| Biallelic (homozygous or compound heterozygous) MUTYH disease variants | - Colonoscopy with polypectomy every 1 to 2 years, starting at 25 to 30 years of age.
- Colectomy in patients with any 1 of the following:
- Documented or suspected colorectal cancer.
- Multiple large/dysplastic polyps and/or significant polyp burden that cannot be effectively managed with colonoscopy and polypectomy.
- In patients with a subtotal colectomy with ileorectal anastomosis, sigmoidoscopy 6 months after surgery and annually thereafter.
- Upper endoscopy and duodenoscopy starting at age 30 to 35 years.
- Complete polypectomy or sampling of duodenal polyps at the time of initial polyp discovery and on each subsequent examination. An abnormal-appearing papilla should be biopsied.
- Interval for subsequent upper endoscopy is based on the severity of duodenal polyposis as classified by the Modified Spigelman score:
- Stage 0: Every 4 to 5 years
- Stage I: Every 2 to 3 years
- Stage II: Every 1 to 3 years
- Stage III: Every 6 to 12 months
- Stage IV: In the absence of surgery (duodenectomy), surveillance every 6 months
- Annual thyroid screening by physical examination and ultrasound.
- Routine screening for other MAP-associated cancers is not recommended.
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