Gardner's Syndrome

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Abstract
The largest family group yet recognized to have Gardner's syndrome is reported. Gardner's syndrome is transmitted as a dominant hereditary disorder, but whether or not more than one gene is involved is not yet established. The main clinical features of the disorder are multiple polyposis of the colon, cystic lesions of the skin, fibrous tissue tumors, osteomatosis, and dental anomalies. Of these, multiple polyposis is the most important because of its malignant potential and because it is amenable to surgical correction before malignancy develops. The most important feature from the standpoint of early recognition of the disorder is the occurrence of disfiguring cystic and fibrous lesions of the skin. Reasonable criteria for evaluation of the colon of patients presenting with the overt manifestations of Gardner's syndrome are reviewed, and some additional criteria are proposed.