Original articleSpinal dermal sinuses and dermal sinus-like stalks analysis of 14 cases with suggestions for embryologic mechanisms resulting in dermal sinus-like stalks
Introduction
A spinal congenital dermal sinus tract (DST) is an uncommon form of spinal dysraphism, resulting in a cutaneous defect along the midline of the spinal axis. Its hallmark is a hollow epithelium-lined tract extending over a variable distance towards the spinal cord, often continuing to the intradural space, where it is mostly attached to the spinal cord.1, 2, 3, 4 Frequently dermoid or epidermoid tumours can be found over the course of these tracts.1, 4 Due to the open connection to the skin, infections are frequent, either superficial or deep with possible meningitis and/or abscess formation.1, 5 Dermal sinuses in the lumbar or lumbosacral region are usually seen in combination with low-positioned conus medullaris, and can be associated with tethered cord syndrome.1, 2, 4
In 1972 similar tracts but lacking an epithelium-lined lumen and dermoid or epidermoid tumours were given the name ‘meningocoele manqué’.6 It was not until 2009 that these tracts were reconsidered as being a histologically, clinically and probably also embryologically different entity and named ‘dermal sinus-like stalks’ (DSLS) by van Aalst.7
This study is a retrospective analysis of 14 cases of DST and DSLS that have been operated by the Neurosurgery department of the Leuven University Hospitals. The aim of our study was to describe the findings in our patients, to subdivide our specimens into DST and DSLS, to compare the characteristics of both groups and to search for possible embryologic mechanisms resulting in DSLS.
Section snippets
Methods
We retrospectively analysed the medical records of all paediatric (aged 16 years or less) patients with a clinical diagnosis of DST in whom intra-operatively a tract from the skin towards the spinal cord was seen between 01-01-1996 and 31-10-2012. Simple coccygeal pits and children that have not been operated upon in our hospital were excluded. Epidemiological and clinical data, imaging, surgical and pathological descriptions and follow-up data were collected and if necessary completed by
Demographics
Fourteen patients were included, 6 boys and 8 girls. Mean age of birth was 39 weeks PMA (range 34–40 w). Mean birth weight was 3000 g (range 1900–5000 g), at P23 according tot the Flemish birth record.9 Mean birth length was 49.0 cm (range 44.0–50.5 cm) and mean birth head circumference was 34.3 cm (range 32.0–36.5 cm). Half of the patients were born by a caesarean section due to various reasons, including foetal stress,2 pelvic dystocia,2 known spina bifida,1 breech presentation1 and the
Incidence and demographics
The incidence of DST is commonly reported as 1 in 2500 live births,1, 10, 11 although this number has been debated, since simple coccygeal pits might have been included in the already skewed patient populations that were used to estimate this number.2 To the best of our knowledge, only 5 specific series of spinal dermal sinuses have been published in the last 30 years (Table 5).1, 2, 4, 12, 13 Elton mainly used the same patients as Rajpal and is therefore not considered as such.11 None of the
Conclusion
We report a series of 14 patients operated upon for spinal DST (n = 5) or spinal DSLS (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, with an average interval of 7 months. Primary reason for referral was skin abnormalities in both groups, though there were two cases of meningitis in DST patients and 2 of recurrent urinary tract infections in DSLS patients.
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