Abstract

Background: Peutz-Jeghers syndrome (PJS) is a hereditary disorder characterized by mucocutaneous pigmentation and hamartomatous polyps. Polyposis mainly occurs in the small bowel, which can lead to intestinal intussusception and bleeding. Using double-balloon enteroscopy (DBE) the whole small bowel can be endoscopically visualized and polypectomy can be performed during the same procedure. The aim of this study was to assess diagnostic and therapeutic efficacy, and safety using DBE for treatment and surveillance in PJS patients. Methods: Retrospectively, PJS patients who had DBE were evaluated, using the DBE-database of our institution. DBE procedural data, location, number and size of small bowel polyps, earlier findings on gastroduodeno- and colonoscopy were evaluated. Large polyps were defined as >10 mm in diameter and with a stalk. Results: Between October 2004 and November 2008 11 PJS patients (7 males, mean age 34 (17-51) years) were examined with DBE. In 9 (82%) patients earlier small bowel surgery was performed due to complicated small bowel polyps. All patients had known (multiple small) polyps in the stomach or duodenum, and, except for 1 patient, all had known colonic polyps. The indications for DBE were surveillance in 13 (54%), abdominal complaints in 7 (29%), and suspicion of small bowel polyps on magnetic resonance enteroclysis in 4 (17%) patients. A total of 24 DBE procedures, 21 proximal and 3 distal, were performed. Mean insertion depth of proximal and distal procedures was 231 (140-300) cm and 145 (140-150) cm, respectively. Mean duration time of DBE was 72 (60-100) minutes. Conscious sedation was used in 14 (58%) procedures and general anesthesia was used in 10 (42%) procedures. Small bowel polyps were found in all patients, and in 22 (92%) procedures. A total of 72 large polyps were found, 68 (94%) of which were located in duodenum and proximal jejunum (within 150cm beyond ligament of treitz). In total 66 (92%) large polyps were removed using a polypectomy snare (maximal diameter 50 mm) after injection of the stalk with a diluted epinephrine-saline solution. No complications occurred. After introduction of DBE as surveillance technique in this PJS patients group, no complicated small bowel obstruction occurred after a follow-up of 252 person months (23 months per patient). Conclusions: DBE seems safe and clinically useful for diagnosis and therapy of small bowel polyps in PJS patients, even in patients with earlier extensive abdominal surgery. As majority of polyps are found in the proximal small bowel, proximal DBE is the preferred route in these patients. DBE can decrease the need for laparotomy in PJS patients.

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