Megacolon in an adult case of hypoganglionosis, a pseudo-Hirschsprung's disease: an autopsy study.
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.
Ito
Takeshi
T
Department of Internal Medicine, Saiseikai Utsunomiya Hospital. ito@jichi.ac.jp
Kimura
Tokuhiro
T
Yagami
Toshiaki
T
Maeda
Naoki
N
Komura
Masayoshi
M
Ohnishi
Nobuki
N
Fujita
Nobuya
N
Arai
Kunihiko
K
Tomioka
Hideto
H
Miyatake
Satoru
S
Kobayashi
Kenji
K
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*