Cantrell综合征的诊断与治疗  

Diagnosis and treatment of pentalogy of Cantrell

作　　者：胡玥(综述) 计晓娟(审校)[1] Hu Yue;Ji Xiaojuan(Department of Cardiology,Children′s Hospital of Chongqing Medical University,Ministry of Education Key Laboratory of Child Development and Disorders,National Clinical Research Center for Child Health and Disorders,China International Science and Technology Cooperation Base of Child Development and Critical Disorders,Chongqing Key Laboratory of Child Infection and Immunity,Chongqing 400014,China)

机构地区：[1]重庆医科大学附属儿童医院心血管内科,儿童发育疾病研究教育部重点实验室,国家儿童健康与疾病临床医学研究中心,儿童发育重大疾病国家国际科技合作基地儿童感染免疫重庆市重点实验室,400014

出　　处：《国际儿科学杂志》2020年第4期235-238,共4页International Journal of Pediatrics

基　　金：重庆市科学技术委员会社会事业与民生保障科技创新专项(cstc2016shmszx130009);中国博士后基金特别资助项目(2018T110949)。

摘　　要：Cantrell综合征是一种罕见的先天性复杂畸形,包括胸骨缺损、膈肌缺损、心包缺损、腹壁缺损、心脏畸形五大特征,也可合并其他多种器官畸形。该病发病率低,预后差,围生儿病死率高,病因及发病机制尚未完全明确。该病早期诊断并及时治疗有着重要意义,利用超声可在孕早期进行产前诊断,而产后则可结合临床表现及超声、CT等影像学手段综合诊断,治疗上以手术矫正为主。其预后与心脏畸形、胸腹壁缺损的严重程度等多种因素相关。该文复习相关文献,总结其诊疗进展。Pentalogy of Cantrell is a rare congenital anomaly characterized by a defect of the sternum,a deficiency of the diaphragm,a defect in the pericardium,an abdominal wall defect and congenital heart disease,with or without involvement of other organ systems.The incidence is low,and the prognosis is poor with high rates of perinatal mortality.The etiology and pathogenesis are still unknown.The early diagnosis and timely treatment are important.The prenatal diagnosis may be made by ultrasound as early as the first trimester,and for infants,it can be diagnosed with clinical features,ultrasound,CT and other imaging findings.Surgical repair is the main treatment.The survival depends mostly on associated cardiac anomalies,degree of thoraco-abdominal defect,and so on.There is a review that the diagnosis and treatment of the disease are summarized.

关 键 词：CANTRELL综合征 病因 诊断 影像诊断 治疗 

分 类 号：R71[医药卫生—妇产科学]