Management of Peutz-Jeghers syndromes in children and adolescents: A Position Paper from the ESPGHAN Polyposis Working Group

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 children may 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 adolescents and 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 polyposis syndromes. 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. Address correspondence and reprint requests to Dr Warren Hyer, Consultant Paediatric Gastroenterologist, St Mark's Hospital Polyposis Registry, St Mark's Hospital, Watford Road, Harrow HA1 3UJ (e-mail: polyposiswg@drhyer). Received 12 July, 2018 Accepted 21 November, 2018 Support and financial interest: None to declare. No external funding sought nor obtained DISCLAIMER ESPGHAN is not responsible for the practices of physicians and provides guidelines and position papers as indicators of best practice only. Diagnosis and treatment is at the discretion of physicians". Role in submitted paper: Andrew Latchford: Created answers, researched answers, editing, expertise Shlomi Cohen: researched answers, editing, expertise Marcus Auth: researched answers, editing, expertise Michele Scaillon: Created answers, researched answers Richard Daniels: Created answers, researched answers Cecile Talbotec: Created answers, researched answers Thomas Attard: researched answers, editing, expertise Jerome Viala: Created answers, researched answers Warren Hyer: Created answers, researched answers, editing, expertise, prepared manuscript. Conflict of interest: None of the authors nor the ESPGHAN Polyposis Working group have any conflicts of interest to declare. Conflict of interest: Andrew Latchford: None to declare Shlomi Cohen: None to declare Marcus Auth: None to declare Michele Scaillon: None to declare Richard Daniels: None to declare Cecile Talbotec: None to declare Thomas Attard: None to declare Jerome Viala: None to declare Warren Hyer: None to declare © 2018 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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