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Long-term outcomes in total colonic aganglionosis: a 32-year experience
Mauricio A Escobar1, Jay L Grosfeld, Karen W West
1Department of Surgery, Indiana University School of Medicine, The J. W. Riley Hospital for Children, Indianapolis, IN 46202, USA.
Insights
This study reviewed 36 children with total colonic aganglionosis (TCA), finding the modified Duhamel procedure offers better outcomes. Long-term survival was 81%, with modified Duhamel showing fewer complications than other surgical methods.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Genetics
Background:
- Total colonic aganglionosis (TCA) is a severe congenital condition affecting the large intestine.
- Hirschsprung disease, specifically TCA, requires complex surgical management.
- Understanding long-term outcomes is crucial for improving patient care.
Purpose of the Study:
- To analyze complications and long-term outcomes in children with total colonic aganglionosis (TCA).
- To evaluate the relationship between surgical procedures and patient outcomes.
- To identify the optimal surgical approach for TCA.
Main Methods:
- Retrospective review of 32 years (1972-2004) of patient data.
- Analysis of 36 children diagnosed with total colonic aganglionosis (TCA).
- Comparison of outcomes based on surgical techniques: modified Duhamel, Martin long Duhamel, Soave, and Kimura patch.
Main Results:
- Eighty-one percent long-term survival was achieved.
- Modified Duhamel procedure had lower complication rates compared to long Duhamel and Soave.
- Significant growth (83%) and continence (81%) were observed in survivors; 19% mortality.
- Short bowel syndrome occurred in 8 patients; Kimura patch benefited 5/6.
Conclusions:
- The modified Duhamel procedure is the preferred surgical approach for total colonic aganglionosis (TCA).
- Long Duhamel and Soave procedures are associated with higher morbidity.
- Bowel transplantation is a potential option for TCA with unresectable short bowel syndrome.
Background/Methods:
A 32-year retrospective review from 1972 to 2004 analyzed complications and long-term outcomes in children with total colonic aganglionosis (TCA) as they relate to the procedure performed.
Results:
Thirty-six patients (27 boys, 9 girls) had TCA. The level of aganglionosis was distal ileum (26), mid-small bowel (8), midjejunum (1), and entire bowel (1). Enterostomy was performed in 35 of 36. Eight developed short bowel syndrome. Twenty-nine (81%) had a pull-through at 15 +/- 6 months (modified Duhamel 20, Martin long Duhamel 4, and Soave 5). Six had a Kimura patch. Postoperative complications (including enterocolitis) were more common after long Duhamel and Soave procedures. Seven (19%; 2 with Down's syndrome) died (3 early, 4 late) from pulmonary emboli (1), sepsis (1), fluid overload (1), viral illness (1), liver failure (1), arrhythmia (1), and total bowel aganglionosis (1). Mean follow-up was 11 +/- 9 years (range, 6 months-29 years). Twenty-four (83%) of 29 patients exhibited growth by weight of 25% or more, 21 (91%) of 23 older than toddler age had 4 to 6 bowel movements per day, and 17 (81%) of 21 were continent. In 5 of 6, the Kimura patch provided functional benefit with proximal disease.
Conclusion:
Long-term survival was 81%. The highest morbidity occurred with long Duhamel or Soave procedures. The modified Duhamel is our procedure of choice in TCA. Bowel transplantation is an option for TCA with unadapted short bowel syndrome.
