The cardinal feature is the development of numerous adenomatous polyps throughout the lower gastrointestinal tract. Since each of the polyps appears to share the predisposition to malignancy, colorectal carcinoma is almost inevitable, usually appearing about 15 years after the development of adenomatosis. Adenomatosis develops during late childhood, may be symptomless for many years, and it is not unusual for the presenting features to be caused by the development of one or more carcinomas. Accepted for publication 30 November 1997. Received 22 July 1997.
Diagnosis is based on clinical findings, barium contrast examination and endoscopic evaluation.
It is the risk of malignant transformation that necessitates the removal of all colonic mucosa. The first recorded operation for polyposis was carried out in 1918. Since then, there have been changes in the management of the disease. The basic principle, however, remains the prevention of colorectal carcinoma, either by removing all diseased tissue or excising the colon and monitoring the rectal remnant. Panproctocolectomy with a permanent ileostomy was one of the first operations performed, and is still in use today.