What is the management for a 23-year-old patient with 100 colorectal polyps and a positive family history of Familial Adenomatous Polyposis (FAP) syndrome?

Management of FAP with 100 Colorectal Polyps at Age 23

This patient requires prophylactic colectomy (Option A) - lifestyle modification alone is inappropriate and Miles operation is not indicated for benign polyposis.

Surgical Intervention is Mandatory

Colectomy is the definitive treatment for this patient and should be performed without delay. 1, 2 The presence of 100 colorectal polyps with confirmed FAP diagnosis (positive family history and colonoscopic findings) represents classical FAP requiring surgical intervention. 3

Why Surgery Cannot Be Deferred:

• Patients with FAP carry a 100% lifetime risk of colorectal cancer if the colon is not removed 3
• Without surgical intervention, FAP patients almost inevitably develop colorectal cancer by mean age 40-50 years 1
• At age 23, this patient is already within the recommended surgical window (ages 16-25 years for most classical FAP patients) 1, 2
• The presence of 100 polyps indicates classical FAP with high cancer risk, and patients with large numbers of polyps should be dissuaded from delaying surgery 1

Surgical Options: Colectomy vs. Miles Operation

The appropriate procedure is either total colectomy with ileorectal anastomosis (IRA) or proctocolectomy with ileal pouch-anal anastomosis (IPAA) - NOT Miles operation (abdominoperineal resection). 1

Decision Algorithm for Surgical Approach:

Choose IPAA (proctocolectomy with ileoanal pouch) if:

• Large number of rectal adenomas (>15-20 polyps) 1
• Rectal polyps >5 mm diameter 1
• High-grade dysplasia in rectal polyps 1
• Patient likely to be poorly compliant with follow-up surveillance 1

Choose IRA (colectomy with ileorectal anastomosis) if:

• Relative rectal sparing (<20 polyps) 1
• All rectal adenomas <5 mm diameter 1
• Patient desires better functional outcomes and can commit to lifelong surveillance 1

Why Miles Operation is Incorrect:

Miles operation (abdominoperineal resection with permanent end colostomy) is reserved for: 1

• Distal rectal cancer requiring radical resection
• Poor sphincter function or incontinence
• Cancers requiring pelvic radiation

This patient has benign polyposis without cancer, making Miles operation unnecessarily morbid and inappropriate. 1

Why Lifestyle Modification Alone is Unacceptable

Option C (lifestyle modification if asymptomatic) is categorically wrong and dangerous. 1, 2

• No lifestyle modifications prevent the inevitable progression to colorectal cancer in FAP 3
• Being asymptomatic is irrelevant - most FAP patients are asymptomatic for years until adenomas become large and numerous or cancer develops 3
• Surgery should be strongly recommended before age 25 years in documented APC gene mutation carriers 1
• Deferring surgery for personal reasons requires intensive surveillance (6-monthly flexible sigmoidoscopy AND annual colonoscopy), not simple lifestyle modification 1

Post-Surgical Surveillance Requirements

After surgery, lifelong surveillance is mandatory regardless of procedure chosen:

If IRA performed:

• Annual endoscopic examination of retained rectum for life (12-29% risk of rectal cancer) 1, 2
• More frequent surveillance (every 6-12 months) if significant rectal polyp burden 1

If IPAA performed:

• Annual surveillance of anorectal cuff for life 1
• Can be performed every 1-2 years in some protocols 1

Upper GI surveillance (regardless of colorectal surgery):

• Begin at age 25-30 years 1, 2, 4
• Frequency based on Spigelman staging of duodenal polyposis 1
• Duodenal cancer becomes major cause of mortality after colectomy 3, 5

Critical Pitfalls to Avoid

• Never delay surgery in classical FAP with 100+ polyps - the cancer risk is too high 1, 2
• Do not confuse this with attenuated FAP - attenuated FAP has <100 polyps (typically 10-100) and later age of onset, allowing consideration of intensive endoscopic management in select cases 1, 6
• Chemoprevention with NSAIDs/COX-2 inhibitors is only adjunctive - it reduces polyp burden but does not prevent cancer or replace surgery 1
• Genetic counseling and APC mutation testing should be performed to confirm diagnosis and allow family screening, but should not delay surgical planning 4, 7

Answer: A. Colectomy