The Hidden Connection - Split Notochord Syndrome with Dorsal Enteric Fistula.

Split notochord with dorsal enteric fistula is a rare congenital disease of the central nervous system. It can be associated with other anomalies. Communication with gastrointestinal tract is one of them. Less than 30 cases of such dorsal enteric fistula with split notochord have been reported till date. Here, the authors describe a unique case of a female infant with split notochord syndrome and dorsal enteric fistula. A comprehensive assessment is essential to plan management of this infrequent disease. She was treated with a multistage surgical repair and is currently thriving well.