A case of split notochord syndrome: presence of dorsal enteric diverticulum adjacent to the dorsal enteric fistula.

Like umblical enteric remnants (eg, umblical sinus and omphalomesenteric fistula), enteric remnants can be seen on the dorsal aspect of the body (dorsal enteric sinus, dorsal enteric fistula IDEF], dorsal enteric diverticulum) in conjunction with complete cleft of the vertebral column. Complete cleft of the vertebral column associated with gastrointestinal tract and central nervous system anomalies is known as "split notochord syndrome" (SNS). The authors present an unreported variant of SNS having dorsal enteric diverticulum adjacent to the DEF. The patient died 17 days after surgical repair.