Congenital dermal sinus with an infected dermoid cyst in the cervico-thoracic spinal cord

Congenital dermal sinuses (CDS) are epithelium-lined tracts that result from incomplete separation of cutaneous ectoderm from the underlying neuroectoderm. CDS may be associated with dermoid cysts and can cause complications by mass effect and by functioning as a pathway for infection. Cervical and thoracic tracts are rare, making up 1 and 10% of all CDS, respectively. We present an unusual case of a cervico-thoracic CDS with concomitant infected dermoid leading to neurological dysfunction. A 1-year-old male with a normal developmental history presented with a several-week history of progressive weakness. Previous visits to two outside emergency departments diagnosed the patient with acute otitis media. After another episode of fever and worsening of neurological symptoms, the patient was correctly diagnosed as having CDS with an infected dermoid cyst. Antibiotics were initiated, the lesion was resected, and the patient improved neurologically. Although cervical and thoracic CDS with infected dermoids are rare, one should have a high index of suspicion when cutaneous stigmata of spinal dysraphism are identified. Due to the risk of neurological deterioration, the recommended treatment of CDS with or without a concomitant intraspinal dermoid is prompt administration of antibiotics and definitive surgical intervention.