Cervical dermal sinus with tethered cord and syringomyelia

Abstract

A 3-year-old girl with a skin dimple in the posterior midline of her neck was admitted to our hospital (Fig. 1). She had difficulty holding objects. Neurological examination demonstrated mild bilateral arm weakness. Magnetic resonance (MR) imaging revealed syringohydromyelia and tethered spinal cord connecting to a dermal sinus tract at C4–5 (Fig. 2). Microsurgically, dermal tract, intradural mass, and tethering bands were removed. Histological examination of the dural tract showed stratified squamous epithelium and that of the intradural mass showed fibrous connective tissue. The patient made an uneventful postoperative recovery, with resolution of her neurological deficit. At 3-month follow-up examination MR imaging revealed collapse of the syringomyelia, the untethered cervical spinal cord, and no evidence of sinus tract (Fig. 3). Congenital dermal sinuses are rarely located in the cervical region. 1 Patients may present with meningitis and/or mass effect secondary to intradural lesions, which can be epidermoid, dermoid, teratoma, or abscess. 2,5 The spinal axis should undergo MR imaging evaluation if a dermal sinus is seen on the midline. Surgery must be performed without delay to prevent infection and neural tissue compression. 3 To the best of our knowledge, the presence of syringomyelia in continuity with a dermal sinus in the cervical region has not previously been reported, although one case has been reported in the upper thoracic region. 4