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Published on: August 20, 2007
Intestinal transplantation in children with chronic intestinal pseudo-obstruction
L Sigurdsson1, J Reyes, S A Kocoshis
1Department of Paediatric Gastroenterology, Children's Hospital of Pittsburgh, 3705 Fifth Avenue, Pittsburgh, PA 15213-2583, USA.
Insights
Intestinal transplantation offers a life-saving option for children with chronic intestinal pseudo-obstruction (CIPO) failing total parenteral nutrition (TPN). Early referral and evaluation are crucial for successful outcomes in these pediatric patients.
Area of Science:
- Pediatric surgery
- Gastroenterology
- Transplantation medicine
Background:
- Children with chronic intestinal pseudo-obstruction (CIPO) often require total parenteral nutrition (TPN), increasing risks of liver failure and infections.
- Intestinal transplantation is a viable option for TPN-dependent children with intestinal failure unresponsive to conventional management.
Purpose of the Study:
- To evaluate the outcomes of pediatric patients with CIPO referred for intestinal transplantation.
- To assess the efficacy and complications of intestinal transplantation in this cohort.
Main Methods:
- Retrospective review of medical records and diagnostic studies.
- Analysis of 27 pediatric patients diagnosed with CIPO referred for intestinal transplantation.
Main Results:
- Eight children underwent intestinal transplantation, with five surviving (median follow-up 2.6 years) and tolerating full enteral nutrition.
- Three transplanted children died post-operatively; complications included dumping syndrome and Munchausen syndrome by proxy.
- Eight children died awaiting transplantation, and six remain TPN-dependent.
Conclusions:
- Intestinal transplantation can be a life-saving intervention for children with CIPO.
- Timely referral and comprehensive pre-transplant assessment are critical for successful transplantation outcomes.
Background:
Children with chronic intestinal pseudo-obstruction (CIPO) often require total parenteral nutrition (TPN) which puts them at risk of liver failure and recurrent line infections. Intestinal transplantation has become a therapeutic option for TPN dependent children with intestinal failure who are failing management with TPN.
Aims:
To investigate the outcome of children with CIPO referred for intestinal transplantation.
Methods:
A retrospective review was carried out of records and diagnostic studies from 27 patients with CIPO referred for intestinal transplantation.
Results:
Five children were not listed for transplantation: two because of parental decision, two because of suspicion of Munchausen syndrome by proxy, and one because he tolerated enteral nutrition. Six are still TPN dependent and awaiting transplantation. Eight children died awaiting transplantation. Eight children underwent transplantation. Three died (two months, seven months, and four years after transplant). Five children are alive with a median follow up of 2.6 years (range two months to six years). All transplanted children were able to tolerate full enteral feedings. The postoperative course was complicated by dumping syndrome, Munchausen syndrome by proxy, narcotic withdrawal, and uncovering of achalasia. Conclusion-Intestinal transplantation may be a life saving procedure in children with CIPO. Early referral and thorough pretransplant evaluation are keys to successful transplantation.
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