Abstract
Hirschsprung's disease has been thought to have genetic factors, and several familial occurrences of Hirschsprung's disease have been reported. Recently, we have experienced Hirschsprung's disease occurred in 3 children of 5 siblings. The eldest sister had a short segment aganglionosis, the younger sister had a total colonic aganglionosis, and the youngest brother had an extensive aganglionosis. Only the youngest boy, who was operated by Z-shaped anastomosis with right colon patch, is alive now. We have survayed 10 affected siblings in 4 families in our department. There have been 98 affected cases in 45 families, including our cases, in Japanese literatures. The cases of familial occurrence showed higher rates of female and of total colonic or extensive aganglionosis than those in sporadic cases.
