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.
[Show abstract][Hide abstract] ABSTRACT: CASE REPORT: We present a female baby with a human tail associated with congenital dermal sinus (CDS) at the caudal site of the tail. Magnetic resonance (MR) images with constructive interference in steady-state (CISS) sequencing clearly demonstrated a lumbosacral lipoma of caudal type, contiguous with the dermal sinus tract and not with the human tail. At 3 months old, the tail was surgically removed. The dermal sinus tract and contiguous lipoma were also resected, and untethering of the spinal cord was performed. Although both the human tail and CDS are frequently associated with spina bifida occulta, coexistence of the tail and CDS is exceedingly rare. CISS-MRI is useful for demonstrating the precise anatomical relationship between these complicated pathologies.
Child s Nervous System 09/2008; 25(1):137-41. DOI:10.1007/s00381-008-0693-6 · 1.11 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Congenital dermal sinus (CDS) usually develops in the midline of the body as a single tract. To date, only a few patients with multiple CDS tracts and ostia have been reported. The authors analyzed the clinical features of patients with spinal CDS and multiple ostia and proposed a novel hypothesis for the pathogenesis of the atypical CDS.
Five patients with spinal CDS and multiple ostia were included. The clinical, radiological, and operative features of these patients were reviewed retrospectively.
Three patients demonstrated bilateral paramedian ostia at the same or a very similar spinal level. One patient showed a paramedian and a midline ostium. One patient had 2 unilateral paramedian ostia at different spinal levels. The layers of the internal ending of CDS tracts were diverse. Complete removal of the tracts was possible in all patients. Two patients had dermoid tumors. All patients had an associated anomaly, such as a lumbosacral lipoma or the Currarino triad. The authors propose a "zipping error" hypothesis for the formation of dual ostia located at the spinal level of primary neurulation. An associated anomaly such as a lumbosacral lipoma may contribute to the formation of dual ostia.
Unilateral or bilateral dual ostia may be present in patients with CDS, especially when an associated anomaly is present. The atypical CDS may develop from aberrant neural tube closure.
[Show abstract][Hide abstract] ABSTRACT: Occult spinal dysraphism (OSD) encompasses various conditions. A dermal sinus tract (DST) consists of a duct communicating to the skin with deep structures that carries an important risk of infection. A different lesion consisting of a translucent skin opening and a fibrous tract that lacks a lumen can also be found in OSD. We termed this lesion pseudo-dermal sinus tract.
We reviewed clinical features of 20 patients with spinal skin orifices. The patients were classified into two categories: group 1 comprised children with true DST and group 2 included patients with skin dimples resembling a DST.
The aim of this study was to analyze differential features of patients in these two groups as they behaved dissimilarly in regard to clinical significance and outcomes, especially those concerning infectious risk.
Children of group 1 (n = 8) presented with tiny skin orifices and with superficial or deep infection. In contrast, group 2 (n = 12) mostly manifested with neurological symptoms. No patient in group 2 developed an infection preoperatively. In both groups, magnetic resonance showed tracts that ended in different structures. Histopathology of the lesions were dissimilar, those of group 1 being hollow tubular structures lined by epithelium while those of group 2 being solid tracts of fibrous connective tissue.
DST constitutes a clinicopathological diagnosis. Although sharing some common cutaneous and neuroimaging findings, both groups behaved differently. Patients of group 1 tended to show up with infection requiring urgent surgery. Patients of group 2 often presented with neurological manifestations and skin lesions, but needed no immediate operation.
Child s Nervous System 10/2010; 27(4):609-16. DOI:10.1007/s00381-010-1308-6 · 1.11 Impact Factor
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