Louisiana State University Health Sciences Center Metairie, LA, United States
Omar Elfeky, MD1, Daniel Wild, MD2, Suraj Panjwani, MD3, David Cave, MD, PhD4, Daniel Raines, MD, FACG5 1Louisiana State University Health Sciences Center, Metairie, LA; 2Duke University Medical Center, Durham, NC; 3UMass Memorial Medical Center, Worcestor, MA; 4UMass Memorial Medical Center, Worcester, MA; 5Louisiana State University New Orleans, New Orleans, LA
Introduction: uetz-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, intussusception, bleeding and malignant transformation. Surveillance of intestinal polyposis in patients with PJS has not been well-described, although centers which perform a significant volume of device-assisted enteroscopy (DAE) encounter multiple cases each year. With the progressive adoption of DAE, examination of the deep small bowel for monitoring and prophylactic polypectomies (hamartomas >15mms) has the potential to prevent complications of PJS and need for repeated laparotomy. We hypothesize that DAE is a minimally invasive, efficacious method of surveillance of intestinal polyposis in patients with PJS.
Methods: After IRB approval, electronic medical records (EMRs) were used to identify all DAE’s performed on patients with PJS at three U.S. referral centers (Duke, LSU, and UMass). EMRs were reviewed to collect and analyze multiple data points. The rate of laparotomy in PJS patients prior to surveillance with DAE was compared to this same rate following enrollment in a DAE surveillance program. As second primary endpoints, we measured the number of hamartomas >15mms, size of hamartomas, location/distribution of hamartomas, procedure time for DAE, complication rate, and rate of total enteroscopy. The utility of adjunctive studies such as VCE and CT/MRE was also evaluated to determine their contribution to management compared to DAE.
Results: 48 total enteroscopies were performed on 23 patients with endoscopic resection of all small bowel polyps >15mm. 50% of patients have had repeat DAE studies within 2-3 years, and 70% of patients had adjuctive imaging studies after initial DAE surviellance which demonstrated efficacy of DAE. Of the study population, the rate of laparatomies due to complications of PJS prior to enrollment in the surviellance program to after DAE monitoring was 85%, while the rate post enrollment in the surviellance program was 4%. The complication rate was 2%.
Discussion: Our results showed that management of patients with PJP using DAE may be safe and effective in the management of intestinal polyposis. We demonstrate a significant decrease in rate of surgical interventions and surgical compilcations in our patient when compared to prior to DAE surviellance.
Omar Elfeky indicated no relevant financial relationships.
Daniel Wild indicated no relevant financial relationships.
Suraj Panjwani indicated no relevant financial relationships.
David Cave indicated no relevant financial relationships.
Daniel Raines indicated no relevant financial relationships.
Omar Elfeky, MD1, Daniel Wild, MD2, Suraj Panjwani, MD3, David Cave, MD, PhD4, Daniel Raines, MD, FACG5. P3007 - Device-Assisted Enteroscopy (DAE) in the Surveillance of Intestinal Polyposis in Peutz-Jeghers Syndrome, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.