[PDF] Management of Peutz-Jeghers syndromes in children and adolescents : a position paper from the ESPGHAN Polyposis Working Group

Source:
European Society for Paediatric Gastroenterology Hepatology and Nutrition
Publisher:
European Society for Paediatric Gastroenterology Hepatology and Nutrition
Publication date:
19 December 2018

Abstract

Peutz-jeghers syndrome (PJS) is a well-described inherited syndrome, characterised by the development of gastrointestinal polyps and characteristic mucocutaneous freckling. Development of small bowel intestinal polyps may lead to intussusception in childrenmay require emergency laparotomy with potential loss of bowel. Gastrointestinal (GI) polyps may lead to bleeding and anaemia. This ESPGHAN position paper provides a guide for diagnosis, assessment and management of PJS in children and adolescentsand guidance on avoiding complications from PJS or from the endoscopic procedures performed on these patients.This is the first position paper regarding PJS published by ESPGHAN. Literature from PubMed, Medline and Embase was reviewed and in the absence of evidence, recommendations reflect the opinion of paediatric and adult experts involved in the care of polyposissyndromes. Because many of the studies that form the basis for the recommendations were descriptive and/or retrospective in nature, some of the recommendations are based on expert opinion. This position paper will be helpful in the appropriate management and timing of procedures in children and adolescents with PJS.