Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
CASE REPORTS
Megacolon in an Adult Case of Hypoganglionosis, a Pseudo-Hirschsprung's Disease: An Autopsy Study
Takeshi Ito, Tokuhiro Kimura, Toshiaki Yagami, Naoki Maeda, Masayoshi Komura, Nobuki Ohnishi, Nobuya Fujita, Kunihiko Arai, Hideto Tomioka, Satoru Miyatake, Kenji Kobayashi
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Keywords: megacolon, pseudo-Hirschsprung's disease, hypoganglionosis, adult, autopsy
JOURNAL OPEN ACCESS

2008 Volume 47 Issue 5 Pages 421-425

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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.

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© 2008 by The Japanese Society of Internal Medicine
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