Peutz Jegher's Syndrome - Double Intussusception in a Teenage Patient in a Single Setting - A Rare Case Report

Abstract

Peutz jegher's syndrome is a rare autosomal dominant disorder with hamartomatous polyps and mucocutaneous melanin pigmentation. This case report shows a case of a teenage Indian boy presented with intestinal obstruction due to double intussusception in a single setting due to the PJS polyps. This is the first case of double intussusception in a teenage patient with PJS in a single setting reported in India and fifth case in the world. A 14-year-old teenage boy presented to a surgical emergency with complaints of abdominal pain, bloody stools, and vomiting with characteristic ink black macules over buccal mucosa. On abdominal examination, sausage-shaped mass was palpable and he was provisionally diagnosed with Peutz-Jeghers Syndrome. Ultrasound abdomen findings favoured the diagnosis of intussusception at two sites. He underwent Emergency Laparotomy with resection & anastomosis of involved segments of the ileum and jejunum. Intraoperative findings showed Jejunojejunal intussusception 50 cm distal to DJ junction with 25 cm of gangrenous jejunum and ileoileal intussusception 30 cm proximal to ileocaecal junction which was histopathologically found to be hamartomatous polyps. The postoperative period was uneventful. He is under surveillance. Periodic surveillance is helpful in patients with PJS.