A RARE CASE OF PEUTZ JEGHER SYNDROME PRESENTING WITH INTUSSUSCEPTION CASE REPORT AND REVIEW OF LITERATURE

Abstract

OBJECTIVE Peutz Jegher's syndrome is a rare entity with an occurrence of 150000 to 1120000 and  intussusception in adults is another rare entity with an  occurrence of 5 of all intussusceptions. Here we report a rare case of intestinal obstruction which turned out to be a case of Peutz Jegher's syndrome with jejunal polyps with co-existing intussusception and oral melanin pigmentation. METHOD Case report and review of medical literature. CASE REPORT A 25 year old male presented to the casualty with complaints of acute abdominal pain, vomiting and constipation of 4 days duration. The pain was intermittent, colicky type of pain which was aggravated with food intake and associated with nausea and vomiting. He had no significant family history. On examination, he had intra oral melanin pigmentation.   Abdomen was distended with restricted movement with     respiration. He had diffuse abdominal tenderness and             guarding and bowel sounds were absent. X ray abdomen erect showed multiple air fluid levels and USG showed target sign and CECT showed sausage shaped mass in small bowel with collapsed distal large bowel suggestive of   Intussusception. Laparotomy was done and intraoperative findings were 2 intraluminal polyps with jejuno-jejunal  intussusception. Limited resection and end to end    anastomosis was done. Post-operative period was uneventful and histopathological examination showed hamartomatous polyp with extensive smooth muscle arborization suggestive of Peutz-Jeghers syndrome. Patient is under regular followup. CONCLUSION Peutz Jeghers syndrome with intra luminal polyps occurring as jejuno-jejunal intussusception in adult life is a rare entity. Sporadic Peutz Jeghers syndrome is an even rarer entity. Intussusceptions require operative management when they present as intestinal obstruction. These patients require lifelong follow-up as they are prone to develop              malignancies in other organs.