New perspective for the management of near-total or total intestinal aganglionosis in infants

K. Sharif, S. V. Beath, D. A. Kelly, P. McKiernan, I. Van Mourik, D. Mirza, A. D. Mayer, J. A C Buckels, J. De Ville de Goyet

Research output: Contribution to journalArticle

Abstract

Background Purpose: Extensive intestinal aganglionosis is rare. The diagnosis and treatment are known to be difficult and it had been considered to be fatal. The aim of this study was to review our experience with children with extensive intestinal aganglionosis. Methods: Retrospective analysis was conducted in patients referred to the intestinal transplantation unit since 1993. Presentation and outcome were analysed looking at 2 groups who had either undergone previous subtotal intestinal resection (group I) or no or limited resection (group II). Results. Eight children were selected (3 patients in group I and 5 in group II). Group I was remarkable in that patients all were referred early in age with progressing liver failure. Parents of one patient refused to accept transplantation as treatment, and he died one month later. Two noncirrhotic patients were maintained in the parenteral nutrition programme and currently progress well with enteral feedings. The other 5 patients underwent transplant, and 4 of 5 are alive after transplantation with a mean follow-up of 22.2 months (range 0.4 to 63.6). Conclusions: Subtotal resection of intestine at the time of diagnosis must be avoided. Conservative management with parenteral nutrition may be associated with long-term good outcome. Small bowel transplant may open new perspective in the management of patients with end-stage liver disease.

Original languageEnglish
Pages (from-to)25-28
Number of pages4
JournalJournal of Pediatric Surgery
Volume38
Issue number1
DOIs
Publication statusPublished - Jan 1 2003

Keywords

  • Hirschsprung disease
  • Intestinal transplantation
  • Intestine surgical resection
  • Parenteral nutrition
  • Surgical techniques
  • Total intestinal aganglionosis

ASJC Scopus subject areas

  • Surgery

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    Sharif, K., Beath, S. V., Kelly, D. A., McKiernan, P., Van Mourik, I., Mirza, D., Mayer, A. D., Buckels, J. A. C., & De Ville de Goyet, J. (2003). New perspective for the management of near-total or total intestinal aganglionosis in infants. Journal of Pediatric Surgery, 38(1), 25-28. https://doi.org/10.1053/jpsu.2003.50004