title: Megacolon in an adult case of hypoganglionosis, a pseudo-Hirschsprung's disease: an autopsy study.
abstract: We report an autopsied 20-year-old man case of intestinal necrosis associated with megacolon from hypoganglionosis, a pseudo-Hirschsprung's disease. The patient had suffered from severe constipation since two years of age, and presented abdominal distention from age ten. Autopsy revealed marked dilatation and necrosis of the entire large intestine. Although ganglion cells in the intestinal plexus were found throughout the large intestine, their number was reduced to 12-20% of that in the normal control. In pseudo-Hirschsprung's disease, there are occasional cases where an acute abdomen first presents itself in adulthood after running its course as chronic constipation.
authors.affiliation: Department of Internal Medicine, Saiseikai Utsunomiya Hospital. ito@jichi.ac.jp
authors.forename: Takeshi
authors.initials: T
authors.lastname: Ito
authors.forename: Tokuhiro
authors.initials: T
authors.lastname: Kimura
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Yagami
authors.forename: Naoki
authors.initials: N
authors.lastname: Maeda
authors.forename: Masayoshi
authors.initials: M
authors.lastname: Komura
authors.forename: Nobuki
authors.initials: N
authors.lastname: Ohnishi
authors.forename: Nobuya
authors.initials: N
authors.lastname: Fujita
authors.forename: Kunihiko
authors.initials: K
authors.lastname: Arai
authors.forename: Hideto
authors.initials: H
authors.lastname: Tomioka
authors.forename: Satoru
authors.initials: S
authors.lastname: Miyatake
authors.forename: Kenji
authors.initials: K
authors.lastname: Kobayashi
mesh_terms: D000328:Adult; D001344:Autopsy; D000013:Congenital Abnormalities / Q000503:physiopathology; D003645:Death, Sudden / Q000209:etiology; D005725:Ganglia, Autonomic / Q000473:pathology*; D006627:Hirschsprung Disease / Q000175:diagnosis; D006801:Humans; D007420:Intestine, Large / Q000473:pathology; D008297:Male; D008531:Megacolon / Q000209:etiology / Q000473:pathology*; D009336:Necrosis / Q000209:etiology / Q000473:pathology; D013368:Submucous Plexus / Q000473:pathology*