Peutz-Jeghers综合征29例诊治分析

目的 探讨Peutz-Jeghers综合征的临床特点及治疗方法。 方法 回顾性分析北京协和医院1984-2008年收治的29例Peutz-Jeghers综合征临床资料。 结果 29例病人均有胃肠道息肉，27例存在黑色素斑点沉着，10例（34%）有明确家族史。临床表现以腹痛、便血为主。并发肠套叠12例（41%），肠梗阻11例（38%）。行手术治疗19例，内镜切除6例，保守治疗4例。病理结果：错构瘤26例，伴不典型增生3例。 结论 Peutz-Jeghers综合征具有较为典型的临床表现，易合并肠梗阻、肠套叠、消化道出血等并发症，治疗上可采用手术治疗与内镜下息肉切除相结合的方式。Peutz-Jeghers综合征病人为恶性肿瘤高风险人群，必须坚持常年随访。

Diagnosis and treatment of Peutz-Jeghers syndrome

Diagnosis and treatment of Peutz-Jeghers syndrome DU Xiao, ZHAO Yu-pei, HE Xiao-dong, et al. Department of General Surgery, Peking Union Medical College Hospital , Peking Union Medical College , Chinese Academy of Medical Science , Beijing 100730 , China Corresponding author : ZHAO Yu-pei , E-mail: zhao8028@263.net Abstact Objective To investigate the clinical feature and treatment of Peutz-Jeghers syndrome(PJS). Methods Twenty-nine cases of PJS admitted in Peking Union Medical College Hospital from 1984 to 2008 were reviewed retrospectively and their clinical characters have been summarized. Results All the 29 patients had polyps in gastrointestinal tract, 27 patients had melanin pigmentation. Definite family history of Peutz-Jeghers syndrome were found in 10 cases (34%). Abdominal pain and hemafecia were the major clinical presentations of this syndrome. 12 cases (41%) complicated with intussusception and 11 cases (38%) with bowel obstructions. Operations were performed in 19 cases, 6 cases underwent polypectomy by endoscopy and 4 patients were given conservative therapy. 26 cases have been proven to be hamartoma of Peutz-Jeghers syndrome by pathologic examination, in which 3 cases had atypical hyperplasia. Conclusion Peutz-Jeghers syndrome has a specific clinical manifestation, and easy to complicated with bowel obstruction, intussusception and digestive tract bleeding. Surgical procedure associating with polypectomy by endoscopy is an appropriate method of treatment. Regular follow-up is indispensable to patients due to the high risk of developing malignant tumors.