Intestinal transplantation in children with chronic intestinal pseudo-obstruction

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 becom e a therapeutic option for TPN dependent children with intestinal failure w ho are failing management with TPN. Aims-To investigate the outcome of children with CIPO referred for intestin al transplantation. Methods-A retrospective review was carried out of records and diagnostic st udies from 27 patients with CIPO referred for intestinal transplantation. Results-Five children were not listed for transplantation: two because of p arental decision, two because of suspicion of Munchausen syndrome by proxy, and one because he tolerated enteral nutrition. Six are still TPN dependen t and awaiting transplantation. Eight children died awaiting transplantatio n. Eight children underwent transplantation. Three died (two months, seven months, and four years after transplant). Five children are alive with a me dian follow up of 2.6 years (range two months to six years). All transplant ed children were able to tolerate full enteral feedings. The postoperative course was complicated by dumping syndrome, Munchausen syndrome by proxy, n arcotic withdrawal, and uncovering of achalasia. Conclusion-Intestinal transplantation may be a life saving procedure in chi ldren with CIPO. Early referral and thorough pretransplant evaluation are k eys to successful transplantation.