Catastrophic Intramedullary Abscess Caused by a Missed Congenital Dermal Sinus

Congenital dermal sinus (CDS) is a type of occult spinal dysraphism characterized by a midline skin dimple. A 12-month-old girl presented with fever and ascending quadriparesis. She had a midline skin dimple in the upper sacral area that had been discovered in her neonatal period. Imaging studies revealed a holocord intramedullary abscess and CDS. Overlooking CDS or misdiagnosing it as benign sacrococcygeal dimple may lead to catastrophic infection and cause serious neurological deficits. Therefore, further imaging work-up or consultation with a pediatric neurosurgeon is recommended following discovery of any atypical-looking dimples in the midline.

Lipoma espinhal associado a seio dérmico congênito: relato de caso / Spinal lipoma associated with congenital dermal sinus: a case report

Os lipomas espinhais são raros, respondendo por 1 por cento de todos os tumores espinhais, estando associados ao disrafismo espinhal oculto em mais de 99 por cento dos casos. Estão divididos em três tipos principais: lipomielomeningocele, lipoma intradural e fibrolipoma do filo terminal. Este relato descreve um caso de lipoma lombossacral congênito associado a estigma cutâneo do tipo seio dérmico lombar congênito.

Spinal lipomas are rare, accounting for 1 percent of all spinal tumors and being associated with occult spinal dysraphism in more than 99 percent of cases. Such lesions are divided into three main types, namely, lipomyelomeningoceles, intradural lipomas, and filum terminale fibrolipomas. The present report describes a case of congenital lumbosacral lipoma associated with cutaneous stigmata of the lumbar dermal sinus type.

Congenital Dermal Sinuses: An Clinical Analysis of 20 Cases

OBJECTIVE: Congenital dermal sinus is a rare congenital disease that results from the failure of the neuroectoderm to separate from the surface ectoderm during the process of neurulation, where there is communication between the skin and the deeper structures. Their pathogenesis, clinical course and treatment strategy are well known. We analyze our series and compare our results with other series. METHODS: Twenty patients were diagnosed as congenital dermal sinus and confirmed pathologically from October 1986 to July 2003 at our hospital. We studied the patients' clinical manifestations, radiological findings and pathological profiles. RESULTS: Seven cases were located in the suboccipital area and 13 cases were located in the spinal area. Interestingly, 4 of 13 spinal lesion cutaneous openings were located lower than the 3rd sacral body level. 8 of 20 lesions were terminated at neural structures, 4 of 20 lesions were terminated at the intradural portion and others terminated at the extradural portion. Nine anomalies were combined with the dermal sinus, including 4 lipomas, 2 Currarino's triad, 1 encephalocele, 1 myelomeningocele and 1 diastematomyelia. Eleven patients had dermoid tumors. CONCLUSION: Congenital Dermal Sinus must be surgically removed immediately if they are diagnosed. The surgical procedure of congenital dermal sinus is complete removal, but in some cases, complete removal is impossible. In those cases, we removed all epithelial tissues. We consider sacrococcygeal dimple almost invariably have no connection with intraspinal structures. But, if other cutaneous manifestations are combined with cutaneous pits, it can communicate with the sacrococcygeal dimple.

A Case of Intradural Dermoid Cyst in the Cervical Region Associated with Congenital Dermal Sinus: Case Report

We report a rare case of a 10-month-old male with a cervical intradural dermoid cyst associated with a congenital dermal sinus. He was presented motor weakness of right upper extremity 1 month before admission. The cervical spinal MRI demonstrated an intradural extramedullary mass from third cervical vertebra to fourth cervical vertebra, which was connected with a skin through a sinus tract. With extensive laminectomy, the mass and the sinus tract were completely removed and the mass was histopathologically diagnosed as a dermoid cyst. six months after surgery, the neurological status return to normal and MR study showed no evidence of recurrence or any deformity. In case of the intradural dermoid cyst associated with a congenital dermal sinus, early diagnosis and total excision of the cyst, including the entire tract may provide an excellent outcome.

Congenital Dermal Sinus at Thoracic Region Associated

Congenital dermal sinus (CDS) is a rare entity widely known to occur as a result of the of the failure of the neuroectoderm to separate from the cutaneous ectoderm during the process of neurulation at the third to fifth week of gestation. The lesion can occur at any level of the craniospinal axis, but are located predominantly at the lumbosacral and occipital region. CDS of thoracic region and cervical region are rare. The patient with CDS presents with meningitis and/or mass effect from the associated inclusion tumor. They are usually dermoid or epidermoid cyst. Teratoma is rarely associated with CDS. We report our experience of CDS an 11-month old boy associated with an intramedullary dermoid tumor at the thoracic region, and together we report a review of the literature.

Spinal congenital dermal sinus--experience of 5 cases over a period of 10 years

Spinal congenital dermal sinus (CDS) is a rare entity which supposedly results from the failure of the neuroectoderm to separate from the cutaneous ectoderm during the process of neurulation. The lesions are most frequent at the lumbosacral followed by the occipital region. CDS of the thoracic region is very rare. The patients with spinal CDS present with meningitis and/or mass effect from the associated inclusion tumor. They are usually dermoid or epidermoid cysts. Teratoma is rarely associated. The authors experienced 5 cases of spinal CDS over a 10 year period. Of the 5 cases, 2 were at thoracic and 3 were at lumbosacral levels. Dermoid cyst, epidermoid cyst and teratoma were associated in one case each. Two cases presented with neurological deficit and meningitis while an additional case presented with neurological deficit and a history of probable meningitis. Pain was present in 2 cases. Magnetic resonance imaging played an important role in the diagnosis of the lesion and planning of surgery. All the cases showed a good response to surgery even though one patient had persistent neurological deficit.

A Case of Congenital Lumbosacral Dermal Sinus Associated with Recurrent Meningitis(Case Report)

Recurrent episode of meningitis in infants and children frequently constitute a frustrating and distressing, both in determination of course and treatment. The authors are reporting a infected case of congenital dermal sinus with dermoid cyst in the subarachnoid space of cauda equina and conus medullaris which was cured after complete removal of cyst and sinus tract.