Peutz-Jeghers Syndrome Complicated by Intestinal Intussusception: A Case Report

Abstract

Peutz-Jeghers Syndrome (PJS) is a rare genetic condition characterized by the presence of hamartomatous polyps in the gastrointestinal tract and mucocutaneous hyperpigmentation. Intussusception is a relatively common complication in these patients, although progression to extensive intestinal necrosis is uncommon and potentially fatal. This report describes a 21-year-old woman with PJS diagnosed in childhood, admitted with acute abdominal pain, hyporexia, and vomiting. Computed tomography revealed extensive jejuno-ileal intussusception, and emergency laparotomy identified 90 cm of invaginated and necrotic small bowel caused by a hamartomatous polyp in the mid-jejunum. Segmental enterectomy with primary side-to-side anastomosis was performed, and the patient had a satisfactory postoperative recovery, being discharged on the fourth day. She remains under outpatient follow-up without signs of short bowel syndrome. This case highlights the importance of structured endoscopic surveillance and early surgical intervention in PJS, especially in settings where advanced enteroscopy techniques are not available.