29例黑斑息肉综合征诊治分析

The Investigation of Diagnosis and Treatment of 29 Cases with Peutz-Jeghers Syndrome

[目的]探讨黑斑息肉综合征（Peutz-Jeghers syndrome，PJS）的发病机制及恶变情况，规范治疗方案。[方法]综合国内外相关文献回顾性分析本院1984～2005年间治疗的29例PJS患者的临床资料。[结果]29例患者中有家族史者18例，占62％（18／29）。恶性肿瘤发生率18％（5／29）。治疗主要以息肉密集段肠切除和术前内镜高频电切除、术中内镜电切除结合息肉密集段肠切除。[结论]PJS患者临床表现类型多，30岁以后及有家族史者恶变率明显上升。术前内窥镜结合术中内窥镜息肉摘除及息肉密集段肠切除术效果好。息肉易复发，术后应定期行肠镜、胃镜及全消化道钡餐检查。

[Objective]To investigate the pathogenesis and caneerauon of Peutz-Jeghers syndrome （PJS） and standardize therapeutic regimen. [Methods]Clinic data of 29 cases of PJS were retrospectively analyzed from 1984 to 2005. [Results]Eighteen cases had family history. The incidence of malignancy was 18 percent. Resection of intestinal segment with intensive polypi combining with endoscopic resection preoperatively and intraoperatively was the principal therapy. [Conclusion]The clinical manifestation of PJS is multitude. The incidence of malignant tumor is obviously higher after 30 years old. Resection of intestinal segment with intensive polypi combining with endoscopic resection preoperatively and intraoperatively has good effects. The polypi are apt to recurrence,it is necessary to have an endoscope and barium meal examination after operation.