Familial adenomatous polyposis syndromes

Patients with familial adenomatous polyposis (FAP)/attenuated FAP treated with prophylactic total proctocolectomy and careful screening for duodenal polyposis have a longer life expectancy than that of earlier generations of people with this disease. However, patients with FAP/attenuated FAP still have a higher likelihood of dying than their age-matched healthy counterparts. Major causes of FAP-related morbidity include perioperative complications, duodenal/periampullary malignancy, and desmoids.[63]Nugent KP, Spigelman AD, Phillips RK. Life expectancy after colectomy and ileorectal anastomosis for familial adenomatous polyposis. Dis Colon Rectum. 1993;36:1059-1062. http://www.ncbi.nlm.nih.gov/pubmed/8223060?tool=bestpractice.com [64]Galle TS, Juel K, Bulow S. Causes of death in familial adenomatous polyposis. Scand J Gastroenterol. 1999;34:808-812. http://www.ncbi.nlm.nih.gov/pubmed/10499482?tool=bestpractice.com