PT  - JOURNAL ARTICLE
AU  - L Sigurdsson
AU  - J Reyes
AU  - S A Kocoshis
AU  - G Mazariegos
AU  - K M Abu-Elmagd
AU  - J Bueno
AU  - C Di Lorenzo
TI  - Intestinal transplantation in children with chronic intestinal pseudo-obstruction
AID  - 10.1136/gut.45.4.570
DP  - 1999 Oct 01
TA  - Gut
PG  - 570--574
VI  - 45
IP  - 4
4099  - http://gut.bmj.com/content/45/4/570.short
4100  - http://gut.bmj.com/content/45/4/570.full
SO  - Gut1999 Oct 01; 45
AB  - 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.CIPOchronic intestinal pseudo-obstructionTPNtotal parenteral nutritionAChEacetylcholinesterase