Tethering tracts in spina bifida occulta: revisiting an established nomenclature.
ABSTRACT The goal of the present study goal was to systematically confirm the previously recognized nomenclature for tethering tracts that are part of the spectrum of occult spinal dysraphic lesions.
The tethering tract in 20 patients with spina bifida occulta underwent histological examination with H & E staining and epithelial membrane antigen (EMA) immunolabeling, and additional selected specimens were stained with Masson trichrome.
All tethering tracts contained fibrous connective tissue. Four tracts were lined with epithelial cells and either originated within a dermoid cyst, terminated at a skin dimple/sinus opening, or had both of these characteristics. No tethering tracts exhibited EMA positivity or meningeal elements. Although all tethering tracts originated in juxtaposition to the spinal cord, their termination sites were variable.
Based on histological findings and presumed embryological origin, the authors broadly classified tethering tracts terminating within the dura mater, epidural space, or lamina as "short tethering tracts" (STTs). The STTs occurred mostly in conjunction with split cord malformations and had a purely fibrous composition. Tethering tracts terminating superficial to the overlying lamina were classified as "long tethering tracts" (LTTs), and the authors propose that these are embryologically distinct from STTs. The LTTs were of two varieties: epithelial and nonepithelial, the former being typically associated with a skin dimple or spinal cord (epi)dermoid cyst. In fact, analysis of the data suggested that not every tethering tract terminating in or on the skin should be classified as a dermal sinus tract without histological confirmation, and because no evidence of meningeal tissue-lined tracts was detected, the use of the term "meningocele manqué" may not be appropriate.
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ABSTRACT: Congenital dermal sinus (CDS) tracts most commonly involve the midline and may communicate with the central nervous system. The authors present a 1y 6 mo-old boy with three pits in the gluteal region noted since birth. Complete excision of the sinus tracts was done. Microscopic examination of the right lateral buttock pit revealed a sinus tract lined by stratified squamous epithelium. Although laterally placed CDS in the buttocks is extremely rare with only six previous cases reported, lateral CDS should be included in the differential diagnosis of a dimple in the buttocks. This location is unusual, and the present report reiterates the fact that congenital dermal sinus tracts prone to having a connection with the central nervous system may present quite laterally and with more than one ostia. To the best of authors' knowledge, this is only the second case of congenital dermal sinus with triple ostia in a single patient.The Indian Journal of Pediatrics 07/2012; DOI:10.1007/s12098-012-0857-y · 0.72 Impact Factor
Article: Spinal inclusion cysts.[Show abstract] [Hide abstract]
ABSTRACT: Dermoid and epidermoid tumours are related though pathologically distinct entities that can be combined under the term inclusion cysts. Most are of developmental origin and in the spine may be intramedullary, intradural extramedullary or extraspinal in location. The terminology, aetiology, radiology and optimal management of spinal inclusion cysts have been the source of ongoing controversy. A retrospective review of surgical cases from a single institution has been conducted, and this experience is discussed in the context of the existing literature. Sixteen cases of paediatric spinal inclusion cysts were treated between 2002 and 2012. Associated anomalies included dermal sinus track (11), previous myelomeningocele (4) and Currarino syndrome (1). The lesions spanned 1-8 spinal levels; in half the cases, there was a history of secondary infection at the time of presentation. The aim of treatment was complete excision, and macroscopic removal was achieved in all cases. The most significant adverse outcome was related to sphincter dysfunction, and this was observed in four cases, two with a prior history of myelomeningocele and two who had presented with active infection. No instances of recurrence were recorded during a median follow up of 7.4 years (range 1-11 years). Spinal inclusion cysts are aetiologically diverse lesions, though in paediatric practice, most occur in association with a dysraphic anomaly. There are no consistent clinical or radiological features that will reliably distinguish between dermoid and epidermoid, and both may result in symptoms from mass effect or infection. The recurrence rate is low if complete macroscopic resection can be achieved.Child s Nervous System 09/2013; 29(9):1647-55. DOI:10.1007/s00381-013-2147-z · 1.24 Impact Factor
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ABSTRACT: BACKGROUND: Spinal dermal sinuses consist of an epithelium-lined tract extending from the skin towards the spinal cord, often resulting in infections or tethered cord syndrome. Recently, a variant called dermal sinus-like stalk was described as an analogous tract but not containing an epithelium-lined lumen. AIMS: We aimed to describe the findings in our patients, subdivide our specimens into both conditions, compare the characteristics of both groups and search for possible embryologic mechanisms of dermal sinus-like stalks. METHODS: We performed a retrospective analysis of all patients operated in our hospital for both conditions between 1996 and 2012. RESULTS: 14 patients were operated upon for spinal dermal sinuses (n = 5) and spinal dermal sinus like-stalks (n = 9). Patients were mainly referred from other hospitals due to skin abnormalities and were evaluated at mean age of 7 weeks and operated upon at mean age of 1 year and 2 months. Primary reason for referral was skin abnormalities in both groups, though there were two cases of meningitis in dermal sinus patients and 2 of recurrent urinary tract infections in dermal sinus-like stalk patients. Consistent with previous findings, dermal sinus-like stalk patients do not have a history of meningitis, lack dermoid or epidermoid tumours along their tract, and are histologically of pure mesodermal origin. Dermal sinus-like stalks might result from interposition of mesenchyme during primary or secondary neurulation. CONCLUSIONS: We consider dermal sinus-like stalks as a rare but currently under diagnosed condition with different clinical, pathological and probably also embryologic characteristics compared to spinal dermal sinuses.European journal of paediatric neurology: EJPN: official journal of the European Paediatric Neurology Society 05/2013; 17(6). DOI:10.1016/j.ejpn.2013.04.003 · 2.01 Impact Factor