Peutz-Jeghers Hamartoma: The Cause of Intermittent Gastric Outlet Obstruction

Abstract

Peutz-Jeghers Syndrome (PJS) is a rare, autosomal dominant inherited disease that manifests with gastrointestinal Hamartomatous polyps and mucocutaneous pigmentation. PJS can cause bleeding, anemia, and intussusception. We report the case of a 21 year old male patient who was diagnosed with Peutz-Jeghers syndrome at age 13 in Ukraine, and after immigration to the US, was referred to our institution for evaluation of intermittent episodes of abdominal pain, vomiting, and anemia. During endoscopic evaluation he was found to have multiple hamartomatous polyps throughout the gastrointestinal tract. One of the largest hamartomas located in the duodenal bulb was approximately 4 cm in diameter, and the culprit of intermittent gastric outlet obstruction. The patient's symptoms of episodic abdominal pain and vomiting resolved after polypectomy of this large duodenal bulb hamartomatous polyp. Peutz-Jeghers Syndrome is usually known to cause intussusception; gastric outlet obstruction, however, is a rare presentation of PJS. Treatment in PJS is geared mainly at removal of polyps, endoscopically, when possible, while minimizing surgical resections. Patients with PJS need close follow ups and monitoring due to increased riskst of gastrointesinal and extra-intestional malignancies during their lifetime.Figure 1Figure 2Figure 3