Peutz-Jeghers syndrome from the point of view of a radiologist and an endoscopist

INTRODUCTION: Peutz-Jeghers syndrome (PJS) is a rare (orphan) hereditary disease with family history, primarily characterized by massive polyposis of the gastrointestinal tract and its complications. Every year, registered in 1 patient per 25,000–300,000 population. OBJECTIVE: To demonstrate effectiveness combined use diagnostic radiology endoscopic imaging techniques diagnosis this A case report aged 47 years given. complained persistent bleeding, thrombosis arteries upper extremity, ventral hernia after elimination intestinal intussusception. was admitted to A. V. Vishnevsky NMRC Surgery Ministry Health Russia (Moscow) determine tactics treatment. Computed tomography abdominal organs CT angiography extremities, esophagogastroduodenoscopy colonoscopy were performed. CONCLUSION: The difficulties treatment PJS associated complications illustrated. brief review literature (21 publications) Russian foreign authors on problems diagnosis, clinical manifestations According performed studies, total polyposis, left limb, postoperative revealed. Taking into account ongoing PJS, prescribed gastrectomy.