Primary treatment of isolated hypoganglionosis in our department

Abstract

Isolated hypoganglionosis (IH) is a disease with a poor prognosis whose main condition is functional intestinal obstruction in the widespread intestinal tract from birth. Clinical practice guidelines suggest upper jejunostomy as a gastrointestinal decompression treatment. Consensus is being reached on the treatment method；however, there are no specific proposals and due to their rarity, initial treatments often vary depending on the institution and the case. In this study, we performed upper jejunal stoma, tube jejunostomy, and lower intestinal stoma construction in the initial surgical treatment and nutrition therapy from the neonatal period for IH five cases, and established enteral nutrition and appropriate nutrition. By controlling enteral inflammation by decompression, we were able to shift to home medical care around a year of age without developing severe complications, such as enterocolitis, in four out of five cases. It was suggested that in the initial treatment of IH, it may be useful to create an appropriate decompression route and enteral feeding route even if there are multiple ostomies.