Surgical approach to juvenile gastrointestinal polyposis

Familial adenomatous polyposis and Gardner syndrome are rare dominantly inherited syndromes characterized by hundreds to thousands of colonic adenomatous polyps. Colonic cancer occurs at a young age in both of them unless the colon is removed. Peutz-Jeghers syndrome and familial juvenile polyposis are inherited hamartomatous polyposis conditions with a low but well defined risk of colonic cancer. These four polyposis syndromes together account less than 1% of cases of colonic malignancies.

Familial juvenile polyposis is an autosomal dominant condition in which affected patients develop upper or lower gastrointestinal juvenile polyps, or both, and have a high possibility to develop a cancer of the gastrointestinal tract. Up to date the risk of G1 cancer in these patients has not been well defined because of the small number of families and lack of follow-up.

Single or few polyps can be frequently found in patients of any age and can occur also in infancy.

Polyps in children are classified as malignant or benign (tab. 1).