Familial Near-Total Intestinal Aganglionosis

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

doi:10.21699/jns.v6i3.562

Familial Near-Total Intestinal Aganglionosis

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

Authors

Hidouri Saida^{1

2}, Zitouni Hayet¹, Chahed Jamila^{1

2}, Mosbahi Sana^{1

2}, Belhassen Samia^{1

2}, Ksiaa Amine^{1

2}, Hmida Badii³, Krichene Imed^{1

2}, Sahnoun Lassad^{1

2}, Mekki Mongi^{1

2}, Belguith Mohsen^{1

2}, Nouri Abdellatif^{1

2}

Affiliations

¹ Department of Pediatric Surgery, Medical School Hospital of Monastir, Tunisia.
² Research Laboratory LR12SP13. Faculty of Medicine of Monastir, Tunisia.
³ Department of Radiology, Medical School Hospital of Monastir, Tunisia.

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