Surgical aspects of hereditary intestinal polyposis

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Abstract
THERE IS PROBABLY no known disease of a more cer- tain premalignant nature than hereditary intestinal pol- yposis. The fact that the entire colonic and rectal mucosa is presumably at risk for the development of carcinoma should remain foremost in the thinking of those charged with the management of patients with this disease. Unfortunately, excision of all mucosal surfaces at risk is currently the only known preventive measure. However, it must be remembered that most polyposis patients are now diagnosed at an early age and at an asymptomatic stage of their disease. Many of them will understandably be reluctant to accept the proctocolectomy which is con- sidered by most physicians to be the ideal operation from the pathologic perspective. Several different surgical approaches to hereditary polyposis have been developed. This paper describes commonly used procedures and attempts to place each into its proper perspective. ingly, among patients in whom carcinoma developed, females outnumbered males almost two to one although the male-to-female ratio of patients in the study was 3.5 to 1. Significantly, those patients in whom rectal carcinoma developed had a very poor outlook, with only 25 per cent surviving five years. One reason for the poor results with ileorectal anastomosis appeared to be the frequency with which early invasive cancers were initially treated by fulguration. This excellent series of 178 patients was reevaluated ten years later. 2 Rectal cancer had not occurred in any of the 35 patients who had no rectal polyps preop- eratively. However, cancer had developed in 46 (32 per cent) of 143 patients with multiple colonic polyposis during a median follow-up of nearly 20 years. There was a significant inverse association between the number of rectal polyps present in patients preoperatively and the amount of time they remained free of rectal cancer. There was also a strong relationship between the presence of cancer in the resected colon and subsequent development of rectal carcinoma. They stressed that cancer risk in the retained segment of large bowel could not be established by extended postoperative observation. Most other studies have shown a low incidence of rectal cancer after ileorectal anastomosis for polyposis, but most have had a short follow-up and are therefore unreliable. The major British study, from St. Mark's Hospital in London, is of interest, as colectomy with ileorectal anastomosis has long been the favored procedure. 3 Rectal cancer developed in only 4 per cent of 73 patients whose progress was followed for up to 25 years. However, only 15 of the patients had more than 15 years of follow-up. The controversy remains unresolved. Most surgeons, however, feel that only a short segment (12 cm) should be retained in patients treated by colectomy and ileorectal anastomosis.