Familial polyposis coli in childhood.

Familial polyposis coli has generally been considered a disorder of adulthood that is rarely identified in children. We report 3 affected kindreds in which the disorder was diagnosed in 6 of 11 potentially affected children between 8 and 16 years of age. Experience with these kindreds demonstrates that polyps frequently develop during childhood in affected individuals. Colonoscopy was found to be preferable to the air-contrast barium enema, in that it was as sensitive a technique for detection of adenomas, and in that it also permitted collection of biopsies for histologic confirmation. We recommend that colonoscopy be performed late in the first decade of life before symptoms develop. Total colectomy, rectal mucosectomy, and ileo-anal anastomosis eliminated the risk of malignancy, preserved anal sphincter function by both clinical and manometric assessment, and was readily adapted to by these children.