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Up-to-date evolution of small bowel transplantation in children with intestinal failure
1Hopital Necker-Enfants Malades, Paris, France.
Insights
Pediatric intestinal transplantation offers a viable solution for intestinal failure beyond short bowel syndrome. Combined liver-small bowel transplants show improved outcomes and reduced rejection rates compared to isolated intestinal transplants.
Area of Science:
- Pediatric surgery
- Transplantation immunology
- Gastroenterology
Background:
- Intestinal failure in children necessitates complex management strategies.
- Short bowel syndrome is a primary indication for intestinal transplantation.
- Combined liver-small bowel transplantation addresses multi-organ failure.
Purpose of the Study:
- To review experience with 26 pediatric intestinal transplantations performed since 1987.
- To evaluate outcomes of isolated intestinal and combined liver-small bowel transplants.
- To assess the impact of immunosuppressive regimens on graft and patient survival.
Main Methods:
- Retrospective analysis of 26 pediatric patients undergoing intestinal transplantation.
- Categorization into three groups based on transplant type and era (1987-1990, 1994-present).
- Comparison of immunosuppressive protocols (cyclosporine vs. tacrolimus) and monitoring strategies.
Main Results:
- Overall patient survival (PS) was 61% and graft survival (GS) was 50%.
- Combined liver-small bowel transplants (Group C) demonstrated higher PS (80%) and GS (80%) with reduced rejection.
- Tacrolimus-based immunosuppression (Groups B & C) improved survival compared to cyclosporine (Group A).
Conclusions:
- Intestinal transplantation is a critical therapeutic option for pediatric definitive intestinal failure.
- Combined liver-small bowel transplantation offers superior functional outcomes with manageable rejection.
- Tacrolimus-based immunosuppression enhances both graft and patient survival in pediatric intestinal transplantation.
Purpose:
The aim of the authors was to report an up-to-date review of their experience with 26 intestinal transplantations in children since 1987.
Methods:
A retrospective study was conducted of 26 patients with a mean age of 5 years (range, 0.3 to 14 years). Three groups were isolated. In group A (1987 to 1990), seven patients received nine isolated intestinal transplants for short bowel syndrome. Immunosuppression therapy consisted of cyclosporine, aziathioprine, and corticosteroids. In group B (1994-current), nine patients received nine isolated intestinal transplants for short bowel syndrom (n = 2), intestinal pseudoobstruction (n = 2), neonatal intractable diarrhea (n = 3), and Hirschsprung' disease (n = 1); hepatic biopsy results showed weak cholestasis or fibrosis. In group C (1994-current), 10 patients received 10 combined liver-small bowel transplants for short bowel syndrome (n = 3), neonatal intractable diarrhea (n = 4), and Hirschsprung' disease (n = 3); hepatic cirrhosis related to total parenteral nutrition (TPN) was shown in all cases. Groups B and C received immunosupressive treatment consisting of tacrolimus, aziathioprine, and corticosteroids. Posttransplant follow-up included intestinal biopsies of the small bowel twice a week and more frequently or combined with liver biopsy if rejection was suspected.
Results:
Overall patient survival (PS) and graft survival (GS) are 61% (16 of 26) and 50% (13 of 26), respectively. In group A, severe intestinal allograft rejection occurred in six patients leading to graft removal (GS, 11%). Five patients died of TPN complications after graft removal (PS, 28%). One survivor is off TPN, and one currently is waiting for a second graft. In group B, six patients survived (PS, 66%). Causes of death include hepatic failure (n = 1), renal and liver failure (n = 1), and systemic infection (n = 1). Severe intestinal allograft rejection occurred in five patients, which neccessitated aggressive immunosuppression (antilymphocyte serum) leading to an incomplete functional recovery of the graft. Only two patients currently are off TPN. In group C, eight patients survived (PS, 80%) all of which are currently off TPN. One patient died during the procedure, and one died of severe systemic infection. Intestinal graft rejection occurred in six patients; rejection of the liver allograft occurred in five patients, yet all rejections were weak and successfully treated by corticosteroids (GS, 80%).
Conclusions:
Intestinal transplantation is a valid therapeutic option for children with definitive intestinal failure and not only for short bowel syndrome. Tacrolimus improves graft and patient survival (group A v group B). The lower severity of graft rejection in combined liver-small bowel transplantation improves functional results of intestinal transplantation in children without additional mortality or morbidity (group B vgroup C).