Intestinal transplantation for total intestinal aganglionosis: a series of 12 consecutive children

Frederique Sauvat, Chiara Grimaldi, Florence Lacaille, Franck Ruemmele, Laurent Dupic, Nathalie Bourdaud, Fabio Fusaro, Virginie Colomb, Dominique Jan, Jean Pierre Cezard, Yves Aigrain, Yann Revillon, Olivier Goulet

Research output: Contribution to journalArticle

31 Scopus citations

Abstract

Background: Management of patients with total intestinal aganglionosis (TIA) is a medical challenge because of their dependency on parenteral nutrition (PN). Intestinal transplantation (ITx) represents the only alternative treatment for patients with irreversible intestinal failure for achieving intestinal autonomy. Methods: Among 66 patients who underwent ITx in our center, 12 had TIA. They received either isolated ITx (n = 4) or liver-ITx (LITx, n = 8) after 10 to 144 months of total PN. All grafts included the right colon. Results: After a median follow-up of 57 months, the survival rate was 62.5% in the LITx group and 100% in the ITx patients. The graft survival rate was 62.5% in the LITx group and 75% in the ITx group. All the surviving patients were fully weaned from total PN, after a median of 57 days. Pull through of the colon allograft was carried out in all patients. Fecal continence is normal in all but one of the surviving children. Conclusion: These results suggest that ITx with colon grafting should be the preferred therapeutic option in TIA. Early referral to a transplantation center after diagnosis of TIA is critical to prevent PN-related cirrhosis and thereby to permit ITx, which is associated with a good survival rate.

Original languageEnglish (US)
Pages (from-to)1833-1838
Number of pages6
JournalJournal of Pediatric Surgery
Volume43
Issue number10
DOIs
StatePublished - Oct 1 2008
Externally publishedYes

Keywords

  • Fecal continence
  • Hirschsprung's disease
  • Intestinal transplantation

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health

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    Sauvat, F., Grimaldi, C., Lacaille, F., Ruemmele, F., Dupic, L., Bourdaud, N., Fusaro, F., Colomb, V., Jan, D., Cezard, J. P., Aigrain, Y., Revillon, Y., & Goulet, O. (2008). Intestinal transplantation for total intestinal aganglionosis: a series of 12 consecutive children. Journal of Pediatric Surgery, 43(10), 1833-1838. https://doi.org/10.1016/j.jpedsurg.2008.03.028