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Peutz-Jeghers Syndrome Presenting as a Large Adenomatous Gastric Polyp and Small Bowel Polyps Causing Intussusceptions


Shah, Samit Student*; Mead, Lisa Student; Trus, Thadeus MD; Rico, Ferdinand MD; Park, Thomas MD; Shah, Ashok MD, MACG

American Journal of Gastroenterology: September 2008 - Volume 103 - Issue - p S39–S40
Supplement Abstracts Submitted for the 73rd Annual Scientific Meeting of the American College of Gastroenterology: STOMACH

Gastroenterology, Surgery, School of Medicine and Dentistry, University of Rochester, Rochester, NY.

Purpose: A 22-year-old Caucasian woman with known Peutz-Jeghers syndrome (PJS) and characteristic perioral hyperpigmentation presented with a two-year history of intermittent postprandial epigastric pain associated with nausea and vomiting. She had undergone EGDs prior to her current presentation, which revealed a large antral polyp. Each time the polyp was resected and found to be a benign hamartoma. An upper GI and small bowel follow-through three months prior again revealed a large antral polyp plus three large polypoid masses in the small intestine with dilated segments proximal to these lesions. Also seen was a nonsustained coiled spring in the duodenum and proximal jejunum between two polypoid masses, which was consistent with intussusception. No complete obstruction was noted. A recent attempt to endoscopically resect the recurrent antral polyp was unsuccessful due to its size. Therefore, a surgical intervention was scheduled. The patient was taken to the operating room for a laparoscopic polypectomy and further examination of the small-bowel intussuscepted segments. An antral gastrotomy confirmed the presence of a large (7.6 × 5.1 cm) antral polyp herniating through the pylorus (Figure) which was subsequently resected. Two segments of intussuscepted small bowel were also noted, only one of which was able to be laparoscopically reduced. The segment of nonreducible intussusception was resected and several polyps, which were potential lead points for other intussusceptions, were removed via enterotomies. EGD afterwards revealed easy passage of the scope into the pyloric region. Over the subsequent 2 weeks after the operation, she reported a remarkable improvement and tolerated a regular diet without discomfort. Pathologic evaluation of the antral polyp revealed a hamartoma that contained extensive adenomatous change and focal high-grade dysplasia. The other polyps from the small intestine were hamartomas. Given the dysplastic changes in her antral polyp and her increased risk of cancer, we recommended annual surveillance EGDs.



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