Surgical Neurology International

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1 Surgicl Neurology Interntionl SNI: Peditric Neurosurgery, supplement to Surgicl Neurology Interntionl OPEN ACCESS For entire Editoril Bord visit : Spinl dorsl derml sinus trct: An experience of 21 cses Ishwr Singh, Seem Rohill 1, Prshnt Kumr 2, Surh Shrm Editor: Sndi Lm, M.D. Bylor College of Medicine; Houston, TX, USA Deprtments of Neurosurgery, 1 Rdiodignosis, nd 2 Anesthesiology nd Criticl Cre, Pt. B.D. Shrm University of Helth Sciences, Rohtk, Hryn, Indi E mil: *Ishwr Singh drishwrsingh@yhoo.co.in; Seem Rohill seemrohill@yhoo.co.in; Prshnt Kumr pk.pgims@yhoo.com; Surh Shrm shu @gmil.com *Corresponding uthor Received: 26 Jnury 15 Accepted: 16 June 15 Pulished: 07 Octoer 15 This rticle my e cited s: Singh I, Rohill S, Kumr P, Shrm S. Spinl dorsl derml sinus trct: An experience of 21 cses. Surg Neurol Int 2015;6:S Copyright: 2015 Singh I. This is n open ccess rticle distriuted under the terms of the Cretive Commons Attriution License, which permits unrestricted use, distriution, nd reproduction in ny medium, provided the originl uthor nd source re credited. Astrct Bckground: Spinl dorsl derml sinus is rre entity, which usully comes to clinicl ttention y cutneous normlities, neurologic deficit, nd/or infection. The present study ws undertken to know the clinicl profile of these ptients, to study ssocited nomlies nd to ssess the results of surgicl intervention. Methods: Medicl records of 21 ptients treted for spinl dorsl derml sinus from Septemer 2007 to Decemer 2013 were reviewed. Results: We hd 21 ptients with mle: femle rtio of 13:8. Only 2 ptients were elow 1 yer of ge, nd most cses (15) were etween 2 nd 15 yers (men ge = 8.2 yers). Lumr region (11 cses) ws most frequently involved, followed y thorcic (4 cses), lumoscrl, nd cervicl region in 3 ptients ech. All of our ptients presented with neurologicl deficits. Three ptients were dmitted with cute meningitis with cute onset prplegi nd hd intrspinl scess. The motor, sensory, nd utonomic deficits were seen in 14, 6, nd 8 ptients, respectively. Scoliosis nd congenitl tlipes equinovrus were the common ssocited nomlies. All ptients underwent surgicl explortion nd repir of dysrphic stte nd excision of the sinus. Overll, 20 ptients improved or neurologicl sttus stilized nd only 1 ptient deteriorted. Postopertive wound infection ws seen in 2 cses. Conclusions: All ptients with spinl dorsl derml sinuses should e offered ggressive surgicl tretment in the form of totl excision of sinus trct nd correction of spinl mlformtion, s soon s dignosed. Access this rticle online Wesite: DOI: / Quick Response Code: Key Words: Compliction, derml sinus, dysrphism, presenttion, spine INTRODUCTION Spinl dorsl derml sinus trct (DST) is rre congenitl dysrphism tht occurs in pproximtely one in every 2500 live irths. [1,7,12,17,18] It includes trct lined y epithelium, which trverse for vrile depth into the underlying structures nd in mny instnces, terminte within the thecl sc. [2,3] They re seen more frequently t the extremes of neurxis with the mjority of spinl DSTs occurring in the lumoscrl region. [6,7,9,17] Spinl DSTs my hve diverse nd occsionlly serious presenttions; in fct, mny cses come to clinicl ttention y neurologic deficit nd/or infectious complictions including life thretening conditions such s meningitis. [6] In ddition, DSTs re frequently ssocited with other nomlies of the centrl nervous system such s tethered cord, inclusion tumors, nd S429

2 split cord mlformtions (SCMs). [2,6] So despite its enign externl ppernce, it my hror gret risks to the ptients helth if not timely ddressed. The neurologicl exmintion is reported to e norml in the erly childhood. However, s the ge increses, there is more chnce of neurologicl deficit, which tends to e more profound. There re few pulished series in literture which emphsize minly the mode of presenttion, rdiologicl findings, ssocited nomlies nd tretment; however, the symptom wise outcome is not studied in detil. [1,3,6,12,17,18] The present study ws undertken to know the clinicl profile, ssocited nomlies nd detiled symptom wise outcome of the ptients presenting with spinl DST. PATIENTS AND METHODS This is retrospective study conducted in Pt. B.D. Shrm University of Helth Sciences, Rohtk from Septemer 2007 to Decemer Medicl records of ll ptients treted for spinl DST were reviewed. Informtion regrding ptients demogrphic vriles, type of presenttion, symptoms, physicl exmintion, rdiologicl nd surgicl findings, nd histopthologicl evlution were collected. Mgnetic resonnce imging (MRI) ws the investigtion of choice nd ws performed in ll cses. MRI reveled the reltionship of the derml sinus to the durl sc nd lso gve informtion regrding ssocited normlities in the cord like dysrphic stte of spine or inclusion tumor. Surgicl intervention The im of surgery ws to excise the sinus trct completely nd to correct the dysrphic stte in the sme sitting. Surgery ws performed in ll cses through midline incision with encircling the sinus. DST ws followed through the sucutneous tissue nd muscle lyer sinus trct ws trced until its end nd excised completely. The course of DST ws invrily rostrl through the incompletely formed lmin or underneth the norml lmin. After doing the lminectomy, dur ws opened in ll cses irrespective of end of DST. In cses where DST ws intrdurl, prt of dur encircling the DST ws excised. Intrspinl pthologies like SCM were delt ccordingly tht is, dermoid nd epidermoid were decompressed or excised; myelocele nd lipomeningomyelocele were repired; dringe of scess in intrmedullry scess, removl of rchnoid dhesion in rchnoiditis nd detethering of the cord ws done in cse of tethered cord. Those ptients presenting with infectious complictions were mnged with pproprite ntiiotics nd then fter recovery surgery for resection of DST, nd correction of ssocited nomlies ws performed. Postopertive follow up rnged from 6 months to 5 yers (men 2.8 yers). RESULTS Records of totl 21 ptients were nlyzed, of which 13 were mle, nd 8 were femle. Ptients ge on dmission rnged from 9 months to 15 yers (men 8.2 yers). Every ptient underwent detiled neurologicl exmintion nd complete rdiologicl workup to delinete ny underlying/ ssocited spinl normlities. DST ws locted most frequently in lumr region (11 cses) [Figure 1], followed y thorcic (4 cses), [Figures 2 nd 3] cervicl [Figure 4] nd lumoscrl region in 3 ptients ech [Tle 1]. It ws stonishing to note tht ll our ptients presented with neurologicl deficits [Tle 2]. Three ptients presented with cute meningitis nd cute onset prplegi. History of recurrent meningitis ws lso positive in two of these cses. Grdully progressing motor deficit ws seen in 14 cses. The deficit ws in the form of lim wekness nd trophy, with or without git disturnce. The sensory deficit ws seen in 6 cses. Eight ptients hd ldder/owel involvement t presenttion out of which five were incontinent t the time of presenttion. Associted skeletl nomlies were noticed in 5 cses. Scoliosis ws the most common finding nd ws seen in 4 cses, followed y congenitl tlipes equinovrus in 2 cses. In the mjority of the ptients (15), sinus ostium ws ssocited with nother skin normlity, the most common of which ws norml pigmenttion. Some ptients hd comintion of these findings. Derml sinuses were seen in conjunction with lipomyelomeningocele in 2 ptients. MRI ws the investigtion of choice nd ws performed in ll cses. It reveled the reltionship of the derml sinus to the durl sc nd lso gve informtion regrding ssocited normlities in the cord [Tle 3]. Epidermoid [Figure 2] nd dermoid tumor [Figure 3] were seen in 2 nd 8 cses respectively. SCM ws seen in 6 cses nd filum normlity in 2 cses [Tle 4]. Motor deficit (present in 14 cses) stilized in 7 cses nd improved in 6 cses [Tle 5]. Two ptients developed fresh deficits in the postopertive period, nd one of them improved to preopertive sttus 3 months lter. The sensory improvement ws seen in 2 cses nd sensory deficits stilized in 4 cses. Neurologic function grdully returned to ner norml stte postopertively in 2 of 3 ptients who presented with cute prplegi with incontinence of urine nd stool, ut there ws no improvement in ldder nd owel function in ll 3 ptients. Of the other 5 incontinent ptients, 1 improved, nd 4 remined the sme. Overll, 11 ptients showed neurologicl improvement, 9 ptients stilized neurologiclly while 1 ptient deteriorted [Tle 6]. Improvement in ny of the neuorologicl prmeters viz motor, sensory or owel/ ldder symptoms ws considered to e n improvement. S430

3 Figure 1: () Spinl derml sinus trct of lumr region with lipomyelomeningocele. () Mgnetic resonnce imging (T1-weighted imge) showing the tethered cord due to stlk extending from the sinus to the indrdurl spce long with lipomyelomeningocele Figure 2: () Spinl derml sinus trct of thorcic region. () Mgnetic resonnce imging (T2-weighted imge) showing intrdurl epidermoid tumor ttched to the thin stlk extending from the sinus Figure 3: () Spinl derml sinus trct of thorcic region. () Mgnetic resonnce imging (gdolinium enhnced T1-weighted imge) showing intrdurl dermoid tumor with intrmedullry scess Tle 1: Distriution of spinl DSTs (n=21) Spinl level Frequency (%) Cervicl 3 (14.2) Thorcic 4 (19) Lumr 11 (52.3) Lumoscrl 3 (14.2) DST: Derml sinus trcts Tle 2: Neurologicl signs nd symptoms in ptients with derml sinus (n=21) Sign nd symptoms Frequency (%) Acute meningitis with cute prplegi 3 (14.2) Motor wekness 14 (66.6) Sensory wekness 6 (28.5) Clu foot/scoliosis 5 (23.8) Incontinence of urine/stool 5 (23.8) Constiption 3 (14.2) DISCUSSION A spinl DST consists of trct lined y strtified squmous epithelium found on or ner the midline nd is thought to result from the norml dhesions (or incomplete disjunction) etween the neuroectoderm (destined to form the neurl tue) nd Figure 4: () Spinl derml sinus trct of cervicl region with rudimentry meningocele. () Mgnetic resonnce imging (T2-weighted imge) showing the tethered cord due to stlk extending from the sinus to the indrdurl spce nd ttch to the ony spur the cutneous ectoderm. [4,10,11,15] The inwrd extent of the trct depends upon the extent of dhesions nd my vry from deep fsci to the spinl cord. The trct elongtes during the development, due to scent of the cord nd my trverse severl levels within the epidurl spce efore entering the surchnoid spce. Disorder of the notochord formtion with sgittl splitting of the spinl cord nd persistence of the dorsl cutneo endo mesenchyml fistul hs lso een suggested s cuse of derml sinus formtion. [11,16] The squmous lining of spinl DST my e encsed in derml nd neurologicl tissue. Within the trct, one my find nerve or gnglion cells or ft, lood vessels, crtilge nd meningel remnnts. [14] Spinl DST my e ssocited with other normlities of the ectoderml, mesoderml or neurl crest derivtives such s meningomyelocele or lipomeningomyelocele, reflecting common ontogenic disorder. Nerly, 60% of the DSTs enter the surchnoid spce nd 27% re ttched to the neurl elements of the conus, cud equin or filum terminle. [1,5,17,19] The trct my end lindly within the extrdurl spce in 10 20% cses. [6,17] Sinus trcts cn occur nywhere from occiput to scrum. Different studies showed tht cervicl re is lest involved (<1% cses). Thorcic re is involved in 10% cses, lumr nd lumoscrl re in 40% nd 12% ptients respectively, scrum in 23% nd scrococcygel junction in 13% of cses. [6] In the present study, the cervicl region ws involved in 14.2% cses, S431

4 the lumr region in 52.3% cses, thorcic region in 19% cses nd lumoscrl region in 14.2% cses. The higher incidence of DST in cervicl re in the present study could e due to selection is s cervicl DST cses re lwys symptomtic while other uthors might include symptomtic lumoscrl cses. Derml sinuses should e distinguished from the more common coccygel pits. Derml sinuses re locted ove the interglutel cleft nd hve cephliclly oriented course nd re often ssocited with other pthologies. On the contrry, coccygel dimples re usully simple lind sinuses with no ssocited cutneous normlities tht lie within interglutel cleft few millimeters crnil Tle 3: MRI finding in ptients with derml sinus (n=21) MRI finding Frequency (%) Epidermoid 2 (9.5) Dermoid 8 (38) Thick filum 5 (23.8) SCM 5 (23.8) Lipomeningocele 2 (9.5) Syrinx 6 (28.5) Intrspinl scess 3 (14.2) MRI: Mgnetic resonnce imging, SCM: Split cord mlformtion Tle 4: Intropertive finding in ptients with derml sinus (n=21) Intropertive finding Frequency (%) Tethered cord 13 (61.9) Epidermoid 2 (9.5) Dermoid 8 (38) Intrmedullry scess 2 (9.5) Intrdurl grnultion tissue 1 (4.76) SCM 5 (23.8) Archnoiditis 4 (19) SCM: Split cord mlformtion Tle 5: Symptom wise surgicl outcomes in ptients with derml sinus (n=21) Function Numer Improved (%) Sme/ stilized (%) Worsened (%) Bldder nd owel 8 1 (12.5) 7 (87.5) Sensory 6 4 (66.6) 2 (33.4) Motor 14 6 (42.8) 7 (50) 1 (7.1) Totl (39.2) 16 (57.1) 3.5 to the tip of coccyx. They re oriented cudlly or stright nd re not ssocited with other intrdurl pthologies nd thus do not wrrnt further evlution. [2,3,15] They my rrely hve intrspinl extension, so it should e rememered tht not ll coccygel pits cn e dismissed. Another chrcteristic tht differentite coccygel dimple from DST is loction. Coccygel pits re lwys in midline while DST is not strictly midline nd should e investigted with high qulity MRI. If scrl or coccygel dimple is ssocited with other cutneous normlities such s hypertrichosis or soft tissue mss, they should e investigted ccordingly. Derml sinuses provide portl of entry for cteril gents into the intrspinl comprtments tht cn cuse meningitis or scess formtion tht my e extrdurl, sudurl, nd intrmedullry or infection of ssocited tumor. Also, septic meningitis cn occur y spillge of inclusion tumor contents or other derml elements into the cererospinl fluid. [7,9,19] Therefore, one should hve high level of suspicion for DST nd dermoids when encountering ny young child presenting with septic meningitis. In the study conducted y Jindl nd Mhptr [6] only 1 ptient presented with infection out of 26 ptients. Ackermn nd Menezes [2] lso hd low rte (10%) of infectious complictions. In the series of Rdmnesh et l., [17] 37.1% hd meningitis on dmission or hd experienced it efore while 25.7% hd scess formtion. The incidence of infection (meningitis) in our ptients ws 14.2%, ll our infected ptients hd scess of which two were intrmedullry. It hs een sid tht nerly ll children with spinl DSTs hve intct neurologicl function t irth. [6] However, due to the reltively high rtes of ssocited pthologies such s tethered cord, infection, nd inclusion tumors, neurologicl deteriortion ecomes more common with incresing ge. It hs een shown tht the chnces of developing neurologic deficit re higher in ptients who present in older ges. [2] Ackermn nd Menezes [2] studied the referrl pttern mong their ptients nd noted tht ptients who were younger thn 1 yer were more likely to e neurologiclly intct thn older ones, concluding tht dely in the dignosis llows for development of neurologic sequele. Proly this my e the reson tht ll of our ptients presented with neurologicl deficits s 90% of our ptients were more thn 1 yer of ge which my due to lck of wreness t the primry helth cre level, which leding to delyed referrl. Unfortuntely, Tle 6: Comprison of surgicl outcome with different previous series Ackermn (%) Jindl (%) Rmnryn (%) Mete (%) Presentseries (%) Neurologiclly symptomtic 11 (39) 3 (13) 1 (11) 12 (75) Neurologiclly improved 12 (43) 8 (34.7) 4 (44.4) 11 (52.3) Neurologiclly stilized 2 (7) 14 (60.8) 4 (44.4) 4 (25) 9 (42.8) Neurologiclly worsened 3 (11) 1 (4.3) 1 (5) S432

5 once ptient develops neurologic deficit, there is reltively high chnce of permnent defect. [5,6] Spinl derml sinuses my e ccompnied y other forms of spinl dysrphism such s lipomyelomeningocele nd myelomeningocele, reflecting possile common ontogenic pthwy. [17] Gupt et l. showed n ssocition of 11.34% etween derml sinus nd other forms of spinl dysrphism. [5] The proposed mechnism for lipomyelomeningocele emryogenesis lso includes disorders of disjunction tht occurs premturely in this entity. It is possile tht there re some shred moleculr pthwys responsile for concurrence of these nomlies. Derml sinuses re occsionlly ssocited with tethered cord, lthough only 1% of ptients with tethered cord hve dorsl derml sinus. [2] In ptients with DST, the trct or ssocited tumor my cuse trction on spinl cord resulting in low lying conus nd tethered cord syndrome. [8] In our study, 13 ptients (61.9%) hd tethered cord. It is reported tht up to 40% of ptients with DST cn hve SCM. [3,17] Conversely, DSTs re seen in 15 40% of SCM. [19] Among our ptients, five hd SCM, three with Type 1, nd two with Type 2. The incidence of filum terminle normlities ws descried y Jindl nd Mhptr [6] nd Rdmnesh et l. [17] Jindl nd Mhptr [6] found filum normlities in 22% of his ptients while Rdmnesh et l. [17] found filum terminle normlity in 40% cses. In the present study, the filum terminle normlities were encountered in 5 cses (22%). The term tight filum terminle refers to set of conditions in which low lying conus medullris is ssocited with short thickened filum without evidence of other tethering pthologies. [7,8] This entity tht rises from filed regression of cudl spinl cord during secondry neurultion cuses typicl signs nd symptoms of tethered cord. Approximtely, hlf of ll derml sinuses re ssocited with dermoid or epidermoid tumor, usully t the termintion of these trcts, ut they my e locted nywhere etween the skin nd the neurl tue. [2,6,13,17] Derml sinuses nd dermoid tumors seem to shre common origin. [9] They re elieved to result from focl expnsion of these ectoderm derived trcts. However, only pproximtely 30% of intrspinl dermoid tumors hve n ssocited sinus trct. [7] DSTs re ssocited more frequently with dermoid tumors (83%) thn with epidermoid (13%). [5] In the present study, two of our ptients (9.5%) hd epidermoid tumors while eight hd dermoids (38%) proved y histology. Postopertive complictions were few nd esy to mnge. Our results indicte tht once ptient developed owel/ ldder incontinence, there ws out 12.5% chnce of improvement in deficit while in ptients with sensory or motor deficits; the chnce of improvement ws 66.6% nd 42.8%, respectively [Tle 5]. The risk of neurologicl deteriortion ws only 3.5%. The ptients presenting t lter ge hd more chnce of developing deficits. We hve lso compred the neurologicl outcomes in the different previous series with our study [Tle 6]. In our study, the overll neurologicl improvement is etter thn the previous study tht my e due to selection is s ll our ptients were symptomtic. However, owel/ldder improvement ws seen in only 1 ptient due to the delyed presenttion. Since none of imging modlities cn ccurtely show intrspinl detils, ll derml sinuses ove the scrococcygel region should e explored opertively regrdless of neuroimging findings. [3,7,17] One should hve high index of suspicion for ll the dimples ove the interglutel fold, despite norml exmintion or neurordiologic studies. Midline should e crefully exmined whenever child suffers from meningitis, especilly when n unusul orgnism is cultured. Conservtive tretment of spinl DST is not recommended. Surgery should e crried out prophylcticlly in dvnce of deficits, to mintin norml neurologicl function. CONCLUSION Spinl DST is n innocuous ppering spinl dysrphism tht my contriute to devstting moridities if not timely ddressed. 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