Split Cord Malformation Type 2 with Double Dorsal Lipoma: A Sequela or a Chance

An 11-month-old baby girl, with normal perinatal history, presented with a congenital lumbosacral swelling and a sacral dimple. Imaging revealed a split cord malformation (SCM) type 2 at L1–L3 with a lipomeningocoele extending intradurally and tethering both the hemicords, the conus was noted to be at L4. She underwent excision of the lipoma and detethering of the hemicords. Intraoperatively, the split cord was seen from L1 to L3 with an aberrant median vessel passing between the hemicords. Two lipomas were seen separately attached to each of the hemicords, the lipomas were dissected off the hemicords, and the hemicords were neurulated. The case helps revisit the unified theory proposed by Pang for SCM as well as the theory of premature disjunction in the pathogenesis of lipomeningocoele. Formation of the endomesenchymal tract splits the developing cord into two, whereas the premature detachment of neuroectoderm from the cutaneous ectoderm can lead to lipomeningocoeles. The present case is only the fourth case ever reported of an SCM type 2 with double lipoma. In the case report, we discuss the embryological basis of this condition and surgical nuances of management.