HIGH-TECH DIAGNOSTIC METHODS AND AN ENTEROSCOPIC TREATMENT OF CHILDREN WITH PEUTZ-JEGHERS SYNDROME

Abstract

Peutz-Jeghers syndrome (PJS) is an autosomal dominant hereditary hamartomatous polyposis with predominant localization in the jejunum and ileum. Because of PJS polyps differ from adenomatous ones, the performing polypectomy is associated with a high risk of bowel perforation. During 2015-2017 18 PJS children were comprehensively examined including esophagogastroduodenoscopy, colonoscopy, and video capsule endoscopy There was developed a step-by-step procedure for the removal of polyps common in all parts of the small intestine, including the creation of a “persistent pillow”; electroexcision of a polyp and application of a clip to the region of the womb of a removed polyp. Polyps more than 7 mm detected in the stomach and duodenum, were removed during endogastroduodenoscopy, and in the colon - under a colonoscopy. During video capsular endoscopy, polyps with a diameter of 2 mm to 2.5 cm were detected in the deep parts of the small intestine. For their surgical removal, during a therapeutic single-balloon enteroscopy, an electroscission of all polyps was performed. The postoperative period was uneventful. The combination of a single balloon enteroscopy with an electroexcision of polyps is suggested by the authors as the optimal method for the diagnosis and treatment of PJS in children, which allows avoiding delayed perforations of the small intestine in the postoperative period. Thanks to this technique, enteroscopy becomes the only possible alternative to bowel resection in PJS children.