Intestinal and multivisceral transplantation in children with severe gastrointestinal dysmotility

Carmelo Loinaz, Maria M. Rodríguez, Tomoaki Kato, Naveen Mittal, Rita L. Romaguera, Jocelyn H. Bruce, Seigo Nishida, David Levi, Juan Madariaga, Andreas G. Tzakis

Research output: Contribution to journalArticlepeer-review

36 Scopus citations

Abstract

Background/Purpose: 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.

Original languageEnglish (US)
Pages (from-to)1598-1604
Number of pages7
JournalJournal of Pediatric Surgery
Volume40
Issue number10
DOIs
StatePublished - Oct 1 2005

Keywords

  • Intestinal dysmotility
  • Intestinal transplantation
  • Multivisceral transplantation
  • Pseudoobstruction

ASJC Scopus subject areas

  • Surgery

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