Gardner's syndrome

Abstract

Summary Two unrelated cases of Gardner’s syndrome are reported and complications and problems involved in their management are reviewed. The first patient had recurrent partial obstruction of the small bowel, caused by intramesentery fibrosis which will probably cause his death. The second condition was diagnosed correctly because the patient had an osteoma of the mandible. From a review of the literature, a plan of management has been established as follows. All patients more than 12 years old should have a colectomy with an ileoproctostomy unless carcinoma of the rectum is present. Postoperatively, proctoscopic examination should be performed at intervals of 3 to 6 months to discover if there are new polyps or if carcinoma is present in the rectal stump. A high incidence of desmoid tumors and abnormal fibrosis should be expected and patients who have carcinoma should not be denied further therapy on the assumption that the masses are recurrent carcinoma. In the management of relatives and children, a plea is made for investigating, for polyposis, all patients who have any accompaniments suggestive of Gardner’s syndrome. Here, we believe, is a chance for real cancer prophylaxis.