title: Megacolon in adulthood after surgical treatment of Hirschsprung's disease in early childhood.
abstract: Hirschsprung's disease (HD) is a disorder associated with congenital malformation of the enteric nervous system with segmental aganglionosis. Prevailing therapy includes a resection of the affected part of the bowel. However, patients often do not obtain complete functional improvement after surgical treatment. We present the case of a 25-year-old woman who had surgical treatment of HD in early childhood. After that procedure she had clinical features of constipation for years in the end, passing of stool once a week, requiring laxatives and enemas. We diagnosed an incomplete resection of the aganglionic bowel via rectal biopsy and resected the remaining aganglionic segment. Two months after surgery the patient's bowel function improved to a frequency of 1-4 stools per day. We conclude that regular follow-up is required to identify HD patients with persistent alterations of bowel function after surgery. In patients presenting with constipation, recognition of a remaining aganglionic segment or other alterations of the enteric nervous system should be aimed at in an early stage.
authors.affiliation: Department of Medicine, Division of Hepatology and Gastroenterology, Charité, Campus Virchow-Klinikum, Universitätsmedizin Berlin, Augustenburger Platz 1, Germany.
authors.forename: Christoph R
authors.initials: CR
authors.lastname: Werner
authors.forename: Gisela
authors.initials: G
authors.lastname: Stoltenburg-Didinger
authors.forename: Henning
authors.initials: H
authors.lastname: Weidemann
authors.forename: Christoph
authors.initials: C
authors.lastname: Benckert
authors.forename: Marco
authors.initials: M
authors.lastname: Schmidtmann
authors.forename: Ivo R
authors.initials: IR
authors.lastname: van der Voort
authors.forename: Viola
authors.initials: V
authors.lastname: Andresen
authors.forename: Burghard F
authors.initials: BF
authors.lastname: Klapp
authors.forename: Peter
authors.initials: P
authors.lastname: Neuhaus
authors.forename: Bertram
authors.initials: B
authors.lastname: Wiedenmann
authors.forename: Hubert
authors.initials: H
authors.lastname: Mönnikes
mesh_terms: D000328:Adult; D003106:Colon / Q000294:innervation / Q000473:pathology* / Q000601:surgery*; D005260:Female; D006627:Hirschsprung Disease / Q000150:complications* / Q000601:surgery*; D006801:Humans; D007223:Infant