to resume his work s lyout designer with no cosmetic ltertions). (Benndorf 2002)

232 8.3 Results of Trnsvenous Emboliztions 223 b c d Fig e. Cse VI: Direct SOV puncture, initil DSA nd trnsvenous occlusion.,b Left ICA injection lterl views, erly nd lte rteril phse: Smll rteriovenous shunt t the left nterior CS (sterisk), supplied by recurrent meningel brnch of the ipsilterl ophthlmic rtery (rrow), drining exclusively into the SOV (short rrow). The SOV ppers prtillly thrombosed in its nterior segment (rrowheds), possibly relted to the previously ttempted unsuccessful fcil vein pproch. No opcifiction of the IPS, the ctheriztion of which filed s well. c Rod mp, lterl (mgnified): After upper eye lid incision, stenotic segment (short rrow) prevented dvncement of the microguidewire (Trnsend 0.014, rrow). Hence, no conventionl ccess to the CS ws fesible. d,e Considering the ptient`s criticl clinicl condition with uncontrollble IOPs up to 76 mm Hg nd imminent loss of visison, direct deep introrbitl puncture of the SOV ws performed: Using bi-plne rod mp, 21G needle (rrowheds) ws very slowly nd gently dvnced long the medil side of the globe until the SOV ws reched in its posterior (intrconl) segment

233 224 8 Endovsculr Tretment b c d e Fig e. Cse VI: Trnsvenous occlusion nd follow-up. Non-subtrcted lterl view: Documenttion of the needle position in the posterior third of the orbit. b Rod mp, lterl view: After dvncing microctheter (Trcker-10, rrowheds) to the fistul site, pltinum coils were deployed (GDC) nd densely pcked in the venous comprtment of the fistul. Control ngiogrms showed complete occlusion of the fistul by the end of the procedure. c Left ICA injection, lterl view: This result ws confirmed stble fter 3 months (sterisk=coils). d, e A 59-yer-old techer with exophthlmos, severe chemosis ssocited with n incresed IOP (76 mm Hg). At 1 dy fter tretment (E), the eyelid is still swollen. (F), 3 months fter tretment, the ptient shows complete resolution of his symptoms (no cosmetic deformities) (Benndorf et l. 2001).

234 8.4 Discussion of Trnsvenous Occlusions 225 lst resort. After thorough discussion of potentilly ssocited risks with the ophthlmologist, neurosurgeon nd mxillo-fcil surgeon, 21-G needle (Terumo) ws very slowly dvnced long the medil wll into the posterior third of the orbit using bi-plne rod mpping. While pproching the SOV, the direction of the needle ws controlled nd corrected by crefully wtching the fluoroscopy in both plnes. The puncture ws successful in the first ttempt nd llowed the introduction of smll microctheter (Trcker-10) nd its nvigtion to the fistul site. Detchble coils (GDCs- 10) were gently deployed until control injection showed reduced filling of the AV shunt. After completing the occlusion, ctheter nd needle were removed nd the incision ws surgiclly closed. No postopertive compliction occurred. The ptient ws dischrged with norml IOP fter 1 week nd recovered completely within 3 months. A followup rteriogrm fter 12 months confirmed complete occlusion. 4 yers lter, the ptient reported miniml tinnitus, but hd no ophthlmologicl symptoms nd IOP of 16 mm Hg. Another control ngiogrm showed miniml opcifiction in the re of the coil pcking tht ws not interpreted s recnliztion Cse Report VII: Direct Puncture of the Sylvin Vein After Crniotomy (Figs. 8.27, 8.28) A 57-yer-old womn presented (My 1992) with pinful exophthlmos nd chemosis on the left side. There were no neurologicl deficits. Computed tomogrphy reveled dilted right SOV due to durl AVF involving the left CS t the lower edge of the suprorbitl fissure. The fistul ws supplied by meningel brnches of ECA (MMA) nd the ICA (ophthlmic rtery nd cvernous brnches), while the venous dringe ws directed posteriorly vi the sphenoprietl sinus nd the Sylvin vein into the vein of Lbbé nd the trnsverse sinus. A microctheter ws introduced first into the MMA nd then the feeding pedicle ws blocked by n injection of 40% NBCA/lipiodol mixture without occluding the AV shunt itself. Becuse of the tortuous ntomy of the ICA feeder nd the ophthlmic rtery, distl position for nother sfe glue injection ws not obtinble. None of the ttempted venous pproches, such s the IPS nd fcil vein (SOV pproch), proved successful, due to thrombosis nd tortuousities. Becuse the ptient developed progressive visul deteriortion, emergency mesures hd to be considered. Becuse, surgicl exposure of the ngulr vein or SOV ws not n estblished prctice in our institution t tht time, n open surgicl pproch ws chosen. In June 1992, following lterl crniotomy, the Sylvin vein ws exposed by neurosurgicl collegue. The vein ws clipped nd punctured using n 18-G needle (plstic cnnul). This veinicth ws imbedded in Plcos (methylmethcrylte, bone cement) nd nchored t the skull for stbiliztion. After ttching Y-connector, Trcker-18 microctheter could be dvnced without ny problems through the sphenoprietl sinus into the CS under fluoroscopic control. Plcement of two smll pushble fibered coils cused n immedite noticeble reduction of the AV shunting. After witing further 10 min, second control ngiogrm showed complete disppernce of the AV shunt so tht the cnnul ws removed nd the procedure ws finished. The clinicl symptomtology resolved during the following 2 weeks nd control exm confirmed clinicl nd ntomic cure. The ptient ws seen for follow-up exm 5 yers lter which confirmed stble occlusion Cse Illustrtions (Figs ) Additionl Cse Illustrtions show lterntive venous routes, the use of liquid embolic gents, trnsrteril emboliztions nd other methods to tret DCSFs (Figs ). (Text continues on p. 228)

235 226 8 Endovsculr Tretment b c d e f Fig f. Cse VII: Sylvin vein pproch. Initil DSA, intropertive trnsvenous occlusion nd finl result. (), left ICA injection, lterl view (sequentil subtrction, imges were obtined using mobile C-rm used for intropertive ngiogrphy): Arteriovenous shunt of the right CS (sterisk), supplied by recurrent meningel brnch of the enlrged ophthlmic rtery. Anterior dringe into the SOV (rrow) nd corticl dringe vi the SPPS (short rrow) into the Sylvin veins (double rrow). b Selective MMA injection left, lterl shows the enlrged MMA (double rrowhed) shunting directly into the CS (sterisk) nd drining into dilted corticl veins: Sylvin veins (double rrow), vein of Lbbé (thin rrow). c,d Intropertive rod mp, lterl: After crniotomy, the rterilized Sylvin vein ws crefully punctured using n 18-G needle. The needle ws ttched to Y-connector nd imbedded in Plcos (methylmethcrylte) for stbiliztion. The microctheter (Trcker -18) could be esily dvnced to the fistul site (short double rrow, d). After deploying only two 4/30 mm fibered coils (rrowhed), significnt reduction of the AV-shunting flow ws noted, which fter 10 more min, resulted in complete occlusion of the fistul (e). Note tht ll ttempts to occlude the fistul, including IPS nd SOV pproches filed. f An 18-G needle with Y-connector (rotting hemosttic vlve) imbedded in Plcos (bone cement) s used for fixtion t the skull, providing stbiliztion during dvncement of the microctheter into the CS

236 8.3 Results of Trnsvenous Emboliztions 227 b c Fig c. Cse VII: Angiogrphic nd clinicl follow-up.,b Left ICA nd ECA 5-yer follow-up rteriogrms, lterl views: Persistent occlusion of the fistul. c A 57-yer-old ptient with complete resolution of her symptoms (left-sided exophthlmos nd chemosis t the time of presenttion in 1992) (Benndorf et l. 1997, 1999b)

237 228 8 Endovsculr Tretment 8.4 Discussion of Trnsvenous Occlusions Approches IPS Approches (Cse Reports I III, Cse Illustrtions III nd XI) The IPS pproch ws initilly described s n ccess route to the cvernous sinus (CS) for tretment of direct CSFs (CCF) (Debrun et l. 1981; Mnelfe nd Berenstein 1980). After its introduction s venous route for emboliztion of indirect (durl) CSFs by Hlbch et l. (1989) in 1989, the IPS hs been incresingly used by numerous other groups (Cheng et l 2003; Theudin et l. 2007; Hlbch et l. 1989; Gobin nd Guglielmi 2000; Kiyosue et l. 2004; Kim et l. 2006; Meyers et l. 2002; Ymshit et l. 1993; Annesley-Willims et l. 2001, Yu et l. 2007). Its frequent utiliztion s venous pproch to the CS is bsed on the following: The IPS represents the ntomiclly shortest, thus techniclly lest complicted nd sfest route. Prticulrly in ptients where this sinus is involved in the venous dringe, the IPS pproch is fst nd strightforwrd in the mjority of cses. Antomiclly, its course is reltively stright, with horizontl nd verticl segment forming wide ngle tht does not cuse luminl nrrowing or ctheter kinking with friction tht hinders dvncements of coils. Another dvntge is the mechnicl stbility of the sinus. Its course long the petro-occipitl suture (petroclivl fissure), covered by the dur mter provides stbility resembling pipe-like structure. This stbility is prticulrly importnt when lrger mount of coils need to be pushed through tortuous ntomy (e.g. like the SOV), eventully cusing friction within the microctheter, which my esily become procedure-limiting fctor. The IPS llows for dvncing guidewire or microctheter even when its lumen is nrrowed, irregulr or completely occluded due to thrombosis. The topogrphicl ntomy of the IPS opening into the jugulr vein llows reltively stble positioning of lrge guide ctheter within the vein, or sometimes even into the IPS itself. This mkes nvigting microctheter into the ipsilterl or contrlterl CS considerbly esier compred to the retrogrde nvigtion through the SOV, where positioning of guiding ctheter is less stble (Oishi et l. 1999; Hlbch et l. 1989, 1997; Ymshit et l. 1993). It seems logicl tht the IPS ws utilized erly on s n lterntive to trnsrteril blloon plcement in direct CCFs with posterior dringe (Mnelfe nd Berenstein 1980). Despite its obvious dvntges, some opertors prefer lternte venous routes, commonly when the IPS is ngiogrphiclly not recognizble (Goldberg et l. 1996; Hnneken et l. 1989; Hsuo et l. 1996), despite n incresing number of reported successful ctheteriztion of such non-visulized IPSs (Benndorf et l. 2000; Cheng et l. 2003; Hlbch et l. 1989; Ymshit et l. 1993; Mlek et l. 2000; Roy nd Rymond 1997; Quinones et l. 1997). Hlbch et l. (1988) described first the successful ctheteriztion of n ngiogrphiclly occluded, but eventully pssble IPS in two ptients. Further, Ymshit et l. (1993) were ble to successfully ctheterize the sinus in two cses, despite it being ngiogrphiclly occult. Quinones et l. (1997) reported on 30% success rte for IPS ctheteriztion, including ngiogrphiclly occluded sinuses. It is noteworthy tht in those unsuccessful cses the venous dringe pttern ws usully not nlyzed in detil. A fistul tht would not opcify the IPS during the erly rteril phse ws commonly referred to s not ngiogrphiclly demonstrted (Ymshit et l. 1993; Quinones et l. 1997), ngiogrphiclly invisible (Yu et l. 2007), not opcified (Klisch et l. 2003; Kuwym et l. 1998), not ptent (Goldberg et l. 1996; Monsein et l. 1991; Krisht nd Burson 1999), not spontneously opcified (Monsein et l. 1991) or hypoplstic (Mizuno et l. 2001). The question of whether or not the IPS ws opcified during the venous phse of the ngiogrm ws not ddressed. However, in my experience, it is helpful to clerly distinguish between two types of negtive IPS dringe ptterns: (1) non-opcifiction only during the erly rteril phse (= not drining the fistul), but during the lte venous phse (= drining the brin), nd (2) no opcifiction t ll. Cses with the former disposition my be considered not worth ttempting by some opertors, lthough they hve high chnce of successful ctheter nvigtion into the CS (Cse Reports II III). An IPS tht does not serve s venous

238 8.4 Discussion of Trnsvenous Occlusions 229 outflow t ll hs likely undergone sub-totl or complete thrombosis. As demonstrted in Cse Report I nd Fig. 8.3, this does not preclude the existence of ntomic communiction, nd thus the possibility of reching the fistul site with suitble tool. The IPS my lso serve s drining pthwy for the posterior foss vi the SPS; therefore, reding ngiogrms for decision-mking must include the vertebrl injections (Figs nd 7.30). The only relible proof of thrombosed IPS is direct sinogrm vi microctheter, reveling n irregulr, nrrowed lumen, which nevertheless might be pssble. A lrge volume jugulr phlebogrm, using severl forceful injections from different levels of the proximl internl jugulr vein, provides vluble dditionl ntomic informtion. It my revel n either widely open IPS (Figs. 7.7 nd 8.19), or tiny residul portion of the thrombosed IPS, often visible s smll notch, (Figs. 7.42, 8.3, 8.14). According to Shiu et l. (1968), four min vrints of the IPS/IJV junction exist nd my ply role in the overll success rte of retrogrde IPS ctheteriztions. Becuse of its complex rchitecture nd the lck of connection to the IJV, the IPS could not be ctheterized in 31% of the cses. This rte my be lower tody with dvnced ctheteriztion tools, improved imging techniques nd better ntomic understnding of this region. It cn be ssumed tht some of the plexiform-like ppering connections between IPS nd IJV in this erly work, performed in the 1960s, represented in fct thrombosed IPSs. The chnces of success when using this route in n individul cse remin hrd to predict. As illustrted in Chp. 7, the existence of n berrnt IPS needs to be considered since it my be used s n pproch s well (Benndorf nd Cmpi 2001b; Clzolri 2002). Identifying such berrnt IPS (unusul deep termintion) tht enters s smll opening into lrge vein, cn be difficult if the ctheter position is not t the right level, or not proximl enough to fill the IPS (Figs nd 8.3). Severl uthors (Hnneken et l. 1989; Hsuo et l. 1996; Gupt et l. 1997; Jhn et l. 1998) hve stted tht ctheter nvigtion through the thrombosed IPS my crry n dditionl risk of rupture, cusing subrchnoid hemorrhge (SAH). The current literture revels 14 cses of IPS perfortion, of which some resulted in SAH nd others in cerebellr or extrdurl hemtoms (see Tble 8.3). Ten cses were successfully mnged by endovsculr mens (Kim et l. 2006; Hlbch et l. 1991; King et l. 1989). Hlbch et l. (1988) observed rupture with SAH in two ptients with direct CCF; both were successfully treted by deployment of coils t the rupture site. This group lso reported on ptient with DCSF nd intrcerebellr hemorrhge fter perfortion of the IPS who prtilly recovered (Hlbch et l. 1991b). King et l. (1989) published rupture of the IPS during trnsvenous pproch to crotid cvernous fistul, cusing minor SAH tht ws subsequently mnged by surgicl exposure of the CS nd injection of IBCA. Ymshit et l. (1993) described IPS rupture with extrdurl extrvstion of contrst which stopped spontneously without clinicl consequences. Ingw et l. (1986) reported ptient who deteriorted fter n unsuccessful occlusion vi IPS pproch. This deteriortion ws ttributed to thrombosis of the IPS, nd the ptient ws treted trnsrterilly by blloon occlusion. Kim et l. (2006) recently presented series of 56 ptients with three IPS perfortions. The rte of sinus perfortion is higher thn reported by others but ws controlled in ll three cses by coil deployment t the rupture site with no significnt neurologicl sequele. The uthors emphsize tht sinus rupture my occur even when using supple guidewires nd microctheters without providing detils of the devices being used. It rises the question of how gently (or forcefully) hve these tools been mnipulted. In the 45 ptients with DCSFs studied here, one perfortion during ctheteriztion of the inferior petrosl sinus ws observed (Benndorf et l. 2004) tht remined cliniclly silent. Aprt from trnsvenous emboliztion of DCSFs, IPS ctheteriztions hve been used extensively s dignostic mens for venous blood smpling (petrosl smpling) in ptients with Cushings disese. The literture shows tht this procedure my be ssocited with complictions s well (Shiu et l. 1968; Bonelli et l. 1999; Miller et l. 1992; Sturrock nd Jeffcote 1997; Seyer et l. 1994; Lefournier et l. 1999; Gndhi et l. 2008) (Tble 8.4) Bonelli et l. (1999) recently reported bout ptient who developed elevted blood pressure, hedche nd confusion during the procedure due to sinus rupture cusing severe SAH, fter using Trcker-18 microctheter. The uthors lso describe hedches nd nuse without sequele in three other ptients (Bonelli et l ) Shiu et l. (1968) lredy documented the rupture of pontine vein during cvernous sinogrphy for dignosis of pituitry denom. As demonstrted in Tbles 8.3 nd 8.4, more serious complictions re observed during petrosl smpling thn during trnsvenous ctheteriztion for CSF occlusion.

239 230 8 Endovsculr Tretment Tble 8.3. Adverse events during IPS ctheteriztion for endovsculr tretment of CSFs (modified fter Benndorf et l. 2000) Nunber of Cses Author Pthology Ctheter/Wire Compliction Therpy Outcome 1 Ingw et l Type-A 5F Deteriortion 1 Blloon Improved 1 King et l Type-A SAH IBCA 0 1 Hlbch et l Type-A 3F/5F SAH Coils 0 1 Hlbch et l. 1991b Type-A SAH Coils 0 1 Hlbch et l. 1991b DCSF ICH Coils Recovered 1 Ymshit et l DCSF Trcker-18 EDH Spontneous 0 1 Oishi et l DCSF Dissection of dur Conservtive CN plsy 1 Klisch et l DCSF SAH Conservtive Improved 1 Benndorf et l DCSF EDH Conservtive 0 3 Kim et l DCSF SAH Coils 0 0 = no deficit; Recovered = no detils, remining symptoms improved; Improved = ptient showed improvement with residul minor diplopi. 1 Symptoms progressed, but resolved fter trnsrteril blloon occlusion prtilly. SAH = subrchnoid hemorrhge; EDH = epidurl hemtom; ICH = intrcrnil hemorrhge; IBCA = Isobutyl-2-cynocrylte; F = French. Type A = direct CSF (Brrow); DCSF = indirect CSF (Type B-D Brrow); = no detils reported. Tble 8.4. Adverse events during IPS ctheteriztion for petrosl smpling (modified fter Benndorf et l. 2000) N Author Ctheter/Wire Compliction Therpy Outcome 1 Shiu et l. (1968) 0.05/0.031 EDH Conservtive 0 4 Miller et l. (1992) 5 F Brin stem lesion Pons hemtom Medull infrction Brin stem lesion Conservtive Conservtive Conservtive Conservtive 0 Remining deficit Remining deficit 0 1 Seyer et l (1994) 5 F Hemipresis, CN presis Conservtive Remining deficit 1 Sturrock nd Jeffcote (1997) 5 F Brin stem lesion Conservtive Remining defici 1 Bonelli et l. (1999) Trcker-18 SAH, Hydrocephlus VD Remining deficit 1 Lefournier et l. (1999) 4 F/6 F 6th nerve plsy Conservtive 0 3 Bonelli et l. (2000) Hedches, nuse Conservtive 0 1 Gndhi et l. (2008) 4 F Brin stem lesion Conservtive Remining deficit SAH = subrchnoid hemorrhge; VD = ventriculr dringe; EDH = epidurl hemtom; CN = crnil nerve; 0 = no residul deficit; F = French. While SAH occurs here less frequently, brin stem infrction due to compromised venous dringe is observed more often. The reson for tht my be the use of (reltively lrge) 5-F dignostic ctheters, especilly when plced bilterlly s is often the cse for dignostic smpling in ptients with Cushings disese. Gndhi et l. (2008) observed ptient who developed prtilly reversible brinstem infrction, requiring intubtion nd trcheostomy. The uthors discuss whether vrint venous dringe pttern or n outflow obstruction, induced by the dignostic ctheters, could hve been the cuse. Unfortuntely, their figures re not supportive of either theory nd pper misinterpreted with regrd to the venous ntomy. However, it seems quite plusible tht plcement of 4-F ctheter becomes occlusive, forcing not only contrst into the pontine nd mesencephlic veins, but lso compromising norml venous circultion (Fig. 1b there). Overll, the risk of venous ischemi due to ctheter mnipultion during endovsculr therpy is reltively low, since smll ctheters (2- to 3-F microctheters) re employed nd re commonly used unilterlly. Consequences my be serious though

240 8.4 Discussion of Trnsvenous Occlusions 231 when 5-F ctheter is introduced into the IPS blocking the posterior venous outflow (Theudin et l. 2007). Furthermore, it cn probbly be ssumed tht in cse of thrombosed IPS, the norml venous circultion of the pons nd brin stem hs lredy ccommodted nd uses collterl circultion. Thus, the blockge of this sinus by guidewire or microctheter will less likely cuse relevnt compromise of the norml venous dringe with subsequent venous infrction. A rupture of the IPS cusing cliniclly significnt intrcrnil hemorrhge my only occur if this sinus lso drins n rteriovenous fistul nd is thus exposed to incresed intrvsculr pressure (King et l. 1989). Two possible mechnisms my cuse hemorrhgic complictions during endovsculr mnipultions (Hlbch et l. 1991b): First, sudden increse in intrvenous pressure during contrst injections; second, perfortion of the thin venous wll during dvncement of the shrp guidewire tip, stiffened by the ctheter nd nrrow vsculr structure. The first cn be voided by performing creful injections s test under blnk rod mp to control ctheter position (Benndorf et l. 2000). The second should remin extremely rre if microguidewire is dvnced very slowly under bilterl fluoroscopy. Under these circumstnces, the use of loop t the tip while dvncing the wire within the thrombosed IPS ws found to be very helpful. The softness of smller devices such s or the guidewires, provides better conformbility to the specific ntomy of the IPS nd prevents entnglement in n irregulr, trbeculted or even thrombosed venous lumen (Benndorf et l. 2000). Advncing supple wire tht cn esily be formed into loop is less trumtic thn using stright tip, prticulrly if the ctheter is lredy wedged. Becuse the IPS is minly locted in the extrdurl spce, or is t lest prtilly covered by the dur, rupture of the IPS resulting in hemorrhge is hrd to imgine unless the dur mter is perforted. Perfortion of the dur mter requires criticl level of mechnicl stress, s pointed out by Shiu et l. (1968), who found it lmost impossible to perforte the dur djcent to the IPS in studies performed on post mortem mteril. To reduce the risk of perfortion, the uthor prefers not to use stiff wire stiff s suggested by Goto et l. (1999) nd others (Oishi et l. 1999; Kirsch et l. 2006). As reported by Oishi et l. (1999), this my cuse dissection of clivl dur nd result in permnent 6 th CN plsy, due to dmge of the nerve when pssing Dorello s cnl or due to direct injury by the guidewire. A wire cnnot be used s loop nd hs to be dvnced in Kuru- Kuru technique, like drill, to recnlize the IPS. This technique, lthough dvocted by some uthors (Oishi et l. 1999; Goto nd Goto 1997; Nemoto et l. 1997), my crry the risk of perfortion or dmge to the 6 th CN, especilly when its lumen is nrrowed nd the wire tip becomes smll sper. A so-clled microctheter pull-up technique using second microctheter with snre hs been recently suggested by Hnok et l. (2006) s n lterntive. Gobin et l. (2000) used left IPS-ICS pproch to plce microctheter in the right IPS fcilitting ctheter nvigtion from the right IJV into the CS in bilterl DCSF. Cse Illustrtion II (Fig. 8.31) shows tht in some cses, even two microctheters cn be nvigted through thrombosed IPS. Such dul IPS pproch cn be very useful in cses with multidirectionl venous dringe. It enbles one to selectively disconnect one venous dringe (e.g. leptomeningel, corticl) while securing ctheter position in nother (nterior) comprtment nd help to void comprtmentliztion of the fistul. Cse Illustrtion X (Fig. 8.38) shows the stged mngement of complex AVF involving the bilterl CS nd the clivus. The CS AV shunts were pproched nd obliterted utilizing the (thrombosed) IPS pproch. The introsseous lesion ws successfully occluded in second session using n introsseous venous chnnel 3 months lter Thrombosed Cvernous Sinus (Cse Report III) As demonstrted, stndrd 2D-DSA my esily fil to opcify the IPS or the CS. Beside hemodynmic fctors, thrombotic processes within the CS or the IPS my ply role in the filure to visulize the sinus s well (Hlbch et l. 1997). Highly flexible, supple microctheters nd guidewires re not only useful when nvigting through thrombosed IPS, but lso when pssing intrcvernous trbecule or thrombi tht my not be esy to identify, but re often present in DCSFs. The CS cn be divided into nteroinferior nd posterosuperior comprtments, seprted by the intrcvernous ICA. Despite intrcvernous septi, mnipultion nd dvncement of microctheters is usully not very difficult in most directions, ipsi- to contrlterl or nterior to posterior. Despite the importnce of modern n-

241 232 8 Endovsculr Tretment giogrphic equipment, profound knowledge of the complex ntomic structures nd good three-dimensionl understnding of the CS re required for optiml use of tools nd devices. Trditionl crosssectionl imging techniques, lthough improved over the lst yers (Schuknecht et l. 1998), still do not provide stisfctory sptil resolution. Even currently vilble high-resolution DSA tht enbles depiction of the smllest AV shunts with certinty is limited in its cpbility to disply smll venous structures. Angiogrphic computed tomogrphy (ACT, DynCT) my improve CS visuliztion nd will likely contribute to better understnding of such complex vsculr structures (see Chp. 7). The resons for incomplete CS visuliztion by 2D-DSA re threefold. First, non-opcified blood drining from other tributries vi the sme venous structures, cusing wshout effects. This becomes obvious in cses where CS tht is ngiogrphiclly silent (fter rteril contrst dministrtion) becomes clerly visible in jugulr phlebogrm. Second, thrombotic processes, frequently ssocited with DCSFs (50%) (Mironov 1994), my be responsible for non-opcified CS comprtments in the sme wy they re for non-opcified IPS. It hs been suggested tht thrombosis of the IPS is one cuse for cute progression of the ophthlmologicl symptoms (Hlbch et l. 1992, 1997), s the blood drining here is rerouted towrds the SOV (Stomi et l. 2005). Acute thrombosis of the IPS is the min reson for most ngiogrphiclly not-visulizble sinuses tht re still pssble with microctheter (Hlbch et l. 1997). Third, locl hemodynmics ply role, since the fistulous comprtment, usully with higher pressure, is more difficult to fill using mnul intrvenous contrst injection, even when performed vi microctheter plced in the CS (Hlbch 1997). Finlly, lthough some uthors doubt tht true trbeculr structure exists (Tpts 1982; Bedford 1966), seprtion by intrcvernous filments nd septi cusing comprtmentliztion ( Debrun et l. 1981; Ingw et l. 1986; Chloupk et l. 1993; Mulln 1979; Butler 1957; Ridley 1695; Winslow 1734; Teng et l. 1988) my influence ngiogrphic ppernce of the CS s well. Thus, mnipultion nd nvigtion of microctheters inside the CS cn be difficult in some cses. Plcement of microctheter into the desired loction vi one single pproch my be impossible (Ymshit et l. 1993). If more thn one fistul site exists tht cnnot be reched using one venous route, two or more pproches my be used simultneously in one session (Cse Illustrtion II nd III). Chloupk et l. (1993) reported CS comprtmentliztion in ptient with DCSF in whom the fistul site in the nterior comprtment ws not rechble from the posterior CS. This prticulr disposition ws clled true ntomicl comprtmentliztion, since no communiction ws seen either in the ngiogrms or in the selective venogrms vi microctheter plced within the CS. However, this sitution resembles the one described in Cse Report III, where ctheter dvncement ws eventully possible indicting intrcvernous thrombosis rther thn ntomic seprtion. Thus, it ppers doubtful tht such conclusion cn be mde bsed on contrst injections nd guidewire resistnce only. Quinones et l. (1997) reported one cse (Figure 2 therein) in which petrosl venogrm did not show direct connection between the IPS nd the CS nd tht subsequently underwent SOV emboliztion. Tking into ccount the limittions of ngiogrphic ppernce due to locl hemodynmics nd thrombotic processes, ctheter nvigtion into seemingly occluded CS comprtments my be fesible nd is justified if gently performed, especilly when more ggressive endovsculr or surgicl techniques cn be voided Trnsfemorl Fcil Vein/Superior Ophthlmic Vein Approch (Cse Report IV) The trnsfemorl pproch through the fcil vein or the SOV ws introduced by Komiym et l. (1990) nd hs been used by vrious groups with different success rtes since (Cheng et l. 2003; Theudin et l. 2007; Kim et l. 2006; Annesley-Willims et l. 2001; Biondi et l. 2003). It my be n effective lterntive route nd is indicted when the SOV is significntly enlrged due to nterior dringe, nd the IPS cnnot be pssed. This pproch is usully fesible when the SOV is dilted enough nd llows even cross-over nd pcking of the contrlterl CS. It my be techniclly chllenging though, if the SOV is tortuous, stenosed or thrombosed (Cse Report V). Two spects seem importnt. First, the SOV pproch is in generl less fesible thn the IPS pproch, due to the less fvorble ntomy. There is longer distnce to be ctheterized combined with tortuousities of the ngulr nd superior ophthlmic veins. Furthermore, the guiding ctheter usu-

242 8.4 Discussion of Trnsvenous Occlusions 233 lly hs mechniclly less stble position within the IJV or the fcil vein thn for the IPS pproch. Unstble position of the guiding ctheter my esily become procedure-limiting fctor in cses where ctheter dvncement is lredy difficult due to the friction in the loops of the SOV. Thus, it is dvntgeous to plce 4-F guiding ctheter in the fcil vein, close to its connection with the ngulr vein. In contrst, some opertors recommend positioning the guiding ctheter in the IJV to void impirment or stsis of the venous outflow tht my cuse ophthlmic or neurologicl complictions (Biondi et l. 2003). Cheng et l. (2003) suggested rubbing over the ptient s fce towrd the orbit to support dvncement of the microctheter. The trnsfemorl SOV pproch ppers prticulrly fesible in ptients with enlrged ophthlmic nd fcil veins. It my be techniclly chllenging or even impossible in cses with low-flow fistuls nd significnt elongtions of the ngulr nd ophthlmic veins, or when ssocited with stenotic segments or thrombosis (Miller et l. 1995; Teng et l. 1988b). Biondi et l. (2003), in the first series of seven ptients, chieved ntomic cure in six cses (85.7%), clinicl cure in four (57%) nd improvement in two (28.5%). In one ptient ctheteriztion of n occluded SOV filed. It ws emphsized to remember the two ntomic roots of the SOV origin for full understnding of the ngiorchitecture (Figs. 3.9, 3.10 nd 7.31). Although the inferior SOV root should be esier to ctheterize from the fcil vein becuse of its reltively stright course, in most cses successful nvigtion is performed through the superior route. The reson for this my be tht the inferior root is often poorly visulized (Biondi et l. 2003), in ddition to vlves tht hve been reported by some uthors (Biondi et l. 2003; Testut nd Jcob 1977; Hou 1993). The uthor s personl experience confirms the one lredy mde by Mulln et l. (1979) nd Hlbch et l. (1989), who struggled with technicl difficulties when nvigting through the loops of the ngulr nd ophthlmic veins. Although this hs become techniclly esier with improved ctheteriztion tools, the SOV ntomy not only remins tortuous compred to the IPS, but lso my show n brupt ngle or nrrowing t the level of the superior orbitl fissure (Hnfee et l. 1968). Mnipultion of ctheters nd guidewires in the SOV my led to temporry venous outflow restriction, thrombosis ssocited with trnsient ggrvtion of exophthlmos nd vision loss (Gupt et l. 1997; Biondi et l. 2003; Devoto et l. 1997). When the CS cnnot be reched or the fistul is not occluded in the sme procedure, serious clinicl condition my develop. This ltter observtion ws mde by other opertors (Hlbch et l. 1989; Devoto et l. 1997), nd in two of the uthor s ptients, who experienced trnsient ggrvtion (Cses V nd VI), nd represents cler disdvntge of this pproch. Another potentil compliction of the pproch through the SOV is the rupture of the vein with introrbitl bleeding nd vision loss (Wldis et l. 2007; Leibovitch et l. 2006; Hyshi et l. 2008). Uflcker et l. (1986) emphsized tht the ge of the AV shunting plys n importnt role for the development of thickened venous wll. Although niml experiments hve shown tht histologicl chnges of rective wll hypertrophy strt to develop pproximtely 7 10 dys fter estblishment of n AV shunt, ctheterizing the SOV in cses with longer-stnding AV shunts my be sfer. Interestingly enough, Sttler (1905) lredy pointed out tht the thickness of the venous wll my increse four times nd numerous elstic fibers develop due to the elevted pressure. DCSFs re often longstnding shunts, nd fresh rterilized veins re to be found more often in direct CCF fistuls with short history. Wldis et l. (2007) reported recently n cute introrbitl hemorrhge during trnsfemorl SOV pproch tht led to n orbitl comprtment syndrome with vision loss due to trnsient flow rrest in the ophthlmic rtery. This compliction, erroneously clled obstruction by the uthors, ws most likely cused by cute increse in introculr pressure, exceeding the perfusion pressure of the orbit. Stgntion of ophthlmic rtery flow my result in centrl retinl rtery occlusion, which cn cuse significnt irreversible retinl dmge when exceeding 240 min (Hyreh et l. 2004). In cses, where the fcil vein cnnot be reched by trnsfemorl pproch, direct puncture t the mndible hs been suggested s n lterntive route (Nito et l. 2002). Scott et l. (1997) reported five ptients undergoing ultrsound-guided direct stick of the fcil vein (trnscutneous pproch) Approches vi the Middle Temporl Vein or the Frontl Vein (Cse Illustrtion I) Bsiclly ll fferent nd efferent veins connected to the ngulr or ophthlmic vein, or directly to the

243 234 8 Endovsculr Tretment CS (depending on their size), my be used s either trnsfemorl or direct percutneous pproches. Ctheteriztion of n enlrged drining middle temporl vein, tht communictes with the ngulr vein for exmple, is not difficult (Komiym et l. 1990; Kzekw et l. 2003; Agid et l. 2004, Yu et l. 2009). Agid et l. (2004) divided the fcil venous dringe into medil nd lterl (representing the middle temporl vein), using the ltter in one cse fter trnsfemorl ctheteriztion. Kzekw et l. (2003) recently reported on two cses successfully embolized with such n pproch fter direct puncture with n 18-G needle. The communictions between the SOV nd the suprorbitl nd frontl veins were used extensively in the pst for orbitl phlebogrms (Brismr nd Brismr 1976,b; Cly et l. 1972). Ctheter mnipultions re not different from other pproches, with the exception of the direct puncture itself being technicl chllenge. However, this puncture is usully successful when the vein is involved in the fistul dringe. When difficult to plpte, n dditionl tourniquet my help nd hs been used by the uthor on severl occsions. Venturi et l. (2003) used this trnscutneous pproch in one ptient, emphsizing the usefulness of this technique dpted from trditionl dignostic exms of the 1960s. The uthors distended the frontl vein by posterior flexion of the hed nd compression of the jugulr vein in the neck, nd cnnulted with n 18-G needle to llow for introduction of guidewire nd microctheter. In the group of ptients studied by the uthor, TVO hs been performed using this pproch in one cse Trnsfemorl Superior Petrosl Sinus Approch (Cse Illustrtion II) The use of the SPS ws mentioned by Mulln in 1979 s surgicl ccess to the CS for tretment of CSFs. As described first by Mounyer et l. (2002), trnsfemorl pproch of the SPS my be successfully used for ctheter nvigtion into the CS nd is n lterntive to IPS or SOV pproch. The uthors used jugulr stick, 5-F sheth nd guidewire (Rdiofocus; Terumo, Tokyo, Jpn) in combintion with n Excelsior microctheter (BSC), supported by 5-F hydrophilic guiding ctheter (Terumo). After reching the CS, the fistul site ws pcked with mechniclly detchble coils. The SPS is, lthough rrely opcified, usully not difficult to ctheterize, even when it is not involved in the dringe of the fistul. If not ccessible from the ipsilterl side becuse of the cute ngle between trnsverse sinus nd SPS, nvigtion from the contrlterl side my be nother option nd sometimes esier. This lterntive hs been communicted by Andreou et l. (2007) in ptient where the AV shunt drined primrily vi the contrlterl SPS. Theudin et l. (2007) reported the use of the SPS in two ptients of which one procedure ws successful. The uthors emphsize the fct tht the SPS should be ptent for ctheteriztion becuse recnliztion my become hzrdous due to ntomic proximity to the vein of Lbbé. Cse Illustrtion II shows the simultneous ctheter nvigtion to the CS by using the IPS pproch from one side nd the SPS from the other, llowing effective occlusion of bilterl DCSF Trnsfemorl Pterygoid Plexus Approch (PP Approch) This pproch hs been described by Debrun (1993) for mngement of direct CCF, nd by Jhn et l. (1998) for tretment of DCSF. The PP cn be used like ny other femorl route if fvorble ntomy is present. Ctheteriztion of the pterygoid plexus my techniclly be more difficult thn of the IPS, but when successful, it my provide stble positioning for microctheters, even when contrlterl pproch is necessry. Klisch et l. (2003) reported on two successful ctheteriztions of the CS vi the PP in three cses with direct CCF, which my show venous dringe through this efferent pthwy more frequently (29%) thn DCSFs Trnsfemorl Corticl Vein Approch Although reported for DAVFs in the trnsverse sinus (Mironov 1998), the trnsfemorl ctheteriztion of corticl drining vein is extremely rre in DCSFs. Bellon et l. (1999) reported cse in which Prowler-14 microctheter ws successfully dvnced from the IJV through the nterior SSS, into corticl vein nd into the CS vi the sphenoprietl sinus. This llowed pcking with GDCs, leding to complete oblitertion of the fistul. The potentil risks include perfortion or rupture of the frgile rterilized corticl vein, pressure chnges due to ctheteriztion or indvertent thrombosis.

244 8.4 Discussion of Trnsvenous Occlusions 235 Even if possibly lowered by the use of modern smll nd supple tools, these risks should be of serious concerns when choosing such n pproch. Although s for the SOV, cses with long-stnding corticl venous dringe my hve developed thickened nd less frgile wlls (Kuwym et l. 1998), it is currently unknown whether such trnsformtion is regulr process nd involves the entire vein or only certin vessels segments Trnscutneous SOV Approch (SOV Cnnultion, Cse Report V) In 1969, Peterson et l. reported the direct cnnultion of the SOV fter upper eyelid or sub-brow cut reported for tretment of trumtic CCF. This ws one of the first venous pproches to the CS ever described. Tress et l. (1983) published most likely the first DCSF treted using this pproch, which ws the repeted by Uflcker et l. (1986) nd Lbbé et l. (1987) nd used by severl groups in the 1990s with incresing success (Oishi et l. 1999; Goldberg et l. 1996; Miller et l. 1995). Teng et l. (1988b) reported on the tretment of five ptients with DCSFs using this pproch. Monsein et l. (1991) described successful tretment of four ptients using detchble coils vi surgiclly exposed SOV with no complictions. Miller (1995) reported on two CCFs nd 10 DCSFs, who were successfully treted vi SOV pproch. Detchble coils s well s thrombogenic coils were used, which ws successful in nine cses. In one cse, dditionl trnsrteril emboliztion hd to be performed. Except for one ptient, who developed persistent sixth nerve plsy, there were no complictions in this series. Goldberg et l. (1996) published series in which 9/10 fistuls were cliniclly nd ntomiclly occluded using the direct SOV pproch. In one ptient in this group, severe introrbitl hemorrhge occurred during direct puncture of smll cliber vein. The sme group of ptients ws expnded by two more ptients nd reported 1 yer lter by Quinones et l. (1997) with 11/12 (91%) lesions permnently occluded. The uthor hs reported erlier on 4/5 ptients (80%) mnged successfully using this pproch with complete occlusion of the fistul nd resolution of their symptoms (Benndorf et l. 2000b). The nterior cnnultion nd ctheteriztion of the SOV ws possible in two cses despite prtil thrombosis of the nterior SOV (Fig. 8.23). Compred with trnsfemorl SOV ctheteriztion, this technique is less time-consuming. The shorter distnce to the CS cuses less friction, which my be of importnce when numerous coils hve to be pushed, or when the fistul site hs to be pproched on the contrlterl side. While the nvigtion of the ctheter into the CS is usully mtter of only few minutes, the surgicl exposure nd cnnultion of the SOV, often performed in the ngio-suite under sterile conditions (Benndorf et l. 2004; Goldberg et l. 1996; Miller et l. 1995), represents the ctul technicl chllenge. For successful procedure, skilled surgeon (ophthlmic, mxillo-fcil or neurosurgeon) with experience in performing micronstomoses is required. Some uthors hve even reported on bilterl SOV cnnultion in either the sme (Miller et l. 1995; Berlis et l. 2002) or subsequent session (Quinones et l. 1997). After successful puncture of the vein, coil plcement should be performed using high-resolution bi-plne system. This strtegy differs from the one reported by Goldberg et l. (Goldberg et l. 1996), who suggested the emboliztion be performed in the ophthlmologicl operting room. Although, in generl, fesible nd simple technique, the trnsophthlmic SOV pproch my be ssocited with complictions such s infection, grnulom, dmge to the trochle nd vessel rupture with introrbitl bleeding nd subsequent vision loss (Goldberg et l. 1996; Oishi et l. 1999; Hlbch et l. 1989; Quinones et l. 1997; Miller et l. 1995). Introrbitl bleeding ws lso observed by Giouleks et l. (1997) who treted five ptients with DCSF nd observed mjor introrbitl bleeding cused by control injection tht ruptured the distl SOV. The sme cse, in which n 8-F sheth nd 5-F ctheter were dvnced into the SOV nd CS, hd been reported erlier by this group (Tress et l. 1983). Gupt et l. (1997) reported on ptient with unilterl vision loss nd neovsculr glucom fter ttempted emboliztion of DCSF vi the SOV. The fistul could not be occluded nd the vein ws ligted t the end of the procedure, which resulted in uncontrollble elevted introculr pressure. Oishi et l. (1999) observed in three out of eight ptients (37%), whose fistule were occluded using the SOV pproch, persistent complictions with forehed dysesthesi (n=1) nd blephroptosis (n=2) due to injury of the levtor plpebre muscle fter prolonged surgicl SOV exposure. These complictions were not seen in the mteril studied by the uthor.

245 236 8 Endovsculr Tretment In recent retrospective nlysis of 25 ptients undergoing the SOV pproch, Leibovitch et l. (2006) found significnt difficulties in six (24%), mong which three hd frgile or very smll vein tht could not be cnnulted. In two other ptients, ctheter nvigtion ws impossible due to clotting or the smll size of the vein nd emboliztion could not be performed, but the ptients recovered over 2 3 months. In one ptient with tortuous nd clotted SOV, ligtion of the distl vein ws performed tht lso resulted in resolution of the symptoms. It is interesting to note tht this group, which is mong the most experienced t using this technique hs recently recommended considering lternte trnsfemorl techniques becuse of the potentil risks ssocited with SOV cnnultion (introrbitl hemorrhge, 8%). Difficulties in cnnultion with subsequent uncontrollble bleeding hs lso been observed by others (Hyshi et l. 2008). Perforting the SOV cn be ssocited with potentil loss of vision, nd cn only be controlled by emergent cnthotomy (Wldis et l. 2007). Becuse of its ssocited risks, the trnsfemorl or trnsophthlmic SOV pproch should probbly not, s suggested by some opertors (Miller 2007), be considered the method of choice, but probbly more s n lterntive strtegy fter consequently using the much esier nd sfer IPS pproch first Trnsorbitl SOV Approch (Direct Puncture, Cse Report VI) The decision for this more ggressive pproch in one of the ptients ws mde only s lst resort fter ll other pproches filed. The incresed risk of direct puncture ws discussed in detil with collegues from ophthlmology, mxillo-fcil nd neurosurgery nd hd been crefully blnced ginst the potentil risks ssocited with open surgery. The ptient s significntly elevted introculr pressure nd his imminent risk of complete vision loss justified the SOV puncture in the deep orbit. In cses where surgicl exposure nd cnnultion of the SOV is not n option, or dditionl mechnicl obstcles prevent ctheter nvigtion, retrobulbr puncture of the SOV hs been suggested ( Goldberg et l. 1996; Teng et l. 1988b). Currently smll cliber microctheters re vilble, which ccommodte the use of smll 21-G needles to fcilitte such n invsive pproch (Benndorf et l. 2001). However, even though the degree of vessel trum is minimized, the risk of cusing mjor compliction such s n introrbitl hemorrhge is nevertheless higher thn using ny other pproch (Goldberg et l. 1996; Leibovitch et l. 2006; Uflcker et l. 1986; Teng et l. 1995). Goldberg et l. (1996) observed mssive introrbitl hemorrhge fter ttempting to puncture n pprently frgile SOV in the center of the orbit (see Leibovitch et l. 2007). Trnscutneous puncture of the SOV ws pioneered by Teng et l. (1988b) who treted five ptients without complictions. The uthors used steel spring coils, gel fom strips nd IBCA for fistul occlusion. According to the uthors, mong trnsfemorl routes, only the IPS ws ttempted. If surgicl dissection of the nterior SOV filed, the vein ws punctured. Although the uthors need to be congrtulted on this erly work, it is interesting to note tht in t lest one cse (Cse 3), the IPS ppered widely open in their ngiogrm nd thus would hve been suitble route for sfer trnsfemorl occlusion. It is lso not relly cler where exctly the vein ws punctured, in the nterior orbit or deeper (intrconl) s bi-plne high-resolution fluoroscopy or rod mpping ws not yet vilble. Also, the use of 16-G or 18-G needles, inch guidewires nd 5-F ctheters, ppers reltively invsive s it my esily cuse serious vessel trum. Despite the dvntge of deploying lrger coils nd reducing time nd costs, choosing smller needles, guidewires, microctheters nd coils ppers dvisble tody (Ong et l. 2009). Chn et l. (2006) reported recently on novel pproch by performing puncture of the intrconl SOV using n 18-G needle fter filed ttempts of femorl ccess nd nterior SOV cnnultion. This technique is however similr to the deep orbitl puncture reported previously by the uthor ( Benndorf et l. 2001) White et l. (2007) recently described ptient in whom the inferior ophthlmic vein ws trnsorbitlly punctured with n 18-G needle nd successfully used for ccessing the CS. The ntomy of the IOV my vry nd the vein my occur not s singulr vein, but rther plexus of veins (Rhoton 2002). Kuettner et l. (2006) treted two ptients using cnnultion of the SOV with 16-G needle fter plpebrl incision. The fistuls were successfully occluded nd symptoms resolved within severl weeks. Both ptients developed persistent moderte blephroptosis.

246 8.4 Discussion of Trnsvenous Occlusions 237 Modern high-resolution fluoroscopy nd bi-plne rod mpping, provide excellent visul control of needle positioning nd dvncement nd hve improved the fesibility nd sfety of introrbitl puncture techniques. Nevertheless, precutions considered for trnsfemorl ctheter nd wire mnipultions in rterilized veins re even more importnt when directly puncturing n rterilized SOV (Uflcker et l. 1986). The risk of lcerting, dissecting nd rupturing this vein by shrp needle is certinly greter nd must not be neglected ( Wldis et l. 2007). Even when exposed to rteril pressure for longer periods of time, n AV shunting vein is frgile structure. The risk of perfortion by even minor nd gentle needle or guidewire mnipultions remins unpredictble, nd thus should be minimized by using the smllest possible devices for this technique (20- to 21-G needles nd 2.5- to 3-F microctheters) Direct Puncture of the Formen Ovle This pproch is performed on nother efferent vein from the CS: the emissry vein of the formen ovle (formen ovle plexus). A direct puncture of the CS through the formen ovle to deflte detchble blloon in the CS ws reported by Jcobs et l. (1993). Using n 18-G needle tht ws guided into the CS, 21-G Chib spinl needle ws introduced nd used to puncture ltex blloons. The technique of puncturing Meckel s cve through the formen ovle under fluoroscopy for tretment of trigeminl neurlgi is well known procedure. The successful use of this pproch for coil occlusion of DCSF hs been performed recently by E. Houdrt (personl communiction) Direct Puncture of the CS vi the SOF (Cse Illustrtion XI) The cse described herein refers to technique tht ws initilly reported by Teng et l. (1988) s n ultimte tretment option for direct CCF with multiple (10 ) recurrences. The sme group lter published series of 11 ptients, successfully treted using this technique, nd reported trnsient ptosis in two ptients s the only complictions (Teng et l. 1995). All ptients hd undergone previous tretment by either crotid ligtion or blloon detchment; some of them hd lredy lost their vision (see bove). An 18-G needle nd 5-F ctheter were used, while 2/11 ptients developed ptosis nd in 1/11 introrbitl bleeding hd to be mnged by immedite plcement of blloon in the ICA. The uthors emphsized tht this pproch should be used s lst option, nd in cses where lterntive methods hve filed, the ICA is occluded or the ptient hs vision loss. It is interesting to note tht the ngiogrms of some of the ptients showed n pprently ptent IPS (Figs. 1 nd 3 there), nd thus this cses hve ctully been cndidtes for less ggressive trnsfemorl pproch. Teng et l. (1995) rgued tht puncture of the CS is techniclly esier thn tht of the SOV becuse of better orienttion using ntomic lndmrks under fluoroscopy such s the suprorbitl fissure (SOF), sell turcic nd nterior clinoid processes. Such justifiction, s well s the fct tht the SOV cn only be visulized by repeted ngiogrms, pper lesser vlid resons tody with the vilbility of high-resolution bi-plne rod mp on ll modern ngiogrphic systems. It could be rgued whether or not the risk of n introrbitl puncture is higher thn tht of n intrcrnil puncture. The former my cuse mngeble introrbitl hemorrhge with potentil vision loss, while the ltter my led to life-thretening subrchnoid or intrcrnil hemorrhge. Workmn et l. (2002) more recently described long-stnding, direct CCF due to gunshot wound, tht reoccurred fter 26 yers of unsuccessful ligtions nd ws treted with n introculr trns-sof pproch. A 16-G ngiocth ws used to deploy 20 Ginturco coils. The uthors discuss in detil the ntomic bsis for this pproch nd its potentilly ssocited complictions. Cse Illustrtion III is n exmple of the use of the trnsorbitl CS puncture in DCSF. This ptient ws referred from nother institution where trnsvenous ccess through the IJV hd filed. Direct CS puncture ws performed s lst tretment resort. Even though performed successfully, subrchnoid hemorrhge due to trnsgression of the subrchnoid spce, introrbitl hemtom nd vision loss, direct injury of the optic nerve, ophthlmic rtery or intrcvernous crnil nerves, globe puncture nd infection re potentil serious complictions (White et l. 2007; Workmn et l. 2002). Furthermore, when the ICA is ptent, s is commonly the cse in DCSFs, the risk of lcerting or (Text continues on p. 251)

247 238 8 Endovsculr Tretment b c d e f Fig f. Cse Illustrtion I: Frontl vein pproch nd TVO with NBCA.,b Right ICA injections, AP views: Arteriovenous shunt of the right CS (sterisks) supplied by brnches of the MHT. Venous dringe vi the right nd left SOV (rrows), both suprorbitl (short rrows) nd both frontl veins (FrV, thick rrows). No opcifiction of the IPS or the fcil vein. c Percutneous puncture of the FrV using 18-G needle llows nvigtion of microctheter (rrow) to the cvernous sinus nd subsequent occlusion of the fistul with intrvenous injection of 20% NBCA (d). e,f Right nd left CCA injection t the end of the procedure demonstrting complete occlusion of the fistul. Insets in nd e Lterl ICA projections pre- nd postemboliztion. Note: Attempts to ctheterize the CS using IPS nd SPS pproches filed. (Courtesy: J. Moret, Pris) Author: Puncture of frontl or suprorbitl veins ws widely used technique in the dys of orbitl phlebogrphy (1960s- 1970s). The use of torniquet cn be helpful to get ccess

248 8.4 Discussion of Trnsvenous Occlusions 239 b c d Fig d. Cse Illustrtion II: Simultneous IPS nd SPS pproch in bilterl fistul.,b Right nd left CCA injection, AP view: Arteriovenous shunt t the right (white sterisk) nd left CS (blck sterisk), supplied by ICA nd ECA brnches. Venous dringe vi ICS (rrowheds) into the right SOV (rrow) c Non-subtrcted AP view. Coilpcking of the right nd left fistul site (sterisks) fter ctheriztion of both CSs using the right IPS (double rrow) nd the left SPS (single rrow). d Left CCA injection, AP view: Complete occlusion of the fistul t the end of the procedure. The ptient recovered fully. (Courtesy: J. Moret, Pris) Author: In cses where bilterl occlusion is necessry (usully bilterl fistuls, lthough rre) nd cross-over nvigtion is not possible, different venous pproches cn be used during the sme session to chieve complete occlusion. The SPS my be difficult to ctheterize when not drining the fistul nd not clerly opcified. If not ccessible ipsilterlly due to n cute ngle between SPS nd trnsverse sinus, nvigtion from the contrlterl side my be nother option

249 240 8 Endovsculr Tretment b c d e f Fig f. Cse Illustrtion III: Dul IPS pproch, TVO using coils. A 48-yer-old womn presenting in Mrch 2009 with eye redness nd diplopi.,b ICA nd APA injection, lterl views show fistul with nterior nd posterior dringe. The IPS is tpered nd prtilly occluded. The nterior/inferior CS comprtment drins into the SOV (rrow) nd corticl veins (thin rrows). The posterior/superior comprtment drins into the leptomeningel nd deep veins (double rrowheds). c,d In order to block the nterior nd posterior dringe independently, two microctheters (Excelsior SL-10, Echelon ) were dvnced into the CS using the IPS pproch (thrombosed IPS). This llowed pcking of the posterior comprtment (white sterisk, double rrows), while keeping ccess to the nterior CS nd SOV (blck sterisk, single rrows). e,f Complete occlusion of the AV shunt t the end of the procedure. Note, the missing ophthlmic rtery in e tht ppered hemodynmiclly occluded, but ws spontneously reconstituted through the ECA (inset, rrowhed: choroidl blush, rrow: OA). This phenomenon ws reversed in the 3 months FU. The ptient s vision ws not ffected. A dul pproch to the CS cn be useful to void rerouting or worsening of venous dringe into leptomeningel or corticl veins

250 8.4 Discussion of Trnsvenous Occlusions 241 b c d e f g h Fig h. Cse Illustrtion IV: Direct puncture of the cvernous sinus through the SOF. A 42-yer-old womn with rpid left visul deteriortion following trnsrteril nd trnsvenous emboliztion in nother institution tht remined incomplete due to technicl issues. The ptient ws urgently trnsferred; upon rrivl, no light perception. Attempted ccess vi fcil vein filed due to thrombosis. Left CCA injection lterl view erly nd lte (b) phse: Arteriovenous shunt t the left CS ( sterisks), supplied by brnches from the ICA nd ECA. Dringe minly vi SOV (rrow), no opcifiction of the IPS, but dringe vi SPS (thin double rrow) nd leptomeningel veins (thin rrows). c,d Left orbit (dry skull) AP view: View onto the the SOF (rrow) through which the SOV courses to rech the CS region (sterisk); short rrow: Optic cnl. e,f Control injection directly into the CS (sterisk), fter direct puncture using 16-G needle, introducing ctheter nd plcement of the first Ginturco coils, shows prtil occlusion with residul dringe into the SOV (rrow). g,h Finl control, left ICA injection, lterl (g) nd AP (h) views: Control rteriogrms reveling complete occlusion of the fistul t the end of the procedure. The ptient never recovered her vision; fundoscopic exm reveled venous infrction of the retin, probbly cused by prolonged venous hypertension following the initil unsuccessful emboliztion. (Courtesy: J. Dion, Atlnt) Author: Direct puncture of the CS through the SOF is fesible but reltively gressive pproch, ssocited with n incresed risk of introrbitl or intrcrnil hemorrhge, nd thus should remin technique of lst resort. Courtesy: J. Dion, Atlnt

251 242 8 Endovsculr Tretment b c d e Fig e. Cse Illustrtion V: Use of Hydro Coils. A 53-yerold femle with recent onset of mild redness nd diplopi of the left eye. Right internl crotid rteriogrm shows Type D fistul of the right posterior cvernous sinus (sterisk), supplied by the meningeohypophysel trunk nd drining into the SOV (double rrow). b Right externl crotid rteriogrm showing supply from the rtery of the formen rotundum (rrow), the ccessory meningel nd middle meningel rteries (rrowhed nd long rrow). c Microctheter (rrow) nvigted to the CS (sterisk) through the fcil nd superior ophthlmic veins. d Tight pcking of numerous bre pltinum coils (TruFill) hd only miniml effect on the AV shunting. e Following deployment of four HydroCoils, significnt flow reduction ws observed. A control ngiogrm 15 min lter showed complete oblitertion of the fistul. (Courtesy: M.E. Mwd, Houston) Author: Becuse it reduces the totl mount of coils, the rdition exposure nd possibly the risk of secondry CN deficits, dditionl use of HydroCoils for indirect (nd direct) CSFs cn be very useful djunct

252 8.4 Discussion of Trnsvenous Occlusions 243 b c d Fig d. Cse Illustrtion VI: Use of HyperSoft coils. High-flow fistul in 64-yer-old ptient suffering from Wegener s grnulomtosis with 6-month history of eye redness nd proptosis on the left side. Right ICA injection, lterl view shows fistul t the posterior CS, supplied minly by the TMH, shunting into the left CS nd drining into the left SOV ( rrow), IOV (short rrow) nd IPS (double rrow). b Left IPS pproch using n Echelon microctheter nd plcement of the first coils. c,d Complete occlusion of the AV shunt by extremely dense pcking with totl of 51 HyperSoft coils (MicroVention) Author: The use of softer coils hs the dvntge of creting very dense pcking, reducing the risk of comprtmentliztion s well s of secondry CN deficits, potentilly cused by incresed mechnicl pressure of the coils. (Courtesy: R. Klucznik, Houston)

253 244 8 Endovsculr Tretment b c d e f Fig f. Cse Illustrtion VII: Trnsrteril emboliztion using NBCA (Glubrn ). A 55-yer-old womn presenting with exophthlmos nd chemosis of 6 months stnding, incresed oculr pressure nd decresed visul cuity for 2 dys prior to the procedure (trnsvenous ttempts filed).,b Left ICA injection AP nd lterl views, showing smll AV shunt (sterisks), fed by the ILT (rrowhed) drining into the SOV (rrow). c Rod mp during selective ctheteriztion. Smll rrowhed: Tip of microctheter (rrowhed) t the origin of the ILT. d Selective sinogrm fter nvigtion of Mgic 1.2 (Blt Extrusion) using Mirge (EV3). Smll rrowhed: Tip of the microctheter 3 mm inside the ILT (rrowhed: Microctheter in the ICA lumen). e,f Control rteriogrms fter injection of 20% Glubrn (NBCA) mixed with Lipiodol showing complete oblitertion of the AV shunt. (Courtesy: A. Biondi, Pris) Author: Becuse the microctheter is only few mm inside the ILT, the risk of reflux is quite high nd glue injection should be performed only by n experienced opertor. A lower glue concentrtion is dvisble

254 8.4 Discussion of Trnsvenous Occlusions 245 b c d e Fig e. Cse Illustrtion VIII: Trnsvenous occlusion using NBCA. A 90-yer-old femle with significnt exophthlmos nd red eye. Right CCA injection, lterl view: Arteriovenous shunt t the right CS (sterisk), supplied by brnches from both ICA nd ECA territories. b Venogrm of the cvernous sinus (sinogrm), lterl view: Becuse the right IPS could not be pssed, n pproch from the contrlterl side through the ICS ws performed, llowing for plcement of microctheter t the fistul site (white rrow) (c). Note tht 4-F guide is dvnced into the left IPS (double rrow). d Injection of 1 cc of glue ( sterisk, 50% Glubrn/lipiodol mixture) resulted in complete occlusion of the AV shunt (E). (Courtesy: J. Moret, Pris) Author: Intrvenous injection of NBCA into the CS cn be very effective, but crries the risk of untowrd migrtion into pil veins. A higher concentrtion of the glue is recommended

255 246 8 Endovsculr Tretment b b b Fig f. Cse Illustrtion IX: Trnsrteril Onyx injection using flow control. A 76-yer-femle presenting with right exophthlmus, chemosis nd glucom, of 2 months stnding., b Right ICA injection, lterl-oblique views, erly rteril nd venous phse shows smll Type D fistul of the right posterior cvernous sinus (sterisk), supplied by the MHT (thin rrow) nd drining into the SOV (rrow). Insets: RAO views, erly nd lte phse. c Right ECA injection, lterl view showing feeding pedicle (rrow) from the AMA (long rrow). Insets: Lte rteril phse. d Superselective injection fter microctheter nvigtion into the AMA (rrow) shows fistul supply vi the posteromedil rmus of the ILT (thin rrow). e Control rteriogrm fter single trnsrteril Onyx injection (0.3 cc) into the AMA pedicle, during which n occlusion blloon (4 20 mm HyperGlide EV3, double rrow) ws inflted cross the MHT origin. Onyx cst seen in the posterior CS; less dense in the nterior CS (sterisks). Note tht some reflux is seen in the feeding pedicle (thin rrow). The blloon ws inflted for short period of time, only when the Onyx reched the CS to prevent reflux into the ICA vi the TMH. The injection ws stopped s soon s ngiogrphic controls showed fistul occlusion. f Finl control ngiogrm showing complete oblitertion of the AV shunt. Insets: Lte rteril phse. (Courtesy: G. Gl, Würzburg) Author: Such indirect flow control cn be very helpful to void Onyx migrtion into the cerebrl circultion

256 8.4 Discussion of Trnsvenous Occlusions 247 b c d e Fig e. Cse Illustrtion X: Use of covered stent. Right ICA injection, erly rteril phse reveling very smll AV shunt, supplied by brnches of the MHT (thin rrow). b Right ICA injection, venous phse shows very slow filling of the CS (sterisk). No opcifiction of drining veins. c Plcement of covered stent ( mm JOSTENT Coronry Stent Grft, Abott Vsculr, IL), bridging the MHT origin (rrows). White rrow: Wingspn Stent plced for tretment of n M1 stenosis (Inset in ). d,e Control ngiogrm t the end of the procedure showing complete occlusion of the AV shunt. (Courtesy: R. Klucznik, Houston) Author: In cse with ICA supply only (Type B fistul), the use of covered stent cn be fesible nd very effective. However, becuse this device (JOSTENT) is reltively stiff nd requires 7 F or 8 F guiding ctheter, its ppliction is currently limited to fvorble ntomy. A dedicted covered stent suitble for neurovsculr ntomy is needed. Long-term dul ntipltelet therpy is required nd represents nother disdvntge

257 248 8 Endovsculr Tretment b c d e f g h i j

258 8.4 Discussion of Trnsvenous Occlusions 249 k l m n o p Fig p. Cse Illustrtion XI: Stged TVO of complex fistul with introsseous comprtment (perfomed together with Dr. A. Biondi in 10/2008 nd 01/2009 t Pitié-Slpêtrière Hospitl, Pris). A 72-yer-old womn presented in Mrch 2008 with intrcrnil bruit, bilterl chemosis, presis of left 3rd crnil nerve nd plsy of the right VIth nerve.,b Right ECA injection AP nd lterl views show complex AV shunting lesion, involving both CSs (blck sterisks) nd midline comprtment tht ppers to be locted withing the clivus (white sterisk). There is venous dringe into both SOVs (rrows), leptomeningel nd corticl veins on the left side (smll rrows) nd into lrger chnnel mimicking the left IPS (thick rrow). Insets: Left jugulr phlebogrm revels tht the IPS (double rrows) is in fct occluded (thrombosed), but cn be pssed using the loop technique (c,d). Asterisk: Left CS, rrow: Left SOV. e Superselective injection vi microctheter fter crossing the midline (AP view) shows the thrombosed right IPS (double rrow) nd right CS (sterisk). No fistul dringe is identified. f Repositioning of the microctheter revels the fistulous communiction with the right SOV (rrow). Dense coil pcking (g) resulted in occlusion of the AV shunt t the right CS. h The midline lesion (white sterisks) ws not ffected nd continued to drin (thick rrow) directly into the IJV s well s into the left CS (blck sterisk), the SOV (rrow) nd leptomeningel veins (thin rrows). i,j Ctheter nvigtion into the left nterior CS shows the fistul dringe into the SOV (rrows) nd corticl veins (smll rrows). Cudl-oblique ngultion in AP (k) nd crnil-cudl ngultion in lterl views (l) help to better visulize the reltionship between the CS-SOV junction nd the exiting corticl veins. This is difficult in stndrd views, but my be crucil to find working projection for disconnection of these veins from the AV shunting. m,n In this cse, the corticl veins enter the CS slightly posterior (white sterisk) to the CS-SOV junction (blck sterisk). For disconnection of corticl nd leptomeningel dringe see lso Figure 8.4. o, p Right nd left ECA injections AP nd lterl views: Complete occlusion of the AV shunt involving the CS on both sides. Note lso the disppernce of the corticl venous dringe on the left side. There is persistent AV shunting involving the midline lesion nd drining vi seprte venous chnnel (thick rrow) directly into the IJV

259 250 8 Endovsculr Tretment b c d e f Fig e. Cse Illustrtion XI: Stged TVO of complex DCSF with introsseous comprtment (2 nd session).,b Right ECA injection, AP nd lterl views prior to the 2 nd procedure in 01/09 shows some diminshed flow, but otherwise unchnged residul AV shunting within the clivus. c, d Ctheteriztion of the introsseus venous pouch in the midline nd subsequent coilpcking. e, f Finl control demonstrting complete occlusion of the AV shunt t the end of the procedure. Thick rrow: Venous chnnel drining the introsseus AV shunt into the jugulr vein tht ws used s pproch in c,d (not to be mistken s IPS). The 3-months FU exm confirmed stble occlusion nd recovery of the ptient. (See the ACT imging of this cse in Fig. 7.78)

260 8.4 Discussion of Trnsvenous Occlusions 251 perforting the rtery within the CS nd creting direct CCF is lwys present. As stted bove, the risks ssocited with the use of 16-G needle need to be weighed ginst the dvntge of pushing coils llowing for fst pcking of the CS, reducing the overll rdition exposure nd durtion of the procedure. Another recent pper summrizes the experience of the sme group with this prticulr pproch in totl of seven ptients in whom complete occlusion ws ccomplished in single session with one trnsient decrese in visul cuity tht improved mrkedly fter 48 h (White et l. 2007). No other complictions hve been reported, nd no further detils of the ptients or procedures re communicted. This technique ws recently pplied in three cses by Ong et l. (2009) without periprocedurl or delyed complictions. The uthors used 22-G spinl needle for puncture under biplne fluoroscopy tht ws exchnged for 5-F sheth nd used to inject Onyx-34. The importnce of voiding withdrwl of needle or ctheter before the AV shunt is fully occluded is prticulrly stressed Sylvin Vein Approch: Combined Surgicl/Endovsculr Tretment (Cse Report VII) The puncture of the Sylvin vein following crniotomy ws first described in 1997 by the uthor ( Benndorf et l. 1997, 1999b) nd repeted by others (Krisht nd Burson 1999). Although puncturing cerebrl vein is techniclly not difficult for n experienced vsculr neurosurgeon, performing open surgery to gin this prticulr ccess crries its inherent risks nd should be voided whenever possible. Schmidbuer et l. (2001) reported on two ptients, who were successfully mnged using cnnultion of the Sylvin vein following pterionl crniotomy. Previous ttempts of TAE nd TVO filed to control the AV shunts. Both fistuls were occluded; one ptient recovered well, the other suffered from thrombembolic stroke due to postopertive ngiogrphy with residul hemipresis. Cnnultion of corticl vein fter crniotomy ws successfully performed in one of the uthor s ptients. This combined surgicl-endovsculr pproch ws chosen s n ultimte option fter ll trnsrteril nd trnsvenous ttempts of tretment repetedly filed. In exceptionl cses, where indeed ll possible trnsvenous routes hve been ttempted, surgicl explortion of n rterilized vein with subsequent puncture nd ctheteriztion of the CS cn become n lternte option. Kuwym et l. (1998) described the cse of 48-yer-old mn who suffered from ICH cused by DCSF drining only vi the Sylvin vein, which ws intropertively punctured, llowing for complete occlusion. Even though reports on such n pproch re scrce, it represents vluble technique in otherwise hopeless cses. The cse presented here (Benndorf et l. 1997) is lso good exmple of interdisciplinry tem work in difficult-to-tret lesion. The use of similr pproches for tretment of cerebrovsculr diseses ws reported erly by Mulln (1979) nd Tress et l. (1983) for mngement of DCSFs. In cse with limited endovsculr options due to ntomicl vrints of the CS, Chloupk et l. (1993) discussed the need for combined neurordiologicl nd neurosurgicl tretment options. As n lterntive, Krischt et l. (1999) suggest pretemporl extrdurl pproch, through which the nterior CS cn be punctured to plce coils. Brker et l. (1994) described similr technique using trnssphenoidl, trnsethmoidl microsurgicl pproch, through which successful emboliztion ws performed. Klisch et l. (2001) reported on trnsnsl-trnssphenoidl pproch to the posterior CS. Hr et l. (2002) reported n interesting surgicl pproch vi the petrosl vein in two ptients. Previous incomplete EVT hd resulted in dngerous rerouting of leptomeningel venous dringe cusing hemipresis in one. Both ptients underwent suboccipitl crniectomy nd combined surgicl endovsculr pproch to the CS. The AV shunts were occluded using GDC pcking of the CS leding to recovery in both ptients. In order to cnnulte the deep SOV, Bdill et l. (2007) recently suggested temporl, extended superior eye-lid incision combined with lterl mrginectomy of the orbitl rim. This pproch ws eventully successful fter trnsfemorl IPS nd SOV ctheteriztion filed due to comprtmentliztion of the CS nd tortuousities of the ophthlmic veins. Although still ssocited with less morbidity thn unroofing the orbit, this technique ppers lso reltively invsive. DCSFs without corticl dringe re considered benign vsculr lesions nd should be mnged with the lest invsive pproch first. While seldom seen,

261 252 8 Endovsculr Tretment cses of persistent corticl dringe in DSCFs cn be ssocited with neurologicl symptoms nd severe hemorrhge, justifying more ggressive tretment options. Besides other potentil complictions such s seizure or infection, puncture nd ctheteriztion of n rterilized corticl vein crries considerble risk, remins lst resort procedure nd should be crried out only by n experienced neurordiologicl-neurosurgicl tem. It should be reserved for ptients in whom clinicl symptoms re progressing nd every possible lternte pproch (trnsrteril, trnsvenous, trnsophthlmic, etc.) ws indeed ttempted nd clerly filed. Choosing ggressive ( heroic ) procedures other thn s n ultimte option in n otherwise hopeless cse is not cceptble nd to be bndoned. The cse shown here ws ctully treted very erly in the uthors experience (1992) nd reported few yers lter. In the erly 1990s, ctheter technology s well s ngiogrphic equipment were in less developed stge, thus limiting opertors cpbilities. It is hrd to imgine tht with endovsculr rmmentrium nd modern high resolution imging of 2009 such n ggressive pproch would still be necessry or justified. In order to understnd preferences for certin pproches dvocted by other opertors, one must lso consider their clinicl bckground. Opertors with surgicl bckground my be in fvor of opertive or combined opertive/endovsculr techniques (Miller 2007; Goldberg et l. 1996; Hnneken et l. 1989; Kuwym et l. 1998; Leibovitch et l. 2006; Brker et l. 1994; Bdill et l. 2007; Debrun et l. 1989). It is presumbly no coincidence tht the lrger series of TVO in DCSFs reported by interventionl neurordiologists recommend using trnsfemorl routes, in the vst mjority the IPS pproch (Cheng et l. 2003; Oishi et l. 1999; Theudin et l. 2007; Wkhloo et l. 2005; Benndorf et l. 2004; Kim et l. 2006; Meyers et l. 2002; Klisch et l. 2003; Kirsch et l. 2006). This is owing to their extensive experience in trnsvenous ctheteriztions nd occlusion techniques for durl rteriovenous lesions. In conclusion, trnsfemorl ctheteriztions of the IPS, or direct IJV stick, re currently considered the techniques of choice for trnsvenous occlusions of DCSFs. Only when ttempts of ipsi- nd contrlterl IPSs remin unsuccessful, trnsfemorl SOV pproch, or SOV cnnultion fter surgicl exposure, should be performed. Depending on the dringe pttern, less invsive pproches such s trnsfemorl ctheter nvigtion through the MTV, PP or FV my be ttempted. As shown in the tretment lgorithm in Tble 8.5, more ggressive techniques, such s direct puncture of the SOV, the IOV or even the CS, s well s combined neurosurgicl-endovsculr pproches should be considered only fter ll other options hve truly been exploited. This rtionl is bsed first on sfety nd second on efficcy of the vrious techniques of nvigting microctheter, veinicth or needle into the CS. Tble 8.5. Tretment lgorithm for TVO in DCSFs [modified fter Benndorf et l. (1999)] Trnsfemorl Inferior petrosl sinus (IPS, lso vi puncture of IJV) Fcil vein (FV)/superior ophthlmic vein (SOV) Pterygoid plexus (PP) Middle temporl vein (MTV) Corticl vein Trnscutneous Puncture of frontl vein Puncture of fcil vein (US-guided) Surgicl exposure nd cnnultion of SOV Puncture of CS through the formen ovle Trnsorbitl Puncture of SOV or IOV Puncture of CS through the orbitl fissure Crniotomy Surgicl exposure nd puncture of corticl (Sylvin) vein Depending on the dominnt venous dringe Embolic Mterils Prticles PVA prticles hve been used to limited degree by the uthor for trnsrteril emboliztions (TAE). Their dditionl use prior or fter TVO hs not been found necessry in the vst mjority of cses, including the ones showing subtotl occlusion t the end of the procedure Coils Cse Reports I VI nd Cse Illustrtions II, IV, V demonstrte how proper choice of coils my llow dense pcking tht results in subtotl or complete

262 8.4 Discussion of Trnsvenous Occlusions 253 occlusion by the end of the procedure (Benndorf et l. 2000). Vrious embolic mterils hve been used in the pst for occlusion of CSFs, including cellulose, cotton, silk threds, Ginturco coils nd blloons (Mulln 1974; Hlbch et l. 1989; Tress et l. 1983; Debrun et l. 1975; Serbinenko 1974). Mulln (1974) initilly used bronze-phosphor wire, which he inserted directly into the CS. Hosobuchi (1975), using direct insertion of copper wire into the CS ws ble to induce electrothrombosis in four ptients with CSF. The concept of electrothrombosis for occlusion of CSFs ws initilly described by Peterson et l. (1969), who were ble to cure direct CCF by introducing copper wires nd pplying 2 ma positive current. Seril ngiogrms demonstrted progressive occlusion of the fistul over 4-h period. Tkhshi et l. (1989) reported on the successful tretment of five ptients in whom copper wires were introduced into the CS vi trnsvenous pproches. By dvncing copper wires using microctheter, the uthors ccomplished complete occlusion of the fistul in three cses (60%) nd disppernce fter 8 months in two (40%). Tress et l. (1983) occluded DCSF with Ginturco steel coils. In the lte 1980s, pltinum ws discovered s new embolic mteril (Yng et l. 1988) nd introduced to replce the commonly used Ginturco coils (Anderson et l. 1977, 1979; Chung et l. 1980; Ginturco et l. 1975). Yng et l. (1988) first described the use of pltinum wires for occlusion of rteriovenous fistuls nd chieved complete occlusion in 6/9 cses. The bility to deliver these thin pltinum wires through microctheters with size of 2.2 F into the desired territory pushed their intrvsculr use. Hlbch et l. (1989) reported on the first lrge series using Ginturco coils nd coils mde of gold nd pltinum. In 9/13 ptients (69.2%) ngiogrphic occlusion could be documented, in five of these (38.5%) only coils were used, nd in four (30.8%), coils were combined with IBCA. Coils with thrombogenic fibers hve been incresingly utilized for trnsvenous fistul occlusions. However, the use of pushble, fibered coils my be problemtic if they re not correctly plced t the fistul site or t the connection between the CS nd its tributries, or if sufficiently dense pcking of coils cnnot be chieved. The ltter my result in n incomplete occlusion, requiring subsequent tretment ttempts with more difficult ntomic ccess. Therefore, despite higher costs, it hs become incresingly more cceptble to use minly, or even exclusively detchble coils. Electrolytic detchble pltinum coils re the most widely used embolic mteril in the endovsculr therpy of cerebrovsculr diseses. For occlusion of n AV shunt, the fct tht pltinum is bout 3 4 more thrombogenic thn stinless steel my be more importnt thn the ctul electrothrombosis itself. Electrothrombosis is thought to be cused by the negtive chrge of white nd red blood cells, pltelets nd fibrinogen, ttrcted by the positive chrge of the electrode (Qureshi et l. 2000). In ddition to the fibrotic rection tht replces the intrluminl clot, n inflmmtory process cn be observed in the grnultion tissue, consisting of neutrophil, polymorphs, eosinophile grnulocytes nd lymphocytes (Byrne et l. 1997). The use of electrothrombosis for tretment of vsculr lesions is not new. The induction of intrluminl clot by introducing metllic needles ws documented in the 19 th century (Velpeu 1831; Philips 1832). It is quite interesting to note tht electrothrombosis for occlusion of CSFs hd probbly lredy been used by Petrequin in 1846, lthough t tht time with n unfvorble outcome for the ptient. As erly s 1880, Sttler mentioned relted to the pulsting exophthlmus, our expecttions re tht in the nterior tumor like segment of the ophthlmic vein n extensive, firm thrombus, nd by slow progression of the thrombosis towrds the cvernous sinus, occlusion of the ruptured wll of the crotid will occur. Lter, in 1930, Sttler gin described electrolysis, clled glvnopuncture tht ws performed in the following mnner: In the unipolr electrolysis, needle, connected to the positive pole is introduced into the pulsting tumor, while the wet, negtive lrge indifferent needle [is] plced onto the body of the ptient. In the bipolr electrolysis, both needles connected to the different poles re introduced nd the current is incresed up to 1 5 Millimpere. Becuse of the occurring pin, nesthesi is necessry. Permnent heling by glvnopuncture ws reported by severl uthors t tht time (Eversbusch 1897; Mencho 1907) with three sessions of 15 min ech in 2-week intervls being sufficient, such s in Eversbusch s cse (1897). A number of uthors hve reported the results of endovsculr tretment of CSFs with GDC ( Nesbit nd Brnwell 1998; Mwd et l. 1996; Guglielmi et l. 1995; Bvinzski et l. 1997; Siniluoto et l. 1997) or other detchble coils ( Oishi et l. 1999; Yoshimur et l. 1995; Terd et l. 1996).

263 254 8 Endovsculr Tretment Guglielmi et l. (1992) were the first to report on direct CSF tht ws treted using GDCs fter filed trnsrteril blloon occlusion. The uthors discussed occlusive fctors nd emphsized electrothrombosis s n importnt one, prticulrly when ptient is heprinized. Whether or not electrothrombosis indeed plys promoting role in the thrombotic trnsvenous occlusion of the AV shunt is open to question ( Guglielmi et l. 1995). Some DCSFs my occlude immeditely fter plcement of few bre pltinum coils. In other cses, the AV shunting remins prtilly open (subtotlly occluded), even fter pushing numerous coils or dditionl fibered coils nd show n occlusion only in the follow-up exmintion (Benndorf et l. 2000). Blood thinning due to hepriniztion during the emboliztion procedure, (normliztion cn tke hours post procedure) my ffect this process (Guglielmi et l. 1995). Nevertheless, hepriniztion throughout trnsvenous emboliztion ppers resonble, becuse it helps not only to void thromboembolic complictions on the rteril side, but lso to prevent untowrd progressive thrombosis on the venous side, especilly inside the SOV tht my cuse visul deteriortion. The complete interruption of the fistulous communiction by dense mechnicl coil pcking ppers more importnt thn electrothrombotic effects. Mechnicl blockge obtined with less thrombogenic but softer bre pltinum coils likely compenstes for the lower thrombogenicity. This ssumption is supported by the fct tht in four ptients who were treted with mteril more thrombogenic thn GDC, none showed n immedite complete occlusion (Benndorf 2002). Although there is no study llowing for direct comprison of different coil systems, nd prt from the uthor s opinion, it ppers justified to stte tht dense mechnicl pcking is more effective thn loosely pcked thrombogenic mteril. The fct tht the effective mss per deployed coil unit is higher thns compred to fiber coils is considered nother contributing fctor (Tomsick 1997). The key dvntge of ll detchble coils is the possibility to correct their positioning, voiding untowrd migrtion into venous comprtments, where occlusion cn cuse clinicl problems. Even unintentionlly, premture occlusion of the IPS my cuse ccess problems or rerouting of venous dringe. During TVO of fistul with nterior dringe, the im is to interrupt the communiction between SOV nd CS without dislodging coils deep into the ophthlmic vein, thus preventing n impirment of the norml orbitl nd oculr venous dringe. The erly experience of Guglielmi et l. (1995) hs been confirmed by severl other investigtors (Nesbit nd Brnwell 1998; Bvinzski et l. 1997; Siniluoto et l. 1997) who were ble to effectively occlude direct CSFs using trnsrteril coil plcement. In four cses of smll to medium fistuls (3 mm), Sinoluto et l. (1997) chieved complete occlusion in two t the end of the tretment, nd in two others t follow-up. Bvinsky et l. (1997) treted six ptients with Type A fistuls nd chieved complete occlusion in ll cses, including one ptient embolized trnsvenously. The uthors observed serious compliction due to mssive thrombosis leding to unilterl visul impirment nd incresed sixth nerve plsy tht ws explined by overpcking of the CS. Nesbit nd Brnwell (1998) were ble to occlude 12 high-flow fistuls, including three Type A CCF. There ws no coil migrtion requiring repositioning of the coil, s seen when using mechniclly detchble coils (Yoshimur et l. 1995). Initilly, only few series described the use of GDCs in DCSFs (Jnsen et l. 1999; Oishi et l. 1999; Mwd et l. 1996; Nkmur et l. 1998). Mwd et l. (1996) reported their results in eight ptients (87%), in whom complete occlusion ws ccomplished, while in one cse tretment filed due to intrcvernous septe. The dvntges of ll detchble coil systems re obvious in the trnsvenous tretment of DCSFs, where the criticl tsk is the precise plcement of the coils t the beginning of the procedure (Benndorf et l. 2000; Oishi et l. 1999). This cn be chieved by deploying coils tht re slightly lrger (2 3 mm) thn the trgeted venous comprtment (Fig. 8.4). For the SOV-CS junction, helicl coils with pproximtely 5 mm dimeter, or with sphericl configurtion, proved helpful in creting smll bsket with sufficient pposition to the venous wll. In order to chieve most effective blockge of the fistulous flow in the SOV erly during the procedure, this bsket should be immeditely filled with smll nd soft coils. The rtionle behind this strtegy hs lredy been pointed out by Hlbch et l. (1997). Using coils with complex shpe helps void overpcking the sinus, which my result in crnil nerve deficits. Further, in order to prevent venous infrction, selective occlusion is of importnce in cses where the norml venous dringe vi the Sylvin vein or the SPPS needs to be mintined ( Nkmur et l. 1998). Such trgeted comprt-

264 8.4 Discussion of Trnsvenous Occlusions 255 mentl emboliztion of DCSFs hs been suggested by Agid et l. (2004). The softness of tody s pltinum coils produced by vrious mnufcturers is dvntgeous becuse it llows denser pcking even in smll interstices nd trbeculr comprtments. To crete such dense coil mesh my be more difficult using fibered coils, especilly in smll fistuls or when the CS is dditionlly thrombosed. Despite its higher costs, the use of softer nd more plible coils minimizes the risk of creting smll pockets in the CS tht could complicte the procedure by rerouting the venous dringe nd cusing venous corticl hypertension (Oishi et l. 1999). Softer coils lso minimize undesirble ctheter buckling or kickbck nd gurntee more stble ccess throughout the procedure. Unstble position of the microctheter in the CS my led to loose coil pcking nd comprtmentliztion. Finlly, the use of softer coils lowers the overll risk of CN irrittions cused by loclly incresed mechnicl pressure (Roy nd Rymond 1997). Becuse the CS is embedded in rdiogrphiclly dense bony structures of the skull bse nd the middle crnil foss, good fluoroscopic visuliztion of pltinum coils is nother dvntge compred to stinless steel coils (Oishi et l. 1999). The sfety nd relibility of modern detchble systems is very high. Premture detchments re seldom, nd secondry coil migrtion usully does not occur (Benndorf et l. 2000; Oishi et l. 1999; Jhn et l. 1998; Nesbit nd Brnwell 1998; Mwd et l. 1996; Guglielmi et l. 1995; Bvinzski et l. 1997). The use of soft nd plible coils in combintion with improved brided microctheters reduces friction, so tht ctheter dmge or unrveling of coils occurs less often. An unrveled coil my be difficult to brek, necessitting ctheter exchnge tht cn be problemtic in cses with difficult ntomy (e.g. cross-over pproches). Chnging ctheters my even led to complete loss of ccess to the fistul site, especilly if some coils hve lredy been deployed. If ctheter dislodges (e.g. during n IPS pproch), coils my get deployed too erly into the posterior CS comprtment or within the IPS itself. This should be voided, in prticulr when the CS-SOV junction is not yet occluded nd the AV shunting is still open. Although the lrgest experience in coiling of DCSFs probbly exists with GDCs, ll of the forementioned dvntges re eqully true for other coil types with controlled detchment such s Micro- Plex coils (MicroVention, Terumo), TruFill TM DCS Orbit Detchble Coil (Cordis Neurovsculr, Mimi Lkes), Detch-18/-11; (Willim Cook Europe, Bjerverskov, Denmrk) nd others. Bre pltinum coils cn be combined with more thrombogenic coils (Nesbit nd Brnwell 1998; Jnsen et l. 1999), s well s with liquid embolic gents such s NBCA (Troffkin nd Given 2007; Wkhloo et l. 2005; Roy nd Rymond 1997) or Onyx (Suzuki et l. 2006). Such combintion of GDCs nd VortX coils hs been in severl erly cses iming to ccelerte thrombosis in lrger CS comprtments (Benndorf et l. 2000). Whether or not fibered coils ccelerted the fistuls occlusion remined difficult to ssess. One should lso keep in mind tht the comptibility between vrious coil types nd microctheters could be problemtic. Pushing fibered coils through smll microctheter my cuse friction to degree tht the subsequent use of bre pltinum coils cn be compromised. The dditionl injection of Onyx requires the use of DMSO comptible ctheters from the beginning of the procedure, while endovenous injection of crylic glue should be performed t the end of the coiling becuse it necessittes immedite ctheter removl. In the erly TVO experience, mechniclly detchble coils mde of tungsten (MDS, Blt Extrusion, Montmorency, Frnce) hve been used s well. Tungsten hs slightly higher thrombogenicity thn pltinum (Byrne et l. 1997); however, it is softer thn pltinum nd thus cnnot be s densely pcked. This nd the less relible detchment system mke these coils less suitble for trgeted emboliztions including DCSFs. In ddition, coil corrosion hs been reported (Weill et l. 1998) nd the mteril hs menwhile been replced by pltinum. Newer mechnicl systems such s the Detch-18 nd Detch-11 coils (Willim Cook, Europe) showed stisfctory performnce s demonstrted by Kiyosue et l. (2004b) in five ptients with DCSFs. In prticulrly, the J-shped coil of this system ppered useful for occluding the trbeculted CS. HydroCoils (Hydrogel) The cse shown in this monogrph (Cse Illustrtions V), demonstrtes the usefulness of this coil system when pplied to venous occlusions. Morsi et l. (2004) were first to report the use of Hydro- Coils in ddition to bre pltinum coils for trnsvenous occlusion of DCSF. Numerous pltinum coils (TruFill, Cordis) tightly pcked in the CS did not show noticeble effects on the AV shunting.

265 256 8 Endovsculr Tretment After plcement of four HydroCoils TM, significnt flow reduction ws evident. A control ngiogrm obtined 15 min lter confirmed complete occlusion of the fistul tht likely would hve tken longer using only bre pltinum coils. Longer procedure time cn be ssocited with higher rdition dose (Gb et l. 2006), which my be prticulrly importnt for lengthy TVO procedures. Klurfn et l. (2006) recently reported on series of 10 ptients with DAVFs, including five DCSFs, treted with combintion of bre pltinum nd HydroCoils, chieving complete ntomicl cure in ll. Angiogrphic follow-up ws performed using MRA in two ptients; two underwent DSA nd one ws lost to follow-up. In one (20%) ptient, persistent 6 th nerve plsy ws seen fter 10 months. Although dt re currently limited, it cn be ssumed tht HydroCoils will likely cuse less CN deficits thn bre pltinum coils. The totl mount of coils could be reduced, thereby decresing costs, procedure time nd rdition dose NBCA (Histocryl TM, Glubrn TM, Trufill TM ) The use of liquid dhesives is well estblished in the tretment of AV shunting lesions, minly for trnsrteril emboliztion of brin AVMs nd DAVFs (Liu et l. 2000; Henkes et l. 1998). The use of crylic glue for emboliztions of DCSFs hs been mostly limited to trnsrteril injections for occluding feeding ECA pedicles, IMA or the cvernous ICA (Liu et l. 2000b; Vinuel et l. 1984; Debrun et l. 1988; Hlbch et l. 1987). Isobutyl-2-cynocrylte (IBCA) nd lter N-butyl-2-cynocrylte (NBCA) re liquid dhesives, primrily used in erly TAE series (Vinuel et l. 1984; Picrd et l. 1987; Kupersmith etl. 1988). Limited experience nd less dvnced imging nd ctheter technology in the 1970s nd 1980s led to serious complictions cused by pssge of the glue through ECA/ICA collterl brnches in the durl cvernous network. A few reports describe the use of intrvenous injections of NBCA directly into the cvernous sinus (Wkhloo et l. 2005; Roy nd Rymond 1997). In 1979, Kerber et l. were the first to use crylic glue (IBCA) for the tretment of direct crotid rtery fistul with the help of clibrted lek blloon ctheter. The uthors observed temporry neurologicl disturbnces but no permnent crnil nerve deficit despite the intercvernous deposition round crnil nerves in ll three ptients. In 1988, Teng et l. (1988) mentioned the endovenous injection of IBCA to promote occlusion of direct CCF. The djunctive use of glue during TVO hs lso been described by Roy nd Rymond (1997), who injected NBCA into the coil bsket in two high-flow DCSFs without complictions, recommending the simultneous compression of the eye bulb to void glue migrtion into the SOV. In the group of ptients studied, only few (n=3) ptients were treted with either dditionl or sole injection of glue. Cse Illustrtions VII nd VIII show tht glue cn be injected either trnsrterilly or trnsvenously with very good results nd led to n immedite occlusion of the fistul due to the thrombogenic effect of NBCA. The high thrombogenicity of glue is the mjor contributing fctor in occlusions of AVFs, where coil occlusion lone is insufficient to induce thrombosis nd occlusion. In prticulr, when no occluding effect occurs despite the deployment of criticl coil mss in the CS, dding glue my be vluble option. When injected trnsrterilly, flow control my be chieved by nvigting microctheter into so-clled wedged position. Flow control is unlikely chieved in the CS, except when plcing n occlusion blloon on the rteril side (Gl et l. 2008). As venous collector, the cvernous sinus is ntomiclly nd hemodynmiclly open in vrious directions, depending on incoming nd outgoing flow ccording to the AV shunting. The ltter my chnge t ny time during the injection. The flux of liquid embolic gent is difficult to predict. In some cses with prtil occlusion by coils, smll mounts of dditionl glue my be enough to induce thrombosis nd chieve complete occlusion. A coil bsket formed initilly my help to void spillge of glue into the SOV or corticl veins (Wkhloo et l. 2005). Similr to other uthors (Roy nd Rymond 1997; Teng et l. 1995), CN irrittions hve not been observed in the studied mteril. Nevertheless, this potentil compliction my occur nd needs to be considered before injecting Histocryl directly into the CS. In my erly personl experience, one ptient developed persistent pin round his lower nose (mxillry division of 5 th crnil nerve) fter NBCA injection into the rtery of the formen rotundum supplying CSF. Roy nd Rymond (1997) injected NBCA into the coil bsket in two high-flow durl fistuls without

266 8.4 Discussion of Trnsvenous Occlusions 257 complictions nd recommend the simultneous compression of the eye bulb to void glue migrtion into the SOV. Shibni et l. (2007) demonstrted tht NBCA injection into smll comprtment of the CS my close the AV shunt when n intrcvernous stenosis does not provide ctheter positioning for proper coil deployment. Wkhloo et l. (2005) reported the lrgest experience so fr with 14 ptients; six treted with NBCA lone, seven with combintion of NBCA nd coils, nd one with combintion of trnsrteril PVA injection nd intrvenous NBCA. The uthors chieved complete cure in ll cses, observing two technicl complictions (14%) nd one trnsient 6 th nerve plsy (7%). There ws one indvertent glue migrtion into the MCA, nother into the SOV; both without clinicl sequele. Intrvenous glue injection should be performed with the gretest of cre to void uncontrolled migrtion into the norml venous circultion or retrogrdely vi ILT nd MHT into the crotid lumen, cusing stroke. The ltter hs been observed by Meyers et l. (2002) who injected glue under pressure exceeding pressure in the smll durl brnches. More recently, modified type of crylic glue (Glubrn TM, GEM, Vireggio Itly) hs become vilble tht consists of monomer NBCA nd monomer MS (owned by GEM). It hs lower therml polymeriztion temperture thn NBCA nd is thought to be better controllble (see cse Illustrtion VII). Preliminry experience in the tretment of brin AVM nd DAVFs hs been reported in one single study so fr (Rffi et l. 2007) Ethylene-Vinyl Alcohol Copolymer (Onyx TM ) The use of the liquid, non-dhesive embolic gent Onyx TM, (EV3, Irvine CA), mixed with dimethyl sulphoxide (DMSO) nd tntlum (Jhn et l. 2001), ws initilly trgeted for brin AVMs (Weber et l. 2007,b; Song et l. 2007; vn Rooij et l. 2007) nd hs only recently been extended to DAVFs (Suzuki et l. 2006; Toulgot et l. 2006; Cognrd et l. 2008; Rezende et l. 2006; Nogueir et l. 2008). Regrdless of its loction, DAVFs hve been considered the most difficult lesions to tret, especilly by trnsrteril pproch, becuse of their often elongted, tortuous ECA feeders, preventing distl ctheteriztion for effective glue injections. Trnsrteril emboliztion with NBCA hs been one min tretment modlity for treting crnil DAVFs, but its efficcy remins limited to cses with fvorble ntomy, in which either distl or wedged positions were obtinble, llowing for deposition of glue downstrem t the desired loction. The dependence on highly experienced opertors for effective nd sfe use of liquid embolic gents hs been widely overcome with the introduction of Onyx (Cognrd et l. 2008). After proper trining, the flux of Onyx is, in generl, esier to control, shortening the lerning curve for its optimized hndling. The ntomicl results currently obtinble with Onyx pper superior to wht cn be ccomplished with NBCA. Creting so-clled embolic plug ( reflux-hold-reinjection technique, reflux nd push technique ) mitigtes too much proximl reflux, while the embolic mteril penetrtes slowly ntegrde to the trgeted vsculr re. After csting the nidus itself, retrogrde ( trnsnidl ) filling of djcent feeding pedicles is possible, llowing for complete oblitertion, not only of the nidus but lso of complete rteril network surrounding the AV shunt. Thus, the recruitment of new feeding vessels tht form fter prtil or incomplete oblitertion is minimized nd will likely improve long-term results of emboliztions in DAVFs, including DCSFs. Similrly, direct endovenous ppliction of Onyx TM is esier to control thn tht of crylic glue, nd cn be used s n djuvnt technique to coils (Suzuki et l. 2006; Toulgot et l. 2006; Rezende et l. 2006; Nogueir et l. 2008; Wrkulle et l. 2003; He et l. 2008; Lv 2009). Toulgot et l. (2006) treted six ptients with DAVFs in vrious loctions with Onyx TM 18, ccomplishing complete occlusions in ll ptients with ctheteriztion of single feeder nd one injection. Nogueir et l. (2008) reported on 12 consecutive ptients with DAVFs embolized with Onyx TM 18 or combintion of Onyx TM 18 nd Onyx TM 34, chieving complete oblitertion in 11 ptients (91.7%) with totl of 17 procedures. The uthors observed no significnt morbidity or mortlity nd sw one recurrence tht required retretment. Cognrd et l. (2008) chieved complete closure in 80% of their ptients (n=30), observing clinicl complictions in two (6.6%). The combined use of coils nd Onyx for endovenous occlusion of DCSFs in three ptients hs been described by Suzuki et l. (2006). The uthors chieved complete occlusion nd considered the controlled nd excellent penetrtion of Onyx to be

267 258 8 Endovsculr Tretment superior for blocking the intricte communictions in these fistuls. Art et l. (2004) performed trnsvenous injection of Onyx for tretment of durl crotidcvernous fistul following n unsuccessful emboliztion using detchble coils nd liquid dhesive gents resulting in complete resolution of symptoms fter 3 month. The intrcvernous injection ws performed using totl of 0.6 ml of 8% ethyl-vinyllcohol-copolymer (Onyx 34) in single injection csting not only of the CS, but lso prt of the SOV (see Fig. 3 in Art et l. 2004). The ltter should probbly be voided, since it my cuse SOV thrombosis with possible loss of vision, the preservtion of which is the gol of EVT in DCSFs. A number of disdvntges nd limittions should be considered when using this mteril ( Suzuki et l. 2006; Jhn et l. 2001) including: It requires DMSO-comptible microctheters Angiotoxicity with ngionecrosis or vsospsm Microctheter retention due to entrpment Costs exceed tht of NBCA (in certin geogrphic regions, except US) Discomfort in ptients when treted without generl nesthesi Another, more generl issue relted to the use of Onyx TM is tht the reltive ese of control my led to n underestimtion of its potentil of untowrd penetrtion into venous comprtments or exits tht should not be occluded. In prticulr, within the CS, the degree of occlusion of only selected comprtment or ll connections to fferent nd efferent veins, nd overfilling of the SOV re difficult to predict. Cse Illustrtion IX depicts how elegntly Onyx cn be used in DCSF with only miniml rteril supply through the ipsilterl AMA nd MHT, when supported by indirect flow control. A blloon plced cross the MHT nd inflted during the Onyx injection into the AMA pedicles llows trgeted deposition of Onyx with the CS. This prevents distl migrtion of the embolic gent into the SOV or reflux into the ICA. Trnsrteril emboliztion of DAVFs my be ccompnied by clinicl compliction due to proximl reflux cusing CN deficits, or overinjection nd subsequent extensive venous thrombosis (Cognrd et l. 2008). Recent literture provides n incresing number of smller series reporting the successful use of Onyx for both trnsvenous nd trnsrteril emboliztion of DCSFs (Ong et l. 2009; Lv et l. 2008; Gndhi et l. 2009; Elhmmdy 2009; Bhti 2009; He 2008). Although the chieved high rtes of technicl success nd occlusion re promising, the use of Onyx TM in CSFs is not without potentil hzrds. He et l. (2008) treted six ptients using Onyx nd coils observing two trnsient 6 th nerve plsies fter TVO (33%) nd one trnsient fcil nerve plsy (17%) fter TAE. In nother series, complete occlusion ws chieved in 11 ptients undergoing TVO using combintion of coils nd Onyx (Lv et l. 2009). Two ptients (18%) developed brdycrdi during the DMSO injection; trnsient or permnent CN plsies were not observed. Bthi et l. (2009) reported five ptients undergoing TVO using Onyx-34 nd chieved complete occlusion with resolution of clinicl symptoms in ll. One ptient (20%) showed persistent 6 th crnil nerve plsy tht resolved fter 3 months. The risk of potentil reflux into cvernous ICA brnches is emphsized s mjor disdvntge. It cn be minimized to some degree by using higher viscosity (Onyx-34), nd pusing the injection whenever reflux occurs. Similrly, Elhmmdy et l. (2009) treted group of 12 CSFs, with 11 DCSFs, using Onyx for rteril nd endovenous injections. The uthors used combintion of coils nd Onyx in five nd Onyx only in three ptients for TVO, chieving complete occlusion in ll. In three other ptients, TAE ws performed resulting in complete oblitertion s well. The uthors observed two trnsient CN plsies (18%) nd one permnent fcil nerve plsy (9%) in the ptients undergoing trnsrteril emboliztions. This reltively high morbidity my be cused by ischemi in the rteril territory supplying the crnil nerves in the CS, by ggrvted CS thrombosis nd swelling, or is possibly relted to direct ngiotoxic effects of DMSO. These newer observtions my tone down bit the previling Onyx enthusism nd illustrte severl importnt spects: First, cution is wrrnted before injecting DMSO nd Onyx directly into the CS s it seems currently uncler to wht degree the crnil nerve function my be ffected. Second, the use of Onyx must be performed under the sme rules used for injecting NBCA. This includes voiding dngerous nstomoses nd respecting the norml rteril supply to the crnil nerves. Third, lrger series with long-term clinicl nd ngiogrphic FU re needed to vlidte efficcy nd procedurl morbidity of Onyx emboliztions (Wkhloo 2009).

268 8.4 Discussion of Trnsvenous Occlusions 259 Murugesn et l. (2008) recently reported severe dverse pulmonry rection following emboliztion of cerebrl AVM. The ptient developed cute respirtory distress syndrome with hypoxemi following extubtion, necessitting mechnicl ventiltory support for 44 h. This unusul compliction my be relted to the excretion of DMSO through the lungs. Incresed rdition exposure due to prolonged injection time [men injection time 45 min (Cognrd et l. 2008)] is nother controversil issue, but must be counterblnced ginst long fluoroscopy time, often necessry for more distl ctheter nvigtion nd repeted sessions in the use of NBCA (Nogueir et l. 2008). In DCSFs, procedure time nd rdition exposure my ctully be reduced compred to time-consuming coil pcking (Gndhi et l. 2009; Bhti et l. 2009). Ctheter entrpment my cuse clinicl complictions (Crlson et l. 2007), but will likely be solved by technologicl dvncements, such s the recent introduction of detchble tip for the Sonic ctheter from Blt (Thon et l. 2008). Overll, the experience with Onyx is still limited but encourging, s it llows for better nd more controlled penetrtion of the complex network of n AV shunt s compred to NBCA, nd cn be considered vluble djunct to the EVT rmmentrium for the mngement of DCSFs, either lone or in combintion with detchble coils. It should be mentioned here tht mong liquid embolic mterils, the use of lcohol hs lso been reported (Koebbe et l. 2003). In group of six ptients, intrrteril lcohol injection ws performed under temporry blloon infltion in the ICA. Technicl success ws chieved in ll cses, clinicl improvement in five. One ptient experienced worsening of her sixth nerve plsy due to CS thrombosis. Although known s highly effective for vsculr occlusion, this technique crries high risk of dmging the ICA endothelium s well s the crnil nerves trversing the CS, nd thus should be used only s lst resort Stents nd Covered Stents The use of stents in the tretment of CSFs hs been reported in limited number of cses (Moron et l. 2005; Archondkis et l. 2007; Felber etl. 2004; Gomez et l. 2007), minly in ssocition with direct CSFs. Moron et l. (2005) demonstrted tht preservtion of the prent rtery ws chieved using stent-ssisted coiling in five ptients. The ctul benefit in using stents lies in the possible reduction of the rteril inflow when deploying stents with low porosity, or idelly, covered stents. Angiogrphic follow-up in eight ptients with trumtic CCFs treted with covered stents (JoStent Coronry Stent Grft) demonstrted improved symptoms or complete regression in ll. In two, residul filling of the AV shunt ws found t the end of the procedure. Six ptients showed good ptency of the crotid lumen, while one presented with n symptomtic occlusion (Archnondkis et l. 2007). For effective use of covered stents, good wll pposition is crucil to fully sel the defect in the crotid wll of CCF, or the TMH/ILT origins in DCSF. If the stent is mlpposed, the AV shunt my sty open (Lv et l. 2008). Cre is necessry when further expnding the stent in CCF, since n injured rteril wll provides little resistnce. Thus, when stent grft is over-inflted, dditionl dmge could potentilly enlrge the defect in the wll (personl observtion in one cse). Lv et l. (2008) observed the development of complex AVF 9 months fter plcement of covered stent for occlusion of trumtic CCF. The use of covered stents in durl cvernous sinus shunts hs been described in one cse by Kim et l. (2006). Residul slow flow directed towrd pontomesencephlic veins occurred fter trnsvenous coil pcking, resulting in brin stem congestion ssocited with dysrthri. Bilterl plcement of stent grft resulted in complete occlusion of the shunt. Although neurologicl deteriortion fter TVO certinly justifies such n ggressive pproch, number of potentil disdvntges hve to be considered. First, no covered stent dedicted for intrcrnil circultion is currently vilble. The stiffness of the JoStent coronry grft is n inherent limittion tht my not llow esy nvigtion nd cn cuse spsm or even dissection (Archondkis et l. 2007). Second, s with ny stent plcement, dul ntipltelet therpy (spirin nd clopidogrel) 2 3 dys prior to the stent plcement nd 2 3 months post stenting, then spirin only for 12 months or even indefinitely, is recommended. The required lrge size of the guiding ctheter (7 or 8 F) in combintion with gressive nticogultion my cuse dditionl complictions t the puncture site. Third, the short- nd long-term ptency of these stents is unknown. The PTFE lyer my cuse cute inflmmtion nd ingrowth of fibrous connective tissue (Geremi et l. 1997; Link et l. 1996). On

269 260 8 Endovsculr Tretment the other hnd, evidence suggests tht PTFE might reduce the rte of intiml hyperplsi (Redekop et l. 2001; Gercken et l. 2002). Fourth nd not lest, plcing covered stent my be effective in CSFs with solely ICA supply (Type B fistuls, see Cse Illustrtion XI), while in Type C nd D fistuls subsequent recruitment of ECA feeders my develop nd the tretment remins ineffective. Longer follow-ups nd the development of softer stent grfts dedicted for neurovsculr use will show whether or not covered stents ply role in the future rmmentrium of endovsculr tretment for DCSFs. The VIABAHNN Endoprosthesis from Gore (Gore & Assocites, Inc., Arizon) hs been FDA pproved nd my represent n lterntive to the JoStent Antomic Results, Clinicl Outcome nd Complictions of Trnsvenous Occlusions nd Trnsrteril Emboliztions Due to the pucity of erly reports on trnsvenous tretment of DCSF, the vlidity of chieved results in ntomic nd clinicl cures s well s complictions rtes hs been limited. Tble 8.6 provides n overview of the results chieved by vrious groups nd shows tht more dt hve become vilble in recent yers. While most groups reported series of between ptients in the 1980s, the number of series studying more thn 20 ptients hs incresed. Thus, sttements bout efficcy nd procedure relesed complictions ssocited with TVO hve become more vlid. In 1989, the first relevnt series reporting trnsfemorl venous pproches ws published by Hlbch et l. (1989) (n=13). The uthors primrily used trnsfemorl pproch through the IPS, the SOV nd the bsilr plexus, chieving 90% ngiogrphic cure nd 77% clinicl cure. They observed two complictions; one ptient developed stroke fter plcement of blloon, while nother suffered from trnsient vision loss. Ymshit et l. (1993) chieved complete ngiogrphic cure in 14 of 16 (88%) cses, interpreting the filure in two cses with coils tht were not optimlly plced within the CS. This my hve been cused by intrcvernous trbecule, thrombosis or unfvorble ntomy of the SOV. There were 12% trnsient nd 6% permnent deficits. Goldberg et l. (1996) presented the first lrger series of SOV cnnultion nd chieved immedite improvement or clinicl cure in 100% of their ptients (n=10). They were unsuccessful in two dditionl ptients nd observed one cse of severe introrbitl bleeding. Quinones et l. (1997) chieved 92% occlusion rte in 12 successfully ctheterized ptients nd clinicl recovery in ll but two ptients (83%). The uthors observed two delyed complictions. One ptient developed plpebrl silk grnulom, nother trnsient contrlterl cvernous sinus syndrome (6 th nerve plsy). With the exception of two dditionl cses (Cse 11 nd 12), the forementioned represents the sme series s the one published by Goldberg et l. (1996) 1 yer erlier. It should be noted, however, tht the 82-yer-old femle who suffered from severe introrbitl hemorrhge fter ttempted trnsorbitl deep puncture ws not included in their results, nd hs not been mentioned in the second report of this group (Quinones et l. 1997). Identifible on the ngiogrphic imges [Figs. 6 nd 3, respectively, in Leibovitch et l. (2006)], this ptient ws recently presented by Leibovitch et l. (2006) s n 86-yer-old femle in more detil with n pprently more complicted post-crniotomy course suffering from permnent left hemiplegi nd vision loss. Such n unfortunte outcome fter TVO of DCSF is rther unusul nd will presumbly remin n exception, if less invsive routes nd mens re considered first. Roy nd Rymond (1997) reported on 24 ptients with DAVFs; 12 with DCSFs. Nine were treted using trnsvenous occlusions only; 89% demonstrted complete ntomic occlusion. In this series, trnsient CN deficits occurred reltively frequently (50%), nd in one ptient permnent sixth nerve plsy ws seen (8%). The uthors explined these complictions with locl thrombosis inside the CS tht led to CN irrittions, nd considered mechnicl pressure less likely the reson, since the symptoms were irreversible. Oishi et l. (1999) reported results nd complictions tht my occur when employing different venous pproches. They chieved complete ngiogrphic cure in 89% of cses with reltively high compliction rte of 32%. These complictions consisted of trnsient 6 th nerve plsies (n=3), dissections of the IPS (n=1), blephroptosis (n=2), s well s permnent dysesthesi of the forehed (n=1) due to upper lid incision for the SOV pproch. The uthors were ble to reduce this compliction rte with

270 8.4 Discussion of Trnsvenous Occlusions 261 gined experience nd more frequent use of the IPS pproch. Gobin et l. (2000) reported ngiogrphic cure in 24 of 26 ptients (92%) nd complete clinicl cure in 25 ptients (96%). The uthors observed two complictions (7%): one trnsient 6 th nerve plsy (4%) nd one cse of visul loss due to thrombosed centrl retinl vein (4%). Cheng et l. (2003) treted 27 ptients with TVO chieving complete ngiogrphic oblitertion 89% (30% immedite) nd clinicl cure in 96%. Two ptients presented with recurrent symptoms nd underwent second procedure. The uthors observed trnsient 6 th nerve plsy in three ptients (11%), which occurred with dely in two, suggesting progressive thrombosis nd inflmmtion inside the CS, s discussed by others (Roy nd Rymond 1997). Meyers et l. (2002) recently reiterted the UCSF experience in 135 ptients followed over period of 15 yers. The mjority (76%) of ptients undergoing EVT were treted by trnsvenous pproch, chieving ngiogrphic nd clinicl cure rtes of 90%. Eight ptients (6%) experienced symptomtic complictions, including infrction (n=1), visul deteriortion (n=2), dibetes insipidus (n=1) nd orbitl ecchymosis (n=1), but it remins uncler if these occurred during TVO or TAE. The overll procedurerelted permnent morbidity ws 2.3%; however, the uthors do not specify whether it ws relted to TVO or TAE. Angiogrphic follow-up ws obtined in 54%; one third of the ptients required more thn one intervention. More recently, Theudin et l. (2007) reported on 27 consecutive ptients undergoing trnsvenous occlusions (n=16) or trnsrteril emboliztions (n=4). Complete occlusion ws chieved in 14/16 ptients (88%) with erly-improved symptoms in 12 (75%). One ptient (6%) developed temporl lobe hemorrhge immeditely following trnsvenous occlusion in fistul with corticl venous dringe, possibly due to blockge of the fistul dringe by plcement of the guiding ctheter into the IPS. The ptient fully recovered without permnent deficit when seen t 1 yer follow-up. Yu et l. (2007) reported series of 61 ptients undergoing 64 successful TVO procedures nd chieved ntomicl cure in 95%. In 38 ptients the fistul ws occluded immeditely fter the procedure, in 20 mild residul fistul ws documented nd completely occluded in FU exms (3 16 months). Three ptients showed persistent symptoms nd underwent repet TVO, while 16 ptients showed cure within 2 weeks, 22 fter 3 months. There were two ptients with trnsient 6 th nerve plsy. Using ctheteriztion of the SOV either vi the fcil or the middle temporl veins the uthors were successful in 11/11 cses. It is interesting to note tht the uthors technicl success rte incresed from 71.6% to 86.5% fter dpting this trnsfcil SOV pproch. Furthermore, it is worth mentioning tht in 7/8 ptients with residul symptoms the IPS ws used s initil route. Kim et l. (2006) chieved n immedite 75% occlusion rte (complete or nerly complete) with cure or improvement of symptoms in 91% of ptients. A totl of 11 complictions (20%) were observed, including six crnil nerve plsies, three venous perfortions nd two ptients who developed brinstem congestion. Although this rte is reltively high, most of the dverse events were trnsient or cliniclly silent. These uthors discuss in detil potentil mechnisms nd possible mngement strtegies (Kim et l. 2006): First, trnsient CN plsies seen in six cses (10.7%) were likely due to overpcking of the cvernous sinus or extensive thrombosis within the CS. This ws lso observed by us in one cse fter coil pcking the left nd right CS with totl of 16 GDCs-10, nd two GDCs-18-VortX. The ptient presented with exophthlmos nd chemosis nd developed new diplopi due to 6 th nerve plsy the dy fter tretment. She ws heprinized for 3 dys nd treted with corticosteroids for 1 week. The ptient recovered completely within 8 weeks. A follow-up rteriogrm fter 6 months confirmed complete occlusion of the fistul. It cn be ssumed tht the reltively stiff VortX-18 coil my hve cused nerve compression. Second, venous perfortions during IPS ctheteriztions were seen in 5.4% (three cses), none of which resulted in clinicl sequele due to immedite recognition nd coil emboliztion. Extrvstion during IPS ctheteriztion occurred in one of our ptients; however, it remined cliniclly silent. Rupture of n IPS my not cuse serious clinicl complictions s most perfortions occur when ctheter is dvnced through thrombosed or occluded IPS with miniml or no AV shunt flow. Finlly, venous congestion in ssocition with DCSFs hs been reported by number of uthors (Iwski et l. 2006; Ki et l. 2004; Uchino et l. 1997; Teng et l. 1991). Kim et l. (2006) demonstrted rte in 3.6%, ttributed to rerouted venous dringe fter coil pcking within the CS.

271 262 8 Endovsculr Tretment Such rerouting of venous dringe towrds the posterior foss following TVO of DCSFs hs not been observed in the mteril studied. In ll cses with preexisting corticl or leptomeningel dringe, it ws possible to disconnect the communiction between the CS nd the efferent venous dringe t the beginning of the procedure. However, venous rerouting fter TVO of direct high-flow CCF using coils without chieving complete occlusion hs occurred nd resulted in n intrcrnil hemorrhge severl hours fter the procedure. In summry, the results chieved by the vrious groups (Tbles 8.6 nd 8.7) show ntomic cure rtes rnging from 52% 100%, depending on whether or not immedite complete or ner complete (subtotl) oblitertion of the AV shunt is considered the endpoint. Similrly, the rte of clinicl cure rnges from 63% 100%, with the mjority of groups chieving more thn 80% 90%. This is higher thn wht ws ccomplished in the erly er of EVT nd lies bove results obtinble using trnsrteril emboliztions ( Goldberg et l. 1996; Hlbch et l. 1987; Sonier et l. 1995), thus reflecting the experience gined Tble 8.6. Antomic nd clinicl results of TVO in DCSFs reported in the literture (600 ptients) Author N Approch Result DSA (complete cure) Prtil None Result (clinicl cure) Improved Teng et l. (1988) 5 SOV 4 (80%) 1-5 (100%) - - Hlbch et l. (1989) 13 IPS 9 d (90%) 1-10 e (76%) 3 - Ymshit et l. (1993) 16 IPS 14 (88%) (88%) 1 1 Miller et l. (1995) 10 SOV 9 (90%) - 1 r 10 (100%) - - Roy nd Rymond (1997) 9 IPS; - 8 (89%) (89%) 1 - Quinones et l. (1997) 12 SOV 11 s (92%) 1-10 (83%) - 2 Oishi et l. (1999) 19 IPS, SOV 17 (89%) 2-17 (89%) 2 - Gobin et l. (2000) 26 IPS, SOV, PP 24 (92%) 2-25 (96%) 2 - Annesley-Willims et l. (2001) 11 IPS, SOV 7 f (64%) 5 g - 8 (73%) 5 j 3 - Meyers et l. (2002) 101 t IPS, SOV 121/135 (90%) o % - 4% Biondi et l. (2003) 7 FV, SOV 6 (100) q (67%) 2 - Cheng et l. (2003) 27 IPS, SOV, CV 24 (89%) (96%) - - Klisch et l. (2003) 11 IPS, SOV, FV - h (72%) 3 - Benndorf et l. (2004) 45 IPS, SOV, SPS, SV, FrV 42 (93%) (91%) - - [100%] i Wkhloo et l. (2005) 14 IPS, SOV 12 (86%) 2 (100%) m - 14 (100%) - - Kim et l. (2006) 56 IPS, FV, SOV 29 (52%) 13 (75%) n (46) k (91%) - - Kirsch et l. (2006) 141 u IPS, SOV 114 (81%) u 18 (13%) 6 (4%) 94% l - 5% Yu et l. (2007) 61 b IPS, SOV, FV, MTV 58 (95%) 20 (33%) 3 (5%) 58 x p 16 w Theudin et l c IPS, SPS, SOV 14 (88%) (63%) 6 IPS = inferior petrosl sinus, SPS = superior petrosl sinus, SOV = superior ophthlmic vein, PP = pterygoid plexus, FrV = frontl vein, FV = fcil vein, CV = clivl venous plexus no ngiogrphic result or FU reported successfully ctheterized out of 13, b technicl success out of 71, c ptients undergoing TVO out of 27, d out of 10 seen for ngiogrphic FU nd occluded, e out of ll 13 ptients, f one bilterl fistul, g not specified if immedite or long term occlusion, h group mixed with direct CCFs, TCCD or MRPA for FU, i ll ptients seen for ngiogrphic FU showed complete occlusion, j immeditely improved, k FU vilble, l 55% of the ptients seen for long term FU, m fter 6mos FU, n complete nd nerly complete occlusion together (n=42), o not specified for TVO or TAE, ngiogrphic FU obtined in 54%, p immeditely occluded, q out of 6 ptients with technicl success, r required TAE for complete occlusion, s 3 ptients seen for ngiogrphic FU, t undergoing TVO out of 135, u TVO filed in 3, TAE performed in 32 ptients, v FU in 4 ptients pending, w =cured in 2 weeks, x cured fter 3 months None

272 8.4 Discussion of Trnsvenous Occlusions 263 Tble 8.7. Reported procedure-relted complictions during TVO in the literture Author N Approch Complictions Rte Hlbch et l. (1989) 13 IPS S (1), VL (1) 15% [8%] Ymshit et l. (1993) 16 IPS VI (2), III (1), W (3), VP (1) 44% [0] Miller et l SOV VI (1) 10% [10%] Roy nd Rymond (1997) 9 i IPS ;- VI (1) VI (5) 66% [11%] Quinones et l. (1997) 12 SOV VI (1) 8% [0] Oishi et l. (1999) 19 IPS,SOV VI (3), D (1), BP (2), VP (1) e 32% [16%] Gobin et l. (2000) 26 IPS,SOV,PP VI (1), TCRV (1) 8% [4%] Annesley-Willims et l IPS, SOV W (2) 18% [0] Meyers et l. (2002) 101 IPS S (1) VL (2) 5% [1%] d OE (1) VP (1) Cheng et l. (2003) 27 IPS, SOV,CV VI (3) 15% [0] MND (1) Klisch et l. (2003) 11 IPS, SOV,FV S (1) SAH (1) 9% [0] Benndorf et l. (2004) 45 IPS,SOV, SPS,SV,FrV VI (1) W (2) VP (1) b 9% [0] 14% [0] Wkhloo et l. (2005) 14 IPS,SOV, VP (1) VI (1) f Kim et l. (2006) 56 IPS,FV,SOV CN (5) 20% [2%] VI (1) c VP (3) b BC (2) Kirsch et l. (2006) 141 IPS, SOV W (4) VP (5) b AE (5) RH (2) PE (2) g 13% [0] Leibovitch et l. (2007) 25 SOV IOB (2) j S (1) h, VL (1) h 12% [4%] Yu et l. (2007) 61 SOV, FV VI (2) 3% [0] Theudin et l. (2007) 16 IPS, SPS, SOV ICH (1) 6% [0] IPS = inferior petrosl sinus, SPS = superior petrosl sinus, SOV = superior ophthlmic vein, PP = pterygoid plexus, FrV = frontl vein, FV = fcil vein, CV = clivl venous plexus S = stroke, VI = permnent 6th CN deficit (VI = trnsient), B = bleeding, BP = blephroptosis, D = dysesthesi, TCRV = thrombosis of the centrl retinl vein, W/W = permnent/trnsient ophthlmologicl worsening, CN = crnil nerve plsy (CN = trnsient), VP = venous perfortion, BC = brinstem congestion, ICH = intrcrnil hemorrhge, IOB = introrbitl bleeding, OE = orbitl ecchymosis, VL = vision loss, AE = 5 rteril emboli detected by MRI, RH = retinl hemorrhge, MND = minor neurologicl deficit, PE = pulmonry embolism, TIA = trnsient ischemic ttck [ ]= rte of procedure relted permnent morbidity, [?] = not reported Retroperitonel hemtoms, femorl rtery pseudoneurysms or deep venous thrombosis re not considered sme ptient, b cliniclly silent, c mild residue fter 42 months FU in one of six ptients, d not specified if cused by TVO or TAE, e dissected dur cused trnsient 6th CN plsy, f 2 ptients with NBCA migrtion into SOV nd ICA without sequele, g cusing non-life thretening dyspnoe, h sme ptient s reported by Goldberg et l., i undergoing TVO only out of 12 with DCSF, j unremrkble recovery in one cse; stroke nd vision loss in nother

273 264 8 Endovsculr Tretment nd the skill level in utilizing trnsvenous occlusion techniques. When exmining ntomic (ngiogrphic) results, one should consider the following confounding spects. First, criteri used for determining ngiogrphic endpoints my vry. Second, in some series, TAE is used prior to or in combintion with TVO, skewing results (Cheng et l. 2003; Oishi et l. 1999; Wkhloo et l. 2005; Kim et l. 2006; Goldberg et l. 1996; Quinones et l. 1997; Kirsch et l. 2006). Third, in severl series, complete occlusion ws chieved fter multiple multi-chnnel pproches were used, either in the sme or sequentil sessions (Benndorf et l. 2004; Klisch et l. 2003; Cheng et l. 1999; Yu et l. 2009). This ws emphsized by Klisch et l. (2003) who studied 31 DAVFs including 11 DCSFs, whereby some were mnged by either severl trnsvenous occlusion steps or by combintion of EVT with surgicl techniques. The compliction rte of TVO, s documented by some groups, ppers reltively high (31%) ( Oishi et l. 1999). However, one should note tht serious complictions, such s stroke or introrbitl/ intrcrnil hemorrhge, seldom occur (Hlbch et l. 1988; Theudin et l. 2007). Furthermore, mny complictions hve been reported s necdotl cses (Tble 8.8) versus lrger series, underlining the fct tht with incresing experience nd skills, their rte cn be reduced considerbly. This becomes cler when looking t overll trnsient nd permnent compliction rtes reported in relevnt studies (Tble 8.9), reveling tht the overll rte of trnsient nd permnent complictions is in fct very low (11.6% nd 1.8%, respectively). The perfortion of the IPS, to dte the most frequent compliction (2.1%), often remins cliniclly silent nd cn be effectively mnged with endovsculr techniques (Benndorf et l. 2004; Kim et l. 2006). The mjority of CN deficits consist of 6 th nerve plsies (overll 4.1%), most of which re trnsient. The bducens nerve my be more exposed to mechnicl pressure cused by overpcking of the CS s the only true intrcvernous nerve. Another explntion of this predilection could be its embedment into the wll of the IPS tht is mostly used s venous pproch. The reltively low rte of CN deficits in our series (2%) could be relted to the more frequent use of soft coils (GDC Soft coils) tht my crete less mechnicl pressure to neuronl structures. CN deficits due to overpcking my likely further decrese with incresing use of softer pltinum coils (or HydroCoils TM ) nd liquid embolic gents. A permnent CN is rrely seen (0.5%) nd hs not been observed in our group, despite using ggressive techniques such s deep puncture of the SOV, crniotomy nd puncture of the Sylvin vein. Permnent neurologicl deficits due to stroke during or fter TVO occurred in 0.5% of ptients nd re below the rte reported by TAE series in 75 ptients (5.3%) ( Vinuel et l. 1984; Hlbch et l. 1987; Picrd et l. 1987; Kupersmith et l. 1988). The reported compliction rtes lso vry due to the fct tht some investigtors include femorl or retroperitonel hemtoms, femorl vein thromboses nd pulmonry embolisms (Meyers et l. 2002; Klisch et l. 2003; Kirsch et l. 2006). Furthermore, one of the lrgest series to dte reporting on 101/135 treted ptients using TVO does not spec- Tble 8.8. Reported necdotl complictions during TVO of DCSFs Author Tress et l. (1983) Hlbch et l. (1991) Hlbch et l. (1991b) Golnik et l. (1991) Fukmi et l. (1996) Arki et l. (1997) Devoto et l. (1997) Gupt et l. (1997) Giouleks et l. (1997) Aihr et l. (1999) Wldis et l. (2007) Hyshi et l. (2008) Event Introrbitl hemorrhge Dibetes insipidus fter CS ctheteriztion (n=1) b Cerebellr hemorrhge fter IPS (n=1) b Angle-closure glucom fter TVO Centrl retinl vein occlusion fter TAE/TVO Intrcerebrl extrvstion during TVO Acute exophthlmos during TVO Severe vision loss nd neovsculr glucom Introrbitl bleeding during SOV cnnultion Deteriortion of oculr motor dysfunction fter TVO Introrbitl hemorrhge nd vision loss during TVO Introrbitl bleeding during SOV cnnultion (n=2) Sme cse s Tress et l. (1983) b Both cses reported in lrge series of EVT (1200 cses) with totl of15 rteril nd venous perfortions

274 8.4 Discussion of Trnsvenous Occlusions 265 Tble 8.9. Overll compliction rte in lrger TVO series (from Tble 8.7, totl of 613 ptients) Complictions Trnsient Permnent Crnil nerve deficit 25 (4.1%) 3 (0.5%) Venous perfortion (IPS) 13 (2.1%) - Stroke - 3 (0.5%) Blephroptosis - 2 (0.3%) Worsening of ophthlmic 11 (1.8%) - symptoms Visul loss 4 (0.7%) 1 (0.2%) Introrbitl bleeding 2 (0.3%) - Subrchnoid hemorrhge 1 (0.2%) - Intrcrnil hemorrhge 1 (0.2%) - Trnsient ischemic ttck 1 (0.2%) - Orbitl ecchymosis 1 (0.2%) - Brinstem congestion 2 (0.2%) - Thrombosis of the - 1 (0.2%) centrl retinl vein CRV Minor neurologicl deficit 1 (0.2%) - Pulmonry embolism 2 (0.3%) - Retinl hemorrhge 2 (0.3%) - Arteril emboli 5 (0.8%) - Dysesthesi - 1 (0.2%) TOTAL 71 (11.6%) 11 (2.0%) Untreted ptient ify whether the observed permnent neurologicl deficits were relted to TVO or TEA (Meyers et l. 2002). The discussion of trnsvenous emboliztions must consider ngiogrphic nd clinicl results of TAE, representing the trditionl wy of treting rteriovenous shunting lesions (Vinuel et l. 1984; Picrd et l. 1987; Fermnd 1982; Brrow et l. 1985). One of the first series ws reported by Vinuel et l. (1984), describing 10 ptients with Type D fistuls, of which seven were occluded by embolizing the ECA feeders using PVA prticles or IBCA. Cure ws documented in five ptients (50%) fter 5 months. One ptient developed hemiplegi nd phsi due to reflux of IBCA through the FRA into the ICA nd MCA. Another experienced cute deteriortion of his vision. Brrow et l. (1985) chieved good results in three of five ptients (60%) treted by embolizing the ECA supply. Grossmn et l. (1985) reported complete resolution of symptoms fter prticulte emboliztion in five of seven ptients. Picrd et l. (1987) communicted results in group of 32 ptients; 25 (78%) underwent superselective emboliztion, chieving complete clinicl nd ntomicl cure in 18 (72%) nd demonstrted clinicl cure without ntomic cure in six (24%). One ptient (4%) suffered from stroke due to IBCA migrtion into the cerebrl circultion nd died fter 3 months. Hlbch et l. (1987) chieved clinicl cure in 77% nd n improvement in 18% (n=22) ptients treted between 1978 nd 1986 by TAE of ECA brnches using IBCA. One permnent deficit (4.5%) ws seen in ptient who developed stroke due to clot formtion in the guiding ctheter, s well s trnsient deficits in three cses (13%). The group lter reported (Hlbch et l. 1992) complete cure rte of 78%, improvement in 20%, nd compliction rte of 4%. In the erlier series published by Debrun et l. (1988), who treted 25 ptients with either PVA or Histocryl, complete occlusion ws reported in 48%. In two cses (8%), enlrgement of ECA feeders occurred; dditionl TVO hd to be performed in two others (8%), nd one ptient (4%) required surgicl exposure of the SOV. Sonier et l. (1995) reported 61% success rte by prticulte emboliztion of IMA brnches. In two cses (25%), TAE hd to be repeted to chieve complete occlusion, nd trnsient fcil edem ws observed in nother. Kupersmith et l. (1988) reported the successful emboliztion of ECA brnches in 88% of their ptients using PVA nd IBCA with two recnliztions (12%) nd four complictions (25%), including hemipresis nd heminopi, permnent 12 th nerve plsy nd persisting visul field defect. Vinuel et l. (1997) reiterted their experience bsed on 74 ptients, reporting complete cure in 31% nd positive clinicl response in 85%. The morbidity ws 3.2%; in two ptients untowrd glue migrtion into the intrcrnil circultion occurred, leding to hemipresis nd phsi in one. Liu et l. (2001) communicted the only lrger series (n=55) in ptients who were followed prospectively. In subgroup of 41 ptients (75%), TAE ws performed using PVA ( μm) nd Histocryl, injected into the distl ECA brnches. The involved ICA brnches were not pproched. A 70.9% complete clinicl cure rte ws chieved, with improved symptoms in 14.5%. The uthors do not specify whether these results were ny different in the embolized (41 ptients), or the non-embolized group (14 ptients). Further, in 24 of these ptients (58.5%), trnsient worsening ws observed tht might be

275 266 8 Endovsculr Tretment considered trnsient dverse event, or even compliction (Benndorf et l. 2004; Meyers et l. 2002). In four ptients (9.7%) there ws no improvement, while four demonstrted ggrvtion of symptoms (9.7%). It is not reported whether procedure relted trnsient or permnent neurologicl deficits were seen. The uthors suggest the use of TAE to convert Type D into Type B fistuls nd to shorten the time to complete cure with conservtive mngement. This tretment strtegy ppers questionble, however, since no evidence exists to dte demonstrting tht chnging Type D into Type B fistul improves the overll prognosis of DCSF. Furthermore, it is importnt to note tht in some TVO series, ptients initilly underwent inefficient TAE. Goldberg et l. (1996) performed PVA emboliztion of the ECA feeders prior to the SOV pproch. Although the ptients benefited cliniclly from the tretment due to reduction of the AV shunt, or even trnsient occlusion of the fistul, ll demonstrted recnliztion t ngiogrphic followup nd underwent subsequent TVO. Theudin et l. (2007) recently reported success rte of only 25% (1/5 ptients) in ptients undergoing TAE with μm, followed by μm until flow in the internl mxillry rtery stopped. It is emphsized tht none of these ptients becme completely symptomtic, lthough clinicl improvement ws seen. A reltively lrge prticle size (>300 μm) is recommended by some opertors for ECA emboliztions to void crnil nerve dmge ( Hlbch et l. 1992), while others suggest μm ( Vinuel et l. 1997). Lrger prticles will more likely produce n occlusion proximl to the fistul site, triggering recruitment of collterls nd recnliztion. It must be borne in mind tht the durl brnches feeding DCSF my lso be involved in the norml blood supply of CN (4 th, 5 th, 6 th, 7 th, 9 th, 10 th, 11 th, 12 th ) (Hlbch et l. 1992). As eluded to in Chp. 2, detiled knowledge of rteril ntomy in the CS re, ECA-ICA collterls nd ntomic vrints is essentil for performing TAE effectively nd sfely. Gregoire et l. (2002) recently published the interesting cse of 60-yer-old femle presenting with diplopi nd git disturbnce 3 dys fter rteril emboliztion due to cerebellr dysfunction cused by reversible pontine venous congestion. It demonstrtes tht the thrombotic process within the CS, triggered by prticle emboliztion, my lso be unpredictble. Prdoxicl worsening cused by SOV thrombosis my occur following TAE ( Sergott et l. 1987), nd hs been documented in significnt number (61.5 %) of ptients (Liu et l. 2001). Aside from pssge of PVA prticles or liquid embolic gents into the brin circultion vi ECA-ICA nstomoses, mjor risk ssocited with trnsrteril injections of embolic gents is reflux of the embolic gent from cvernous ICA brnches. Due to their size (norml dimeter pproximtely 0.3 mm), they cn be difficult or impossible to ctheterize, even when enlrged nd supplying n AV shunt. Even when possible, due to tortuousities, only their most proximl segments re usully ccessible, mking sfe nd effective injection of prticles or liquid dhesives difficult to control (Gobin et l. 2000; Picrd et l. 1987; Phtouros et l. 1999; Hlbch et l. 1989b). The TMH give rise to the inferior hypophysil rtery, thus injection of embolic gents my cuse mlfunction of the pituitry glnd resulting in dibetes insipidus, s ws observed in one cse fter injection of 50% dextrose nd pure lcohol mixture (Phtouros et l. 1999). The occlusion of durl ICA brnches using GDCs hs been suggested (Vinuel et l. 1997) s sfer lterntive to prticles or liquid dhesives nd my be effective in selected cses, but my be followed by recnliztion. Although liquid dhesives hve been lrgely replced by PVA prticles nd microspheres for TAE, their use cn be fesible option under certin conditions. Liu et l. (2000b) injected 10% 15% mixtures of NBCA with lipiodol nd tungsten into ECA brnches, chieving complete resolution of symptoms fter 1 month without definite neurologicl compliction. If ntomy of the rteril supply is fvorble, complete cure my be chieved by injecting even smll mounts vi n enlrged ILT or MHT. This technique, however, is not without risk nd requires n experienced opertor (Illustrtive Cse VII). Another elegnt lternte solution for difficult-to-tret cses is demonstrted in Illustrtive Cse IX, where n occlusion blloon, inflted cross the ipsilterl TMH supply, llows for controlled injection of Onyx vi single pedicle from AMA (indirect flow control), resulting in complete oblitertion of the fistul (Gl et l. 2008). Gndhi et l. (2009) recently communicted cse of Type D fistul tht ws successfully mnged by TAE vi the distl IMA using Onyx-18 in single injection. The ptient showed significnt immedite improvement, but developed novel 6 th nerve plsy tht resolved over 12 weeks. The figures of this cse show n extensive Onyx cst not only

276 8.5 Conclusion 267 in the CS itself, but lso in the proximl supplying pedicles of the IMA. Such proximl occlusion my led to indvertent migrtion of embolic mteril into dngerous ECA-ICA nstomoses or smll rteries supplying the CN nerves in the CS. Such mechnism is most likely responsible for the permnent fcil nerve prlysis seen fter TAE in the series of Elhmmdy et l. (2009) Bsed on the literture, the occlusion rte of TAE rnging from 31% 88% lies below the results obtinble tody with trnsvenous occlusion techniques. Recnliztion hs been observed in 25% 100% of the cses, while pproximtely two thirds of the ptients my experience trnsient worsening of the symptoms. Although improvement of symptoms cn be chieved with shunt reduction of bout 50% 85% (Vinuel et l. 1997; Grossmn et l. 1985), the potentil for recnliztion (Goldberg et l. 1993; Lsjunis nd Berenstein 1987), nd migrtion of embolic mteril cusing presis, phsi, CN deficits, intrcrnil hemorrhge ( Vinuel et l. 1984; Hlbch et l. 1987), worsening of ophthlmologicl symptoms (Golnik et l. 1991) or thrombosis of corticl veins (Cognrd et l. 1999; Tomsick 1997b), nevertheless remin significnt deterrents. Modern endovsculr tools nd dvnced ngiogrphic visuliztion in the 1990s hve improved results nd outcomes of TAE (Vinuel et l. 1997; Liu et l. 2000b; Mcho et l. 1996; Lnzs et l. 1996; Robinson et l. 1999); however, even in experienced hnds, the forementioned residul risks must be tken into ccount. Thus, the role of TAE in the mngement of DCSFs hs chnged s it hs become more n djunctive tretment option, employed in cses where TVO cnnot be utilized ( Vinuel et l. 1997), or prior to rdiosurgery. Mjor dvnces in of ngiogrphic imging technology with three-dimensionl nd cross-sectionl imging cpbilities contribute to better understnding of complex ngiorchitecture of the CS nd its fferent nd efferent drining veins. Due to its ntomicl topogrphy, short length, stright course nd ttchment to the dur long the petroclivl fissure, the IPS pproch represents the most preferred ccess route to the CS, followed by the trnsfcil pproch through the SOV. Improved ctheter nd guidewire technology enbles explortion of virtully every possible venous ccess route to the CS, incresing the technicl success rte. High-resolution bi-plne fluoroscopy nd rod mpping fcilitte direct percutneous puncture of drining veins such s the SOV, the IOV, the frontl vein or other tributries providing dditionl ccess. Improved nd novel embolic gents such s softer detchble pltinum coils or HydroCoils further increse efficcy nd sfety of trnsvenous occlusions. Whether newer liquid embolic gents such s Onyx will further enhnce the efficcy nd sfety of TVO nd possibly replce mechnicl coil pcking needs to be shown in future studies. In greement with most centers, it cn be concluded tht there is currently little reson to devote significnt time nd effort to the techniclly chllenging ctheteriztions of durl cvernous ICA brnches. More invsive techniques such s direct puncture of introrbitl veins, the cvernous sinus, or open surgery will ply decresing role in the therpeutic mngement of DCSFs. They should be reserved for combined surgicl-endovsculr pproches in truly intrctble cses. 8.5 Conclusion In conclusion, results obtined tody with TVO of DCSFs demonstrte tht overll rtes of ntomicl nd clinicl cure hve improved considerbly over the lst 15 yers. At the sme time, complictions nd morbidity ssocited with TVO hs become very low, prticulrly s seen in lrger series nd when tretment is performed by experienced opertors with pproprite trining nd skills in ctheteriztion techniques. References Agid R, Willinsky RA, Hw C, Souz MP, Vnek IJ, ter- Brugge KG (2004) Trgeted comprtmentl emboliztion of cvernous sinus durl rteriovenous fistule using trnsfemorl medil nd lterl fcil vein pproches. Neurordiology 46: Ahn JY, Lee BH, Joo JY (2003) Stent-ssisted Guglielmi detchble coils embolistion for the tretment of trumtic crotid cvernous fistul. J Clin Neurosci 10:96 98 Aihr N, Mse M, Ymd K, et l. (1999) Deteriortion of oculr motor dysfunction fter trnsvenous emboliztion of durl rteriovenous fistul involving the cvernous sinus. Act Neurochir (Wien) 141: ; discussion

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282 References 273 Mounyer C, Hmmmi N, Piotin M, et l. (2007) Nidl emboliztion of brin rteriovenous mlformtions using Onyx in 94 ptients. AJNR Am J Neurordiol 28: Mulln S (1974) Experiences with surgicl thrombosis of intrcrnil berry neurysms nd crotid cvernous fistuls. J Neurosurg 41: Mulln S (1979) Tretment of crotid-cvernous fistuls by cvernous sinus occlusion. J Neurosurg 50: Murugesn C, Srvnn S, Rjkumr J, Prsd J, Bnkl S, Murlidhr K (2008) Severe pulmonry oedem following therpeutic emboliztion with Onyx for cerebrl rteriovenous mlformtion. Neurordiology 50: Nesens R, Mestdgh C, Breemersch M, Defreyne L (2006) Direct crotid-cvernous fistul: cse report nd review of the literture. Bull Soc Belge Ophtlmol (299):43 54 Nito I, Mgrisw S, Wd H (2002) Fcil vein pproch pproch by direct puncture t the bse of the mndible for durl crotid cvernous fistul. 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283 274 8 Endovsculr Tretment Scott JA, De Nrdo AJ, Horner T, Liepzig T, Pyner T (1997) Fcil venous ccess to the cvernous region AV-fistuls: sfe nd relible technique. In: ASITN/WFITN Scientific Conference; Sept.13-16; New York, p 99 Serbinenko FA (1974) Blloon ctheteriztion nd occlusion of mjor cerebrl blood vessels. J Neurosurg 41: Sergott RC, Grossmn RI, Svino PJ, Bosley TM, Schtz NJ (1987) The syndrome of prdoxicl worsening of durlcvernous sinus rteriovenous mlformtions. Ophthlmology 94: Seyer H, Honegger J, Schott W, et l. (1994) Rymond s syndrome following petrosl sinus smpling. Act Neurochir (Wien) 131: Shibni A, Rohny M, Prkinson R, et l. (2007) Primry tretment of n indirect crotid cvernous fistul by injection of N-butyl cynocrylte in the durl wll of the cvernous sinus. Surg Neurol 67: ; discussion 408 Shiu PC, Hnfee WN, Wilson GH, Rnd RW (1968) Cvernous sinus venogrphy. 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(2006) [Trnsrteril embolistion of intrcrnil durl rteriovenous mlformtions with ethylene vinyl lcohol copolymer (Onyx18)]. J Neurordiol 33: Tress BM, Thomson KR, Klug GL, Mee RR, Crwford B (1983) Mngement of crotid-cvernous fistuls by surgery combined with interventionl rdiology. Report of two cses. J Neurosurg 59: Troffkin NA, Given CA, 2 nd. (2007) Combined trnsrteril N-butyl cynocrylte nd coil emboliztion of direct crotid-cvernous fistuls. Report of two cses. J Neurosurg 106: Uchino A, Kto A, Kurod Y, Shimokw S, Kudo S (1997) Pontine venous congestion cused by durl crotid-cvernous fistul: report of two cses. Eur Rdiol 7: Uflcker R, Lim S, Ribs GC, Piske RL (1986) Crotid-cvernous fistuls: emboliztion through the superior ophthlmic vein pproch. Rdiology 159: vn Rooij WJ, Sluzewski M, Beute GN (2007) Brin AVM emboliztion with Onyx. AJNR Am J Neurordiol 28: ; discussion 178

284 References 275 Velpeu A (1931) Memoire sur l piqure ou làcupuncture des rteres dns le tritment desnevrisme. Gz Med Pris 2:1 4 Venturi C, Brcco S, Cerse A, et l. (2003) Endovsculr tretment of cvernous sinus durl rteriovenous fistul by trnsvenous embolistion through the superior ophthlmic vein vi cnnultion of frontl vein. Neurordiology 45: Vinuel F, Fox AJ, Debrun GM, Peerless SJ, Drke CG (1984) Spontneous crotid-cvernous fistuls: clinicl, rdiologicl, nd therpeutic considertions. Experience with 20 cses. J Neurosurg 60: Vinuel F, Duckwiler G, Guglielmi G (1997) CCF: types B, C, nd D rteril embolistion. In: Tomsick TA (ed) Crotid cvernous fistul. Digitl Eductionl Publishing, Cincinnti, pp Wkhloo AK, Perlow A, Linfnte I, et l. (2005) Trnsvenous n-butyl-cynocrylte infusion for complex durl crotid cvernous fistuls: technicl considertions nd clinicl outcome. AJNR Am J Neurordiol 26: Wkhloo AK (2009) Endovsculr tretment of durl crotid cvernous sinus fistuls. J Neuroophthlmol 29:1 2 Wrkulle DR, Aviv RI, Niemnn D, Molyneux AJ, Byrne JV, Teddy P (2003) Embolistion of spinl durl rteriovenous fistule with Onyx. Neurordiology 45: Weber W, Henkes H, Berg-Dmmer E, Esser J, Kuhne D (2001) Cure of direct crotid cvernous fistul by endovsculr stent deployment. Cerebrovsc Dis 12: Weber W, Kis B, Siekmnn R, Jns P, Lumer R, Kuhne D (2007) Preopertive emboliztion of intrcrnil rteriovenous mlformtions with Onyx. Neurosurgery 61: ; discussion Weber W, Kis B, Siekmnn R, Kuehne D (2007b) Endovsculr tretment of intrcrnil rteriovenous mlformtions with onyx: technicl spects. AJNR Am J Neurordiol 28: Weill A, Ducos V, Cognrd C, Piotin M, et l. (1998) Corrosion of Tungsten spirls: disturbing finding. Intervent Neurordiol 4: White JB, Lyton KF, Evns AJ, et l. (2007) Trnsorbitl puncture for the tretment of cvernous sinus durl rteriovenous fistuls. AJNR Am J Neurordiol 28: Winslow JB (1734) Exposition Antomique de l Structure du Corpus Humin. London Wldis EJ, Peebles TR, Weinberg DA (2007) Mngement of cute orbitl hemorrhge with obstruction of the ophthlmic rtery during ttempted coil emboliztion of durl rteriovenous fistul of the cvernous sinus. Ophthl Plst Reconstr Surg 23:57 59 Workmn M, Dion J, Tong FC, Cloft HJ (2002) Tretment of trpped CCF by direct puncture of the cversnous sinus by infroculr trns-sof pproch. Interventionl Neurordiology 8: Wright KC, Anderson JH, Ginturco C, Wllce S, Chung VP (1982) Prtil splenic emboliztion using polyvinyl lcohol fom, dextrn, polystyrene, or silicone. An experimentl study in dogs. Rdiology 142: Ymshit K, Tki W, Nishi S, et l. (1993) Trnsvenous emboliztion of durl croticocvernous fistule: technicl considertions. Neurordiology 35: Yng PJ, Hlbch VV, Higshid RT, Hieshim GB (1988) Pltinum wire: new trnsvsculr embolic gent. AJNR Am J Neurordiol 9: Yoshimur S, Hshimoto N, Kzekw K, Nishi S, Smpei K (1995) Emboliztion of durl rteriovenous fistuls with interlocking detchble coils. AJNR Am J Neurordiol 16: Yu SC, Cheng HK, Wong GK, Chn CM, Cheung JY, Poon WS (2007) Trnsvenous emboliztion of durl crotid-cvernous fistule with trnsfcil ctheteriztion through the superior ophthlmic vein. Neurosurgery 60: ; discussion Zink WE, Meyers PM, Connolly ES, Lvine SD (2004) Combined surgicl nd endovsculr mngement of complex posttrumtic durl rteriovenous fistul of the tentorium nd stright sinus. J Neuroimging 14:

285 Alterntive Tretment Options 9 CONTENTS 9.1 Spontneous Thrombosis Mnul Compression Therpy Controlled Hypotension Cse Report VIII Rdiotherpy Surgery 287 References 289 With regrd to the reltively high rte of spontneous thrombosis of durl cvernous sinus fistuls (DCSFs) in some studies, number of investigtors consider conservtive mngement the first line of tretment, especilly if symptoms re mild, no corticl venous dringe is present nd the ngiogrphic evlution revels low-flow shunt. This my include observing nd following ptients, while they re regulrly exmined by n ophthlmologist to monitor their visul cuity nd IOP. Retroorbitl pin my be treted with stndrd nlgesics, diplopi my be coped with by using prism therpy nd elevted IOP (if necessry) by dministrting topicl gents such s Ltnoprost for few weeks (Miller 2007). Worsening of symptoms my indicte n increse in AV shunting flow, but cn be lso prt of the heling process tht is ccompnied by thrombosis of the CS nd my involve the SOV to some degree. Administrtion of corticosteroids my help to cope with these symptoms nd lessen their se- verity (Sergott et l. 1987). This mngement my lso be useful until elective endovsculr tretment is scheduled. If IOP continues to increse, exophthlmos progresses or chemosis develops, definite occlusion of the AV shunt becomes inevitble. Although conservtive mngement with or without mnul compression therpy is recommended by numerous investigtors (Miller 2007; Grove 1984; Phelps et l. 1982; de Keizer 2003), glucom tretment my be insufficient nd IOP cn be difficult to control (Kupersmith et l. 1988). While nticogultion is used by some investigtors to void postopertive thrombosis of the SOV nd the centrl retinl vein, heprin is dministered by others to control the thrombosis s prt of the nturl history of DCSFs. Binchi-Mrzoli et l. (1996) recently reported improvement in four ptients fter low-dose heprin dministrtion, nd deteriortion in two other ptients in whom the heprin ws stopped. Yousry et l. (1997) observed n interesting cse in which the AV shunt completely disppered following systemic nticogultion for 3 months. Other vible non-invsive options for conservtive mngement include mnul compression nd controlled hypotension (see below). 9.1 Spontneous Thrombosis Erly reports on spontneous occlusion of CSFs include the monogrphs from Sttler (1930), Dndy (1937) nd Hmby (1966), which reported 5.6% 10% occlusion rtes. These reltively low numbers re presumbly due to the inclusion of lrge number of high-flow direct CCFs (Prkinson 1965, 1987).

286 278 9 Alterntive Tretment Options Sttler (1930) reported tht lthough he found occlusion in 18/322 cses (5.6%), this my be n underestimtion, becuse in mny treted nd untreted cses, the outcome remined unknown. In two trumtic fistuls (0.6%), slow regression of symptoms ws seen without ny therpeutic mesure. The remining 16 ptients (10 trumtic nd six spontneous) underwent spontneous thrombosis of the orbitl veins, ccompnied by severe inflmmtory rections. Some dt suggest tht bout 30% of the ptients in ll series show spontneous occlusion of the AV shunt, lthough the published mteril is quite heterogeneous (Tomsick 1997). For exmple, Nukui et l. (1984) nd Sski et l. (1988) studied 20 nd 26 ptients, respectively, who were conservtively followed between 4 nd 108 months (9 yers). A regression of symptoms ws noted in 18/20 (90%) nd 19/26 (73%) cses, respectively, nd ws delyed in ptients older thn 60 yers of ge, in slow-flow fistuls nd in cses with multiple drining veins. There ws unfortuntely no informtion on the ntomicl outcome nd whether disppernce of the symptoms correlted with complete ngiogrphic occlusion. It ppered in these dt tht closure of the fistuls followed pttern with hlf-life of 18 months (Brci-Slorio et l 2000). Dt on the nturl history of DCSFs re in generl incomplete, becuse some spontneous occlusions occurred following cerebrl ngiogrphy (Nukui et l. 1984; Sski et l. 1988; Tkhshi et l. 1989; Newton nd Hoyt 1970; Voigt 1978; Seeger et l. 1980), mnul compression therpy (Ki et l. 2007), or in groups of ptients undergoing trnsrteril emboliztions (Kurt et l. 1998; Stomi et l. 2005). Angiogrphy-triggered occlusion ws likely the underlying cuse in the cse of n AVF involving the IPS with symptoms mimicking CSF, observed by the uthor. Following bgtelle trum, the 72-yer-old gentlemn developed right eye redness, chemosis nd incresing diplopi due to 6 th nd 4 th CN plsy. The DSA reveled smll rteriovenous shunt t the posterior CS, but minly involving the IPS nd exclusively supplied by bilterl durl brnches of the APA. This ptient, shown in Cse Illustrtion XII, is one of the rre cses I hve observed with possibly relted trum in their history (see Sect ). Due to respirtory infection on initil dmission, endovsculr therpy hd to be postponed nd ws rescheduled 6 dys lter. The ngiogrm t the beginning of this endovsculr procedure showed prtil occlusion of the AV shunt, while the symptoms hd regressed (Fig. 9.1). This occlusion ws confirmed by second control ngiogrm 3 months lter, when the clinicl exm demonstrted complete resolution of conjunctivl engorgement nd diplopi. Such spontneous occlusions of CSFs tht follow intrvsculr contrst dministrtions hve been described by severl investigtors (Seeger et l. 1980; Nishijim et l. 1985; Isfort 1967; Voigt et l. 1971; Ymmoto et l. 1995; Potter 1954; Prsons et l. 1954; Fromm nd Hbel 1965; Toennis nd Schiefer 1959). Voigt et l. (1971) reported the spontneous occlusion of bilterl DSCF ssocited with cerebrl ngiogrphy. The uthors questioned the role of vsoconstrictor effect of the contrst medium triggering locl thrombosis. They fvored theory of stsis following chnges in pressure grdients during ngiogrphy. The role of generl nesthesi ws explined usully ccompnied by lowered systemic blood pressure. The ltter theory hs been considered by others s well (Potter 1954; Prsons et l. 1954) nd revels some evidence in fistul occlusions chieved by induced hypotension (see below) (de Miquel et l. 2005; Ornque et l. 2003). In 1980, Seeger et l. presented six ptients with spontneous occlusions. They discussed the role of contrst medium tht likely induces thrombosis by direct interction with the endothelium tht cuses ggregtion of pltelets nd white blood cells, ccelerting the clumping of erythrocytes nd thrombosis. Phelps et l. (1982), reporting the red eye shunt syndrome, observed six (32%) fistul occlusions. In this group, 7/19 ptients (37%) underwent ngiogrphy, three of them (43%) obliterted soon fter the exm. Spontneous occlusions my be ccompnied by excerbtion or regression of the symptoms, becuse fresh thrombus in the CS my redirect AV shunting flow towrds the SOV, incresing IOP. Tht is why prdoxicl increse of symptoms due to ongoing thrombosis cn be seen in some of the ptients (Seeger et l. 1980; Hwke et l. 1989; Grossmn et l. 1985). Hwke et l. (1989) documented tht ptient with initilly isolted posterior dringe developed new ophthlmologicl symptoms fter the IPS thrombosed. Kurt et l. (1993) emphsized tht ptients with singulr SOV dringe nd likely existing IPS thrombosis, demonstrted more significnt chemosis nd exophthlmos thn those with more open efferent veins.

287 9.1 Spontneous Thrombosis 279 b c d e f Fig. 9.1 f. Cse Illustrtion XII: Spontneous occlusion of DCSF. Right durl cvernous sinus fistul in 71-yer-old mle who presented 10/98 with eye redness nd diplopi due to 4th nd 6th nerve plsy., b The initil DSA (selective APA injection, lterl view) reveled smll AV shunt minly involving the right IPS (double rrow), supplied exclusively by the APA (rrow) nd drining into the ipsilterl CS (sterisk) nd SOV (rrowheds) s well s into the IJV (thick rrow) c, d EVT hd to be postponed due to respirtory infection nd ws rescheduled 6 dys lter. At tht time, the DSA showed n occlusion of the CS nd residul AV shunting into the IJV (lterl nd AP views). His symptoms hd improved.e Diplopi due to 4th nd 6th nerve plsies on dmission tht resolved completely over 3-month FU period (f)

288 280 9 Alterntive Tretment Options As lredy discussed by Sttler (1930), spontneous occlusion of CSF, if cused by thrombosis of the ophthlmic nd the retinl veins, my cuse visul loss when the fistul hels (Sergott et l. 1987; Knudtzon 1950; Miki et l. 1988; Suzuki et l. 1989). Choroidl effusion with incresing orbitl congestion nd crnil neuropthy due to uncontrolled spontneous thrombosis my occur, leding to drmtic worsening of the symptoms. When ptients undergo TVO, trnsient ggrvtion of symptoms due to induced CS thrombosis ffecting orbitl veins my lso occur. However, the mount of thrombus forming within the CS nd potentilly cusing pseudoinflmmtory deleterious effects is much lrger during spontneous occlusions. In ddition, the elevted venous pressure is t lest prtilly reduced s long s coils re effectively blocking the AV shunting flow. While iming for this reduction of the venous pressure hs priority during endovsculr mngement, excessive thrombosis of the SOV nd the centrl retinl vein must be voided. Thus, nticogultion for 48 h, even fter complete trnsvenous coil pcking of the CS, my be required (Kupersmith et l. 1988; Tomsick 1997). The uthor utilized n nticogultion regimen if emboliztion visibly ccelerted thrombosis, or when ptient developed progressive symptoms or incresing introculr pressure post procedure. 9.2 Mnul Compression Therpy The most widely used non-invsive conservtive mngement of CSF ptients is intermittent mnul compression therpy (MCT). This involves simple mneuver to reduce the rteril inflow nd the venous outflow of CSF nd ws used by Scott in 1834 for dignostic purposes. In 1846, Vnzetti from Pdu communicted verblly simple digitl compression between hert nd tumor, nd tught the sme from 1853 onwrds. Gioppi, one of Vnzetti s peers nd professor of ophthlmology in Pdu, reported in 1856 more defined technique (Gioppi 1858). He described 42-yer-old femle with pulsting exophthlmos tht developed fter pregnncy, who ws treted with 15 min of intermittent externl compression. After 4 dys, pulstion nd bruit were diminished. After 6 dys, slight recovery of the complete vision loss ws observed. Gioppi suggested four different compression techniques: (1) from nterior to posterior between the two heds of the sternocleidomstoid muscles, (2) using the 2 nd, 3 rd, nd 4 th finger of the left hnd long the lterl mrgin nd the thumb long the medil mrgin of the sternocleidomstoid muscle, while the right hnd pushes the hed to the involved side, (3) using the second finger t the nterior mrgin of the sternocleidomstoid muscle posteriorly nd slightly lterl, nd (4) slight compression ginst the lrynx or trche. While performing prt of the compression therpy herself, Gioppi s ptient ws not lwys ble to grb the crotid nd hd to use his third technique. Two yers lter, in Veron, Scrmuzz treted ptient using intermittent digitl compression for no longer thn 4 5 min, 5 6 times per dy over 18 dys. After the 3 rd dy, prtil regression ws noted. After 16 dys, complete regression of the exophthlmus occurred, nd fter 26 dys complete heling of the fistul ws observed [reported by Vnzetti (1858)]. Sttler (1880) mentioned tht crotid compression might be less effective in trumtic fistuls thn in idiopthic cses (spontneous CSFs). In 29 ptients in which digitl or instrumentl crotid compression ws performed, only four (14%) showed success. Despite these somewht discourging results, he suggested beginning tretment of pulsting exophthlmus with compression method, either digitl, using mechnicl instrument (Fig. 2.7) or vi tourniquet, s ws utilized by Nelton. In spontneous ( idiopthic ) cses, such intermittent compression my lredy be sufficient to chieve improvement or cure. Bed rest nd other mesures to lower systemic blood pressure were lso recommended. In 1924, Locke mentioned rte of cure or improvement of 37% in 27 ptients nd 26.4% in 106 ptients, respectively. Mnul compression therpy s tretment option for CSFs hs been dvocted by numerous investigtors s minimlly invsive procedure since. It ws further supported by the encourging experience reported by Hlbch et l. in Ptients with DCSFs were sked to compress their crotid rteries nd jugulr veins with their opposite hnds while sitting for 10 s severl times per hour. When tolerted, the compression ws incresed 30 s over totl of 4 6 weeks. Ptients with ngiogrphic evidence of corticl venous dringe were excluded. The uthors chieved complete cure in 7/23 (30%) ptients undergoing crotid jugulr compression, while the

289 9.2 Mnul Compression Therpy 281 b c d Fig. 9.2 d. Cse Illustrtion XIII. Worsening symptoms fter mnul compression of the SOV in 53-yerold womn. ECA injection, lterl view demonstrtes smll AV shunt t the CS (sterisk), filling very slowly the SOV (rrow) nd prtilly the IOV (short rrow). b, c Ptosis nd significnt eye redness with dilted epibulbr veins indicting deteriortion cused by the compression of the SOV. d ECA injection, lterl view fter TAE with PVA prticles shows complete occlusion of the AV shunt. (Courtesy: W. Lim, Singpore) sme group (Higshid et l. 1986) chieved occlusion of direct CCFs in eight of 48 ptients (17%). Since then, this occlusion rte (pproximtely one third) hs been cited in numerous publictions. Thus, mnul compression therpy hs been recommended s n djunct or even lternte tretment option. In 1992, complete cure ws reported in 34% of 53 ptients (Hlbch et l. 1992). Quite surprisingly, in the most recent report on DCSF ptients from the sme investigtors, including ll ptients from , mnul compression therpy ws curtive in only one ptient (0.74%), while it ws used s n djunctive technique to endovsculr tretment in 34%. This rises questions bout its true efficcy. Unfortuntely, no vlidtion of its ctul tretment effects exists to dte. Ki et l. (2007) studied group of 23 ptients, chieving complete resolution of symptoms in eight cses (35%). The uthors identified lower oculr pressure, shorter intervl between symptom onset nd compression tretment nd venous dringe solely vi the superior ophthlmic vein without involvement of the inferior petrosl sinus s fctors tht would fvor complete occlusion chievble by this technique. Becuse the ptients in this group underwent MRI/MRA for FU, it is not cler in how mny cses ntomicl occlusions were indeed chieved. It is lso known tht resolution of ophthlmologicl symptoms my occur while the venous dringe is rerouted posteriorly towrds corticl veins, in fct creting, more dngerous lesion.

290 282 9 Alterntive Tretment Options Mnul compression therpy ws performed in only four of the uthor s ptients, who were considered complint. The therpy ws performed s described previously. Although ll ptients reported some improvement over this period of time, none showed notble reduction of the shunt flow in the control ngiogrm. In one ptient, fter 3 weeks compressing the SOV in the eye ngle, n increse in symptoms occurred. All ptients underwent subsequent endovsculr therpy, which led to complete ntomicl nd clinicl cure. The effect of compression therpy ppers unpredictble, s there is no control on the chnge in the venous pressure or flow during this tretment. In fct, ggrvtion of symptoms such s retinl hemorrhge, induced by centrl retinl vein occlusion nd hypoxic retinopthy preceding the spontneous regression of spontneous cvernous sinus fistuls hs been reported (Miki et l. 1988). One fctor tht my possibly influence the efficcy of mnul compression therpy is the dependence of the cerebrl venous dringe on the posture of the ptient. Recent studies hve shown tht the internl jugulr vein serves s min dringe vessel only in the supine position. When stnding, this function is minly tken over by the vertebrl venous plexus (Gisolf et l. 2004). Consequently, compression of the IJV when stnding or sitting will likely be less effective. Thus, until controlled dt become vilble, it remins doubtful whether or not mnul compression therpy is in fct effective s single tretment modlity. There is otherwise no question tht reducing the flow nd pressure within the AV shunting communiction is useful djunctive therpy to both trnsrteril nd trnsvenous occlusion techniques. If consequently performed by coopertive ptient, it my even be effective s single (conservtive) tretment modlity in direct CCF (Spinnto et l. 1997). Some investigtors recommend the externl compression of the SOV t the inner eye ngle before the vein joins the ngulr vein (Locke 1924) or direct oculr compression (Ismt et l. 2000). Blocking the venous outflow in cse with nterior dringe my hve similr untowrd effects, cusing either ggrvtion of ophthlmic symptoms or rerouting the venous flow nd inducing corticl dringe. Cse Illustrtion XIII is n exmple in which mnul SOV compression resulted in worsening of the symptoms (Fig. 9.2). The ptient hd to undergo subsequent trnsrteril emboliztion with prticles to reduce the AV shunting. 9.3 Controlled Hypotension Cse Report VIII (Fig. 9.3.) A 72-yer-old womn presented with right chemosis, exophthlmos, glucom nd loss of visul cuity of 0.05 in the right eye (09/17/1999). Performing mnul crotid compressions, she felt subjectively better nd hd visul cuity of 0.2 (09/29/1999). An ngiogrm ws performed on 10/11/99, reveling durl AVF of the left CS fed by the left meningohypophysel trunk nd drining into the right SOV. Ophthlmologiclly, there ws suspicion of superior ophthlmic vein thrombosis nd thus, low-moleculr heprin ws prescribed. Two weeks lter, left retinl hemorrhges were found, nd nticogultion ws suspended. Tretment of the AVF becme necessry. Since emboliztion of the smll ICA pedicle ws considered dngerous, controlled hypotension ws proposed. Her BP ws lowered from 160/100 to 80/45, while the men rteril pressure ws mintined round 60 mmhg using propofol nd nitroglycerine. After 8 min of hypotension, the ptient suddenly noticed tht her vision ws clerer nd better. In fct, her visul cuity improved over severl dys nd ws 0.4 in the RE nd 0.5 in the LE t ophthlmologic FU 3 weeks lter. The glucom tretment ws stopped. The ptient remined symptom free until 5 yers lter t the end of The induction of controlled hypotension for occlusion of DCSFs is new pproch, which hs not been widely communicted (de Miquel et l. 2001; 2005; Ornque et l. 2003). The first description ws from De Miquel et l. (2001), who reported eight consecutive ptients with DAVFs, including two DCSFs who underwent 30 min of controlled hypotension using sodium nitroprussite, esmolol nd nitroglycerine. In four ptients, the symptoms lessened; in two, ngiogrphy confirmed occlusion. In 2003, Ornque et l. reported DAVF of the trnsverse sinus for which controlled hypotension ws performed by lowering the blood pressure under generl nesthesi utilizing propofol. The uthors discovered the possible influence of low blood pressure on DAVF thrombosis nd occlusion in ptient with hypovolemic shock due to mssive hemtom fter unsuccessful trnsfemorl emboliztion. A totl of 13 ptients were treted without generl nesthesi using vrious blood pressure lowering drugs, including nitroglycerin, urpidil nd nitroprussi-

291 9.3 Controlled Hypotension 283 b c d Fig. 9.3 d. Cse Report VIII: Occlusion of DCSF using controlled hypotension. Right durl cvernous sinus fistul in 72-yer-old womn who presented with chemosis nd high introculr pressure. The fistul evolved over severl weeks nd led to prtil superior ophthlmic vein thrombosis., b Left ICA injection, lterl view shows smll trnssellr brnch (rrowheds) tht supplies n AV shunt t the right CS (sterisk). The fistul drins only nteriorly leding to sluggish filling of the right SOV (rrow). c, d Right nd left ICA injection, AP view: Complete resolution of the AV shunt fter totl of 30 min of controlled hypotension. (Courtesy: M. des Angeles de Miquel, Brcelon) te. This group included spinl AVFs (n = 3), DAVFs (n = 2) nd DCSFs (n = 8). In four ptients (31%), complete occlusion ws ccomplished, in four others the symptoms improved, in five (over 38%), there were no chnges. The men rteril pressure ws lowered to mmhg for durtion of min. The ptient reported by the uthors ws 48-yer-old mle with DAVF of the right trnsverse sinus initilly undergoing prtil emboliztion nd ws plnned for surgery. Controlled hypotension during the generl nesthesi using propofol for 90 min resulted in complete occlusion prior to surgicl explortion. An rteriogrm confirmed tht the fistul remined closed for 15 dys nd during the following 6 months.

292 284 9 Alterntive Tretment Options Propofol (Diprivn) is short-cting nesthetic gent with miniml side effects commonly used for induction or mintennce of generl nesthesi nd for sedtion of ICU ptients. It is very fesible to induce short periods of controlled hypotension. Recently, the sme group reported on the longterm outcome in 14 ptients with DAVFs in different loctions (de Miquel et l. 2005). Five ptients (36%) hd very low-flow DAVFs nd four (28.5%) resolved completely. In one ptient, the procedure could not be completed due to hemodynmic instbility of the ptient. Out of three ptients (21.4%) with low AV shunt flow, two (14.3%) occluded nd one (7%) significntly improved. In five cses (35.7%) where flow ws considered medium, two (14.3%) occluded, one (7%) improved nd one remined unchnged. In one high-flow fistul, the flow chnged only slightly nd the ptient hd to be treted by coil occlusion. The uthors concluded tht controlled hypotension is good djuvnt tool in low-flow DAVFs nd my led to long-term occlusions. The Cse Report (Fig. 9.3) shown here depicted tht induced hypotension cn be effective, nd thus should be considered in cses with smll or residul AV shunting. The pthophysiologicl mechnism is still not fully understood nd the role of venous pressure in the pthogenesis of DAVF is discussed in more detil in Chp. 5. Pressure chnges, tmospheric or systemic blood pressure, especilly when combined with intrrteril contrst ppliction, my possibly promote thrombosis nd occlusion in DCSFs, prticulrly in low-flow cses. Kupersmith et l. (1988) reported two cses where occlusion of DCSF occurred following ir trvel. One ptient developed choroidl detchment nd 3 rd nerve plsy; the ngiogrm performed the next dy showed complete thrombosis of the DCSF, ipsilterl CS nd SOV. Another ptient demonstrted complete resolution of the bruit nd bilterl 6 th nerve presis fter plne flight confirming ngiogrphic occlusion the next dy. It is noteworthy tht the role of lowered systemic blood pressure hs been considered custive fctor in cses tht developed spontneous occlusion following ngiogrphic procedures performed under generl nesthesi (Potter 1954; Prsons et l. 1954). Echols nd Jckson (1959) observed occsionl success with bilterl crotid compression under hypothermi. They reported 25-yer-old ptient with trumtic CCF who underwent hypothermi with body temperture reching 85.5 F, leding to unttinble blood pressure nd peripherl pulstion for 45 min. During this period, the bruit could not be herd, but returned fter 1 h, lthough to lesser degree. Potter (1954), who observed cse of spontneous cure fter period of severe hemorrhge nd syncope, lso discussed the role of hypotension for occlusion of CCFs. Due to the generl risks of dverse rections ssocited with nesthetic drugs, the use of controlled hypotension under generl nesthesi s single mens for therpeutic mngement is probbly not justified. It ppers, however, resonble s n djuvnt mesure in ptients undergoing endovsculr tretment, excluding those with cerebrovsculr, renl or crdic insufficiency. 9.4 Rdiotherpy Irrdition for tretment of durl cvernous sinus fistul ws introduced by Brci-Slorio et l. in 1979 in 65-yer old ptient suffering from chemosis, exophthlmos nd diplopi. After stereotctic rdiosurgery using single coblt source delivering 40 Gy, the ptient ws reported to be symptom free 2 months lter. In 1982, Bitoh et l. (1982), successfully treted two ptients with 60 Co (Coblt) using 32 Gy nd 30 Gy chieving complete occlusion, documented by ngiogrphy. Rdiotherpy of DCSFs hs been dvocted ever since s n lternte tretment option for DCSFs by severl groups (Bitoh et l. 1982; Sodermn et l. 2006; Hiri et l. 1998; Ysung et l. 1987; Ymd et l. 1984; Pierot et l. 1992; Mizuno et l. 1989; Hsuo et l. 1996; Guo et l. 1998; Moriki et l. 1993; Onizuk et l. 2003; Pollock et l. 1999). Conventionl frctionted irrdition vi liner ccelertor (Brci-Slorio et l. 2000; Hiri et l. 1998; Ysung et l. 1987), nd more recently, 60 Co source (Bitoh et l. 1982; Ymd et l. 1984) vi gmm knife rdiosurgery (Guo et l. 1998; Moriki et l. 1993; Pollock et l. 1999; Link et l. 1996) hve been suggested. In some series, rdiotherpy hs been combined with pre- or post-procedure emboliztion (Hiri et l. 1998; Pierot et l. 1992; Hsuo et l. 1996; Pollock et l. 1999; Link et l. 1996). In 1982, Brci-Slorio et l. described gmm knife rdiosurgery in four ptients s bloodless

293 9.4 Rdiotherpy 285 opertion without nesthesi, with no surgicl risk tht cn be pplied independent of ge nd generl condition of the ptient. Twelve yers lter, the sme group (Brci- Slorio et l. 1994), reported 25 ptients treted with modified 60 Co therpy, pplying n estimted dose of Gy. In ll, 22 ptients hd DCSFs, of which 20 (90%) could be completely obliterted during n verge post-tretment period of 7.5 months (2 20). In Type B fistuls, 100% occlusion rte ws chieved, compred to 75% nd 86% in Type C nd D fistuls, respectively. In two cses, retretment ws required to chieve complete occlusion. The sme group lter reported n improved overll occlusion rte in DCSFs of 91.6% (Type B: 100%, Type C: 90%) in 24 ptients, fter men follow-up intervl of 7.2 months (Brci-Slorio et l. 2000). There were no post-irrdition injuries to the opticl or oculr motor nerves. The occlusive effect is explined by erly endothelil swelling tht my evolve to prtil or complete thrombosis ssocited with bsl membrne rupture, necrosis, interstitil exudtes nd leukocyte invsion. This leds to fibroblstic nd endothelil cell prolifertion resulting in intiml hyperplsi tht cn be observed fter ppliction of doses between Gy (Jones et l. 1991). Hyline degenertion my occur fter 60 Gy (Jones et l. 1998). Link et l. (1996) reported on results of gmm knife therpy in 29 ptients with DAVFs, mong which 10 were locted t the CS. In 17 (59%) of these ptients who hd either pil or corticl dringe, dditionl trnsrteril emboliztion within 48 h fter rdiotherpy ws performed. A reduction of the AV shunting by trnsrteril emboliztion my decrese the risk of bleeding in the ltency period (post-tretment period) in these high-risk ptients. By using this combined pproch, complete occlusion ws ccomplished in 75% nd prtil occlusion in 29.5% of cses. One of the ptients with DCSF developed mild trnsient expressive phsi fter the emboliztion. Hsuo et l. (1996) reported on nine ptients with type D fistuls, who were treted with 30 Gy fter prticulte emboliztion, chieving immedite improvement nd complete resolution of the symptoms over 4 19 months. The uthors recommend this tretment s thetherpy of choice in type D fistuls with only mild symptoms. Guo et l. (1998) recently published series of 18 ptients with DCSFs (B: n = 1, C: n = 7, D: n = 10), who were treted primrily with gmm knife surgery (22 38 Gy). Trget levels were kept t 50% 90% isodose, while neurl structures such s the optic nerve were kept t 8 Gy. Complete occlusion ws chieved in 15 ptients (83%). The remining three showed prtil oblitertion with no complictions or worsening of symptoms observed over period of 27 months. The uthors concluded tht gmm knife tretment is fesible nd sfe lterntive for ptients with DCSFs. Hiri et l. (1998) reported the use of multifrctionted rdition, performed vi liner ccelertor dministering totl dose of 30 Gy over 15 sessions (2 Gy per session) in ll but one ptient, who received 40 Gy over 20 sessions. In 12 ptients undergoing irrdition lone, cure ws chieved in 75% of cses. Two cses with fst-flow fistuls showed no chnge; one demonstrted occlusion. In ll, 14 ptients underwent TAE or TVO. In six of these ptients, endovsculr tretment ws combined with irrdition, chieving cure in 12 ptients (86%). Two ptients with fst-flow type fistuls demonstrted either no chnge or improvement of symptoms despite undergoing combined tretment. One ptient suffered from ischemic stroke fter TAE nd recovered within 1 yer, while nother presented with trnsient epiltion following TVO. Pollock et l. (1999) treted 20 DCSFs with either rdiosurgery lone (n = 7), or rdiosurgery followed by prticulte trnsrteril emboliztion (n = 13), chieving improvement of symptoms in 19 (95%), nd totl ngiogrphic occlusion in 87%. Two ptients who did not show initil ngiogrphic occlusion experienced recurrence of the symptoms nd underwent repeted TAEs. One ptient underwent TVO. None of the ptients who demonstrted ngiogrphic occlusion (14/15) showed recurrent symptoms. Three ptients (15%) in this group experienced complictions: one ptient developed stroke during stereotctic ngiogrphy, one developed venous ischemi nd nother permnent 6 th nerve plsy due to cute CS thrombosis s consequence of the emboliztion procedure. Sodermn et l. (2006) recently reported on 53 ptients with 58 DAVFs treted with gmm knife surgery chieving totl occlusion in 68% (ngiogrphiclly proven oblitertion) nd significnt flow reduction in 24%. Five ptients (9%) with DCSFs were included receiving miniml dose of either 10 Gy or 12 Gy resulting in complete oblitertion (Cse Illustrtion XIV). One ptient (18.8%) with preexisting 6 th nerve plsy received mximl dose of 50 Gy nd developed trnsient worsening of his symptoms. The entire series lso contined cse

294 286 9 Alterntive Tretment Options of lte rection (10 yers) with hemorrhge (1.9%), one ptient with focl lopeci nd two ngiogrphy relted minor complictions. It is emphsized tht DAVFs with corticl venous dringe my not respond to rdition, which is disdvntge, s these ptients hve n incresed risk of intrcrnil hemorrhge during the post-tretment period. DCSFs in generl my hve lower risk of intrcrnil hemorrhge thn DAVFs in other loctions; neurologicl deficits due to venous congestion my develop during this ltency period. Some investigtors see this s mjor downside of rdiosurgery nd fvor emboliztion therpy insted (Cognrd et l. 2008). It should lso be mentioned tht in some cses of DCSFs, the time to occlusion is reltively short compred to the time required for oblitertion of cerebrl AVMs (Heros 2006). In one of the lrgest series to dte, 100% of Type B fistuls closed fter men period of 5.9 months, 75% of Type C fistuls closed fter 12.6 months nd 85.7% of Type D fistuls closed fter 8.16 months (Brci-Slorio et l. 1994). Sodermn et l. (2008) reported the sme phenomenon, ssuming tht the loction within the dur mter nd the usully nrrow vessels my ply role in this reltively fster oblitertion. Although complictions directly relted to rdiosurgery of DCSFs re rre, side effects my occur. Lu et l. (2006) reported on prdoxicl worsening in ptient with DCSF undergoing rdiotherpy who developed signs of SOV thrombosis nd centrl retinl vein occlusion. As the rdition trget includes not only the dur mter, but lso the venous CS comprtments, progressive oblitertion in n unpredictble mnner might be of generl concern. As stressed for other indirect tretment modlities, benign DCSF my trnsform into more ggressive lesion, exposing the ptient to incresed risk of neurologicl deficit or intrcrnil hemorrhge nd deth. Such n untowrd chnge my occur during the reltively long follow-up period, especilly when rdiosurgery is used s single tretment option. To monitor chnges in direction of the SOV flow, intermittent imging follow-up using Doppler ultrsound my be recommended (Chiou et l. 1998). Definite occlusion of the AV shunt should be ffirmed by intr-rteril DSA rther thn MRA, s ws recently reported by Chiou et l. (1998), who successfully treted four ptients, one with corticl venous dringe. Smll remining fistulous comprtments re not detectble using MR ngiogrphy. Although stted otherwise by some investigtors relying on symmetric dimeters of the SOVs or ppernce of the orbitl ft tissue (Struffert et l. 2007), MRA ws completely unrelible to rule out smll AV shunts, especilly when drining posteriorly in severl of my own cses. Non-invsive imging follow-up, if t ll, should rther be used for ptients undergoing TVO, in whom complete occlusion is mostly chieved t the end of the procedure or will occur within dys or weeks post procedure in the mjority of cses. Although undue effects of rdition on the optic or oculomotor nerves hve not been reported in ptients with DCSFs, they my occur t rte of 6.3% following rdiosurgery of CS tumors (Tishler et l. 1993). A dose limit of 8 10 Gy ppers to be ccepted for the optic nerve nd the brin stem (Guo et l. 1998; Tishler et l. 1993; Brci-Slorio et l. 1994b), while such limits for intrcvernous crnil nerves remin to be determined (Brci-Slorio et l. 2000). In summry, rdiotherpy of DCSF represents n effective lterntive (up to 90%) (Brci-Slorio et l. 2000) nd should be considered vluble complement of the therpeutic spectrum for DCSFs. In selected cses in which endovsculr mens remin unsuccessful, ineffective, contrindicted or considered too risky, irrdition using liner ccelertor or gmm knife should be considered. In elderly ptients with comorbidities, lengthy endovsculr procedures under generl nesthesi could be voided, especilly when they hve lredy filed in previous sessions. Rdiosurgery should focus primrily on smll, low-flow shunts nd, if possible, be combined with trnsrteril emboliztion or mnul compression. The efficcy in high-flow lesions or direct CCF ppers questionble (Brci-Slorio et l. 2000). Newer dt on the nturl history seem to indicte tht hemorrhge rtes for DAVFs with corticl dringe my be lower thn previously expected (Sodermn et l. 2008). Nevertheless, rdiotherpy should probbly not be considered in those cses, or when neurologicl mnifesttion is lredy evident. Although not seen by the uthor, so-clled intrctble cvernous sinus fistuls hve been reported by others (Link et l. 1996; Guerro et l. 2006). Due to continuous dvncement of endovsculr tools nd devices, improved visuliztion techniques nd enhnced ntomic understnding, their number will lso remin reltively smll in the future.

295 9.5 Surgery Surgery Direct surgicl tretment of CSFs ws the primry tretment modlity in the pre-endovsculr er; however, it ws ssocited with significnt morbidity nd mortlity (Dndy 1937; Sttler 1930; Locke 1924; Hmby nd Grdner 1933). Ligtion of the ophthlmic veins ws successfully performed by Lnsdown in 1874 (Lndsdown 1875) nd repeted by others (Sttler 1880; Locke 1924) during subsequent yers. Locke (1924) reported 68.4% cure or improvement rte with this tretment, with mortlity of 5.3%. About 100 yers fter Ashley Cooper performed the first CCA ligtion, De Schweinitz nd Hollowy reviewed 114 ptients communicted in the literture to tht dte, nd found good outcome in 56%, no improvement in 17.5% nd recurrence rte of 20%. The overll mortlity rte ws 11.7%. Locke (1924) documented cure or improvement in 61.9% of the ptients undergoing crotid ligtions for tretment of pulsting exophthlmus nd mortlity of 14.3%. Becuse of such high recurrence rtes nd poor, or even ftl outcomes, ligtion of intrcrnil crotid rteries ws proposed by Hmby nd Grdner in The introduction of microsurgicl techniques enbles direct surgicl pproches to the CS for oblitertion of direct nd indirect CSFs ( Prkinson 1965, 1987; Dolenc 1990; Ismt et l. 1986; Frncis et l. 1995; Vinuel et l. 1984). Ismt et l. (1986) described four ptients (three trumtic, one spontneous), whose CS ws exposed vi pterionl crniotomy llowing the introduction of muscle frgments nd/or fibrin selnt. The sme uthors reported on seven dditionl ptients treted by trnsmurl injection of fibrin selnt into the CS chieving complete occlusion in ll, preservtion of the ICA in 4/4 cses nd observing one trnsient postopertive hemipresis (14.2%) (Ismt et l. 2000). It is emphsized tht control of the hemodynmic condition by plcing temporry clip on the suprclinoid crotid nd trnsient occlusion of the cervicl ICA using mnul compression or rubber bnd cn be useful djunct. With the onset of trnsrteril emboliztion techniques, severl investigtors pioneered their use of direct nd indirect CSFs (Hlbch et l. 1987; Kerber et l. 1979; Mnelfe nd Berenstein 1980; Bnk et l. 1978; Picrd et l. 1987; Chermet et l. 1977). Prticulrly since Serbinenko s introduction of blloons for occlusion of direct CCFs in 1974 ( Serbinenko 1974), direct surgicl oblitertion of CSFs hs been incresingly bndoned. Despite the estblishment of EVT s primry tretment modlity tody for direct fistuls nd indirect fistuls, open surgery of the CS is still considered useful in certin cses (Ismt et l. 2000; Tu et l. 1997; Dy nd Fukushim 1997; vn Loveren et l. 1991). In the Tu et l. (1997) series, 19/78 (24.3%) ptients with type A D CSFs who initilly underwent EVT were definitively treted by subsequent microneurosurgery. The uthors reported n occlusion rte of 100% nd n ICA ptency rte of 94%. Among these 19 ptients, three (15.8%) hd DCSF in which either the smll fistul size or multiple ECA brnches were reported s resons for filure of endovsculr techniques. Interestingly enough, no trnsvenous techniques were ttempted in ny of these ptients. Eight ptients (42%) experienced trnsient 3 rd nerve plsy, while in one ptient (5.2%) permnent 6 th nerve plsy ws noted. Vn Loeveren et l. (1991) performed direct surgery in eight ptients with Type A fistuls between 1979 nd 1996, chieving complete occlusion in 75% nd reduction of the fistuls in 25%. There were immedite postopertive CN deficits in five ptients (63%), mong whom two were permnent (one ptient ws lost to long-term FU). Dy et l. (1997) published series of nine Type D fistuls treted by combined extr-intrdurl pproch to the CS nd consecutive oblitertion of the fistulous communiction. In this group, endovsculr tretment hd been ttempted using TAE (de Keizer 2003) nd trnsvenous pproches (Phelps et l. 1982), but filed. It is stted tht Type D fistuls represented chllenge for endovsculr tretment. This my be true for trnsrteril emboliztion, but is not for trnsvenous occlusion techniques using ll vilble routes. It remins uncler to wht extent endovsculr tretment options were fully explored in some of the cses (Illustrtive Cse 1). While resolution of the symptoms nd ngiogrphic occlusion were complete in 100% of ptients, trnsient diplopi nd trigeminl hypoesthesi developed in ech ptient, resolving over 6 months. In ddition, there ws high procedure-relted morbidity of 22% [trnsient hemipresis in one (11%) nd permnent hemipresis in nother (11%)]. The success of this method, which ws originlly described by Mulln (1979), relies on superb knowledge of the cvernous tringles while sensing how to void

296 288 9 Alterntive Tretment Options b c d Fig. 9.4 e. Cse Illustrtion XIV: Occlusion of DCSF using rdiosurgery., b DSA of n 80-yer-old gentlemn with red eye on the right side cused by left DCSF, drining over the midline towrds the right CS (sterisk) nd secondrily into the right Sylvin vein (red rrow). A previous endovsculr tretment ttempt hd filed due to technicl difficulties to ctheterize the IPS nd SOV on either side. The trget volume ws delineted in white nd indexed with 1. c Dose plnning for gmm knife rdiosurgery: The prescription dose ws 20 Gy with mximum dose of 40 Gy (50% isodose), which is common dose pln. The gmm knife collimtors were plugged for optiml coverge of the trget volume while the rdition dose to the optic nerve ws kept below 8 Gy, nd thus should not ffect the vision of the ptient. d, e At 24-month follow-up showing occlusion of the AV shunt. The ptient hd fully recovered from his eye redness within few months. (Courtesy: M. Soedermn, Stockholm) e

297 9.5 Surgery 289 overpcking. The ltter cn be problemtic mtter nd ppers even in experienced surgicl hnds not to be completely under control. To prevent overpcking is no less importnt thn voiding underpcking tht will result in persistence of the fistul. These concerns pper to be n inherent problem of direct surgicl tretment for DCSFs. Krisht et l. (1999) described pretemporl pproch to the nterior cvernous sinus for tretment of n intrctble CS fistul tht could not be treted by trnsvenous pproch becuse of thrombosis or bsence of the petrosl sinuses. The uthors used n extended pterionl pproch, dissecting the dur in the pretemporl region nd drilling the posterior orbitl roof to gin ccess to the nterior extension of the cvernous sinus, where they were ble to introduce n trumtic intrvenous cnnul. Intropertive ngiogrphy demonstrted complete fistul oblitertion fter introduction of thrombogenic coils. The sme group (Guerrero et l. 2006) recently reported nother cse of n intrctble Type D fistul using pretemporl direct pproch combined with ssisted coiling. The ptient ws reported to hve undergone seven unsuccessful ttempts t trnsrteril nd trnsvenous emboliztion. The uthors pproch represents n elegnt surgicl technique to overcome the complex ntomy in this region s well s block the fistul flow provided by the ECA brnches. In this prticulr cse, however, detils of the fistul s ngiorchitecture nd why ttempts t trnsvenous ctheteriztions remined unsuccessful re not reported. The only preopertive imge shows n erly rteril phse insted of dringe pttern s erroneously mentioned in the text. All other figures show stepwise increse of the coil pcking. The finl rteriogrm does not llow one to determine whether complete or subtotl occlusion ws chieved with ny certinty. No follow-up rteriogrm ws provided. Thus, it remins somewht questionble whether such direct open emboliztion ws indeed indicted, nd whether the performed coil pcking ws sufficient to chieve complete oblitertion. Although it is dvntgeous to be ble to trget different CS comprtments with coils, dense pcking my be less esy to chieve while the CS is in fct open nd its wlls cnnot be used s buttress for the coils. It cn be stted tht tody so-clled technicl chllenges in the EVT of Type D fistuls re mostly mngeble with modern trnsvenous ctheteriztion techniques nd sophisticted bi-plnr imging. Stte-of-the-rt endovsculr techniques must not be neglected when considering direct surgicl pproch in DCSF. Reported postopertive deficits, prticulrly with combined opertive nd technicl efforts (generl nesthesi, crniotomy, opening of the CS, etc.), hve contributed to the current trend of endovsculr mngement (vn Loveren et l. 1991) nd recently invlidted the need for direct surgicl tretment of DCSFs. In summry, direct surgery, if t ll necessry, should be pplied in selected cses only to fcilitte endovsculr pproches. Coopertion between operting nd endovsculr collegues is essentil for chieving optiml clinicl nd ntomicl results. Current endovsculr tools nd high-resolution imging cpbilities hve minimized the need for direct surgery in the therpeutic mngement of DCSFs. References Bnk WO, Kerber CW, Dryer BP, Troost BT, Mroon JC (1978) Crotid cvernous fistul: endrteril cynocrylte occlusion with preservtion of crotid flow. J Neurordiol 5: Brci-Slorio JL, Broset J, Herndez G, Bllester B, Msbout G (1979) Rdiosurgicl tretment of crotid-cvernous fistul. Cse report. In: Szikl G (ed) Stereotctic cerebrl irrditions. Elsevier, Amsterdm, pp Brci-Slorio JL, Herndez G, Broset J, Gonzlez-Drder J, Ciudd J (1982) Rdiosurgicl tretment of crotidcvernous fistul. Appl Neurophysiol 45: Brci-Slorio JL, Soler F, Brci JA, Hernndez G (1994) Stereotctic rdiosurgery for the tretment of low-flow crotid-cvernous fistule: results in series of 25 cses. Stereotct Funct Neurosurg 63: Brci-Slorio JL, Soler F, Brci JA, Hernndez G (1994b) Rdiosurgery of crotid-cvernous fistule. Act Neurochir Suppl 62:10 12 Brci-Slorio JL, Brci JA, Soler F (2000) Rdiosurgery for Crotid-Cvernous Fistuls. In: M.B. E, Al-Mefty O, eds. The Cvernous Sinus. Phildelphi: Lipincott Binchi-Mrszoli S, Righi C, Cisc P (1996) Low dose heprin therpy for durl cvernous sinus fistuls. Neurordiology 38:15 (Suppl.) Bitoh S, Hsegw H, Fujiwr M, Nko K (1982) Irrdition of spontneous crotid-cvernous fistuls. Surg Neurol 17: Chermet M, Cbnis EA, Debrun G, Hut J (1977) [Crotidocvernous fistul treted with infltble blloons]. Bull Soc Ophtlmol Fr 77: Chiou HJ, Chou YH, Guo WY et l. (1998) Verifying complete oblitertion of crotid rtery-cvernous sinus fistul: role of color Doppler ultrsonogrphy. J Ultrsound Med 17:

298 290 9 Alterntive Tretment Options Cognrd C, Jnuel AC, Silv NA Jr, Tll P (2008) Endovsculr tretment of intrcrnil durl rteriovenous fistuls with corticl venous dringe: new mngement using onyx. AJNR Am J Neurordiol 29: Dndy W (1937) Crotid-cvernous neurysms (pulsting exophthlmos). Zentrl bl Neurochir 2: Dy JD, Fukushim T (1997) Direct microsurgery of durl rteriovenous mlformtion type crotid-cvernous sinus fistuls: indictions, technique, nd results. Neurosurgery 41: ; discussion de Keizer R (2003) Crotid-cvernous nd orbitl rteriovenous fistuls: oculr fetures, dignostic nd hemodynmic considertions in reltion to visul impirment nd morbidity. Orbit 22: de Miquel MA, Myorl V, Ornque I, Souto JM, Arrug J, Cmbr R (2001) Controlled hypotension s co-djuvnt tretment in durl very low flow durl fistule. In: ASNR. Boston 303 de Miquel MA, Cmbr R, Ornque I et l. (2005) Controlled hypotension s n djuvnt tretment for closure of very low flow durl fistule. Long term outcome. 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Engelmnn, Leipzig, pp Sttler H (1930) Pulsirender Exophthlmus. In: Grefe A, Semisch T (eds) Hndbuch der Gesmten Augenheilkunde. Springer-Verlg, Berlin Heidelberg New York, pp Seeger JF, Gbrielsen TO, Ginnott SL, Lotz PR (1980) Crotid-cvernous sinus fistuls nd venous thrombosis. AJNR Am J Neurordiol 1: Serbinenko FA (1974) Blloon ctheteriztion nd occlusion of mjor cerebrl blood vessels. J Neurosurg 41: Sergott RC, Grossmn RI, Svino PJ, Bosley TM, Schtz NJ (1987) The syndrome of prdoxicl worsening of durlcvernous sinus rteriovenous mlformtions. Ophthlmology 94: Sodermn M, Edner G, Ericson K et l. (2006) Gmm knife surgery for durl rteriovenous shunts: 25 yers of experience. J Neurosurg 104: Sodermn M, Pvic L, Edner G, Holmin S, Andersson T (2008) Nturl history of durl rteriovenous shunts. Stroke 39: Spinnto S, Tlcchi A, Perini S, Dolenc V, Bricolo A (1997) Conservtive tretment of trumtic direct low-flow crotid-cvernous sinus fistul: cse report. Act Neurochir (Wien) 139: Struffert T, Grunwld IQ, Mucke I, Reith W (2007) [Complex crotid cvernous sinus fistuls Brrow type D: endovsculr tretment vi the ophthlmic vein, imging control with stndrdized MRI, long-term results]. Rofo 179: Suzuki Y, Kse M, Yokoi M, Arikdo T, Miysk K (1989) Development of centrl retinl vein occlusion in durl crotid-cvernous fistul. Ophthlmologic 199:28 33 Tkhshi A, Yoshimoto T, Kwkmi K, Sugwr T, Suzuki J (1989) Trnsvenous copper wire insertion for durl rteriovenous mlformtions of cvernous sinus. J Neurosurg 70: Tishler RB, Loeffler JS, Lunsford LD et l. (1993) Tolernce of crnil nerves of the cvernous sinus to rdiosurgery. Int J Rdit Oncol Biol Phys 27: Toennis W, Schiefer H (1959) Zirkultionstoerungen des Gehirns im Serien Angiogrm. Springer, Berlin-Goettingen- Heidelberg

300 292 9 Alterntive Tretment Options Tomsick TA (1997) Typ B,C, & D CCF: etiology, prevlence nd nturl history. In: Tomsick TA (ed) Crotid cvernous fistul. Digitl Eductionl Publishing Tu YK, Liu HM, Hu SC (1997) Direct surgery of crotid cvernous fistule nd durl rteriovenous mlformtions of the cvernous sinus. Neurosurgery 41: ; discussion vn Loveren H, Keller J, Fl-Klliny M, Scodry DJ, Jr TJ (1991) The Dolenc technique for cvernous sinus explortion (cdveric prosection). Technicl note. J Neurosurg 74: Vnzetti (1858) Secondo cso di neurism dell rteri oftlmic gurito coll compressione digitli dell crotide, e cenni prtici intorno questo metodo di curre gli neurismi. Pdov, nd Annli universli di medicin. CLXV:151 Vinuel F, Fox AJ, Debrun GM, Peerless SJ, Drke CG (1984) Spontneous crotid-cvernous fistuls: clinicl, rdiologicl, nd therpeutic considertions. Experience with 20 cses. J Neurosurg 60: Voigt K (1978) [Neurordiologicl dignosis nd tretment of durl crotid-cvernous sinus fistule (uthor s trnsl)]. Arch Psychitr Nervenkr 225: Voigt K, Suer M, Dichgns J (1971) Spontneous occlusion of bilterl croticocvernous fistul studied by seril ngiogrphy. Neurordiology 2: Ymd F, Fukud S, Mtsumoto K, Yoshii N (1984) [Effect of rdiotherpy on durl rteriovenous mlformtion. Long-term follow-up study nd clinicl evlution]. Neurol Med Chir (Tokyo) 24: Ymmoto T, Asi K, Lin YW et l. (1995) Spontneous resolution of symptoms in n infnt with congenitl durl croticocvernous fistul. Neurordiology 37: Ysung T, Tkd C, Uozumi H et l. (1987) Rdiotherpy of spontneous crotid-cvernous sinus fistuls. Int J Rdit Oncol Biol Phys 13: Yousry TA, Kuhne I, Strube A, Bruckmnn H (1997) [An unusul combintion of crotid rtery-cvernous sinus fistul nd sinus thrombosis. Successful therpy with nticogultion]. Nervenrzt 68:

301 Hemodynmic Aspects of DCSFs 10 CONTENTS 10.1 Introduction Bsic Hemodynmic Principles Invsive Assessment of Hemodynmics Flow Velocity nd Pressure Mesurements in Brin AVMs nd DAVFs Hemodynmics nd Pthophysiology in CSFs Flow Velocity nd Pressure Mesurements in DCSFs 300 Comments Introduction References 305 The study of blood flow in rteriovenous shunting lesions goes bck to Shenkin et l. (1948), who were ble to demonstrte flow velocity elevted by fctor of three from the ICA into the IJV in ptients with rteriovenous mlformtions (AVMs). Murphy (1954) defined the stel phenomenon ssocited with clinicl findings in AVMs such s seizures or psychic ltertions. He concluded tht the AVM shunt perfusion works t the expense of cerebrl tissue perfusion. Further reserch by severl investigtors focused minly on mesure- ments of regionl blood flow (Feindel et l. 1967; Heggendl et l. 1965; Ingvr nd Lssen 1972; Lssen et l. 1963). Nornes (1979, 1980) pioneered the use of Doppler ultrsound to mesure flow velocity in cerebrl rteries nd mesured intropertively elevted perfusion pressures up to 50%. The use of Doppler techniques for more detiled ssessment of blood flow in AVMs is extensively described in the monogrph by Hssler (1986). He studied flow chrcteristics in AVMs using trnscrnil Doppler sonogrphy (TCD) s well s intrvsculr probes by mesuring flow nd pressure intropertively before nd fter removl. Nornes (1972) lso used Doppler mesurements in the mngement of five ptients with CSFs (four trumtic, one spontneous). He found stel flow rnging from ml/min, forwrd flow rtes in the ICA between ml/min nd reverse flow between ml/ min. Nornes used the rtio of reverse flow/forwrd flow s n indictor of sufficient collterl cpcity of the cerebrl circultion in the cse of permnent ICA occlusion. Mny studies on hemodynmics of CSFs utilized trnsophthlmic ultrsound for detecting nd monitoring ptients with AV fistuls (Kwguchi et l. 2002; de Keizer 1982, 1986) or duplex sonogrphy of the ICA nd ECA flow (Lin et l. 1994; Chen et l. 2000). Lck of proper imging tools is reson for the scrce literture on AV shunting flow in CSFs tht minly focused on flow pttern on the rteril side pre- nd post emboliztion. Very little is known bout rteriovenous shunt flow nd pressure in DAVFs in generl, or in DCSFs in prticulr. Dt on intrsinus flow nd pressure is mostly lcking. Criteri for hemodynmic clssifictions re minly bsed on the speed of contrst filling in ngiogrms, nd thus re to lrge degree subjective nd impossible to quntify (see Sect. 4.3).

302 Hemodynmic Aspects of DCSFs 10.2 Bsic Hemodynmic Principles Blood flow through vessels follows the lws nd principles of fluid dynmics, whose key prmeters re s follows: Vessel rdius: r Vessel cross-sectionl re: A Velocity: ν Flux of fluid: Q Viscosity: μ Density: ρ Resistnce: R s Pressure grdient: ΔP L = Tube length: L Reynolds number: R e = ρνl μ The flow rte Q depends on the difference in pressure between both ends of tube nd the resistnce to tht flow. Only 2% of the hert ction is trnsformed into kinetic energy, while 98% is used to overcome frictionl force (Nornes nd Grip 1980). Q = ΔP / R s (1) Conservtion of mss embodied in the continuity eqution is one of the most bsic concepts in fluid dynmics nd thus in hemodynmics (SECCA nd GOULAO 1998). To mintin constnt flow, when the dimeter of the tube gets smller, the velocity will increse. More precisely, Q = Aν = constnt in tube (2) Conservtion of energy is nother key concept in the physics of flow nd cn be expressed in Bernoulli s eqution: P = 1 ρ v 2 (3) 2 This eqution is vlid only for fluids without viscosity, nd therefore does not directly pply to blood flow. However, it hs been usefully pplied to the estimtion of pressure drops in lrger rteries (crotid, ort). Viscosity of blood is inversely relted to temperture. Hgen-Poiseuille s lw is third cornerstone lw nd is written s: Q = ΔP π r4 (4) 8Lμ The flow rte vries with the fourth power of its rdius nd is inversely proportionl to its length nd to the viscosity. Thus, the vessel s dimeter is criticl fctor for regulting blood flow in the humn body. This lw, however, is only strictly pplicble to rigid, stright tubes with constnt dimeter nd homogenous fluids under lminr (s opposed to turbulent) flow conditions. In relity blood is inhomogeneous nd flows through complint vessels with chnging dimeters nd curvtures. It hs vrible viscosity chnging with sher rte nd the concentrtion of red blood cells (hemtocrit). Under norml tempertures nd pressures nd 40% hemtocrit, blood viscosity is pproximtely four times tht of wter. Very low flow sher rtes my result in reltive vlues of more thn 1000 or prestsis (Hssler 1986). Flow nd resistnce (Rs) re inversely proportionl to ech other (Eq. 2) nd for Hgen-Poiseuille s flow, the resistnce to the flow is written s: R s = 8 L η (5) πr 4 In closed vessel under constnt pressure grdient, the flow will increse with lrger rdius, decresing length nd lower viscosity (Secc nd Goulo 1998). In rteriovenous shunting lesions velocity is incresed nd the flow my become unstedy or turbulent, especilly when the Reynolds number R e exceeds 400. The flow becomes less lminr nd develops mrginl swirls; lminr flow completely disppers for R e bove 2,000 (Hssler 1986). While studies on hemodynmics in brin AVMs hve been of interest for number of investigtors (Nornes nd Grip 1979, 1980; Miysk et l. 1994; Norbsh et l. 1994; Hssler nd Thron 1994; Duckwiler et l. 1990), there is only scnt reserch of this type in DAVFs or DCSFs.

303 10.3 Invsive Assessment of Hemodynmics Invsive Assessment of Hemodynmics In order to study hemodynmics in intrcrnil rteriovenous shunting lesions, direct invsive mesurements of rteril nd venous flow velocities were performed by the uthor during emboliztions of AVMs, DAVFs nd DCSFs (Benndorf et l. 1994,b, 1995). In ddition, simultneous pressure mesurements in drining sinuses of AVMs (Benndorf et l. 1994b) or DCSFs (Benndorf nd Wellnhofer 2002) were recorded. For ssessments of flow nd pressure, two different sensor-tipped micro-guidewires were used: 1. The FloWire system from Crdiometrics (Mountin View, CA) ws developed in the 1990s for invsive studies of coronry blood flow velocities, pre- nd post ngioplsty (Serruys et l. 1993; Di Mrio et l. 1995; Lbovitz et l. 1993; Wellnhofer et l. 1997). A few investigtors utilized this technology for hemodynmic stud - ies in other vsculr territories, minly the cerebrovsculr circultion (Benndorf et l. 1994, 1995, 1997; Murym et l. 1996; Henkes et l. 1993). This Doppler guidewire consists of (0.036 mm) micro-guidewire, onto which Doppler probe is mounted with smple volume of 5 mm, provided by 10-MHz trnsducer. The SmrtWire ws initilly modified version of the FloWire (Doppler Wire) with more flexible tip for use in the tortuous cerebrl circultion (Fig. 10.1). The SmrtMp enbled rel-time disply nd recording of flow velocity nd flow pttern. The system is currently mnufctured by Volcno (Lgun Hill, CA) nd menwhile offers new Smrt-II Wire for pressure mesurements nd ComboWire (ComboMp) for ssessment of coronry flow reserve (CFR) nd frctionl flow reserve (FFR). The ltest wire versions hve specil core for better trckbility, PTFE coting nd re comptible with stndrd 18-microctheters. The following prmeters cn be recorded: MPV = Mximum (over pulstile cycle) pek velocity APV = Averge (over pulstile cycle) pek velocity PI (CPI) = Pulstility index (PI) = (IPV mx-ipv men) / APV IPV = Instntneous pek velocity 2. The PressureWire (Rdi Medicl Systems AB, Uppsl Sweden) is guidewire-mounted highfidelity fiberoptic pressure sensor, locted 3 cm proximl to the shpeble rdiopque tip (Fig. 10.1). Similr to the FloWire the PressureWire ws lso initilly developed for intrcoronry use (Di Mrio et l. 1993, 1995; Gorge et l. 1993) nd pplied to other territories to only some degree (Benndorf et l. 1994b; Abildgrd et l. 1995). It ws intended for use with PGA interfce tht provided pressure vlues, but no curve. The ltest version (Certus) comes s hydrophilic-coted (0.036 mm) guidewire in 175 cm/300 cm length nd mesures pressures between 30 nd 300 mmhg. Using the principle of thermodilution, monitoring intrvsculr temperture is possible nd flow velocities cn be clculted, lthough wveform is not obtined. The wire cn lso be dvnced through vrious stndrd microctheters (e.g. Tcker-Excel). The initil version of Rdi s PressureWire ws reltively stiff compred to the FloWire nd could not be dvnced through the crotid siphon. Flexibility my be improved with the ltest versions, but hs not been tested by the uthor for this purpose. The bove described wire configurtions with two sensors hve only recently become vilble. In erlier studies, two seprte wires (one for flow, nother for pressure) hd to be used. Both systems re employed to mesure pressure grdients, intrcoronry flow nd the frctionl flow reserve (FFR) tht is currently used s stndrd dignostic tool in crdic ctheteriztion lbortories (Wellnhofer et l. 1997; Abildgrd et l. 1995; Mrques et l. 2002; Nisnci et l. 2002; Alfonso et l. 2000; Briguori et l. 2001). A few reports describe non-coronry interventions (Mhmud et l. 2006; Cvendish et l. 2008) mesurements of crotid rtery pressure during ngiogrphy (Knzw et l. 2008) nd even ssessment of cerebrospinl fluid pressure in Chiri I niml models (Turk et l. 2006). In order to obtin dt on venous flow nd pressure in AV shunting lesions, the uthor used the two sensor-tipped guidewires, which were simultneously or lterntingly dvnced into the gret durl cerebrl sinuses, such s the sigmoid sinus (SS), trnsverse sinus (TS), stright sinus (StS), superior sgittl sinus (SSS) or the CS.

304 Hemodynmic Aspects of DCSFs b c Flow sensor d Pressure sensor Pressure sensor Flow sensor e PTFE Coting SmrtWire Floppy JET Coting JET Coting SENSOR CORE WIRE TIP f 1.5 cm 11.5 cm 3.0 cm 2 0 cm g Fig h. Sensor-tipped guidewires for invsive mesurements of pressure nd flow. Originl FloWire /SmrtWire (rrow, Crdiometrics) crrying miniturized 10 Mhz Doppler probe next to stndrd guidewire, here introduced into microctheters s used by the uthor. b A 5 mm smple volume of the Doppler probe. c, d Mgnified views of the currently vilble ComboWire with two sensors: Doppler probe t the tip nd pressure sensor either with 1.5 cm offset or next to the flow sensor ( rrows, Volcno Therpeutics Inc., Lgun Hills, CA). e, f Scheme of the current SmrtWire Floppy nd the SmrtMp (ComboMp ) for rel time monitoring of pressure nd flow. g, h Recent version of the (0.36 mm) PressureWire (Rdi Medicl Systems AB, Sweden) with the opening for the sensor 3 cm proximl to the tip, nd the RdiAnlyzer. Using thermodilution, flow dt cn lso be estimted h