Entire colon aganglionosis,and extensive aganglionosis: Analysis of 94 cases in Japan

Incidence, sex distribution, length of the involved bowel, associated malformations, treatment and operative results were analysed in 94 cases of entire colon aganglionosis, 87 of which were collected from 42 institutions in Japan and seven treated in our department. In this series, 62 were entire colon aganglionosis and 32 were extensive aganglionosis. Incidence of entire colon aganglionosis was four per cent of all aganglionosis seen, and that of extensive aganglionosis was two per cent. Male: female ratio was 2∶1 in cases with entire colon aganglionosis and 1.3∶1 in extensive aganglionosis. Associated malformation was rare in both of entire colon, or extensive aganglionosis. Of the 94 cases, 41 cases survived neonatal period, and cure was obtained in 23 of entire colon aganglionosis and three of extensive aganglionosis.