Epidermoid Causing Split Cord Malformation in a Case of Jarcho Levin Syndrome (JLS)

Abstract

Jarcho-Levin syndrome (JLS) (costovertebral syndrome) is a rare congenital anomaly with multilevel vertebrocostal deformities. Intraspinal epidermoid cyst is a rare tumor. The incidence in adults is lesser than 1% and in children lesser than 3%. Epidermoid is predominantly seen at the dorsal spinal level. These may be congenital or acquired with known association with spinal dysraphism. We hereby report a rare case of split cord malformation with epidermoid in a 7-year female with JLS. The patient presented with complaints of progressive scoliosis, since3 years of age. On Neurological examination revealed no motor deficit. Patient had a dorsolumbar scoliosis with a hairy patch. X-ray revealed irregular fusion of left sided ribs and scoliosis of dorsolumbar spine with convexity towards left. Magnetic resonance imaging (MRI) and computerized tomography (CT) of the spine were done which revealed bifid spine from D11-L1, diplomyelia with two hemicords in a single dural sac with no membranous or bony septum. Intra operatively incompletely fused spinous process were seen from D11-L1. The patient underwent D10 to L2 laminectomy; there was a fibrous band which was leading into the bifid spine which was followed till the dura after removal of the laminae. After opening the dura this fibrous band was seen extending into the split cord. To our surprise split cord was continuing above the fibrous band following it revealed an epidermoid at the junction of the split cord, which was excised completely and fibrous band also removed. Histopathology of lesion confirmed as epidermoid.