A 30 year old woman with Peutz-Jegher syndrome presented with nausea, vomiting and crampy left abdominal pain. She had undergone multiple abdominal surgeries since child hood for intussuception of the small bowel due to large hamartomatous polyps. Her physical exam was remarkable for tachycardia, hyperpigmented spots on her lips and an abdomen tender to deep palpation with no rebound tenderness.
A computer tomography of the abdomen and pelvis showed gastric polyps and dilated loops of bowel with obstruction from large polypoid lesions. A target sign was present.
After the benefits and risks of surgery were explained to the patient, she elected endoscopy with double balloon enteroscopy with polypectomy to avoid surgery and maintain small bowel function.
Both upper and lower endoscopy were performed.
Upon entry into the stomach, multiple large polypoid lesions were found throughout the stomach as well as duodenum. The larger polyps had a pedunculated appearance. Using monopolar cautery they were carefully removed.
A colonoscopy with double balloon enteroscopy showed multiple large polypoid lesions throughout the visualized small bowel, causing intussuception. Air was carefully insufflated. Polyps greater than 1 centimeter were snared using monopolar cautery.
According to the current limited data from case series, up to 68% of adults with Peutz-Jegher syndrome undergo laparotomy by the time they are 18 years of age. Of those, 39% undergo repeat laparatomy within 5 years. Removal of small bowel polyps larger than 1cm during laparotomy, a technique referred to as “clean sweep”, delays the need for repeat laparotomy.
Histopathology of polypoid lesions showed arborizing pattern with smooth muscle fibers consistent with Hamartomatous Polyps.
Our case demonstrates endoscopy with double balloon enteroscopy with removal of large polyps is a feasible alternative to laparotomy for intussuception.
? Giardiello FM, Trimbath JD. Peutz-Jeghers Syndrome and Management Recommendations. Clinical Gastroenterology and Hepatology 2006; 4:408-415.
? Hines R, Philp C, Hyer W, et al. Complications of Childhood Peutz-Jeghers Syndrome: Implications for Pediatric Screening. Journal of Paediatric Gastroenterology and Nutrition 2004;39: 219-220.
Michael Babineaux, MD, University of Texas Medical Branch
Gabriel Lee, MD, University of Texas Medical Branch
Andrew W. DuPont, M.D., University of Texas Medical Branch