婴幼儿先天性大血管畸形合并中央气道狭窄18例临床分析

Clinical analysis of infants with congenital vascular malformation associated with central airway stenosis in 18 cases

目的 :探讨婴幼儿先天性大血管畸形合并中央气道狭窄患儿的临床特征和辅助检查特点,提高该病的诊断水平。方法:回顾性分析2008年8月至2012年6月温州医学院附属育英儿童医院因先天性大血管畸形合并中央气道狭窄住院患儿的临床资料,分析其临床表现、体格检查、影像学检查和支气管镜检查特点。结果:18例患儿均以咳嗽、喘息、气促等呼吸系统症状为主要表现,体检吸呼双相哮鸣音11例,心前区杂音6例。超声心动图提示双主动脉弓1例;胸片2例报告提示主动脉弓异常改变;CT血管造影(CTA)提示肺动脉吊带6例,双主动脉弓4例,右位主动脉弓3例,右位降主动脉1例,镰刀综合征1例,主动脉缩窄2例,主动脉弓中断2例。9例患儿接受了支气管镜检查,均提示气管支气管狭窄,伴有气管软化和(或)气管外压改变。3例患儿在手术后出现气道狭窄;其余15例中10例行手术治疗,6例手术后症状逐渐改善,2例术后仍有反复咳嗽喘息,2例术后死亡。结论:大血管畸形合并气道狭窄常以呼吸道症状为主要表现,对反复或持续喘息、呼吸困难,体检发现吸呼双相哮鸣音,尤其是内科治疗效果不佳者,应想到该病可能,CTA联合支气管镜检查有助于早期诊断。

Objective： To explore the clinical, diagnostic and laboratory examination characteristics of congenital vascular malformation associated with central airway stenosis, and to improve the dignostic level for this disease. Methods： All patients due to congenital vascular malformation associated with central airway stenosis were retrospectively analyzed in our Hospital from August 2008 to June 2012, including clinical features, imaging studiess, and bronchoscopy characteristics. Results： Eighteen patients manifested with coughing, wheezing, shortness of breath and other symptoms of the respiratory system. On examination, 11 cases showed biphasic wheeze, and six cases showed precordial murmur. In 18 cases of chest X-ray, only two cases reported abnormal change of aortic arch. One case aortic lesion was shown in 18 cases of echocardiogram. Four cases of double aortic arch associated with tracheal stenosis were shown in 18 cases of chest CTA, 1 case of right descending aorta, 6 cases of pulmonary artery sling, 3 cases of right aortic arch, 1 case of scimitar syndrome, 1 case of coarctation of the aorta, two cases of interrupted aortic arch. 9 patients were given fiberoptic bronchoscopy and showed various degrees of bronchial stenosis, combined with tracheomalacia and/or tracheal or bronchial stenosis acquired by exterior pressure. Three cases of tracheal, bronchial stenosis occured after surgery. Ten patients of the remaining 15 cases underwent surgery, 6 cases gradually returned to normal after surgery, two cases died postoperatively, two cases were still vulnerable wheezing. Conclusion： Congenital vascular malformation associated with central airway stenosis in children is mainly manifested as respiratory symptoms, such symptoms like repeated or persistent wheezing, dyspnea, and physical exami- nation with respiratory duplex wheeze may be the presence of the disease, especially when medical treat-ment fails, we have to be highly suspected this disease, we can make an early diagnosis using chest CTA and the bronchoscope.