Peutz-Jeghers综合征诊治体会（附31例报告）

Objective To improve the level of diagnosis and treatment in patients with Peutz-Jeghers syndrome (PJS) in order to improve the patient's life quality. Methods The clinical characteristics of 31 patients with PJS admitted in Airforce General Hospital from January 2004 to September 2008 were analyzed. The polypi of gastrointestinal tract were resected under the endoscope. Large sessile polypi were resected through operation assisted with endoscope. Results Disseminated blue/black freckling existed on peri-oral, buccal mucosa or the extremities of limbs in 31 patients. The main symptom of 29 patients was paroxysmal abdominal pain accompanied with abdominal distention without defecation, venting and blood stools. The polypi were divided into long pedicle, deuto-pedicle and sessile polypus, which presented like mushroom or cauliflower. There were erosion or ulceration on the surface of some polypi. Among these patients only four patients had hemafecia and one patient had perforation of intestine after three days of resection. Hamartomatous was identified in 29 cases, one case of jejunum adenocarcinoma and one case of duodenum adenocarcinoma. Conclusions Gastroscopy, colonoscopy and double balloon-enteroscopy are important methods in the treatment of patients with PJS. The polypi smaller than 3cm could be resected under the endoscope. While the deuto-pedicle and sessile polypi larger than 3cm, especially with the disposition of cancerization, should be treated by operation. The resection of intestinal polypi by the operation assisted with endoscope is an important complemental method for the treatment of patients with PJS.