儿童先天性支气管动脉-肺动脉瘘的漏诊分析

Congenital bronchial artery-pulmonary artery fistula in children:missed diagnosis and treatment

目的：总结先天性支气管动脉-肺动脉瘘（DPS）非咯血患儿的临床诊治经验.方法：回顾性分析2009年7月至2013年11月间,在北京儿童医院行先天性心脏病（先心病）动脉导管未闭介入手术,诊断为DPS 31例患儿的临床资料,总结其临床表现、胸部X线片、大血管造影及随访情况.结果：男性16例,女性15例,年龄7个月～6岁,均为动脉导管介入手术中发现,术后21例仍有反复上呼吸道感染史.胸部x线片检查：8例未见异常,23例示右下肺内带肺纹理粗多.降主动脉造影均提示支气管动脉扩张、走行迂曲,呈扫帚状或网状,支气管动脉管腔内径≥2mm,在支气管动脉显影的同时有肺动脉同步显影,并可见支气管动脉与肺动脉管道状相交通,形成左向右分流.结论：PDA介入术中发现的细小先天性支气管肺动脉瘘,如无症状可随诊观察.

Objective：The purpose of this study was to summarize the diagnosis of congenital bronchial artery-pulmonary artery fistula in children.Methods：The clinical data of 31 cases with Bronchial artery-pulmonary artery fistula who were diagnosed from July 2009 to November 2013 were summarized,including present history,past history,physical examination,chest X-ray（CXR）,contrast 64-multidetector-row CT（MDCT）,3D-volume rendering（VR）,DSA examination.All cases were found in congenital heart disease：artery catheter arteriosus interventional operation,and still under follow-up until December 2013.Results：All cases were diagnosed as bronchial artery to pulmonary artery fistula,including 16 boy and 15 girls,aged from 7 months old to 6 years old.The major symptom was recurrent chest infections in 21 cases.CXR of 8 cases were negative,and CXR show infiltration in 23 cases.DSA showed tortuous bronchial artery and shunt between systemic artery and pulmonary vessels.Conclusion：bronchial artery-pulmonary artery fistula may be not a rare disease in children.In some children recurrent chest infections occur.It had no specific signs on CXR.