Most patients with familial adenomatous polyposis (FAP) develop colorectal adenomas in the second decade of their life. If they are not treated promptly at this stage they will develop colorectal cancer in there 30s or 40s.
Treatment with NSAIDs may reduce the size and number of adenomas, but the only curative treatment of FAP is surgery.
|With ileo-rectal anastomosis, the risk of dying from rectal cancer by age 65 was 12.5%.|
The two surgical options are proctocolectomy followed by ileum-pouch-anal anastomosis (IPAA) or total colectomy followed by ileorectal anastomosis (IRA).
IRA leaves a risk of rectal cancer remaining, and necessitates continuous endoscopic follow-up.
Dr H Vasen and colleagues assessed the risk of dying from rectal cancer after IRA, and the success of regular endoscopic follow-up in the early diagnosis of these cancers. The research team also compared life expectancy after the two surgical techniques.
The study, published in the latest Gut, found that 47 of the 659 patients undergoing IRA developed rectal cancer. The risk of dying from rectal cancer by age 65 was 12.5%.
75% of patients had a negative rectoscopy within the 12 months before the diagnosis of rectal cancer. This suggests that follow-up endoscopy does not have a sufficient preventative effect on mortality from rectal cancer.
Dr Vasen finds that IPAA would increase life expectancy by 1.8 years compared to IRA.
However, IPAA has its own disadvantages, including a risk of severe post-operative complications. In the worst case the pouch may have to be removed and an ileostomy constructed.
A recent study by Dr Vasen's group found no difference in quality of life between successful IRA and IPAA.