Unfamiliar aspects of familial polyposis coli

Patients with familial polyposis coli or Gardner's syndrome are at risk for a variety of extracolonic manifestations. In a series of patients followed at the University of Washington, we have found several recurring and unusual manifestations, including upper gastrointestinal polyposis, small bowel obstruction secondary to desmoid tumors or adhesions, recurring pancreatitis, and adenoma of the papilla of Vater. In one family with familial polyposis only, a set of twins had different manifestations; one twin had familial polyposis only, whereas the second had classic extracolonic manifestations of Gardner's syndrome. Multiple rectal adenomas developed in a woman with an ileorectal anastomosis with each of three pregnancies. Spontaneous regression occurred after each delivery. Multiple rectal adenomas developed in her daughter on two occasions while taking birth control pills. Physicians caring for these patients should look for such manifestations.