S1571 Device-Assisted Enteroscopy (DAE) in the Surveillance of Intestinal Polyposis in Peutz-Jeghers Syndrome

Abstract

Introduction: Peutz-Jeghers Syndrome (PJS) is an autosomal dominant, genetic disorder characterized by the formation of hamartomatous polyps in the gastrointestinal tract. These polyps are a source of significant morbidity and mortality in this population due to associated complications of intestinal obstruction, bleeding, and malignant transformation. Surveillance of intestinal polyposis in patients with PJS has not been extensively studied, although centers which perform a significant volume of device-assisted enteroscopies (DAEs) encounter multiple cases each year. With the progressive adoption of DAE, examination of the deep small bowel for monitoring and prophylactic polypectomy has the potential to prevent complications of PJS and minimize the need for repeated laparotomy. Methods: After obtaining IRB approval, electronic health records were used to identify all DAEs performed on patients with PJS at 3 US referral centers (LSU, Duke, and UMass) between January 1, 2007 and January 1, 2020. Electronic medical records were reviewed to collect and analyze multiple data points. Primary endpoints included the complications associated with DAE performed for removal of intestinal hamartomas and the rate of laparotomy in PJS patients prior to and after index DAE. Secondary data points included patient characteristics, procedural details, and size/location/distribution of small bowel hamartomas. Results: Twenty-four patients met our inclusion criteria. Of these, 18 (75%) had previously undergone small bowel surgery for complications related to small bowel hamartomas. Between 2007 - 2020, a total of 46 DAEs were performed in these patients with an average of one surveillance exam every 2.5 years. A total of 131 polypectomies were performed during our study period with an observed complication rate of 1.5%, including one bowel perforation requiring surgery. Only one patient underwent surgery related to small bowel hamartomas following initial DAE surveillance exam over a total of 366 years of aggregated follow-up (Figure). Conclusion: Endoscopic management of small bowel polyps in patients with PJS using DAE is an effective strategy for prophylactic removal of hamartomas. DAE surveillance and endoscopic polypectomy is safe and may decrease the need for repeated laparotomy due to complications from intestinal hamartomas in patients with PJS.Figure 1.: Hamartomatous polyp in the ileum removed with saline injection-lift technique and hot snare polypectomy