Case of neonate with total intestinal hirschsprung disease managed with a proximal jejunostomy.

Abstract

Background: Hirschsprung disease (HD) is a congenital disorder of the enteric nervous system, and its management will differ clinically depending on the degree of aganglionosis. Total intestinal HD (TIHD) is a rare variant, accounting for < 1 % of all cases, and is defined as aganglionosis of nearly the entire intestine with less than 20 cm of ganglionated small bowel past the ligament of Treitz (LOT). TIHD can be challenging to manage with high risk for chronic intestinal failure.

Case report: Ours is a neonate confirmed pathologically to have total intestinal aganglionosis, surgically managed with a jejunostomy formed at 40 cm distal to LOT.

Conclusion: Case of TIHD that has benefitted from a proximal diverting jejunostomy.