Intestinal transplantation in children with chronic intestinal pseudo-obstruction

• Published in: Gut 1999-10, Vol.45 (4), p.570-574
• Main Authors: Sigurdsson, L, Reyes, J, Kocoshis, S A, Mazariegos, G, Abu-Elmagd, K M, Bueno, J, Di Lorenzo, C
• Format: Article
• Language: English
• Subjects: Adolescent; Biological and medical sciences; Biopsy; Bladder; Child; Child, Preschool; children; Chronic Disease; chronic intestinal pseudo-obstruction; Female; Follow-Up Studies; Humans; Infant; Infections; Intestinal Pseudo-Obstruction - mortality; Intestinal Pseudo-Obstruction - surgery; Intestinal Pseudo-Obstruction - therapy; intestinal transplantation; Intestines - transplantation; Lifesaving; Liver cirrhosis; Liver diseases; Male; Medical prognosis; Medical sciences; Mortality; Nutrition; Pain; Parenteral nutrition; Parenteral Nutrition, Total; Patients; Quality of Life; Retrospective Studies; small bowel motility; small bowel transplantation; Stomach, duodenum, intestine, rectum, anus; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Surgery of the digestive system; Survival Rate; total parenteral nutrition; Transplants & implants; Treatment Outcome; Urinary tract diseases; Urogenital system
• ISSN: 0017-5749; 1468-3288; 1458-3288
• DOI: 10.1136/gut.45.4.570

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.