Intramedullary epidermoid associated with an intramedullary spinal abscess secondary to a dermal sinus.
Intramedullary spinal abscesses are rare and potentially devastating lesions. A heightened clinical awareness of patients at risk is essential for an early diagnosis and intervention to improve the prognosis of these patients. The first case of spinal abscess was described in 1830, and only 63 cases have been reported subsequently. Intraspinal epidermoids also are unusual lesions. These tumors were reported first in 1829 and represent less than 1% of all intraspinal tumors. We present a case of a 2-year-old girl who had rapidly progressive paraplegia with urinary and bowel retention and was found to have an intramedullary epidermoid and an intramedullary abscess as a result of a dermal sinus. The association of an intramedullary abscess and epidermoid tumor, two rare lesions, has not been reported previously. We also review the literature related to both intramedullary epidermoids and intramedullary abscesses of the spinal cord. Advances in sectional imaging as described should help lead to an early and safe diagnosis of these rare but often debilitating lesions. This case illustrates the importance of a complete neurological assessment of all patients with a congenital dermal sinus because of the potential for intradural extension and the frequent association with other dysraphic abnormalities. Prophylactic surgery is indicated in many cases to prevent dangerous and recurrent infections of the central nervous system.
Benzil
D L
DL
Department of Clinical Neurosciences, Brown University, Providence, Rhode Island.
Epstein
M H
MH
Knuckey
N W
NW
D000038:Abscess / Q000175:diagnosis / Q000209:etiology*; D002294:Carcinoma, Squamous Cell / Q000150:complications*; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D016136:Spina Bifida Occulta / Q000150:complications* / Q000175:diagnosis; D013118:Spinal Cord Diseases / Q000175:diagnosis / Q000209:etiology*; D013120:Spinal Cord Neoplasms / Q000150:complications*