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Total colonic aganglionosis: a surgical challenge. How to avoid complications?
Andrea Bischoff1, Marc A Levitt, Alberto Peña
1Division of Pediatric Surgery, Colorectal Center for Children, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, ML 2023, Cincinnati, OH 45229, USA. andrea.bischoff@cchmc.org
Insights
Total colonic aganglionosis (TCA) is a complex surgical condition in children. This study offers recommendations to prevent complications and improve long-term outcomes for TCA patients.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Medical Genetics
Background:
- Total colonic aganglionosis (TCA) presents significant surgical challenges in pediatric patients.
- Suboptimal long-term results and frequent complications are common in TCA management.
- This study analyzes surgical experience to develop recommendations for improved outcomes and complication avoidance.
Purpose of the Study:
- To analyze surgical experience with total colonic aganglionosis (TCA).
- To formulate recommendations for achieving better surgical results in TCA patients.
- To identify strategies for avoiding complications and sequelae associated with TCA.
Main Methods:
- Retrospective review of medical records for patients with TCA who underwent primary or secondary operations.
- Evaluation of the number of operations, preventable complications, bowel control, stoma status, and clinical follow-up.
- Description of the current surgical approach for TCA based on accumulated experience.
Main Results:
- 27 TCA patients identified (19 males, 8 females); 15 required reoperation.
- Average of 6.8 operations per patient, with common preventable complications including ileostomy issues (21) and severe diaper rash (10).
- 15 patients achieved bowel control; 11 maintained ileostomies (7 temporary, 4 permanent). Recommended approach: colectomy with straight ileoanal anastomosis and ileostomy, with delayed closure.
Conclusions:
- Total colonic aganglionosis (TCA) remains a significant surgical challenge with frequent complications and reoperations.
- Preventable complications can be reduced through proper stoma management, avoiding pouch/patch procedures, and delayed ileostomy closure.
- Meticulous surgical technique, accurate pathology, and dissection above the dentate line are crucial for successful TCA treatment.
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.
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