1130-0108/2016/108/11/742-746 REVISTA ESPAÑOLA DE ENFERMEDADES DIGESTIVAS © Copyright 2016. SEPD y © ARÁN EDICIONES, S.L. REV ESP ENFERM DIG 2016, Vol. 108, N.º 11, pp. 742-746 CASE REPORTS ABSTRACT 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 agangli- onic 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. Key words: Hirschsprung disease. Megacolon. Intestinal obstruc- tion. INTRODUCTION Hirschsprung’s disease (HD) is a malformation of the large intestine characterized by the absence of ganglion cells in submucosal and myenteric plexus, which pro- duces a functional obstruction and dilatation proximal to the affected segment (1). This congenital disease is usually detected in neonatal period. It is rarely diag- nosed in adult age. Approximately 300 cases have been reported in literature. These patients tend to have milder forms of the disease, in which hypertrophy of innervated proximal colon compensates the functional obstruction by lack of innervation of the distal area (2). We report a case of a 54-year-old male patient that was admitted in the Emergency Department because of a massive mega- colon that required a total colectomy with ileum-rectal anastomosis. CASE REPORT A 54-year-old male was admitted in the Emergency Department by generalized abdominal pain accompanied by anorexia of several days duration. Previous history of meningioma operated in 1998, intestinal resection in child- hood (unknown etiology), chronic constipation, smoker of 20 cigarettes per day and moderate drinker. The patient reported difficulty for expelling gases, although he expelled stools without pathological products. No nausea or vomit- ing. No fever. Exploration showed a hemodynamically sta- ble patient, eupneic with blood pressure 138/97 and heart rate 100 beats per minute. Abdomen was very distended and tympanic, with previous laparotomy scar, painful on palpa- tion diffusely. Peritoneal irritation was not found. Peristal- sis was present. Abdominal X-rays showed a dilated colon occupying abdominal cavity. Laboratory studies revealed leukocytosis with left deviation and elevated C-reactive pro- tein. A CT scan was performed, showing a massive dilatation of sigmoid colon, which compresses liver to the right upper quadrant, stomach into the left upper quadrant and bowel loops into the right flank (Figs. 1 and 2). A transition zone was appreciated in distal sigmoid colon. Rectum had a nor- mal size. Peripheral aerial imagery was found in the dilated segment, gas-like wall, which is thickened in this segment. Given these findings, emergency surgery was decided. A midline laparotomy was performed, finding a massive dila- tation of sigmoid colon, attaining a diameter of 44 cm at its more dilated portion. The rest of the colon was also dilat- ed. Rectum had normal caliber. It was decided to perform a total colectomy with ileum-rectal anastomosis. After 24 hours, the patient presented hemodynamic instability, with severe abdominal pain. CT scan was performed, showing Received: 18-05-2015 Accepted: 04-09-2015 Correspondence: José Antonio López-Ruiz. Emergency Surgery Unit. Department of General and Gastrointestinal Surgery. Complejo Hospitalario Virgen Macarena-Virgen del Rocío. Av. Doctor Fedriani, 3. 41007 Sevilla, Spain e-mail: wurdalach@gmail.com López-Ruiz JA, Tallón-Aguilar L, Sánchez-Moreno L, López-Pérez J, Pareja- Ciuró F, Oliva-Mompeán F, Padillo-Ruiz J. Hirschsprung disease with debut in adult age as acute intestinal obstruction: case report. Rev Esp Enferm Dig 2016;108(11):742-746. DOI: 10.17235/reed.2016.3841/2015 Hirschsprung disease with debut in adult age as acute intestinal obstruction: case report José Antonio López-Ruiz, Luis Tallón-Aguilar, Laura Sánchez-Moreno, José López-Pérez, Felipe Pareja-Ciuró, Fernando Oliva-Mompeán and Javier Padillo-Ruiz Emergency Surgery Unit. Department of General and Gastrointestinal Surgery. Hospital Universitario Virgen Macarena. Seville, Spain