Peutz-Jeghers症候群引起腸套疊個案報告

Intussusception Caused by Peutz-Jeghers Syndrome: A Case Report and Literature Review

Peutz-Jeghers syndrome (PJS) is a rare, though well-described, hereditary polyposis syndrome associated with mucocutaneous pigmentation that typically presents in the second decade of life with complications related to intestinal polyps. We report a case of intestinal obstruction associated with intermittent abdominal cramping pain and copious vomiting. Ultrasonography revealed a large target sign lesion over the upper abdomen. Computerized tomography of the abdomen disclosed jejunal intussuception. Exploratory surgery with reduction and segmental resection of the involved jejunum was performed. Histopathologic study revealed numerous hamartomas, with the biggest one measuring 4cm in diameter. The patient’s post-operative course was uneventful. Herein, we report on this rare case, review the literature, and suggest long-term follow up of these kind of cases to early detect malignancy.