WOFAPS 2025 8th World Congress of Pediatric Surgery

Linara Khabibullina

Russian Children’s Clinical Hospital, Moscow, Russia

Background: Familial Adenomatous Polyposis (FAP) is the second most common cause of colorectal cancer including in children and adolescents. Therefore, it is necessary to identify a specific group of patients with FAP who require early colectomy.

Aim: To identify pediatric patients with FAP who require colectomy before age 15, and to analyze clinical and genetic features associated with early surgical intervention.

Methods: This retrospective cohort study included 82 children with FAP managed between 2000 and 2025. Data collected included age at disease onset, family history, and concomitant upper GI polyposis, and genotypes.

Results: Of 82 children with FAP, 37 patients underwent colectomy and 18 underwent colectomy before age 15. Among these patients, 6 had de novo APC mutations. Extensive colonic polyposis (carpeting of the adenomas) was identified in 11 patients. One patient developed colorectal adenocarcinoma at age 14 and that patient doesn’t have family history of FAP. Surgical approaches included total proctocolectomy with ileal pouch-anal anastomosis (12 patients) and colectomy with ileorectal anastomosis (5 patients), total proctocolectomy with ileostomy (1 patient).

Conclusions: Despite guideline recommendations to initiate surveillance at ages 12-14 and delay colectomy until later adolescence, a subset of pediatric patients with FAP require colectomy before age 15 due to aggressive disease features. These findings underscore the need for individualized management strategies and highlight the importance of genotype-phenotype correlation in surgical decision-making