Peutz-Jeghers综合征伴小肠套叠的临床分析

The Clinical Analysis of Peutz-Jeghers Syndrome with the Complication of Intussusception

目的分析Peutz-Jeghers综合征（Peutz-Jeghers syndrome,PJS）并发小肠套叠的临床特征及其诊断与治疗。方法回顾性分析5例PJS并发小肠套叠患者的临床资料,患者以皮肤粘膜色素沉着、腹痛及便血为主要表现,并发肠梗阻、肠套叠5例,消化道出血2例;发生恶变1例。行肠道息肉切除2例,肠部分切除术3例。结果均顺利出院,随访4例,平均时间为23（6-40）个月,未见复发,但1例息肉恶变患者因远处转移而死亡。结论PJS并发肠套叠以小肠套叠为主,手术解除临床症状是最主要的治疗方法;PJS患者发生肿瘤的危险性较高,应加强术后随访,定期复查内镜。

Objective To analyzed the clinical features,diagnosis and treatment of Peutz-Jeghers syndrome（PJS） with the complication of small intestine intussusception.Methods The clinical data of 5 PJS cases was analyzed retrospectively.The main performances of patients were mucocutaneous pigmentation,abdominal pain and blood in the stool.Concurrent intestinal obstruction and intussusception appeared in 5 cases,gastrointestinal bleeding appeared in 2 cases,and malignant transformation was found in one case.Enterectomy was performed in 3 cases,and polypectomy was performed in 2 cases.Results All the patients were successfully discharged.4 cases were followed up or 23 months in average,of which one case died from distant metastases of the colon carcinoma.Conclusions PJS with the complication of intussusception was located mainly in small intestine,and surgical relieving the clinical symptoms is the most important treatment.The patients of PJS have a higher risk of canceration,therefore,postoperative follow-up should be strengthened to regularly review the endoscope.