Familial Near-Total Intestinal Aganglionosis

Authors

  • Saida Hidouri
  • Hayet Zitouni
  • Jamila Chahed
  • Sana Mosbahi
  • Samia Belhassen
  • Amine Ksiaa
  • Badii Hmida
  • Imed Krichene
  • Lassad Sahnoun
  • Mongi Mekki
  • Mohsen Belguith
  • Abdellatif Nouri

DOI:

https://doi.org/10.52783/jns.v6.427

Keywords:

Hirschsprung’s disease, Total intestinal aganglionosis, Genetic, Familial

Abstract

Near total aganglionosis represents the most extreme and rare form of Hirschsprung's disease. It can affect more than one member of family. We report three cases of near total intestinal aganglionosis in a family presenting with intestinal obstruction at birth. All of them were operated and a jejunostomy was performed. Outcome was dismal.

Downloads

Download data is not yet available.

Downloads

Published

2017-08-17

How to Cite

1.
Hidouri S, Zitouni H, Chahed J, Mosbahi S, Belhassen S, Ksiaa A, et al. Familial Near-Total Intestinal Aganglionosis. J Neonatal Surg [Internet]. 2017 Aug. 17 [cited 2026 Mar. 3];6(3):62. Available from: https://jneonatalsurg.com/index.php/jns/article/view/427

Issue

Vol. 6 No. 3 (2017): Journal of Neonatal Surgery

Section

Case Series

License

You are free to:

  • Adapt — remix, transform, and build upon the material for any purpose, even commercially.

Terms:

  • Attribution — You must give appropriate credit, provide a link to the license, and indicate if changes were made. You may do so in any reasonable manner, but not in any way that suggests the licensor endorses you or your use.
  • No additional restrictions — You may not apply legal terms or technological measures that legally restrict others from doing anything the license permits.