Bilateral Ankle Fusion in Leak Syndrome Induced Deformity: A Case Report

Transcription

1 Cse Report Journl of Orthopedi Cse Reports 2017 Mr-Apr: 7(2):52-56 Bilterl Ankle Fusion in Lek Syndrome Indued Deformity: A Cse Report Luis Sorl 1, An C Ângelo 1, Afonso Cetno 1, Joquim Rodei 1, Isel Ros 1 Wht to Lern from this Artile? Systemi pillry lek syndrome my result in severe lower lim deformity. Although it n e treted s omprtment syndrome sequele, more serious intr- nd post-opertive omplitions hve to e onsidered. Astrt Introdution: Systemi pillry lek syndrome (SCLS) is rre ondition of unknown etiology defined y sudden episode of hypotension, high hemtorit, nd low serum protein onentrtion due to pillry hyperpermeility. Severl tretments hve een pulished for this pthology nd eventul omprtment syndrome ut not for the disling onsequenes. This linil se highlights the negtive orthopedi onsequenes of severe systemi ttk nd reports susequent deformity tretment option tht resulted in the ptient qulity of life improvement. To our knowledge, orthopedi surgery for SCLS indued foot nd nkle deformity is presented for the first time. Cse Report: A 40-yer-old Cusin femle ptient ws referred to our institution for ilterl foot nd nkle deformity fter omprtment syndrome during SCLS ttk. She presented ilterl rigid equino-vo-vrus-ddutus deformity using wlk impirment. Bilterl nkle fusion y retrogrde niling ws performed in two-stged proedure. Rigid lw toes were lso surgilly ddressed in n dditionl proedure. Rdiologilly, pprent nkle fusion ourred t 3 months post-opertive in oth sides. Deformity orretion llowed the ptient to regin wlking pity. A right nkle tiiotlr filed rthrodesis ws notied t 24 months post-opertive. Prophylti humn immunogloulin therpy ws initited fter the lst surgery with no reurrent ttk registered during the 38 months follow-up period. Conlusion: Bilterl nkle fusion improved this ptient qulity of life y restituting wlking pity nd lowering the proility of lower lim deformity relted ulers nd infetions. Mjor nd minor omplitions re lso disussed. This report ontriutes to the glol knowledge out this syndrome nd eliits the importne of the indued deformities surgil orretion. Keywords: Systemi pillry lek syndrome, omprtment syndrome, equino-vrus, nkle fusion, nkle retrogrde niling, humn immunogloulin. Author s Photo Gllery Aess this rtile online Wesite: Dr. Luis Filipe Loureiro Sorl Dr. A C Ângelo Dr. A Cetno Dr. J Rodei Dr. I Ros DOI: Deprtment of Trumtology nd Orthopedis, São Frniso Xvier Hospitl, Estrd do Forte do Alto do Duque , Liso, Portugl. Address of Correspondene: Dr. Luis Filipe Loureiro Sorl, Deprtment of Trumtology nd Orthopedis, São Frniso Xvier Hospitl, Estrd do Forte do Alto do Duque , Liso, Portugl. E-mil: luisflsorl@gmil.om Journl of Orthopedi Cse Reports pissn eissn Aville on doi: /jor This is n Open Aess rtile distriuted under the terms of the Cretive Commons Attriution Non-Commeril Liense ( whih permits unrestrited non-ommeril use, distriution, nd reprodution in ny medium, provided the originl work is properly ited. 52

2 Introdution Systemi pillry lek syndrome (SCLS), desried y Clrkson et l. in 1960 [1], is rre ondition defined y sudden episodes of hypovolemi shok, elevted hemtorit, nd hypoluminemi without luminuri [2, 3]. The etiology is still unknown, lthough severl possile triggers hve een reported s upper respirtory trt infetions, sustined physil effort nd menstrution [1, 4]. It results in generlized or segmentl edem nd, in severe ses, omprtment syndrome requiring emergent fsiotomy [2, 3, 4, 5]. The sudden intromprtmentl high pressure often origintes vsulr nd neurologil dmge nd onsequent lower lim deformity [3, 6]. Prolonged hospitl sty my lso ggrvte this ondition with osteoporosis following long-term immoiliztion or lim disuse. Deformity frequently leds to wlk impirment nd deresed qulity of life [7]. A few retrospetive studies report nkle fusion for orretion of omprtment syndrome indued deformity. None of them inluded SCLS etiology for omprtment syndrome [6, 7]. Severl tretments hve een pulished for SCLS nd omprtment syndrome [1, 2, 3, 4, 5] ut not for the disling onsequenes. To our knowledge, orthopedi surgery for SCLS indued foot nd nkle deformity is presented for the first time. Cse Report A 40-yer-old Portuguese womn omplined of severe limittions in dily life tivities due to ilterl foot nd nkle deformity. She hd een previously dignosed with SCLS fter sudden episode of hypotension, high hemtorit, nd low serum protein onentrtion. This ute phse ws mnged y fluid resusittion. The onsequent generlized omprtment syndrome ws treted with extensive fsiotomies of oth rms, forerms, hnds, thighs, nd legs. She styed 3 weeks in the intensive re unit nd 8 months hospitlized. Although n intense physil therpy progrm ws pplied, she ws unle to wlk nd utonomy ws limited to wheelhir. Amerin Orthopedi Foot nd Ankle Soiety (AOFAS) sore ws 20 points. Plin rdiogrphs showed ilterl equinovrus nkle deformity with supinted feet (Fig. 2). Ahilles, posterior tiil, nd flexor digitorum longus tendons lengthening were performed y medil pproh ssoited to right tiiotlolnel rthrodesis with retrogrde loked intrmedullry nil (PANTA - Integr TM ) y lterl pproh. Distl fiul one ws used s n utogrft. At 8 weeks of follow-up, plsti surgery pplied free skin grft over persistent post-opertive medil lister (Fig. 3). Apprent fusion ws otined t 3 months postopertively (Fig. 4). At 6 months fter the initil surgery, fusion surgery of the left nkle ws performed in extly the sme fshion. As in the first proedure, linil nd rdiologil follow-up ws done t 2, 6 weeks nd every 3 months. Surgil wound dehisene ws the short-term omplition nd ws resolved with dressing hnges t 6 weeks post-opertive. Fusion ws otined t 3 months postopertively (Fig. 4). At 12 months fter the first surgery, we doumented reurrent dorsl proximl interphlngel (PIP) joints inflmmtion with shoe wer. Right foot Moerg osteotomy, resetion rthroplsty of PIP joints, nd flexor tendon tenotomy of ll the lesser toes were performed. Left foot Moerg osteotomy, PIP joints fusion of the 2 nd nd 3 rd toes with intrmedullry guide implnt (Ipp-On - Integr TM ), PIP joints resetion rthroplsty of the 4 th toe, nd flexor tendon tenotomy of ll the lesser toes were performed 14 months fter the index surgery. Hrdwre-relted pin used Moerg osteotomy stple removl t 6 weeks post-opertive (Fig. 5). The rehilittion protool inluded progressive weight-ering with wlking oots nd lower lim dringe for 6 months. Prophylti humn immunogloulin therpy ws initited fter the lst surgery. She regined wlking pity with lned shoes nd returned to work 4 months fter left foot surgery. AOFAS sore improved to 61 points. No toes relted omplints, pressure ulers or terminl nerosis of the toes were found. On exmintion, she presented ilterl severe stiff equino-vo-vrusddutus deformity with retrtion due to suutneous nd musle srring nd neurologil defiit. Clw hllux nd lesser toes were lso present ilterlly. Plntigrde weight ering ws not possile (Fig. 1). 53 d Figure 1: Pre-opertive photogrphs showing ilterl nkle equino-vo-vrusddutus deformity. Postero-nterior photogrph of oth foot nd nkle with the ptient sitting in wheelhir simulting weight er (). Photogrphy tken t the onsulttion room, superior view photogrph of oth foot nd nkle with the ptient sitting in wheelhir simulting weight er (). Photogrphy tken t the onsulttion room, lterl view photogrph of the left thigh nd leg showing extensive fsiotomy (). Photogrphy tken t the operting room, superior view photogrph of the right forerm showing extensive nterior fsiotomy (d). Figure 2: Plin rdiogrph showing pre-opertive ilterl nkle: Bilterl nkle X-ry AP view showing equinovrus deformity in weight er simultion (), right foot nd nkle X-ry lterl view showing equinovrus deformity nd supinted foot in weight er simultion () nd left foot nd nkle X-ry lterl view showing equinovrus deformity nd supinted foot in weight er simultion () foot lterl view. Journl of Orthopedi Cse Reports Volume 7 Issue 2 Mr - Apr 2017 Pge 52-56

3 Figure 3: Right nkle posteromedil lister fter deridement (8 weeks fter index proedure). Medil view photogrph of the right nkle posteromedil lister t 8 weeks post-nkle fusion proedure. This photogrph ws tken fter lol deridement nd just efore skin grfting. d Figure 4: Plin rdiogrphs showing: Right nkle X-ry AP view showing pprent tiiotlr fusion t 3 months post- right nkle proedure, right nkle X-ry lterl view showing pprent tiiotlr fusion t 3 months postright nkle proedure (,) nd left nkle X-ry AP view showing tiiotlr fusion t 3 months post-left nkle proedure, left nkle X-ry lterl view showing tiiotlr nkle fusion t 3 months post-opertive (,d). Figure 5: Plin rdiogrphs showing ilterl hmmer toes deformities orreted: Bilterl foot X-ry AP view showing hmmer toes orretion (), right foot lterl view showing hmmer toes orretion () nd left foot lterl view showing hmmer toes orretion, we n lso see ilterl plntr support in weight-ering rdiogrph () foot lterl view (10 months post-opertive). At 24 months fter initil surgery, she omplined of right nkle pin de novo with weight er nd lol swelling. Rdiologilly, filed rthrodesis with tiiotlr nd sutlr one resorption, peri-implnt proximl rdiolueny, nd distl lneus srew frture were shown. No lol dringe or lood test infetion prmeters were found. Only symptomti tretment nd nonsteroidl nti-inflmmtory drugs were presried. At 38 months of follow-up, the ptient refers no signifint right nkle pin, nd no SCLS ttks reurrene ws registered (Fig. 6). Disussion This high-risk ptients nd the unpreditle ourse of the disese required multidisiplinry tretment options disussion. The ptient never epted irreversile utonomy loss nd ws wre of ll the possile negtive surgery onsequenes when she signed the surgery onsent. There re few tretment options for long-term sequele of omprtment syndrome tht inlude rthrosopi ssisted rthrodesis, fusion with n Figure 6: Plin rdiogrphs showing right nkle filed rthrodesis t 38 months post-opertive: Right nkle AP view X-ry showing tiiotlr filed rthrodesis with one resorption nd peri-implnt proximl rdiolueny () nd right nkle lterl view X-ry showing filed rthrodesis with tiiotlr nd sutlr one resorption, periimplnt rdiolueny, nd distl lneus srew frture (). externl fixtor or internl devies suh s pltes or intrmedullry nils. Arthrosopi rthrodesis is n exellent option when spring the soft tissues is needed; however, this orretion is limited to mild deformities. Externl fixtion represents serious risk of pin trt infetion with no 54 Journl of Orthopedi Cse Reports Volume 7 Issue 2 Mr - Apr 2017 Pge 52-56

4 onsensus out prevention tretment [8], nd therefore we dvote s potentil risk for SCLS relpse. Fusion with plte nd srews implies onsiderle ggression to the lredy dmged soft tissues, lthough it represents high stiffness onstrut. The uthors mnged this linil se s n equino-vrus-voddutus deformity fter lower extremity omprtment syndrome. Retrogrde niling voided extensive striping nd Berend et l. hve demonstrted etter iomehnis stiffness ompred to rossed srews [9]. Wng et l. [6] desried exellent stisftion rte fter retrogrde niling for lower extremity omprtment sequele. No tendinous trnsfers were onsidered due to neurologil defiit, srred musles, nd stiff joints. Clw toes deformity hs high risk of pressure ulers y shoe wer onflit. Lol infetion in SCLS ptients my represent potentil trigger for relpse nd therefore must e orreted. Considering the stiff interphlngel joints, hllux lw deformity ws mnged y ilterl Moerg osteotomy. Lesser toes were ddressed y resetion rthroplsty of PIP joints with the exeption of the stiffer left seond nd third toes tht were fixted with n intrmedullry guide implnt [10]. Fusion rte of only 50% (1/2 nkles) ws low ompred to nother pulished study tht shows rtes round 91% of the primry ony union fter ilterl nkle rthrodesis. However, none of these ptients hd post-omprtment syndrome nkle deformity nd relted sequele [11]. With no signs of infetion, lte filed rthrodesis my e relted to omprtment syndrome, lol vsulr nd neurologil dmge, tehnil error or even SCLS per se. Three minor omplitions were registered. One lrge posteromedil lister required skin grfting 6 weeks fter right nkle proedure. A left nkle lterl surgil wound dehisene heled y seondry intention only with dressing hnges. Filure of left hllux Moerg osteotomy stple ws mnged y hrdwre removl. These omplitions re reltively ommon nd hve een previously desried in foot nd nkle deformity orreting rthrodesis [6, 12]. They re usully due to poor skin nd vsulr onditions. No delyed weight ering ws notied in rehilittion progrm due to prompt nd suessful tretment. Conerning deformity orretion, the results were very stisftory. The AOFAS sore inresed from 20 to totl of 61 points even fter tiiotlolnel rthrodesis tht does not llow ny hind foot movement. Moreover, the pinless plntr support permitted wlking without ruthes fter 24 months onfined to wheelhir. Returning to work ws lso n importnt milestone for this ptient. At 38 months of follow-up, the ptient is now symptomti with minor lol right nkle edem nd no wlking limittion. Rdiologilly, the one resorption ppers to hve stilized (Fig. 6). Anedotl evidene hs shown good results in nkle rthrodesis revision fter omprtment syndrome [6]. However, one must onsider unpreditle onsequenes of fusion revision in SCLS ptient, inluding severe ttk during the nesthesi or post-opertive infetion with potentil lethl onsequenes. This ptient hs een dignosed with idiopthi form of SCLS. Although no diret use ws identified, she hd een tking zithromyin for respirtory trt infetion whih my represent potentil trigger [4, 13]. Monolonl gmmopthy ws lso present t follow-up l lood tests s seen in more thn 90% of ses [2, 4]. There re severl possile prophylti tretments, whih inlude B2-gonists, thlidomide, lium hnnel lokers nd hemotherpy with limited evidene onsidering the rrity of the disese. Intrvenous immunogloulin ws dministered in the pst 12 months of follow-up with no relpses dding to the hypothesis tht this my represent n effetive prophylxis [4, 14]. Limittions of the study This study hs severl limittions: (1) It presents the results of only one ptient nd therefore lks the ility to generlize, (2) low qulity evidene-sed knowledge out the syndrome nd its tretment limits the possiility to estlish use-effet reltionship, (3) we present limited follow-up time of 38 months with lte filed rthrodesis tht hs not een reoperted yet, nd (4) it is not possile to predit the result of right nkle filed rthrodesis revision. Conlusion Bilterl nkle fusion improved this SCLS ptient funtionl outome y restituting the wlking pity. One lte filed rthrodesis ws the mjor omplition nd my require seondry proedure lthough the ptient is urrently symptomti. Four surgeries were performed with no SCLS ttks reurrene registered t 38 months follow-up period. Prophylti humn immunogloulin my hve ontriuted to the sene of relpses. Although surgil orretion of SCLS skeletl deformities sequele represents n orthopedi hllenge, we elieve tht this tretment should e onsidered in previously utonomous highly motivted tive ptients. Clinil Messge SCLS is rre ondition tht n led to lower lim deformity, wlk impirment nd deresed qulity of life. Although deformity orretion surgery my represent life-thretening omplitions, lifehnging results re possile nd should e pursued. 55 Referenes 1. Clrkson B, Thompson D, Horwith M, Lukey EH. Cylil edem nd shok due to inresed pillry permeility. Am J Med 1960;29: Atkinson JP, Wldmnn TA, Stein SF, Gelfnd JA, Mdonld WJ, Hek LW, et l. Systemi pillry lek syndrome nd monolonl IgG gmmopthy; Studies in sixth ptient nd review of the literture. Mediine (Bltimore) 1977;56(3): Snghvi R, Anemn A, Prr M, Dunlop L, Chmpion D. Systemi pillry lek syndrome ssoited with omprtment syndrome nd rhdomyolysis. Anesth Intensive Cre 2006;34(3): Gousseff M, Arnud L, Lmert M, Hot A, Hmidou M, Duhut P, et l. The systemi pillry lek syndrome: A se Journl of Orthopedi Cse Reports Volume 7 Issue 2 Mr - Apr 2017 Pge 52-56

5 series of 28 ptients from Europen registry. Ann Intern Med 2011;154(7): Kyeremnteng K, D Egidio G, Wn C, Bxter A, Rosenerg H. Comprtment syndrome s result of systemi pillry lek syndrome. Cse Rep Crit Cre 2016;2016: Wng X, M X, Zhng C, Hung JZ, Gu XJ, Jing JY. Ankle fusion with retrogrde loked intrmedullry nil for sequel of lower extremity omprtment syndrome. Chin J Trumtol 2012;15(3): Lintz F, Colomier JA, Letenneur J, Gouin F. Mngement of longterm sequele of omprtment syndrome involving the foot nd nkle. Foot Ankle Int 2009;30(9): Jennison T, MNlly M, Pndit H. Prevention of infetion in externl fixtor pin sites. At Biomter 2014;10(2): Berend ME, Glisson RR, Nunley JA. A iomehnil omprison of intrmedullry nil nd rossed lg srew fixtion for tiiotlolnel rthrodesis. Foot Ankle Int 1997;18(10): Coillrd JY, Petri GJ, vn Dmme G, Deprez P, Lffenêtre O. Stiliztion of proximl interphlngel joint in lesser toe deformities with n ngulted intrmedullry implnt. Foot Ankle Int 2014;35(4): Henrison A, Kmrd I, Rosengren B, Crlsson Å. Bilterl rthrodesis of the nkle joint: Self-reported outomes in 35 ptients from the Swedish nkle registry. J Foot Ankle Surg 2016;55(6): Asomugh EU, Den Hrtog BD, Junko JT, Alexnder IJ. Tiiotlolnel fusion for severe deformity nd one loss. J Am Ad Orthop Surg 2016;24(3): Amour Z, Ppo T, Ninet J, Htron PY, Guillumie J, Piette AM, et l. Systemi pillry lek syndrome: Report on 13 ptients with speil fous on ourse nd tretment. Am J Med 1997;103(6): Mrr AM, Gignte A, Rosto E. Intrvenous immunogloulin in systemi pillry lek syndrome: A se report nd review of literture. Expert Rev Clin Immunol 2014;10(3): Conflit of Interest: Nil. Soure of Support: None How to Cite this Artile Sorl L, Ângelo AC, Cetno A, Rodei J, Ros I. Bilterl Ankle Fusion in Lek Syndrome Indued Deformity: A Cse Report. Journl of Orthopedi Cse Reports 2017 Mr-Apr;7(2): Journl of Orthopedi Cse Reports Volume 7 Issue 2 Mr - Apr 2017 Pge 52-56