Polyp Progression in Paediatric Patients With Familial Adenomatous Polyposis: A Single-centre Experience
Chukwuemeka C Anele1,2,3, Jinpo Xiang4, Isabel Martin1,5
1The Polyposis Registry, St Mark's Hospital, London North West University Healthcare NHS Trust, Harrow.
Insights
Familial adenomatous polyposis (FAP) management in children shows stable adenoma counts during surveillance. Tailored colonoscopy intervals based on individual FAP phenotype are recommended over fixed schedules.
Area of Science:
- Pediatric Gastroenterology
- Gastrointestinal Oncology
- Clinical Genetics
Background:
- Familial adenomatous polyposis (FAP) management emphasizes prophylactic colectomy at premalignant stages.
- Colonoscopy surveillance is crucial for children diagnosed with FAP before surgical intervention.
Purpose of the Study:
- To investigate the natural history of FAP in pediatric patients.
- To evaluate adenoma progression rates and identify factors influencing the timing of colectomy in children with FAP.
Main Methods:
- Retrospective analysis of pediatric FAP patients (<18 years) with multiple colonoscopies.
- Data collection included demographics, endoscopy, genetics, and surgical history.
- Adenoma counts and polyp progression rates were assessed, considering polypectomies.
Main Results:
- Eighty-four children with FAP were analyzed; 79% had <100 adenomas at initial colonoscopy.
- The median polyp progression rate was 12.5 adenomas/year.
- Polyp progression did not influence the surgical approach in patients who underwent colectomy.
Conclusions:
- Adenoma burden remains stable for most children with FAP undergoing surveillance.
- Personalized surveillance intervals, guided by FAP phenotype, are more appropriate than standardized schedules.
Objectives:
Prophylactic colectomy at a premalignant stage is the cornerstone of management of familial adenomatous polyposis (FAP). Before surgery, colonoscopy surveillance is recommended in children with FAP. This study aimed to examine the natural history of FAP in children by evaluating adenoma progression and factors influencing timing of colectomy.
Method:
Patients with FAP younger than 18 years at first surveillance colonoscopy and who had undergone more than 1 colonoscopy were identified. Demographic, endoscopic, genetic, and surgical data were retrieved. Cumulative adenoma (polyp) counts were obtained while accounting for any polypectomies during the study period. The rate of polyp progression and factors influencing the timing of colectomy were evaluated.
Results:
Eighty-four patients (50% boys; mean age at first colonoscopy 13 years [standard deviation 1.97]) were identified, of which 83 had a family history of FAP. At first colonoscopy, 67 (79%) had <100 adenomas and 29 (35%) had colonic polyps identified despite rectal sparing. The median rate of polyp progression per patient was 12.5 polyps/year (range 0-145). Of the 45 (54%) patients who had undergone surgery, 41 (91%) underwent colectomy with ileorectal or ileodistal sigmoid anastomosis. Polyp progression did not alter the choice of surgical intervention in any patient.
Conclusion:
Our results suggest that adenoma number remains relatively stable in the majority of children under surveillance. Tailored surveillance intervals according to phenotype are a more appropriate strategy as recommended by recently published guidelines.
