Unilateral exophthalmos

Transcription

1 Brit. J. Ophthl. (97) 56, 678 Unilterl exophthlmos Aetiologicl study of 85 cses HANNA-SAMR ZAKHARA, KEVORK ASDOURAN, AND CAMLLE S. MATTA From the Deprtment of Ophthlmology, Americn University Medicl Center, Beirut, Lebnon Unilterl exophthlmos continues to intrigue nd chllenge the ophthlmologist. The condition hs been described by mny uthors (O'Brien nd Leinfelder, 935; Dndy, 91; Drescher nd Benedict, 95; Vn Buren, Poppen, nd Horrx, 957; Bullock nd Reeves, 959; Schultz, Richrds nd Hmilton, 96; Moss, 96; Porterfield, 96; Jones, 96; Reese, 96; Pohjol, 96; Plmer, 965; Silv, 967; Smith, 967) nd the frequency of the different cuses of this sign hve vried ccording to the interest of the uthor nd consequently the type of referrls. n reviewing 85 cses of unilterl exophthlmos seen t the Americn University Medicl Center (AUMC), we considered tht this constituted good representtive smple for the study of this condition for the following resons: (i) The AUMC is generl hospitl, nd ptients with unilterl exophthlmos cme not only from the ophthlmology service, but lso from the rest of the hospitl services. At lest one ophthlmologist sw the ltter cses in consulttion. () Only ptients with unilterl exophthlmos of orbitl origin were included. Unilterl oculr cuses, such s xil high myopi or congenitl glucom, were eliminted. (3) Dignosis of the etiology ws bsed on histopthologicl confirmtion (ioo per cent. of neoplsms), nd clinicl, lbortory, nd rdiologicl evidence. This series does not clim to reflect the reltive incidence of orbitl lesions, since mny such proven cses my not produce significnt exophthlmos or, s is frequently the cse, they re bilterl. Mteril nd methods The medicl files of ll ptients with unilterl exophthlmos t AUMC were studied. Only cses with n exophthlmometric difference of t lest 3 mm. were included, the non-ffected eye being norml by history nd exmintion. All mesurements were mde with the Hertel exophthlmometer. Cses with bilterl involvement, even if they mnifested such n exophthlmometric difference, were not considered. Cses which hd the second eye involved t lter dte were lso rejected. Routine investigtion included creful history nd system review, generl nd ophthlmologicl exmintion, thyroid studies, nd blood counts. Rdiologicl studies included x-rys of the orbit skull, nd sinuses. Dye-contrst orbitogrphy, internl crotid rteriogrphy, tomogrphy of the orbit, nd specil views of the optic formin were performed when it ws considered necessry. Phlebogrphy (Kryenbuhl, 96), rdiologicl exophthlmometry (Bogren nd Tengroth, 967), nd ultrsonogrphy (Gitter, Meyer, Goldberg, nd Srin, 968) were not employed. Received for publiction October i8, 97 Address for reprints: Cmille Mtt, M.D., Deprtment of Ophthlmology, Americn University Medicl Center, Beirut, Lebnon Br J Ophthlmol: first published s /bjo on 1 September 197. Downloded from on 15 August 18 by guest. Protected by

2 Unilterl exophthlmos 679 Resuls The ptients were divided into six ctegories ccording to the etiology. Tble shows the individul dignosis in these ctegories nd their distribution into three ge groups. Tble Dignostic group ncidence of etiologicl dignosis in the three ge groups Neoplstic Meningiom (36) Hemngiom Dermoid cyst Rhbdomyosrcom Metsttic crcinom Adenoid cystic crcinom lc. gl. Neuroblstom, metsttic Retinoblstom with orbitl extension Epiderml cyst Liposrcom Srcom, undifferentited Mlignnt melnom with orbitl extension Cementifying fibrom, mxillry sinus Reticulum cell srcom Nsophryngel trnsitionl cell crcinom Eosinophilic grnulom Osteom of ethmoid sinus nflmmtory (3 ') Orbitl cellulitis/bscess Mucocele/pyocele Pseudotumour Hydtid cyst Trumtic Orbitl hemorrhge (6) V Endocrine () V Vsculr (3) Cvernous sinus thrombosis Crotid-cvernous fistul V Unknown (5) Totl (85) NEOPLASTC Age (yrs) Totl cses o O 3' No. pthologicl This ws the most common in our series (36 ptients). t encompsses primry orbitl tumours nd metsttic mlignncies, s well s secondry extension into the orbit from the globe, the crnium, the prnsl sinuses, nd the nsophrynx. Hemngioms were included in this group rther thn in the vsculr group. Histologicl confirmtion ws obtined in ll cses. NFLAMMATORY There were 31 ptients in this group. Of fourteen cses of orbitl cellulitis, three bscesses hd to be evcuted, nd their contents were studied pthologiclly nd bcteriologiclly Br J Ophthlmol: first published s /bjo on 1 September 197. Downloded from on 15 August 18 by guest. Protected by

3 68o Hnn-Smir Zkhri, Kevork Asdourin, nd Cmille S. Mtt All seven mucoceles were excised; histopthology on one is missing but the x-ry nd opertive findings were conclusive. Of the seven pseudotumours encountered, five were biopsied, nd the other two mnifested typicl clinicl picture nd responded drmticlly to corticosteroids. The three cses of orbitl hydtidosis hve been reported in previous pper (Bghdssrin nd Zkhri, 971). TRAUMATC Six of our ptients presented with history of orbitl trum, unilterl proptosis of rpid onset, nd norml x-rys of the orbit. The dignosis of orbitl hemorrhge secondry to trum ws supported in ech cse by t lest three other hllmrks of periorbitl nd oculr trum: eyelid swelling nd ecchymosis, extroculr muscle presis, subconjunctivl hemorrhge, hyphem, iritis with or without secondry glucom, iridoplegi, multiple sphincterotomies, iridodilysis, vitreous hemorrhge, nd commotio retine. The condition ws self-limiting nd the exophthlmos receded in to weeks. ENDOCRNE There were only four ptients in this group. Two were hyperthyroid, one ws hypothyroid, nd the lst ws euthyroid. This lst ptient ws 55-yer-old mn with unilterl proptosis (exophthlmometric difference 3 mm.) of 15 yers' durtion, norml visul cuity, nd norml fundus. A Berke-Kronlein explortory orbitotomy ws undertken. No mss could be seen nd biopsy of the hypertrophied orbitl tissues reveled chnges consistent with endocrine ophthlmopthy. VASCULAR Two out of seven ptients with cvernous sinus thrombosis hd unilterl exophthlmos. One hd stphylococcl septicemi tht subsided on dequte ntibiotic therpy, though the ptient continued to hve some residul exophthlmos. The other ptient hd complete internl crotid rtery occlusion s demonstrted by rteriogrphy, becme comtose, nd died. An interesting cse in this ctegory ws crotid-cvernous fistul fter trum tht hd closed spontneously. Dignosis ws reched on the bsis of the history, clinicl findings, internl crotid rteriogrphy, retrogrde jugulr venogrphy, nd orbitl tomogrphy. UNKNOWN Of the five ptients in this group, three hd unilterl exophthlmos (3, 5, nd 9 mm. respectively) s the sole clinicl findings. There ws no plpble mss nd the vision nd fundus were norml. No bnormlity could be found nd no surgery ws performed. These ptients were lost to follow-up fter dischrge from the hospitl. The fourth ptient, 3-yer-old womn with mm. forwrd exophthlmos, visul cuity of counting fingers t m., ppilloedem, nd strie, hd mss indenting the scler, choroid, nd retin, which could be seen by indirect ophthlmoscopy. The tenttive dignosis ws hemngiom of the muscle cone. Unfortuntely, she refused surgery. The lst ptient ws 79-yer-old mn with mm. exophthlmos, nd plpble orbitl mss with evidence of intrcrnil extension by crotid rteriogrphy nd brin scn. Two successive generous biopsies showed "lymphoid tissue", nd no tumour cells were identified. Br J Ophthlmol: first published s /bjo on 1 September 197. Downloded from on 15 August 18 by guest. Protected by

4 Unilterl exophthlmos Discussion The reltive frequency of disese conditions cusing unilterl exophthlmos vries in different series. Much depends on the medicl specility of the uthor nd his method of collecting his cses. A neurosurgeon like Dndy (191) mostly hs referrls of tumours of neurl origin invding the orbit secondrily. Bullock nd Reeves (1959) present rdiologicl review of 5 cses of unilterl exophthlmos in which mucocele ppers to be the most common cuse. Drescher nd Benedict (195), with 77 cses, Schultz nd others (96) with 59 cses, nd Moss (96) with 3 cses shre the view tht endocrine ophthlmopthy rnks foremost in symmetric proptosis. Some of these my hve been bilterl but with unequl proptosis! More recently, Silv (967) reported series of 3 consecutive cses of unilterl exophthlmos in which he listed pseudotumour s the commonest cuse followed closely by mucocele. Plmer (965) quoted eight series, mking totl of,73 cses of unilterl exophthlmos, of which orbitl tumours constituted the bulk. Unfortuntely, he included series of 877 eyes for Reese which ctully formed histopthologicl review of orbitl lesions irrespective of the presence of exophthlmos or its unilterlity. n our series, the mjority of cses were of the neoplstic group. This is in greement with most other series (O'Brien nd Leinfelder, 935; Drescher nd Benedict, 95; Vn Buren nd others, 957; Bullock nd Reeves, 959; Moss, 96; Silv, 967; Smith, 967). Hemngioms re generlly regrded s the primry orbitl tumours of highest incidence nd most fvourble prognosis. We hd five cvernous hemngioms, none of the cpillry vriety, which proved to be well encpsulted nd in every cse excision resulted in cure. Predominnce ws in young mles with excellent preopertive vision, ppilloedem, nd strie, usully with no plpble mss or ophthlmoplegi nd norml x-ry findings. Tble (overlef) summrizes the slient fetures. n contrst the meningiom ptients were mostly femles presenting with mrked visul loss (secondry to optic trophy), externl ophthlmoplegi, no plpble mss, nd positive roentgenogrms. The outcome of surgicl excision ws not very rewrding (Tble, overlef). Rhbdomyosrcom remins the most common primry mlignnt orbitl tumour in children nd crries very grve prognosis. Of interest in our group of three cses (ge yrs, i yrs, nd 3 dys) is the lst one; the tumour ws present t birth nd ws excised t 3 dys of ge but recurred less thn yer lter. Out of,ooo cses of orbitl tumour seen t the registry of Ophthlmic Pthology (AFP), Porterfield (96) described 1 cses in children below 5 yers ofge. Rhbdomyosrcom ws the most frequent tumour nd gliom the second. t is worthy of note tht not single cse of gliom of the optic nerve ws seen in this hospitl nd its clinics. Porterfield (96) lso stted tht orbitl metstses of neuroblstoms in children re much overrted in their frequency nd presents three cses. This smll series includes two such ptients, nd 5 yers of ge, who both presented with exophthlmos nd in both the origin ws in the drenl glnd. Sinus osteoms invding the orbit to produce exophthlmos re reltively rre nd usully occur in the frontl or mxillry sinus. The single cse recorded here is n ethmoidl osteom. A relted cse is one of cementifying fibrom of the mxillry sinus. Lesions of the lcriml glnd my or my not produce proptosis. Of six ptients operted in our hospitl, two hd benign mixed tumour, two hd denoid cystic crcinom, one hd tuberculous dcryodenitis, nd the remining chronic nonspecific sclerosing 68x Br J Ophthlmol: first published s /bjo on 1 September 197. Downloded from on 15 August 18 by guest. Protected by

5 68 1 *. " t 9!. 6..c- r N 8 *. E * t * *,v Qu. ti c 5:1 ) V t *n *. 5 *M c._ -. r le ~ U). QNs Zcd Hnn-Smir Zkhri, Kevork Asdourin, nd Cmille S. Mtt _-o o. ".).; 1 l.1* z E_ ol ". i " ~) - E t v u *- cd r) co od vx c -5. -, ~) ) ld.. o - szz - ".). : z - -o " ~ d N Cf - ".) -) ", $. to. -- C.) - * - C* CS. -jc. Br J Ophthlmol: first published s /bjo on 1 September 197. Downloded from on 15 August 18 by guest. Protected by

6 Unilterl exophthlmos *t-. Q). A.e E o E to.5 1 A L. Qox C g 1.g n _._R ~ ( C US c O 8- to S, C E i - t; d co 19 - Q 9 E r3.w S z.) '. ).. O S.DO cs c c.~... ) P. ' '' h ' ~.. 3. E1 C >.,J: u -S wo 1 C C.w. ' o S._ S.o R r.. ow -O r, C._ r.".. -b.._. *o n c~. z.oo A : to 5)..5. c C: o. -g v ic -,.. c cn :~~~ o -~~~ of ' ' m ~ 1 z (. C 'M m ' to U) 683 u ) co _ ) Br J Ophthlmol: first published s /bjo on 1 September 197. Downloded from on 15 August 18 by guest. Protected by

7 68 Hnn-Smir Zkhri, Kevork Asdourin, nd Cmille S. Mtt dcryodenitis. Only the two with denoid cystic crcinom hd exophthlmos, probbly secondry to the more rpid growth of these lesions. Both ptients hd recurrence fter hving refused exentertion. The most common single cuse of unilterl exophthlmos in our series ws orbitl cellulitis or bscess. The portl of entry ws from n ethmoid sinusitis proven cliniclly nd rdiologiclly in seven ptients, ll below 5 yers of ge; two followed conjunctivl lcertion, one lid lcertion, nd one pustule on the upper eyelid. n two, the portl of entry ws not known. The remining ptient hd bcteril ulcer of the nterior stphylom nd developed pnophthlmitis followed immeditely by orbitl cellulitis; eviscertion ws performed. The orgnism cultured from the eviscerted mteril ws Pseudomons pyocyneus. The other cultures reveled Stph. ureus cogulse-positive in four nd Stph. lbus cogulse-negtive in three. n two ptients the cultures were negtive nd in four no cultures were tken. Antibiotic therpy ws effective in clering the infection nd reducing the exophthlmos in eleven ptients; in three n bscess formed nd hd to be drined. The endocrine group in our series is very smll compred to other series in spite of n ctive endocrinology clinic, but is in greement with the findings of O'Brien nd Leinfelder (935). Mny of these cses were bilterl or hd the other eye involved lter nd were excluded. The group of "unknown" might be reduced further if we were to dopt bold pproch, s suggested by Mortd (i 96). He reported on explortion of the orbit in nine ptients presenting with unilterl exophthlmos nd norml lbortory, rdiologicl, nd clinicl findings including norml vision nd fundus, nd no plpble mss. He found hemngiom to be the cuse in seven ptients. Most uthors do not subscribe to this pproch nd do not operte unless there is plpble mss or visul embrrssment relted to fundus findings. We hve subscribed to this pproch so fr. Compring our series to vrious reports in the literture, we find tht the reltive frequency of the vrious etiologies of unilterl exophthlmos is very closely similr in severl respects (Tble V, opposite). The neoplstic etiology seems to rnk first in ll series in spite of the differing interests of the reporters: ophthlmic, otolryngologicl, neurosurgicl, or rdiologicl. n the one series which does not show this prepondernce (Schultz nd others, 96), the difference in fvour of the endocrine group (three cses) is very smll. The second most frequent cuse ws inflmmtory or endocrine in tht order. Summry The etiology of unilterl exophthlmos ws studied in 85 cses. The conditions responsible for the exophthlmos were rrnged into six groups: neoplstic, inflmmtory, trumtic, endocrine, vsculr, nd unknown. The lrgest group ws the neoplstic one, with meningiom nd hemngiom heding the list. The most frequent single cuse in this series ws orbitl cellulitis. References BAGHDASSARAN, S. A., nd ZAKHARA, H. (97) Amer. J. Ophthl., 71, io8i BOGREN, H.,nd TENGROTH, B. (967) Clin. Rdiol., 8, 193 BULLOCK, L. J., nd REEVES, R. J. (1959) Amer. J. Roentgenol., 8, 9 Br J Ophthlmol: first published s /bjo on 1 September 197. Downloded from on 15 August 18 by guest. Protected by

8 Unilterl exopthtlmos 685 (~~~~~~~~~ % ) ( co LO $ N 1 '~~~ ~~~~c co ~ <t > r O N CO e S~~~~~( ~ * 9 o '., k t) (o it t < t N O O1 o N C-O ti O co c co cp ~co co- L _ N t t E E o E E * E ) X U ' c -,~ ~~~~~~~~~ ( il ~~~~~~~~~~~~~~~~~~~~~~~~ -S: >gm i 8. _ ~~~- ( ed = td me bo^~~~~~~~~~~~~i L C) r co ( LO 1 n C L) (o ~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~ ~~~~~~~~~~~~~~~~~~~~~ ~ - ~~~~~~~~ -~~~~~~~~~~~~~~~~~~~ cis C13 ~~~~~~ C ~L ( ( CUn - ( C~~~~~~~~~~~~~~~~~ ~~CC - O Br J Ophthlmol: first published s /bjo on 1 September 197. Downloded from on 15 August 18 by guest. Protected by

9 686 Hnn-Smir Zkhri, Kevork Asdourin, nd Cmille S. Mtt DANDY, W. E. (9) "Orbitl Tumours". Osks Piest, New York DRESCHER, E. P., nd BENEDCT, W. L. (195) A.M.A. Arch. Ophthl.,, O9 GTTER, K., MEYER, D., GOLDBERG, R., nd SARN, L. K. (968) Arch. Ophthl. (Chicgo), 79, 37 JONES,. (96) Highlights of Ophthlmology-96 series (U.S. ed.), 5, KRAYENBUHL, H. (96) J. Neurosurg., 19, 89 MORTADA, A. (96) Brit. J. Ophthl., 6, 369 MOSS, H. M. (96) Amer. J. Ophthl., 5, 76 O'BREN, c. s., nd LENFELDER, P. J. (935) bid., 8, 3 PALMER, B. W. (965) Arch. Otolryng., 8, 15 POHJOLA, s. (96) Act ophthl. (Kbh.),, 56 PORTERFELD, j. (96) nt. Ophthl. Clin.,, 319 REESE, A. B. (96) n "Oculr nd Adnexl Tumors", ed. M. Boniuk, p Mosby, St. Louis SCHULTZ, R. O., RCHARDS, R. D., nd HAMLTON, H. E. (96) Amer. J. Ophthl., 5, 1 SLVA, D. (967) "Dignosis of orbitl tumours" in "Proc. int. Symp. Plst. Reconstr. Surg. of Eye nd Adnex", ed. B. Smith nd J. M. Converse, pp. o8-5. Mosby, St. Louis SMTH, M. E. (967) nt. Ophthl. Clin., 7, 9 VAN BUREN, J. M., POPPEN, J. L., nd HORRAX, G. (1957) Brin, 8o, 139 Br J Ophthlmol: first published s /bjo on 1 September 197. Downloded from on 15 August 18 by guest. Protected by