Case report Paediatrics Today 2015;11(1):59-65 DOI /p

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1 Cse report DOI /p UNUSUAL PRESENTATION OF SPLIT CORD MALFORMATION TYPE I ASSOCIATED WITH MYELOMENINGOCELA Hkij BEČULIĆ 1, Rsim SKOMORAC 1, Aldin JUSIĆ 1, Alm MEKIĆ-ABAZOVIĆ 2, Fhrudin ALIĆ 1, Eldin BURAZEROVIĆ 3, Alm VOLJEVICA 4, Lejl BEČULIĆ 5 1 Deprtment of Neurosurgery, 2 Deprtment of Oncology, Hemtology nd Rdiotherpy Cntonl Hospitl Zenic, Zenic, Bosni nd Herzegovin; 3 Clinic of Neurosurgery Clinicl center University of Srjevo 4 Institute of Antomy, Fculty of Medicine Srjevo, Srjevo, Bosni nd Herzegovin 5 Institute for Occuptionl nd Sports Medicine of Zenic-Dooj Cnton, Zenic Bosni nd Herzegovin Corresponding uthor: Hkij Bečulić Deprtment of Neurosurgery Cntonl Hospitl Zenic Zenic Bosni nd Herzegovin dr_eculichkij@hotmil.com Tel.: Fx.: Received: Septemer 2, 2014 Accepted: Jnury 19, 2015 Copyright 2015 y University Clinicl Centre Tuzl. E-mil for permission to pulish: peditricstody@ukctuzl. Ojective We report n unusul cse of split cord mlformtion (SCM) ssocited with open spinl dysrphism nd other nomlies of the centrl nervous system. Cse report A mle neworn ws dmitted to the Peditric Intensive Cre Unit immeditely fter irth y Cesren delivery. Clinicl exmintion nd dignostics (MRI) showed open spinl dysrphism in the distl prt of the spine (level L5 nd S1), split cord mlformtion type I which seprted two hemicords, tethered cord nd syringomyeli. Two dys fter irth microneurosurgery ws performed. Crnil nd cervicl MRI showed Chiri I nd corpus cllosum hypoplsi. There ws no hydrocephlus. Preopertively the ptient hd prpresis nd discretely moved his left foot. MRI showed dilted ldder so he my hve hd urinry retention. The postopertive recovery ws stisfctory. The ptient did not hve ny dditionl neurologicl deficit. The ptient ws followed up y neurosurgeon, peditric surgeon, peditricin nd physitrist. Control MRI scns showed significnt regression of the syrinx nd some scensus of the medull. Aout 18 months fter opertion the ptient hd discrete presis of the left foot. Conclusion Complex spin ifid is n extremely rre condition. In this pper we descrie this interesting cse of complex spin ifid: split cord mlformtion chrcterized y typicl ony morphology with durl fold into the one septum. Key words: Unusul spinl dysrphism Surgicl tretment Outcome. Introduction Split cord mlformtion (SCM) or distemtomyeli is rre spinl nomly nd refers to sgittl division of the spinl cord into two symmetricl or symmetricl hemicords (1). SCM re of two types. Type I consists of two hemicords, ech contined within its own durl sheth nd seprted y medin ony spur. Type II consists of two hemicords housed in single durl tue seprted y firous medin septum (2). In the sence of ssocited cutneous stigmt tht prompts the clinicins to request screening imging for the spinl cord, SCM nomlies re usully dignosed in reltively older children. Although their precise incidence is unknown, SCMs re rre normlities, representing 3.8% to 5% of ll spinl cord nomlies (2-4). It is form of occult spinl dysrphism, ut it my coexist with open spinl dysrphism, such s meningomyelocele (complex spin ifid) (4). 59

2 Open spin ifid or myelomeningocele (MMC) is devstting congenitl defect of the centrl nervous system. It is chrcterized y protrusion of the meninges nd spinl cord through open verterl rches (5). The incidence of myelomeningocele is out 1 per 1000 irths. SCM my present with MMC (complex spin ifid) or without MMC s n occult nomly. These nomlies re treted surgiclly. The gol of erly surgicl tretment is medul detethering, to prevent cererospinl fluid (CSF) lek nd meningitis, s well s to prevent neurologicl deteriortion in the future (3-6). We report n unusul cse of SCM ssocited with open spinl dysrphism nd other nomlies of the centrl nervous system. Cse report A mle neworn ws dmitted to the Peditric Intensive Cre Unit immeditely fter irth y Cesren delivery. Gesttionl ge ws 39 weeks. His APGAR score ws 8 nd 9 t 1 nd 5 min, his weight ws 3470 grms, length 50 cm, nd hed circumference 34.5 cm. In the lumoscrl re n open spinl dysrphism (MMC) ws detected. The ptient ws exmined y neurosurgeon who indicted Mgnetic Resonce Imging (MRI). MRI showed open spinl dysrphism (MMC) in the distl prt of the spine with n rch defect t L5 nd S1. SCM type I which seprted two symmetricl hemicords, tethered cord nd syringomyeli (Fig. 1). The conus medullris ws locted t the level of inferior prt of the S2 verter. The syrinx ws locted throughout the spinl cord. In the left hemicord it ws 4.2 x 3.7 mm, in the thorcl prt 5.6 x 8.1 mm nd in the cervicl prt of the spinl cord there ws discrete dilttion of the centrl cnl. There were no other spinl nomlies. Two dys fter irth we performed microneurosurgery. Generl nesthesi ws used. The ptient ws plced in the prone position with rolls under the chest nd hips. In the lumoscrl re there ws n MMC (40 x 35 mm) (Fig. 2). We mde n ellipticl incision oriented verticlly (long the xis of the MMC) just outside the norml skin. The incision ws crried through the sucutneous tissue to c 1 Fig. 1 Preopertive MRI of lumoscrl spine. () Sgittl T2 weighted (1) n open spinl dysrphism in the distl prt of the lumoscrl spine; (2) one septum t the level of the L4 verter; (3) syringomyeli () Axil T2 weighted - (1) split cord mlformtion type I with two hemicords (c) Axil T2 weighted (1) n open spinl dysrphism. 60

3 H. Bečulić et l. Split cord mlformtion Everted dur Myelomeningocel c d Spinl cnl deffect Neurl plcode Fig. 2 Surgicl repire of myelomeningocele. the lyer of everted dur nd fscil defect. We moilized the se of the sc medilly until the fscil defect ws visile. The skin nd the epithelium zone were excised round the neurl plcode. We reconstructed the neurl plcode nd nother prt of the neurl tissue nd folded them into the tue. Then we closed the pi-rchnoide junction nd the dur ws closed in wtertight mnner using 4/0 suture (Fig. 2). After this we mde verticl skin incision ove the L3 nd L4 verter nd retrcted the prspinl muscles lterlly. Crefully we mde L4 lminotomy round the one septum nd exposing the durl cleft. Ech hemicord hd its own durl sheth. Using the dissector we detched the septum from the surrounding dur. Then we removed the superficil prt of the one septum using rnger nd showed nother durl fold in the one septum. The durl fold ws prt of the right hemicord nd entered in the one septum t its se (Fig. 3). Fig. 3 An unusul split cord mlformtion. () Tuulr osseous septum which spred long whole sgittl dimeter of the spinl cnl; () durl fold into the osseous septum (which is prt of the right hemicord); (c) the left hemicord; (d) the right hemicord, which is symmetric. We crefully removed the ony septum piece y piece from the verterl ody, Then fsci ws closed s seprte lyer using it lterlly in semicircle on oth sides, elevting it from the underlying muscle nd reflecting it medilly. The fsci ws closed with 4/0 suture. The skin ws moilized y lunt dissection nd closed. After the opertion, the ptient ws redmitted to the Peditric Intensive Cre Unit. In the postopertive period we performed cervicl nd crnil MRI. There were no normlities in the cervicl spine. Crnil MRI showed Chiri I nd Corpus cllosum hypoplsi (Fig. 4). There ws no hydrocephlus. Additionl dignostic nd dominl echosonogrphy did not show other nomlies. The postopertive recovery ws stisfctory. The ptient did not hve ny dditionl neurologicl deficit. After surgery, we strted erly physicl tretment. The ptient ws followed up y neurosurgeon, peditric surgeon, peditricin nd physitrist. At the time of the ltest check-up, the ptient hd discrete presis of the left foot. Control MRI scns (one yer fter surgery) showed significnt regression of the syrinx (in the left 61

4 c d Fig. 4 MRI presenttion of corpus cllosum hypoplsi nd Chiri I mlformtion (Sgitl T1 weighted): - corpus cllosum hypoplsi, - tonsil descensus; c C1 verter, d posterior rim of the formen mgnum. Fig. 6 The ptient 18 months fter surgery. c Fig. 5 Postopertive mgnetic resonnce imging one yer fter surgery. () Sgittl T2 weighted significnt scensus of the medull without one septum nd myelomeningocele; () Axil T2 weighted without ny one septum t the level of the L4 verter; (c) Axil T2 weighted significnt regression of syringomyeli. 62

5 H. Bečulić et l. Split cord mlformtion hemicord 1.1 x 1.2 mm, in the thorcl prt of medull 3.2 x 4.1 mm, complete regression in the cervicl prt of the spinl cord) nd some scensus of the medull (Fig. 5). Now the oy is out 18 months old, wlking with discrete presis of the left foot (Fig. 6). Periodicl check ups y echosonogrphy hve shown complete ldder emptying. Discussion Distemtomyeli ws first descried in 1837 y Olivier. The term is used to descrie developmentl mlformtion of the spinl cord tht is chrcterized y splitting of the cord into t lest two independent segments (6, 7). The term Split cord mlformtion (SCM) hs een used since 1992 (7). SCM usully occurs in the lower thorcic or upper lumr re (6, 7). Mny hypotheses hve een postulted for the genesis of these mlformtions. The most widely ccepted theory out the emryogenesis of SCM ws proposed y Png et l. (7, 8). This theory is founded on the presence of n nomlous neuroenteric cnl nd mintins tht the endomesenchyml trct cuses ll doule cord mlformtions (7). SCM re of two types: type 1 consists of two hemicords, ech contined within its own durl shethed tue nd seprted y medin ony spur, nd type 2 consists of two hemicords housed in single durl tue seprted y firous medin septum (4). SCM comprises 3.8% of ll spinl dysrphisms. SCM is known to e common mong women. In two seprte studies, the femle: mle rtio ws reported s 1.3:1 (10). SCM my e ccompnied y mny congenitl spinl disorders, such s low-lying cord, hydromyeli, lipom, meningomyelocele, thick filum terminle nd dermoid cysts (6, 9). The clinicl presenttions of split cord mlformtions cn e clssified s symptomtic or symptomtic. Asymptomtic presenttions re similr to those of other forms of occult spinl dysrphism. Symptomtic cses often present with progressive signs nd symptoms. The most common presenting symptoms re scoliosis nd tethered spinl cord syndrome (4, 5, 9). Myelomeningocele is form of open neurl tue defect nd it is the most severe congenitl mlformtion of the CNS comptile with survivl (10). A myelomeningocele results from n normlity in fusion of the emryologic neurl tue during the first month of gesttion. Filure of neurl tue closure results in sc-like hernition of the meninges (meningocele) or hernition of neurl elements (myelomeningocele). Adequte folic cid intke in the periconceptul nd erly pregnncy periods significntly reduces the risk of MMC (10-12). MMC most commonly occurs in the lumoscrl re. The verge worldwide incidence of open spin ifid is 1 cse per 1000 irths (10). Neurologicl impirments cused y MMC include muscle wekness of the legs (prlysis), owel nd ldder dysfunction, seizures (crnil normlities, hydrocephlus), orthopedic normlities (feet nd hip normlities, scoliosis) etc. (11, 12). SCM is not merely n entity of occult dysrphism nd it my coexist with open spinl dysrphism, such s MMC (complex spin ifid). Aout 40.8% cses of ll SCM present with MMC, suggesting tht the open nd closed forms of spinl dysrphism my coexist (4). The prognosis of SCM with MMC is not s good s in pure SCM cses. Progressive sensomotor deficit lso hs higher incidence in cses of SCM with MMC (3, 4). Chiri I nd II mlformtions re ssocited with myelomeningocele (out 30% cses), ut not with SCM. Hydrocephlus hs een more frequently oserved in ptients with SCM ssocited with MMC (26%), thn in those with pure SCM (11%) (4, 13). Syrinx is seen in 25% to 40% of p- 63

6 tients with spinl dysrphism, nd is more common in cses with oth SCM nd MMC (13, 15). Type I SCM consists of two hemicords seprted y one septum without the presence of other tissues (4). In this pper we present rre cse of typicl SCM type I, ssocited with myelomeningocele nd other CNS mlformtions (Chiri I, syringomyeli nd tethered cord). In our cse, the third durl fold pssed into the one septum. This durl fold elongs to the right hemicord. During microneurosurgery the one septum ws totlly resected without ny ventrl durl deficit. The septum occurred t the cudl end of the split cord, leving the more rostrl spect of the split free of ttchments. Then myelomeningocel ws repired in the clssicl mnner. In the postopertive period the ptient did not hve ny dditionl neurologicl deficits. After surgery, we strted erly physicl tretment. The ptient ws controlled y neurosurgeon, peditric surgeon, peditricin nd physitrist. Control MRI scns showed significnt regression of the syrinx nd some scensus of the medull. Now the oy is ged out 18 months old, he is wlking without presis nd without owel nd ldder control. In the literture different vritions of one septum hs een descried: doule septum, septum with dorsl or ventrl insertion, septum tht seprtes the durl sc into three segments, septum with one nd firous prts (1, 13-15). In the ville literture we did not find cse with durl fold pssing into the one septum. Conclusion Complex spin ifid is n extremely rre condition. MRI is highly useful tool for determintion of SCM nd its ccompnying spinl nomlies. In this pper we descrie n interesting cse of complex spin ifid: SCM chrcterized y typicl one morphology with durl fold into the one septum. Erly surgicl mngement of the comined split cord mlformtion with myelomeningocele provides good results if mnged dequtely with removl of ll tethering fctors. Authors contriutions: Conception nd design: HB nd RS; Acquisition, nlysis nd interprettion of dt: HB nd AJ; Drfting the rticle HB, AMA nd FA; Revising it criticlly for importnt intellectul content: HB, EB, AV nd LB. Conflict of interest: The uthors declre tht they hve no conflict of interest. References 1. Moriy J, Kked S, Korogi Y, Soejim Y, Urski E, Yokot A. An unusul cse of split cord mlformtion. AJNR. 2006; 27(7): Borkr SA, Mhptr AK. Split cord mlformtions: A two yers experience t AIIMS. Asin journl of neurosurgery. 2012;7(2): Alzhrni GA, Al-Jehni HM, Melncon D. Multilevel Split Cord Mlformtion: Do We Need New Clssifiction? J Clin Imging Sci. 2014;4: Kumr R, Singh V, Singh SN. Split cord mlformtion (occult spin ifid): n Indin scenrio. JK Science. 2005;7(4): Myer S, Weisser M, Till H, Grfe G, Geyer C. Congenitl myelomeningocele-do we hve to chnge our mngement. Cererospinl Fluid Res. 2010;7: Bnskot N, Jh R, Khdk N, Shrm GR, Bist P, Kumr P. Surgicl Mngement of Spinl Dysrphism: Five yer Experience in Centrl Hospitl. J Nepl Peditr Soc. 2014;34(1): Png D, Dis MS, Ah-Brmd M. Split cord mlformtion prt I: Unified theory of emryogenesis for doule spinl cord mlformtion. Neurosurgery. 1992;31: Mhptr AK. Split cord mlformtion A study of 300 cses t AIIMS J Peditr Neurosci. 2011;6: Ozturk E, Sonmez G, Mutlu H, Sildiroglu HO, Velioglu M, Bsekim CC, Kizilky E. Split-cord mlformtion nd ccompnying nomlies. J Neurordiol. 2008;35(3):

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