Surgical treatment of familial adenomatous polyposis in children: cross-sectional study

Author:

Khabibullina L. R.1ORCID,Razumovsky A. Yu.2ORCID,Sherbakova O. V.1ORCID

Affiliation:

1. Russian Children’s Clinical Hospital

2. Pirogov Russian National Research Medical University

Abstract

Familial adenomatous polyposis (FAP) is an autosomal dominant disease caused by the APC gene mutation, characterized by colon adenomas and colorectal cancer, including in children. The issue of timing and indications for surgical treatment of FAP in childhood remains debatable.AIM: to identify predictors of surgery for FAP in pediatric patients.PATIENTS AND METHODS: a retrospective case-control study was conducted. The results of treatment of 50 children with FAP who were in the surgical department of the Russian Children's Clinical Hospital from January 2000 to April 2023 were analyzed. Two groups were formed: patients who underwent surgery of FAP under the age of 18 (case), and patients who did not undergo surgery at this age (control). We analyzed potential predictors: the age of manifestation, the clinical, the characteristics of adenomas, the anemia and family history, polyposis of the upper gastrointestinal tract.RESULTS: in the surgical group, the proportion of patients with more than 100 adenomas was higher (23 (88%) versus 11 (45%) (p=0.002)). It was revealed that the number of adenomas was more than 100 at the time of the first colonoscopy (OR 12 (95% CI (3–80), p=0.02) and the presence of colon bleeding (OR 5.8 (95% CI 1–35, p=0 .03) are independent predictors of colproctectomy in children.CONCLUSION: the number of adenomas over 100 and colon bleeding are independent predictors of colproctectomy in childhood.

Publisher

Russian Association of Coloproctology

Subject

Gastroenterology,Oncology,Surgery

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