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Revista Española de Enfermedades Digestivas

Print version ISSN 1130-0108


LOPEZ-RUIZ, José Antonio et al. Hirschsprung disease with debut in adult age as acute intestinal obstruction: case report. Rev. esp. enferm. dig. [online]. 2016, vol.108, n.11, pp.742-746. ISSN 1130-0108.

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.

Keywords : Hirschsprung disease; Megacolon; Intestinal obstruction.

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