Total colonic aganglionosis (TCA) extended to the ileum is seen quite rare among infants with Hirschsprung's disease. Type and timing of definitive surgery in these patients are controversial. This report was presented to discuss the management of two siblings with TCA. Case 1: A two-day-old girl was operated for partial intestinal obstruction. During laparotomy, serial frozen biopsies proved TCA extended to the terminal ileum and a loop ileostomy was performed. At five months of age, a modified Duhamel-Martin procedure without protective ileostomy was performed. An endo-GIA stapler was transanally used for colo-ileal anastomosis. She is doing well for the last five years. Case 2: A one-day-old boy admitted to the hospital with similar findings to his sister. Frozen biopsies during first laparotomy proved that majority of ileum and entire colon was aganglionic and a proximal ileostomy was performed. At 10 months of age, he underwent a similar Duhamel-Martin operation. He is in a good condition for the last four years. Conclusion: In infants, our modification on Duhamel-Martin procedure, which is based on the use of an endo-GIA stapler transanally for colo-ileal anastomosis without protective ileostomy, may be utilized as an alternative method in the definitive treatment of patients with TCA.
