Department of Gastroenterology and Hepatology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands.
Gastrointest Endosc. 2010 Apr;71(4):768-73. doi: 10.1016/j.gie.2009.11.005. Epub 2010 Feb 25.
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.
To assess therapeutic efficacy and safety of double-balloon enteroscopy (DBE) for detection and treatment of small-bowel polyps in patients with PJS.
Prospective cohort study.
Tertiary-care referral center.
This study involved 13 patients with PJS, defined as a proven STK11 gene mutation or according to international diagnostic criteria.
DBE with enteroscopic removal of pedunculated polyps of > or =10 mm.
Location, number and size of small-bowel polyps, polypectomy data, and complications and long-term complications associated with development of small-intestine polyps.
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.
Small number of patients.
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.
Peutz-Jeghers 综合征(PJS)是一种遗传性疾病,其特征为黏膜皮肤色素沉着和主要发生于小肠的错构瘤。这些息肉可能导致并发症,如肠套叠。
评估双气囊小肠镜(DBE)对 PJS 患者小肠息肉的检测和治疗的疗效和安全性。
前瞻性队列研究。
三级转诊中心。
本研究纳入了 13 例 PJS 患者,定义为 STK11 基因突变的证实或符合国际诊断标准。
行 DBE 并经内镜切除 >或=10mm 的有蒂息肉。
小肠息肉的位置、数量和大小、息肉切除术数据以及与小肠息肉发生相关的并发症和长期并发症。
13 例 PJS 患者(8 例男性,平均年龄 31 岁)共行 29 次 DBE 检查。10 例(77%)患者因小肠息肉而行部分小肠切除术。所有 13 例患者均发现小肠息肉。大多数息肉(94%)位于空肠近端。共发现 82 个 >或=10mm 的息肉,79 个(96%)息肉无并发症地经内镜切除。在 356 人年的随访期间,引入 DBE 后无与小肠息肉相关的并发症发生。
患者数量少。
即使在有广泛腹部手术史的患者中,DBE 对 PJS 患者的小肠息肉的诊断和治疗也是安全且有效的。DBE 可能减少 PJS 患者开腹手术的需求。