本邦における Hirschsprung 病類縁疾患の実態 : 全国93施設アンケート調査成績

概要

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Questinnaires were sent to the councilors of the Japanese Society of Pediatric Surgeons requesting data on Pseudo-Hirschsprung's disease. Two definitions of Pseudo-Hirschsprung's disease were given: 1) congenital, chronic, nonmechanical obstruction of the intestine, and 2) the presence of intramural ganglion cells in the terminal rectum. A total of 93 replies was received, reporting 148 cases of Pseudo-Hirschsprung's disease of which 77 were abnormal in the intramural ganglia and 42 were normal, and 29 were unknoun. In the abnormal group of the intramural ganglia, there were immaturity of ganglia or oligoganglionosis (54), neuronal intestinal displasia (15), and segmental anomaly (8). In the normal group of myenteric plexus, 22 were chronic idiopathic intestinal pseudoobstruction syndorome (CIIPS), 12 CIIPS suspected, and 8 megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS). The most of oligoganglionosis (or hypoganglionosis) or immaturity of ganglia showed negative rectoanal reflex and normal Ach-E activity, while the most of the normal group of intramural ganglia revealed positive rectoanal reflex and normal Ach-E activity. The prognosis of oligoganglionosis (or hypoganglionosis), CIIPS, and MMIHS were very poor, considering that these are benign diseases. Pseudo-Hirschsprung's disease was suggested to be truely one of serious diseases in children.
特定非営利活動法人日本小児外科学会の論文
1988-12-20