Small Intestinal Polyp Burden in Pediatric Peutz-Jeghers Syndrome

Abstract

The management of pediatric Peutz Jeghers Syndrome (PJS) focuses on the prevention of obstruction from intussusception complicating small intestinal polyposis. This hinges on the accurate appraisal of polyp burden to tailor therapeutic intervention. Video capsule endoscopy (VCE) is an established tool to determine small intestinal polyp burden in children but an in-depth characterization of polyp burden in this population with PJS is lacking. Methods: Study design; retrospective longitudinal cross-sectional analysis of VCE studies in pediatric PJS patients followed at our institution 2010 – 2020. Demographic, clinical history, and VCE findings reported by three reviewers in tandem were accrued. Polyp burden variables were modeled as functions of patient and study characteristics using linear mixed models adjusted for clustering. Results:The cohort included 16 patients, mean age 13.4 years at first VCE. Total small bowel polyp count and largest polyp size clustered under 30 polyps and < 20mm with only 4 studies detecting > 10 polyps and polyps > 21 mm in size. Luminal occlusion correlated closely to estimated polyp size. Polyp distribution favored proximal (77%) over distal (66 %) involvement of the small bowel. Adjusted largest polyp size was greater in male over female patients. Double balloon enteroscopy was associated with decreased polyp number and decreased luminal occlusion categories. Discussion: The polyp burden in pediatric PJS favors the proximal third of the small intestine, relatively small numbers, and polyp size amenable to resection through device assisted enteroscopy (DAE). Male gender correlated with larger polyps; older age related to increased polyp number. (247 WORDS)