Juvenile gastrointestinal polyposis.

Two cases of juvenile gastrointestinal polyposis are described and the literature is reviewed. Despite the benign nature of the individual polyps, the disease has a poor prognosis. Of 13 reported patients, 4 had macrocephaly, and one of the authors' 2 patients had large peripheral intracranial cysts. This appears to be the first reported documentation of the etiology of macrocephaly associated with juvenile polyposis. Major clinical problems are related to severe loss of blood and protein from the gastrointestinal tract.