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

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.


Keywords


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

Full Text:

html PDF


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

Refbacks

  • 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 with PUBMED, PUBMED CENTRAL (PMC),  Indexcopernicus International, Google Scholar, NewJour, Open J-gate, Science Central, eDoctoronline, Ulrichsweb, Scirus, JournalTOCs, ResearchGate, Scribed, FreeJournalAct, Anoox, News-Digest, CORE.

EL-MED-Pub Publishers