Familial Near-Total Intestinal Aganglionosis

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


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.


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

Full Text:

html PDF

DOI: http://dx.doi.org/10.21699/jns.v6i3.562


  • There are currently no refbacks.

Copyright (c) 2017 Saida

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.

The Journal of Neonatal Surgery has been indexed in Google Scholar, Worldcat, ROAD (Directory of Open Access Scholarly Resources), THE KEEPERS, Publons, OALib (Open Access Library), NewJour, Open J-gate, Science Central, Cite Factor, eDoctoronline, Ulrichsweb, Scirus, JournalTOCs, Journal Database, GFMER, SJIFactor, Journal Seek, ResearchGate, Scribed, FreeJournalAct, Anoox, News-Digest, CORE, ICI World of Journals.

EL-MED-Pub Publishers