Guidelines on Management of Hereditary Polyposis Syndromes in Pediatric Patients: Agreement, Disagreement and Where It Matters

Hereditary polyposis syndromes are rare but potentially devastating conditions which require multidisciplinary care from an early age. Societal guidelines, which ideally combine expert opinions and medical evidence, serve as the framework for disease diagnosis, surveillance and treatment. However, there is a significant underrepresentation of pediatric gastroenterology input in guideline formulation, and additionally, recommendations can vary significantly between societies, which can have a moderate-to-high clinical impact on patient care. This paper aims to summarize key differences in management based on societal guidelines and identify some of the factors which may contribute to divergence in care in hereditary polyposis syndromes in pediatric patients. The authors review the literature underlying the divergence in recommendations and attempt to reconcile these differences with a closer consideration of the pediatric population, considering the available evidence. This review highlights the need to harmonize recommendations across subspecialties and professional societies and sheds light on the significant underrepresentation of pediatric gastroenterology input in guideline formulation. Given the poor-quality evidence underlying many societal guidelines and the lack of pediatric gastroenterology representation in guideline formulation in these rare syndromes, there is a need for collaborative, multicenter, registry-based studies to refine and improve care standards.