Peutz-Jeghers综合征诊治体会(附31例报告)

The Diagnosis and treatment of patients with Peutz-Jeghers syndrome(31 cases report)

目的加强对Peutz-Jeghers综合征的认识,以提高诊治水平,改善患者的生存质量。方法总结31例Peutz-Jeghers综合征患者的临床资料,对胃肠道息肉行内镜下切除,较大无蒂息肉手术切除,辅以术中内镜检查。结果31例患者口唇、口腔颊黏膜、手指脚趾末端均有散在分布的黑/褐色斑,29例有阵发性腹痛,伴腹胀,无排便、排气、血便。息肉分为无蒂、亚蒂、长蒂息肉,表面呈分叶状或菜花状,部分息肉表面黏膜糜烂,伴溃疡形成。术后第3天发生便血4例,小肠穿孔行手术修补治疗1例。病理证实错构瘤29例,十二指肠黏液腺癌1例,空肠黏液腺癌1例。结论胃镜、结肠镜、小肠镜是Peutz-Jeghers综合征重要的治疗手段,≤3cm息肉行镜下切除,>3cm无蒂或亚蒂息肉或可疑癌变倾向的患者宜手术治疗,术中辅以内镜检查进一步切除小肠息肉是治疗Peutz-Jeghers综合征的重要补充手段。

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 reseeted under the endoscope. Large sessile polypi were resected through operation assisted with endoscope. Results Disseminated blue/black freckling existed on peri - oral,bueeal mueosa 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 hemafeeia 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 adenoeareinoma. Conclusions Gastroseopy,colonoscopy and double balloon - enteroscopy are important methods in the treatment of patients with PJS. The polypi smaller than 3cm could be reseeted 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.