Twenty-three years of follow-up in patients with total colonic aganglionosis.

The main purpose of our study was to evaluate the outcome of patients with total colonic aganglionosis diagnosed and treated in our hospital. Seven of our twelve patients died within 6 month after birth due to infectious complications or underlying other diseases. 5 patients are alive after subtotal colectomy and ileo-rectal anastomosis, and were investigated for late complications, social integration, stool behavior, local status and laboratory parameters 12 and 23 years after surgery. All patients developed almost normally and were socially and professionally integrated. Although all had an accelerated passage of stools, no signs of malnutrition were found. One patient developed a recto-sacral fistulae and sacral osteomyelitis eight years after surgery and needed an ileostomy. After ileo-rectostomy all patients were continent with a grown rectal stump, had no strictures and a normal appearance of the mucosa.