The split notochord syndrome with dorsal enteric fistula

The authors describe a new case of the split notochord syndrome (SNS) with dorsal enteric fistula in an eight-month-old-boy. The child had also central nervous system anomalies including dysgenetic corpus callosum, absence of septum pellucidum, triventricular hydrocephalus, small posterior fossa, and lipomyelomeningocele, gastrointestinal system anomalies such as malrotation, wandering spleen, and right inguinal hernia. The clinical features, embryogenesis, and literature were reviewed briefly.