title: Pseudo-Hirschsprung's disease with rectal hypoganglionosis: an autopsied case of circulatory failure due to severe constipation.
abstract: We report a 21-year-old female patient who suddenly died of circulatory failure due to pressure from megacolon allied with pseudo-Hirschsprung's disease. Since 3 years before her death, she had exhibited the feeling of abdominal distention, which was diagnosed as constipation. However, her constipation did not respond well to the prescribed oral administration of laxatives and enemas. She was found dead at home in the morning, lying on her back. An autopsy revealed a decreased number of ganglion cells in the rectum, suggesting hypoganglionosis. In cases of intractable chronic constipation, careful investigation of the cause of such symptoms is important.
authors.affiliation: Department of Plastic and Reconstructive Surgery, University of Tsukuba, Tsukuba City, Ibaraki, Japan.
authors.forename: Akio
authors.initials: A
authors.lastname: Nishijima
authors.affiliation: Department of Radiology, Tsukuba Medical Center, Tsukuba City, Ibaraki, Japan.
authors.forename: Seiji
authors.initials: S
authors.lastname: Shiotani
authors.affiliation: Department of Forensic Medicine, Tsukuba Medical Examiner's Office, Tsukuba City, Ibaraki, Japan.
authors.forename: Hideyuki
authors.initials: H
authors.lastname: Hayakawa
authors.affiliation: Department of Neurology, Aomori Prefectural Central Hospital, Aomori City, Aomori, Japan; Department of Neurophysiology, Institute of Brain Science, Hirosaki University Graduate School of Medicine, Hirosaki City, Aomori, Japan. Electronic address: hnishijima-tky@umin.ac.jp.
authors.forename: Haruo
authors.initials: H
authors.lastname: Nishijima
mesh_terms: D001344:Autopsy; D003248:Constipation / Q000150:complications*; D005260:Female; D045888:Ganglion Cysts / Q000473:pathology*; D006627:Hirschsprung Disease / Q000150:complications; D006801:Humans; D012002:Rectal Diseases / Q000150:complications* / Q000473:pathology; D012769:Shock / Q000209:etiology*; D055815:Young Adult
keywords: Adult; Autopsy; Hypoganglionosis; Megacolon; Postmortem computed tomography; Pseudo-Hirschsprung’s disease