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Improved quality of life by combined transplantation in Hirschsprung's disease with a very long aganglionic segment
Revillon Yann1, Aigrain Yves, Jan Dominique
1Hôpital des enfants Malades, Paris, France.
Insights
Combined liver, intestine, and right colon transplantation improves quality of life for children with Hirschsprung disease. Patients achieved full independence from parenteral nutrition and improved continence after this complex procedure.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Transplantation Medicine
Background:
- Hirschsprung disease involving the proximal jejunum presents significant surgical challenges.
- Long-term parenteral nutrition in these patients can lead to liver impairment.
- Combined liver, intestine, and right colon transplantation is explored as a treatment option.
Observation:
- A retrospective study analyzed 3 pediatric patients undergoing combined liver, intestine, and right colon transplantation.
- Immunosuppression involved steroids, tacrolimus, and azathioprine.
- Abdominal pull-through procedures were performed post-transplantation.
Findings:
- All 3 patients were weaned off total parenteral nutrition, with improved bowel function (2-3 daily movements).
- Two patients achieved full daytime and nighttime continence; one achieved daytime continence.
- Follow-up ranged from 2 to 6 years, demonstrating sustained positive outcomes.
Implications:
- Combined liver-intestine transplantation is a viable option for complex Hirschsprung disease, even with liver compromise.
- Right colon inclusion in transplantation did not negatively impact outcomes in this cohort.
- This approach significantly enhances the quality of life for affected children.
Background/Purpose:
The treatment of children with Hirschsprung's disease beginning in the proximal jejunum remains a challenge for the pediatric surgeon. These patients need a definitive parenteral nutrition, which could lead to a liver impairment. The goal of this work is to assess the quality of life after combined liver, intestine, and right colon transplantation.
Methods:
This is a retrospective study of 3 patients. Data regarding symptomatology, radiographic and operating findings, postoperating recovery, and quality of life were analyzed and compared with the quality of life before the transplantation.
Results:
The suspicion of a very long intestinal aganglionosis should be derived from the intestinal biopsies. Three combined liver, intestine, and right colon transplantation operations have been performed. The immunosuppression included steroids, tacrolimus, and azathioprine. An abdominal pull-trough (Duhamel procedure 2, Swenson procedure 1) was performed from 6 to 24 months after the transplantation. The follow-up after the transplantation ranges from 2 to 6 years. These 3 patients are completely off total parenteral nutrition with bowel movements 2 to 3 times a day. Two patients are continent day and night, and one is continent during the day only.
Conclusions:
Intestinal transplantation is feasible with good results even when a liver impairment needs a combined intestine and liver transplantation. The right colon transplantation, in our experience, does not impair the results. The quality of life after the transplantation is better than before.
