家族性近全肠无神经节症

Familial Near-Total Intestinal Aganglionosis.

作者信息

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

机构信息

Department of Pediatric Surgery, Medical School Hospital of Monastir, Tunisia.

Research Laboratory LR12SP13. Faculty of Medicine of Monastir, Tunisia.

出版信息

J Neonatal Surg. 2017 Aug 10;6(3):62. doi: 10.21699/jns.v6i3.562. eCollection 2017 Jul-Sep.

DOI:10.21699/jns.v6i3.562

PMID:28920022

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5593481/

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.

摘要

近乎全肠无神经节细胞症是先天性巨结肠最极端且罕见的一种形式。它可能会影响家族中的多名成员。我们报告了一个家庭中三例近乎全肠无神经节细胞症的病例，这些病例在出生时均表现为肠梗阻。他们都接受了手术，并进行了空肠造口术。预后很差。