Duodenal intussusception and pancreatitis due to a giant hamartomatous polyp in an infant. Case report and review of literature

Abstract

Peutz–Jeghers syndrome is a rare genetic disorder characterized by the development of benign hamartomatous polyps in the gastrointestinal tract and mucocutaneous pigmented macules on the lips, hands, and oral mucosa. Only 7% of patients present initially with intussusception, usually at the age of 10 years. Duodenal polyps are exceedingly rare, with only 0.4% of patients affected. We here describe a case of a 1-year-old infant who presented with severe non-bilious vomiting for 2 months and was diagnosed with a giant duodenal hamartomatous polyp. The polyp was pre-ampullary and caused intermittent duodenal intussusception and acute pancreatitis. This case is the youngest PJS patient presenting with duodenal obstructive symptoms and acute pancreatitis.