Open AccessHirschsprung disease with debut in adult age as acute intestinal obstruction: case report
Author(s) -
José Antonio López Ruíz,
L Tallón Aguilar,
Laura Moreno,
José López Pérez,
Felipe Pareja Ciuró,
F Oliva Mompeán,
Javier Padillo-Ruíz
Publication year - 2016
Publication title -
revista española de enfermedades digestivas/revista española de enfermedades digestivas
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.331
H-Index - 38
eISSN - 2340-4167
pISSN - 1130-0108
DOI - 10.17235/reed.2016.3841/2015
Subject(s) - medicine , disease , myenteric plexus , constipation , ganglion , emergency surgery , surgery , gastroenterology , immunohistochemistry , anatomy
Hirschsprung's disease is characterized by absence of ganglion cells in submucosal and myenteric plexus of distal bowel. Most cases become manifest during the neonatal period, but in rare instances, this disease is initially diagnosed in adult age. It usually presents as severe constipation with colonic dilatation proximal to the aganglionic segment. The treatment is surgical, removing the aganglionic segment and restoring continuity of digestive tract. The disease rarely presents as an acute intestinal obstruction. We report a case not previously diagnosed, which presented as a massive colonic dilatation with a maximum diameter of 44 cm, with imminent risk of drilling that forced to perform an emergency surgery. We include a review of existing literature.