State's pull-through for total colonic aganglionosis and GI dismotility.

Background: Total colonic aganglionosis (TCA) is present in 4% - 5% of the patients with Hirschsprung's disease and has a high surgical mortality rate between 13% - 23%. Diagnosis and treatment of TCA is still a major challenge for pediatric surgeons. Many techniques with several advantages and disadvantages were established for its treatment. We have performed State's pull-through as total colectomy and ileo-proctostomy with long posterior rectal myotomy in TCA and severe dysmotility disorders.

Methods: In this retrospective study, 13 patients with TCA were evaluated from 1992 through 2012 in two pediatric surgery centers, which consisted of 10 TCA, one intestinal neuronal dysplasia (IND), and two chronic intestinal pseudo- obstruction syndrome (CIP). All patients underwent total colectomy, resection of part of the involved small intestine, and rectal anastomosis in one layer with 4/0 vicryl with long posterior rectal myotomy. All patients had preoperative barium enema and rectal biopsy for diagnosis. Leveling ileostomy was performed in 12 patients and mid-jejunostomy in one patient. In two of 13 children, proximal diverting loop ileostomy was established after definitive operation. In three patients, trans-rectal myotomy was needed two weeks after the initial operation.

Results: All patients were females with the age ranging from six months to six years. State's pull-through was carried out for all (the mean age of definitive procedure was 5.1 months). Rectal biopsy of 10 patients reported no ganglion cells of which one had extended aganglionosis to mid- jejunum, one had IND, and two had ganglionicbowel bowel with clinical presentation of CIP. The follow- up period was from six months to 10 years (the mean follow- up period was 10 months). There were no significant complications in 12 patients except episodes of diarrhea and severe dehydration which needed hospitalization. One patient with mid- jejunum aganglionosis had severe failure to thrive and needed repeated hospitalization for parenteral nutrition. All patients had acceptable bowel function following operation (two to six times a day). Now, five of the 13 patients are above the age of toilet training, having voluntary bowel movement with little or no medication (Leopromid).

Conclusion: State's pull-through is recommended in all cases of TCA and severe dysmotility problems of the colon. This technique is less difficult to perform, and avoids the complications and disadvantages of removal of the rectum and has satisfactory results.