Table 14.

Statements pertaining to the lower gastrointestinal tract—extended version

Statements
Surveillance
LGI.1 At what age should surveillance commence in classical FAP? A: Surveillance should begin at 12 years of age in asymptomatic patients with a germline PV in the APC gene (for FAP disease), or in asymptomatic patients with first-degree relatives affected by classical FAP (if a genetic test is not available or if no PV is detected in the affected relative). B: In symptomatic patients with germline PV in the APC gene (for FAP disease), or patients with first-degree relatives affected by classical FAP if a genetic test is not available or if no PV is detected) colonoscopy should start at any age and as soon as possible.
LGI.2 At what age should surveillance commence in attenuated FAP? A: Surveillance can start later but no later than 18–20 years of age in asymptomatic patients with a germline PV in the APC gene for attenuated FAP disease and an attenuated proband/family phenotype. Alternatively, surveillance should also begin in asymptomatic patients with first-degree relatives affected by attenuated FAP, if a genetic test is not available or if no known pathogenic mutations are detected. B: Colonoscopy should start at any age and as soon as possible in symptomatic patients with a germline PV in the APC gene for attenuated FAP disease or in patients with first-degree relatives affected by attenuated FAP (if a genetic test is not available or if no known pathogenic mutations are detected).
LGI.3 What is the optimal modality for colorectal surveillance in classical FAP? The optimal modality for colorectal surveillance in classical FAP is high-definition white-light colonoscopy. Flexible sigmoidoscopy can be considered as an initial option, according to the patient’s preference. If adenomas are identified, then a high-definition white-light colonoscopy should be performed.
LGI.4 What is the optimal modality of colorectal surveillance in attenuated FAP? The optimal modality for colorectal surveillance in a-FAP is high-definition white-light colonoscopy.
LGI.5 What are the ideal intervals for endoscopic surveillance colonoscopy before prophylactic surgery in classical and attenuated FAP? A: Endoscopic surveillance of the colon should be adapted according to phenotype, genotype–phenotype, and the severity of the disease B: Repeat endoscopy should be performed within 1 year or less if at least one of the following criteria is present: APC germline PV of codon 1309 associated with a severe phenotype. Presence of ≥100 adenomas at colonoscopy. Presence of large polyps at colonoscopy (≥10 mm). Symptoms. Rapid progression in terms of polyp size.  C: Repeat endoscopy may be performed at 2 years when the phenotype shows all of the following criteria: Presence of 0–20 adenomas. Presence of small adenomas at colonoscopy (1–2 mm) Absence of symptoms.
LGI.6 Should screening colonoscopy routinely include advanced imaging technologies? A: White-light high-definition colonoscopy is sufficient for surveillance colonoscopy in FAP. B: There are insufficient data to recommend the use of advanced imaging technology. C: White-light endoscopy is sufficient in most cases; virtual or dye-based chromoendoscopy could have an advantage in discriminating between the clinical diagnosis of FAP versus a-FAP.
Surgery
LGI.7 When is prophylactic colorectal surgery indicated? Absolute indications for immediate colorectal surgery in FAP are: Certain or suspected cancer Severe symptoms from polyposis Severe disease (≥1000 polyps at colonoscopy) Unfavourable histological features (such as HGIEN, villous adenoma, etc.)  Indications for planned surgery are: Polyps >10 mm in diameter Favourable histological features Substantial increase in polyp number between examinations Sparse disease (100–1000 polyps)
LGI.8 Which patient characteristics support restorative proctocolectomy over total or subtotal colectomy for prophylactic surgery? A: IPAA may be offered to patients with either: 20 or more rectal adenomas. Approximately 500 or more colonic adenomas. APC mutation at codon 1250–1450.  IRA may be offered to: Patients with 5 or fewer rectal adenomas and <500 colonic adenomas.  B: Patients’ preference should be considered in the surgical choice.
LGI.9 Does prophylactic surgery need to include mesocolic/mesorectal excision? There is no conclusive evidence indicating a clear advantage or disadvantage in performing dissection with mesocolic/mesorectal excision.
LGI.10 Should a diverting ileostomy be routinely performed in total proctocolectomy with ileal pouch anal anastomosis? Routine diverting ileostomy is not mandatory when total proctocolectomy with IPAA is performed.
LGI.11 Is subtotal colectomy superior to total colectomy? When the rectum can be preserved, an ileo-sigmoid anastomosis could be considered to diminish the risk of anastomotic leak and improve functional outcome.
Post-surgical management
LGI.12 What is the appropriate management for patients with ileo-rectal anastomosis? A: The optimal modality for surveillance after an IRA is endoscopy. The surveillance interval should not exceed 2 years, starting from the colectomy, and should be individualized based on phenotype. B: All polyps >5 mm should be removed (endoscopically or with transanal excision). C: Secondary proctectomy should be considered when polyposis is no longer conservatively manageable or in the presence of 2 or more polyps with HGD.
LGI.13 When and how frequently should the ileo-anal pouch be surveilled? A: Endoscopic surveillance of an ileo-anal pouch should start 12 months after colectomy. B: Endoscopic surveillance of an ileo-anal pouch should be performed annually.
LGI.14 In the case of pouch adenoma/multiple adenomas/polyposis, what is the recommended treatment? A: Pouch adenomas may be managed endoscopically. B: In the presence of HGD in/of complete polyp resection, the pouch should be surveilled within 6 months. C: In the presence of two or more polyps with HGD, surgery may be considered.
LGI.15 In the case of pouch carcinoma, is pouchectomy/dismantling of the pouch indicated? In the case of pouch carcinoma, pouchectomy/dismantling of the pouch is indicated.
LGI.16 Should pouchoscopy also include the ileum proximal to the pouch? Expanding endoscopy to the more proximal small bowel should be performed during pouchoscopy in FAP patients after total proctocolectomy with IPAA.

a-FAP, attenuated familial adenomatous polyposis; FAP, familial adenomatous polyposis; HGD, high-grade dysplasia; HGIEN, high-grade intraepithelial neoplasia; IPAA, ileal pouch anal anastomosis; IRA, ileorectal anastomosis.