2021 P.J.S. Management Guidelines

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.