Dorsal Enteric Fistula

The dorsal enteric fistula is a rare congenital spinal dysraphism due to an anomaly in notochord integration. It arises from the splitting of the notochord, leading to the division of the spinal cord and vertebral column. All the theories pertaining to the disorder revolve around the presence of the neurenteric canal that leads to the observed malpresentation. The persistence of the connection between the endoderm and the ectoderm is the main culprit which occurs due to the disruption in embryogenesis. The anomaly exhibits itself differently at different stages and involves different viscera such as that of the abdomen and chest, includes different systems, and also encompasses abnormal tissue remnants of enteric origin (posterior enteric remnants). This needs to be taken into account when performing an examination of signs and symptoms and during histopathological analysis. Thus, its manifestations span over a spectrum that comprises an isolated lesion or a combination of a wide variety of anomalies. Dorsal enteric fistula with a bowel ostium is the most severe presentation. The management differs from scenario to scenario depending on the presentation of the disorder and subsequent examination findings. In any case, complete surgical removal is the best treatment option.