先天性双主动脉弓畸形的诊断与外科治疗  

Diagnosis and Surgical Treatment of Congenital Abnormality of Double Aortic Arch

作　　者：刘彩霞[1] 马新平[1] 杨辉[1] 陈瑞[1] 

机构地区：[1]山西省儿童医院,山西太原030013

出　　处：《山西职工医学院学报》2017年第2期4-7,共4页Journal of Shanxi Medical College for Continuing Education

基　　金：山西省儿童医院资助项目(201028)

摘　　要：目的:总结婴幼儿先天性双主动脉弓畸形的诊断与手术治疗经验,分析漏误诊的原因,以进一步提高该疾病的诊治效果。方法:对7例先天性双主动脉弓畸形的诊断过程及外科治疗进行回顾性分析和总结研究。结果:全组7例中伴有咳喘或气促等呼吸系统症状的有6例,进食固体食物困难的1例。合并心内畸形的1例,合并肺动脉吊带畸形的1例,单纯性双主动脉弓畸形5例。超声确诊1例,发现主动脉弓畸形可疑建议CT检查的2例,发现肺动脉走形异常建议CT检查发现同时合并双主动脉弓畸形的1例,漏诊3例。7例均经心脏大血管螺旋CT及三维重建检查确诊。7例中于不同医院就诊3次以上才得以确诊的有6例。7例中6例行手术矫治,其中正中开胸手术2例,后外侧开胸手术4例,无围术期死亡。1例合并肺动脉吊带的患儿因并发"环-吊带复合体"畸形术后脱离呼吸机困难家长放弃治疗。术后出院时4例患儿无残余症状,1例哭闹后有轻微残余呼吸道症状。随访发现该患儿呼吸道症状半年后消失。无需再手术病例。另1例未手术者仍在随访中。结论:对于反复呼吸道感染、咳喘或吞咽困难的患儿,儿科及影像科医生应注意除外双主动脉弓畸形,避免误诊误治;螺旋CT增强扫描和三维重建是诊断双主动脉弓畸形的可靠方法。单纯双主动脉弓畸形手术效果良好。Objective： To summarize the diagnostic methods and surgical experience of congenital abnormality of double aortic arch and to analyze the causes of missed diagnosis and misdiagnosis in infants for the sake of curtive effects. Methods：7 cases of diagnostic process and the surgical treatment experience for the patients with double aortic arch diagnosed between September 2009 and June 2016 in Shanxi Children Hospital were studied retrospectively. Re- suits：6 cases in the group had respiratory symptoms such as cough, cuhy in taking solid food,with one case combined with intracardiac stridor, or shortness of breath. One case had diffi- anomalies. One case was showed pulmonary artery sling as well. 5 cases were only with double aortic arch. One case with double aortic arch was diagnosed definitely by echocardiography. Two cases suggested for CT scan were doubted with aortic anomalies by echocardiography. Double aortic arch was also found by CT except for pulmonary artery sling in one case who was found with pulmonary artery a- nomaly by echocardiography. 3 cases of double aortic arch were missed. Diagnoses of the above 7 cases were all con- firmed by spiral CT scan. In the group ,6 cases had definite diagnoses after visiting doctor for 3 times or more in different hospitals. Six cases were undergone surgical treatment and no perioperative death. Two cases were through mid - sternal incision and 4 ones through posterolateral thoracotomy. One case with pulmonary artery sling had difficulty in being extu- bated postoperatively, so his parents initiated discontinued treatment. At discharge 4 cases were asymptomatic after half a year and one had mild residual respiratory symptom after crying. During follow - up the symptom in this case disap- peared. No one needed reoperations. The one with no procedure was still in follow - up. Conclusions ： Pediatricians andimaging dotors should pay more attention and exclude double aortic arch in the patients with recurrent respiratory infec- tion or ough and stridor. Enhanced CT

关 键 词：双主动脉弓 先天性 血管环 反复呼吸道感染 气管压迫 

分 类 号：R725.4[医药卫生—儿科]