Proximal jejunostomy with or without myectomy-myotomy modification in five infants with total intestinal aganglionosis: An experience with surgical treatments in a single institution.

BACKGROUND Total intestinal aganglionosis is characterized by the absence of intramural ganglion cells, in which the disease's involvement extends from the stomach to the anorectum. This disease was suggested previously to be incompatible with life, but recently an extended small bowel myectomy-myotomy has achieved some prolonged survivors. METHODS Five patients with total intestinal aganglionosis underwent laparotomy at 1 to 5 days of age. Surgery was performed as a simple jejunostomy 60 to 70 cm below the ligament of Treitz in the initial 2, jejunustomy 30 cm below the ligament of Treitz in 1, and jejunostomy with myectomy-myotomy modification 30 to 35 cm below the ligament of Treitz in the remaining 2 infants. RESULTS The initial 2 patients died of sepsis, possibly derived from frequent enteritis and bacterial translocation at 7 and 8 months of age. Another patient had prolonged survival but died of hepatic failure at 1 year, 4 months. The remaining 2 children have survived beyond 2 years of age without any liver dysfunction, receiving a combination of enteral and parenteral nutrition. CONCLUSIONS The more proximal site (30 to 35 cm below the ligament of Treitz) of jejunostomy with myectomy-myotomy modification appeared to be preferable for prolonged survival in these 5 patients with total intestinal aganglionosis.