Management

Colectomy of varying degrees is necessary for familial polyposis coli as otherwise all patients develop adenocarcinoma by the age of 40 years and in about half of all cases, these malignancies are multiple. The surgical options are:

• proctocolectomy and ileostomy:
  • abolishes the risk of large bowel cancer
  • a stoma is required, presenting psychological problems
    
    
• colectomy and ileorectal anastomosis:
  • does not abolish the risk of large bowel cancer
  • lifelong 6 monthly surveillance of the rectal stump is mandatory
    
    
• proctocolectomy and ileoanal pouch:
  • abolishes the risk of large bowel cancer
  • preferred by patients to ileostomy
  • functionally inferior, and more prone to operative complications

The offspring of affected patients should be carefully screened on a periodic basis until about the age of 35 by colonoscopy and double contrast barium enemas. Testing for faecal occult blood is insufficient.

Although polyps rarely develop before puberty, endoscopic surveillance of at-risk individuals should occur from early teens - colectomy is indicated once polyposis has been established (1).

Reference:

1. Pulse (2005); 65(14):55-60.