Child's Nerv Syst (1995) ll: 3 9 Springer-Verlag 1995 Juan EMartinez-Lage Juan A. Esteban Mhximo Poza Carlos Casas Congenital dermal sinus associated with an abscessed intramedullary epidermoid cyst in a child: case report and review of the literature Received: 5 December 1992 Revised: 28 May 1993 J.E Martinez-Lage (IN]) . J.A. Esteban M. Poza Regional Service of Neurosurgery, University Hospital "Virgen de Arrixaca", E1 Palmar, E-30120 Murcia, Spain Fax: 34-68-369678 C. Casas Section of Neuropediatrics, University Hospital "Virgen de Arrixaca", E1 Palmar, E-30120, Murcia, Spain Abstract A 16-month-old male in- fant presented with paraparesis and a high thoracic skin dimple. After myelography the child underwent complete surgical removal of the dermal sinus and of an abscessed tumor located within the cervico- thoracic cord. Histological study showed that the lesion was an in- tramedullary epidermoid cyst. Only four descriptions have previously been published of dermal sinuses associated with intramedullary epi- dermoids, three of them complicat- ed by intramedullary abscesses. This appears to be the first report in the current literature of the asso- ciation of a dermal sinus and an infected intramedullary epidermoid occurring out of the confines of the lumbosacral region. Key words Congenital tumor 9 Dermal sinus. Epidermoid cyst - Intramedullary abscess 9 Intramedullary tumor 9 Staphylococcal infection Introduction Congenital dermal sinuses - anomalous communications between the surface of the skin and deep structures - are the result of developmental anomalies in the separation of the neuroectoderm and the cutaneous ectoderm [2, 6, 10]. They tend to occur in the midline of the occipital and lumbosacral regions [10]. The association of a dermal sinus and an intradural dermoid cyst is not infrequent [6]. The consequence of these abnormal cutaneous openings is infection, in the form either of meningitis or ofintradu- ral abscess. On the other hand, coexistence of an intrame- dullary epidermoid and a congenital dermal sinus seems to be a very rare event [2]. A literature search yielded only four reports on this association [2, 5, 7, 11], and only in two of them did there exist a direct communication from the skin down to the substance of the spinal cord itself [2, 5]. Intradural infection was a constant finding in all four instances. In these previous cases the lesions were situated whithin the lumbosacral region. Our patient thus represents the first documented case of a cervicothoracic epidermoid connected to the skin by a dermal sinus, com- plicated by an intramedullary spinal cord abscess. Case report A 16-month-old male child was admitted to hospital on 28 Septem- ber 1979. During the previous days he had had difficulty and pain whilst walking, and had refused to stand up or walk the day before admission. The previous month, the child had suffered a similar episode that subsided spontaneously. His past medical history was unremarkable. Psychomotor development had been normal, the child having started to walk unaided at the age of 13 months. Examinations On admission the child appeared ill, was fully conscious, and showed moderate neck and spinal rigidity. Ocular fundi were nor- mal. He had severe paraparesis and increased deep reflexes in both legs, along with bilateral ankie clonus. Plantar responses were