Total colonic aganglionosis: a surgical challenge. How to avoid complications?

INTRODUCTION: Total colonic aganglionosis represents a significant challenge for pediatric surgeons. Long-term results are suboptimal and complications are very common. We analyzed our experience to formulate recommendations to achieve better results and avoid complications and sequelae. METHODS: The medical records of patients with total colonic aganglionosis that were operated on by us primarily or secondarily were reviewed. We evaluated: number of operations performed, preventable complications, bowel control or presence of stomas, and clinical follow-up. Based on this experience we describe our current approach for this condition. IRB approval was obtained. RESULTS: 27 patients were identified (19 males, 8 females). 12 patients had the primary pullthrough performed by us and 15 were operated on elsewhere before coming to us for reoperation. The average number of operations per patient was 6.8 (1–40). We identified several preventable complications: ileostomy prolapse or stricture (21), severe diaper rash (10), obstructive symptoms following a pouch or patch-type of pullthrough (9), infection, abscess, and fistula after the pullthrough (5); wrong histologic diagnosis leading to colostomy opening in aganglionic bowel (4) with consequent pullthrough of aganglionic intestine in two of them; anastomotic stricture/acquired atresia (3); and destroyed anal canal and permanent fecal incontinence (2). 15 patients have bowel control; 11 have an ileostomy: temporary (7) and permanent (4); and one is less than 3 years of age. Length of follow-up ranged from 1 to 17 years. Based on this experience, our approach for this condition consists of: colectomy with straight ileoanal anastomosis and ileostomy at presentation, followed by ileostomy closure only when the child is toilet trained for urine and is willing to tolerate rectal irrigations. CONCLUSION: Total colonic aganglionosis remains a serious surgical challenge. Patients suffering from the condition, have multiple complications, sequelae, and often require reoperations. We found that it is possible to prevent many of these by properly fixing the stoma, avoiding pouch or patch procedures, delaying ileostomy closure, having pathology expertise, and with meticulous surgical technique starting the dissection/anastomosis well above the dentate line.