Hirschsprung disease with debut in adult age as acute intestinal obstruction: case report

• Published in: Revista española de enfermedades digestivas Vol. 108; no. 11; pp. 742 - 746
• Main Authors: López Ruiz, José Antonio, Tallón Aguilar, Luis, Sánchez Moreno, Laura, López Pérez, José, Pareja Ciuró, Felipe, Oliva Mompeán, Fernando, Padillo Ruiz, F Javier
• Format: Journal Article
• Language: English
• Published: Spain Sociedad Española de Patología Digestiva 01.11.2016; Aran Ediciones
• Subjects: Abdominal Pain - diagnostic imaging; Abdominal Pain - etiology; Gastroenterology & Hepatology; Geriatrics & Gerontology; Hirschsprung disease; Hirschsprung Disease - diagnostic imaging; Hirschsprung Disease - surgery; Humans; Infectious Diseases; Intestinal obstruction; Intestinal Obstruction - diagnostic imaging; Intestinal Obstruction - etiology; Male; Megacolon; Middle Aged; Oncology; Pathology; Pharmacology & Pharmacy; Radiology, Nuclear Medicine & Medical Imaging; Surgery; Intestinal obstruction; Enfermedad de Hirschsprung; Obstrucción intestinal; Hirschsprung disease; Megacolon
• ISSN: 1130-0108
• DOI: 10.17235/reed.2016.3841/2015

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.