JEJUNO-JEJUNAL INTUSSUSCEPTION IN A PATIENT WITH PEUTZ-JEGHERS SYNDROME: A CASE REPORT AND REVIEW

Abstract

Peutz-Jeghers syndrome is an autosomal dominant genetic disorder associated with pigmented spots on the skin and mucous membrane especially around oral mucosa, lips, nasal alae, palm and soles, as well as hamartomatous polyps in the alimentary canal. Prevalence of Peutz-Jeghers syndrome is between 1 in 8300 and 1 in 280000 people. Here, we present a case of 18 years old lady who presented with complains of peri-umbilical pain and vomiting for 2 months. Physical examination revealed multiple pigmentations over lips, palms and soles. Ultrasonography was normal. On further evaluation using a contrast enhanced computerized tomographic scan of abdomen and pelvis, jejuno-jejunal intussusception was noted. She then underwent exploratory laparotomy along with resection and anastomosis of the involved jejunal segment. Biopsy specimen containing multiple pedunculated polyps was sent for histopathological examination which revealed hamartomatous polyp.