BACKGROUND: Peutz-Jeghers syndrome (PJS) is a hereditary disorder characterized by mucocutaneous pigmentations and hamartomatous polyps mainly in the small bowel. These polyps may cause complications such as intussusception. OBJECTIVE: To assess therapeutic efficacy and safety of double-balloon enteroscopy (DBE) for detection and treatment of small-bowel polyps in patients with PJS. DESIGN: Prospective cohort study. SETTING: Tertiary-care referral center. PATIENTS: This study involved 13 patients with PJS, defined as a proven STK11 gene mutation or according to international diagnostic criteria. INTERVENTION: DBE with enteroscopic removal of pedunculated polyps of > or =10 mm. MAIN OUTCOME MEASUREMENTS: Location, number and size of small-bowel polyps, polypectomy data, and complications and long-term complications associated with development of small-intestine polyps. RESULTS: Thirteen patients with PJS (8 male, mean age 31 years) underwent 29 DBE procedures. Ten patients (77%) had a history of partial small-bowel resection because of small-bowel polyps. Small-bowel polyps were found in all 13 patients. The majority of polyps (94%) were located in the proximal jejunum. A total of 82 polyps of > or =10 mm were detected, and 79 (96%) were endoscopically removed without complications. After the introduction of DBE, no small-intestine-polyp-related complications occurred during a follow-up period of 356 person-months. LIMITATIONS: Small number of patients. CONCLUSION: DBE is clinically useful and safe for diagnosis and therapy of small-bowel polyps in patients with PJS, even in patients with a history of extensive abdominal surgery. DBE may decrease the need for laparotomy in patients with PJS. Copyright 2010 American Society for Gastrointestinal Endoscopy. Published by Mosby, Inc. All rights reserved.
BACKGROUND:Peutz-Jeghers syndrome (PJS) is a hereditary disorder characterized by mucocutaneous pigmentations and hamartomatous polyps mainly in the small bowel. These polyps may cause complications such as intussusception. OBJECTIVE: To assess therapeutic efficacy and safety of double-balloon enteroscopy (DBE) for detection and treatment of small-bowel polyps in patients with PJS. DESIGN: Prospective cohort study. SETTING: Tertiary-care referral center. PATIENTS: This study involved 13 patients with PJS, defined as a proven STK11 gene mutation or according to international diagnostic criteria. INTERVENTION: DBE with enteroscopic removal of pedunculated polyps of > or =10 mm. MAIN OUTCOME MEASUREMENTS: Location, number and size of small-bowel polyps, polypectomy data, and complications and long-term complications associated with development of small-intestine polyps. RESULTS: Thirteen patients with PJS (8 male, mean age 31 years) underwent 29 DBE procedures. Ten patients (77%) had a history of partial small-bowel resection because of small-bowel polyps. Small-bowel polyps were found in all 13 patients. The majority of polyps (94%) were located in the proximal jejunum. A total of 82 polyps of > or =10 mm were detected, and 79 (96%) were endoscopically removed without complications. After the introduction of DBE, no small-intestine-polyp-related complications occurred during a follow-up period of 356 person-months. LIMITATIONS: Small number of patients. CONCLUSION: DBE is clinically useful and safe for diagnosis and therapy of small-bowel polyps in patients with PJS, even in patients with a history of extensive abdominal surgery. DBE may decrease the need for laparotomy in patients with PJS. Copyright 2010 American Society for Gastrointestinal Endoscopy. Published by Mosby, Inc. All rights reserved.
Authors: G Perrod; E Samaha; E Perez-Cuadrado-Robles; A Berger; H Benosman; S Khater; A Vienne; C-A Cuenod; A Zaanan; P Laurent-Puig; G Rahmi; C Cellier Journal: United European Gastroenterol J Date: 2019-09-10 Impact factor: 4.623