Peutz-Jeghers综合征的影像学诊断及临床分析——附两例报道及文献回顾  

Imaging diagnosis of Peutz-Jeghers Syndrome:a report of two cases with literature reviews

作　　者：马爱冬[1] 史冬梅[2] 张千里[3] MA Aidong;SHI Dongmei;ZHANG Qianli(Department of Radiology,fining NO.1 People's Hospital,Jining 272001 China;Department of Dermatology,Jining NO.1 People's Hospital;Radiology department,Guizhou cancer hospital)

机构地区：[1]济宁市第一人民医院放射科,山东济宁272001 [2]济宁市第一人民医院皮肤科 [3]贵州省肿瘤医院影像科

出　　处：《中国辐射卫生》2018年第5期513-516,共4页Chinese Journal of Radiological Health

摘　　要：目的提高Peutz-Jeghers综合征(PJS)的诊断及治疗水平。方法回顾性分析两例Peutz-Jeghers综合征患者的人口学及临床、影像资料。结果两例均为临床确诊的Peutz-Jeghers综合征,均有腹痛病史,均有小肠息肉继发肠套叠,形成肠梗阻病史;其中1例并有乳腺癌和子宫病变,影像学表现与临床症状相一致。结论 Peutz-Jeghers综合征以反复便血、腹痛、特定部位皮肤黏膜黑斑、消化道多发性错构瘤性息肉及家族遗传性为特征,且有息肉恶变及胃肠道恶性肿瘤的可能,其临床及影像学表现具有一定特征性。息肉的切除或者摘除是重要的治疗手段,需定期随访。Objective This study aimed to improve the diagnosis and management of PJS. Methods The clinical data and imaging findings of two PJS cases were retrospectively analyzed. Results Both cases were clinically diagnosed as Peutz-Jeghers syndrome with a history of abdominal pain,secondary intussusception of the small intestine polyps and intestinal obstruction,and one of the cases complicated with breast cancer and uterus diseases. The imaging findings consistent with the clinical manifestations. Conclusion Patients with PJS usually present hematochezia,abdominal pain,bowel obstruction. They also have an increasing risk of developing cancer or transformation to malignant polyps in the gastrointestinal tract and other organs.Imaging finding and clinical features of PJS have certain characteristics. Surgical resection may appropriately be used for the patients with polyps.

关 键 词：PEUTZ-JEGHERS综合征 肠息肉 肠套叠 影像诊断 

分 类 号：R814.4[医药卫生—影像医学与核医学]