Peutz-Jeghers Syndrome: Clinical Presentation and new Therapeutic Strategy

Summary

A new treatment strategy is presented and evaluated in 5 patients with Peutz-Jeghers syndrome, in an effort to preserve bowel integrity and to eliminate the threat of a short bowel. After conventional endoscopic polypectomy of all reachable lesions, peroperative endoscopic polypectomy is scheduled. Endoscopic peroperative panpolypectomy is carried out and only in bulky, broadbased, multiple or invaginating polyps is an enterotomy or a resection - kept to a minimum thanks to the information provided by the endoscopist - performed. The procedure was well tolerated, hospitalization was remarkably short, and the subsequent follow-up period free of readmission - in contrast to previous observation periods. It is, however, a tedious and time-consuming method, and close collaboration between endoscopist and surgeon is urgently required.

Key words:

Peutz Jeghers syndrome - Hamartoma - Intussusception - Endoscopic polypectomy - Peroperative endoscopy