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Case Studies | Surgery | India | Volume 12 Issue 1, January 2023 | Rating: 5 / 10
An Unusual Case of Intussusceptions Revealing Peutz-Jeghers Syndrome
Dr Nishad Ahmed | Dr Shivashankar Bhat | Dr Rakshit Shetty
Abstract: Introduction: Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant disorder characterized by hyperpigmentation on the lips and oral cavity and gastrointestinal (GI) hamartomatous polyps. PJS patients usually present with acute complications such as bleeding, bowel obstruction and intussusception. Presentation of case: We hereby report a case of a 25-year-old male, who presented with complaints of colicky abdominal pain since 4 days. On abdominal ex-amination, tenderness in the right iliac fossa and increased bowel sounds. Patient presented 3 months ago with bleeding pr and rectal mass. Ultra sound of the abdomen demonstrated suspected ileo-colic intussusception. The patient underwent colonoscopy showed pedunculated polyp and polypectomy from ileo-caecal region. The symptoms did not subside after 2 days, hence was taken up for a mid-line laparotomy that showed an ileo-colic and jejuno-jejunal intussusception. Reduction of this intussusception was successfully done with, multiple enterotomy and polypectomy. The specimen further confirmed the diagnosis of hamartomatous Peutz-Jeghers polyps with no malignancy. Physical examination revealed multiple pigmented spots on the oral cavity and palm. Thus, the diagnosis of PJS was made. Discussion: PJS is a rare inherited disease that often remains undiagnosed for many years. Patients often presents with acute complications such as bleeding per rectum, intussusception or obstruction. Conclusion: Early detection and identification of PJS in patients and their family members can improve the prognosis, prevent complications. Patients should be on regular follow up once the diagnosis has been made.
Keywords: Peutz-Jeghers syndrome, Intussusception, Intestinal obstruction, Muco-cutaneous, pigmentation, Hamartomatous polyp
Edition: Volume 12 Issue 1, January 2023,
Pages: 458 - 460