Clin Colon Rectal Surg 2016; 29(04): 321-329
DOI: 10.1055/s-0036-1584089
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Adenomatous Polyposis Syndromes: Diagnosis and Management

Jonathan B. Mitchem
1   Department of Colon and Rectal Surgery, Lahey Hospital and Medical Center, Burlington, Massachusetts
,
Jason F. Hall
1   Department of Colon and Rectal Surgery, Lahey Hospital and Medical Center, Burlington, Massachusetts
› Author Affiliations
Further Information

Publication History

Publication Date:
21 November 2016 (online)

Abstract

Familial adenomatous polyposis (FAP) syndromes make up fewer than 1% of patients diagnosed with colorectal cancer each year. Patients with familial polyposis syndromes including FAP, attenuated FAP, and MYH-associated polyposis (MAP), are an important group often cared for by colorectal surgeons. Registry and screening programs have been shown to improve survival in patients with adenomatous polyposis, as it allows patients to undergo surgical intervention prior to the development of colorectal cancer. There are several surgical options for the treatment of colorectal polyps in patients with adenomatous polyposis, so it is important to choose the appropriate procedure for each patient after discussing the risk of cancer in the rectal remnant, as well as bowel and sexual function in a predominantly young patient group. Regardless of procedure choice, long-term follow-up is important with yearly endoscopic evaluation of the pouch or remnant rectum, as well as appropriate screening for extracolonic malignancy. Adenomatous polyposis patients require an intense care regimen, but can have a normal lifespan with good quality when cared for appropriately.

 
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