Endoscopic Therapy of Small Bowel Polyps in Cowden Syndrome using Double-Balloon Enteroscopy: A Case Series

Abstract

Purpose: Cowden syndrome (CS) is a rare genetic condition with hamartomatous polyposis. It has 30% small bowel involvement which may cause intussusception and other complications. Based on our systematic literature search using PubMed, Embase and Scopus; successful use of double-balloon enteroscopy (DBE) for small bowel polyps in CS patients has only been reported twice. The largest series comprise of only 2 pediatric cases (Urs et al., Arch Dis Child, 2011). In this case series, we aim to report the feasibility and safety of DBE for removal of large intestinal polyps in CS patients. Methods: We retrieved CS cases from a database of 642 DBE procedures at a tertiary referral center. Demographic factors and endoscopy outcomes were recorded. Results: We identified 3 CS patients who underwent a total of 4 DBE procedures. All patients met the National Comprehensive Cancer Network diagnostic criteria for CS. Table 1 summarizes the patients' characteristics. Patient 1 and 3 had small bowel polyps > 1 cm, detected on prior CT enterography. Patient 2 was referred for DBE for surveillance after establishing the diagnosis of CS. Both patients 2 and 3 had prior episodes of intussusception. Patient 3 required partial small bowel resection as a child from intestinal obstruction. DBE was performed by 2 interventional endoscopists using the Fujinon double-balloon enteroscopy system. General anesthesia was used for sedation for all DBE procedures. Endoscopic resection of large intestinal polyps was successful on all 4 DBE procedures. Patient 1 and 3 each had a single antegrade (oral approach) DBE, reaching up to the distal jejunum. Patient 2 required two separate sessions (antegrade followed by a retrograde approach), yielding 30 polyps for each session. Maximum size of resected polyps range from 1 to 3.4 cm. Procedure duration was 2-3.5 hrs. All endoscopically resected polyps were hamartomatous growths, confirmed by histopathology. There were no complications. All 3 patients have been followed for 23 to 36 mos. without any repeat intussusception, bleeding or small bowel obstruction.Table: [1768] Table 1. Patient CharacteristicsConclusion: In this case series, DBE provided successful and safe endoscopic removal of small bowel polyps in patients with CS. Consistent with CS, all intestinal polyps retrieved did not have any evidence of dysplasia.