A 45-year-old man is diagnosed with familial adenomatous polyposis (FAP) confirmed by APC gene mutation. He has >1000 colonic polyps and a 2 cm rectal polyp with high-grade dysplasia. What is the recommended prophylactic surgery?
- A Restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA) given high-grade rectal dysplasia ✓
- B Subtotal colectomy with ileorectal anastomosis (IRA) in all FAP patients
- C Segmental colectomy and surveillance of remaining colon
- D Sulindac and endoscopic polypectomy surveillance
Explanation
In FAP, prophylactic surgery removes the entire colorectal mucosa. The choice between ileorectal anastomosis (IRA) and restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA) depends on rectal burden: IRA is acceptable when <20 rectal polyps and no rectal dysplasia, preserving rectal function but requiring lifelong rectal surveillance. When the rectum has dense polyposis or dysplasia (as here with high-grade dysplasia), IPAA (total proctocolectomy with J-pouch) is recommended to eliminate all at-risk mucosa and prevent rectal cancer. The rectum is at highest risk of cancer in FAP and often dictates the surgical choice.
Reference: Bailey & Love's Short Practice of Surgery, 27th ed.
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Written and medically reviewed by the StethoPrep medical team.