先天性双主动脉弓畸形的外科诊治

Surgical treatment of congenital double aortic arch

目的 总结先天性双主动脉弓（DAA）畸形患儿的临床特点和手术治疗经验.方法 回顾性分析2013年3月至2015年8月共5例双主动脉弓患儿的临床资料.5例患儿均为男性,年龄7～18（11.60±4.21）个月,体质量8.0～9.5（8.50±0.50）kg;其中左右弓均衡型1例、右弓优势型4例.临床表现为气喘、喉鸣、吞咽困难.5例患儿手术经左后外侧切口进胸,切断左弓,同时切断动脉导管或动脉韧带,松解气管、食管周围组织,解除对气管、食管的压迫.结果 5例患儿均顺利完成手术,未出现与手术相关并发症,无围术期死亡病例.呼吸机使用时间2～4（3.30±0.83）h,住院时间9～20（12.40±4.39）d.5例患儿均得到随访,1例患儿术后半年行肺动脉闭锁、室间隔缺损、房间隔缺损根治手术,术后痊愈出院;1例患儿术后半年行双侧隐睾下降固定术.其余患儿临床症状均缓解,生长发育、活动量较健康儿童无明显差异.结论 DAA是一种少见的心血管畸形,易引起气喘、喉鸣、吞咽困难等症状,心脏大血管CT检查可确诊,早发现、早期手术治疗效果良好.

Objective To summit clinical characters and surgical experiences of double aortic arch. Methods The clinical data of 5 double aortic arch patients during Mar. 2013-Aug. 2015 were retrospectively an- alyzed. All the 5 patients were male, whose ages ranged from 9 months to 18 months old, mean （11.60±4.21） months, and whose weight ranged from 8.0 to 9.5 kg, mean （8.50±0.50）kg. Among these patients, one of was double arch symmetrical type and the other were right arch predominant. Clinical symptoms were polypnea, laryn- geal stridor and dysphagia. All 5 patients accepted operation through posterolateral incision of left chest, abscising the left arch and potent ductus arteriosus or material ligament, reliving the pressure of trachea and esophagus. Re- sults 5 patients successfully underwent the operations, and none of them had any complications. The duration of mechanical ventilation was 2-4 hours, mean （3.3±0.83）hours, and length of stay was 9-20 days, mean （12.40± 4.39 ）days. We followed up all the 5 patients, one of those received pulmonary atresia, ventricle and atrial septal defect total correction 6 months after, and discharged. Another received orchiopexy 6 months after. Other patients were relieved from clinical symptoms, and had no significant differences with children of the same age in growth and exercise tolerance. Conclusion DAA is a rare vascular malformation which can cause polypnea, laryngeal stridor and dysphagia, and can be diagnosed by cardiac CT scan. Early investigation and surgical treatment will lead to good outcomes.