摘要
目的总结临床表现为大量咯血的先天性体动脉-肺动脉瘘患儿的临床特点及诊治经验。方法回顾性收集首都医科大学附属北京儿童医院2007年3月至2008年2月诊断为先天性体动脉-肺动脉瘘4例患儿的临床资料,总结其临床表现、胸部X线片、胸部64排CT增强扫描三维容积再现(3D-VR)、数字减影血管造影(DSA)、治疗及随访情况。结果男1例,女3例,最大发病年龄为11岁,最小发病年龄为2个月,主要表现为咯血。1例有杵状指,3例未见特异性体征。胸部X线片检查:3例未见异常,1例示肺间实质浸润。胸部64排CT增强扫描3D-VR检查:1例提示支气管动脉迂曲,但未见异常交通;1例导管栓塞治疗(TCE)后示右侧支气管动脉扩张迂曲,并与肺动脉相通可能性大,考虑支气管动脉-肺动脉瘘;1例提示支气管动脉-肺动脉瘘;1例未见异常。3例DSA提示为支气管动脉-肺动脉瘘,病变部位均位于右下肺,并行TCE。随访至2009年2~5月,1例复发,表现为痰中带血,胸部64排CT增强扫描未见异常,余3例未复发。3例怀疑为遗传性出血性毛细血管扩张症(HHT),1例考虑为HHT高度危险者。结论体动脉-肺动脉瘘可造成大量咯血。胸部X线片一般无特异性表现,胸部64排CT增强扫描3D-VR可显示病变部位,明确诊断需行DSA。可采用TCE治疗。TCE远期效果应进行长期随访予以明确。
Objective The purpose of this study was to summarize the diagnosis and treatment of systemic artery-pulmonary artery fistula (or systemic artery-pulmonary artery malformation) in children.Methods The clinical data of four cases with systemic artery-pulmonary artery fistula who were diagnosed from March 2007 to February 2008 were summarized,including present history,past history,family history,physical examination,complete blood count (CBC),percutaneous oxygen saturation (SpO2)/artery blood gas,chest X-ray (CXR),contrast 64-multidetector-row CT (MDCT),3D-volume rendering (VR),digital subtraction angiography(DSA) for bronchial arteriography and treatment.All cases were still under follow-up until May 2009.Results All cases were diagnosed as bronchial artery to pulmonary artery fistula,including 1 boy and 3 girls,aged from 2 months old to 11 years old. The major symptom was hemoptysis. Three cases had massive hemoptysis.Case 2 had chest pain and case 4 had dyspnea as accompanied symptom All cases had no specific signs of this disease except case 2 with clubbing.CXR showed atelectasis or infiltration caused by hemoptysis in three cases.CXR of case 3 was negative.Contrast MDCT and 3D-VR of case 1 showed tortuous bronchial artery and no shunt between systemic artery and pulmonary vessels. Contrast MDCT and 3D-VR of case 2 after transcatheter embolization (TCE) showed tortuous right bronchial artery and probable shunt to pulmonary artery.Contrast MDCT and 3D-VR of case 3 showed bronchial artery to pulmonary artery shunt.Contrast MDCT and 3D-VR of case 4 was negative.Three cases with bronchial artery to pulmonary artery fistula were confirmed by DSA bronchial arteriography and were treated by TCE. Case 3 didn't receive TCE for the very young age,and had medical treatment instead. There was no evidence of pulmonary artery to pulmonary vein fistula (PAVF) in case 2 although he had clubbing. In follow-up,the disease recurred in case 1 indicated by the recurrence of bloody sputum and did not recur in the other three cases.Three cases were highly suspected as hereditary hemorrhagic telangiectasia(HHT) and the other was higher risk.Conclusions Systemic artery (bronchial artery)-pulmonary artery fistula could be a rare cause for massive hemoptysis in children. It had no specific signs on CXR. Contrast MDCT and 3D-VR could show the lesion. It was confirmed by DSA. TCE could be used for treatment. Follow-up should be recommanded to understand the natural history and the long-term effects of TCE.
出处
《中国循证儿科杂志》
CSCD
2009年第4期368-374,共7页
Chinese Journal of Evidence Based Pediatrics
关键词
先天性体动脉-肺动脉瘘
支气管动脉
咯血
儿童
Congenital systemic artery to pulmonary artery fistula
Bronchial artery
Hemoptysis
Children