Intestinal and multivisceral transplantation in children with severe gastrointestinal dysmotility.
OBJECTIVE
Severe gastrointestinal dysmotility (GID) impairs patients' quality of life and is almost uniformly fatal after complications of parenteral nutrition. Intestinal and multivisceral transplants have been used as alternative treatment of these disorders. We studied patients with GID treated with transplantation in our center, and reviewed their outcome to determine the therapeutic efficacy of multivisceral transplants.


METHODS
The transplant database was searched for patients with GID from 1994 to 2001. We excluded patients with Hirschsprung disease, scleroderma, and diabetic enteropathy. We reviewed explanted organs, histochemistry, and immunohistochemistry and classified cases by etiology.


RESULTS
We selected 12 children with GID from 124 patients transplanted. Nine presented before 1 year and 3 started with symptoms between 2 and 8 years. By combined clinical and histopathological features, 6 were classified as megacystis microcolon intestinal hypoperistalsis syndrome, 4 as chronic idiopathic intestinal pseudoobstruction, and 2 as intestinal neuronal dysplasias. Six patients died during the follow-up from 21 to 546 days after transplant. The Kaplan-Meier actuarial survival rates were 66.7% at 1 year and 50% at 3 years.


CONCLUSIONS
Multivisceral transplantation is a valuable therapeutic alternative for children with severe GID who cannot be adequately managed with parenteral nutrition.
Loinaz
Carmelo
C
Department of Surgery, 12 de Octubre Hospital, Complutense University of Madrid, Madrid 28041, Spain.
Rodríguez
Maria M
MM
Kato
Tomoaki
T
Mittal
Naveen
N
Romaguera
Rita L
RL
Bruce
Jocelyn H
JH
Nishida
Seigo
S
Levi
David
D
Madariaga
Juan
J
Tzakis
Andreas G
AG
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D005767:Gastrointestinal Diseases / Q000601:surgery*; D005769:Gastrointestinal Motility; D006801:Humans; D007223:Infant; D007422:Intestines / Q000637:transplantation*; D008297:Male; D012720:Severity of Illness Index; D014781:Viscera / Q000637:transplantation*