Split Notochord Syndrome: Case Report

Split notochord syndrome includes a cleft of the vertebral column associated with malformation of the central nervous system. Fourteen cases involving the lumbosacral spine have been reported in the literature. We report on a female neonate who had intact function of her lower extremities, a posterior midline lumbar defect bounded by two complete spinal canals that contained two spinal cords. A meningocele, a large bowel fistula, and a mature teratoma protruded through the cleft. The baby is doing well after repair of the anomaly and placement of a ventriculoperitoneal shunt.