Clinical presentations and surgical approach of acute intussusception caused by Peutz-Jeghers syndrome in adults.

INTRODUCTION: Peutz-Jeghers syndrome is a rare autosomal dominantly inherited disease characterized by mucocutaneous pigmentations and gastrointestinal polyps. The polyps are located predominantly in the small intestine and usually cause intussusceptions. Adult intussusception caused by Peutz-Jeghers syndrome occurs very rarely. The purpose of this study was to analyze the clinical characteristics, preoperative diagnosis, and surgical management of Peutz-Jeghers syndrome associated with acute intussusception in adult patients. DISCUSSION: Consecutive patients with the postoperative diagnosis of acute intussusception caused by Peutz-Jeghers syndrome from 1995 to 2010 were reviewed retrospectively for this study. Data concerning clinical considerations, morphological examinations, and surgical procedure were analyzed. Different clinical manifestations were presented in patients with intussusception due to Peutz-Jeghers syndrome. Computed tomography associated or not with ultrasonography may be the most accurate examination for acute intussusceptions caused by Peutz-Jeghers syndrome. Surgical intervention is the first choice regimen in acute intussusceptions caused by Peutz-Jeghers syndrome. Prophylaxis and polypectomy of the entire small bowel is a worthy way in Peutz-Jeghers syndrome patients to reduce the frequency of laparotomies.