单中心60例先天性血管环回顾性分析

Retrospective analysis of 60 cases of congenital vascular ring:a single center study

目的:分析先天性血管环的临床特征、诊断及治疗。方法:对重庆医科大学附属儿童医院2003年1月至2013年1月10年间确诊的60例先天性血管环患儿进行回顾性分析。所有患儿均行心脏彩超、CT血管造影(computed tomography angiography,CTA)、消化道钡餐、纤维支气管镜等确诊。结果:本组患儿男40例,女20例,年龄1 d^6岁1月(中位数年龄7.65月)。其中肺动脉吊带25例(占41.67%),双主动脉弓21例(占35%),右主动脉弓合并左动脉导管/韧带12例(占20%)。临床表现以呼吸困难、吼喘及反复呼吸道感染为主,占87%(52例)。28例(46.7%)合并有心脏畸形,45例(75%)合并有气道狭窄,10例合并有食管狭窄。CTA的诊断符合率81.82%,心脏彩超的诊断符合率53.33%。40例行手术治疗,包括左肺动脉移植术15例,双主动脉弓离断术12例,切断动脉导管/韧带12例,左锁骨下动脉夹闭1例。本组患儿7例死亡:术前、术中各死亡1例,术后死亡5例。血管环合并完全性气管环是手术死亡的高危因素(P<0.05)。术后患儿中,虽半年内仍存在不同程度吼喘、气急等呼吸道症状,但较术前有明显缓解。结论:对于内科治疗效果欠佳的反复肺炎、喘鸣、吞咽困难的婴幼儿,要高度警惕先天性血管环。心脏彩超联合CTA是确诊血管环的最佳方法,早期正确诊断和及时手术是治疗及改善其预后的关键。

Objeetive：To summarize the clinical features,diagnosis and treatment of congenital vascular ring in children. Methods： Clinical data of 60 patients with congenital vascular rings in Children＇ s Hospital of Chongqing Medical University from January 2003 to January 2013 were retrospectively studied. All children underwent computed tomography angiography（CTA）, echocardiography, gastrointestinal barium meal,fiberoptic bronchoscopy. Results：Sixty cases including 40 males and 20 females, aged from 1 day to 6 years, were enrolled in our study. The most frequent diagnosis was pulmonary artery sling（25 cases, 41.67%）, followed by double aorta arch （21 cases, 35%） and right aortic arch with left ductus arteriosus （12 cases, 20%）. Twenty-eight cases （46.7%） combined with heart malformations. Forty five cases（46.7%） combined with tracheobronchial stenosis, 10 cases combined with esophageal stricture. The diagnosis rate of CTA was 81.82% and that of echocardiography was 53.33%. 52 cases（87%） had a history of respiratory symptoms including difficulty in breathing, recurrent cough and wheezing, and recurrent respiratory tract infections. Forty patient who underwent surgery included left pulmonary artery transplantation（15 cases）,double aortic arch devascularization（ 12 cases）,artery catheter/ligament cut-off（12 cases）,left subclavian artery occlusion （1 case）. Seven patients died. Vascular ring combined with complete tracheal ring was a risk factor for patients underwent surgery（P〈0.05）. Patients underwent surgery remained respiratory symptoms for up to 6 months,however,they had significant improvement in the postoperative period. Conclusion：Children with recur- rent respiratory symptoms such as recurrent respiratory tract infections, chronic cough and wheeze, dysphagia should be examined for the presence of congenital vascular ring. Echocardiography combined with CTA is the best way to diagnose vascular ring. Early cor- rect diagnosis and timely surgical treatment are the keys to survival.