摘要
目的提高对遗传性毛细血管扩张症（hereditary hemorrhagic telangiectasia，HHT）造成的大咯血患儿的诊治水平。方法总结1例以大量咯血为临床表现的HHT患儿的临床诊治经过并复习相关文献。结果通过数字减影血管造影（digital subtraction angiography，DSA）及病史诊断为HHT，DSA结果提示右侧支气管动脉中下部发育异常、支气管动脉一肺动脉瘘、左侧支气管动脉发育异常，行经导管栓塞（transcatheter embolotherapy，TCE）治疗，随访2年，无咯血表现。在国内外报道儿童咯血原因中，血管因素属少见原因。40％的HHT患者存在肺动静脉畸形（pulmonary arteriovenous malformation，PAVM），70％的PAVM患者与HHT相关，2％—5％的PAVM患者为支气管动脉与肺动脉瘘。结论遗传性毛细血管扩张症为儿童大量咯血的少见原因。DSA可以明确病变部位。利用TCE治疗并应长期随访。
Objective To improve the diagnosis and treatment of hereditary hemorrhagic telangiectasia （HHT） in children with massive hemoptysis. Methods To summarize the experience of treating one HHT patient with massive hemoptysis and review the related literatures. Results The patient was diagnosed as HHT with bronchial artery to pulmonary artery shunt by medical history and digital subtraction angiography （DSA） under transcatheter embolotherapy （TCE）. DSA showed that the right and left bronchial arteries were aberrant with bronchial artery to pulmonary artery shunt in right middle and low lobe. In 2 years follow-up, the patient had no recurrence. The aberrant vessel in lung was uncommon cause for the massive hemoptysis in children. More than 40% HHT had pulmonary arteriovenous malformation （PAVM）, conversely 70% of PAVM were associated with HHT. 2%—5% of PAVM were characterized as pulmonary arteries with a connection to the bronchial artery. Conclusions HHT can be the rare cause for massive hemoptysis. DSA may confirm the lesions. TCE can be used as treatment and follow-up is recommended.
出处
《北京医学》
CAS
2010年第1期7-9,共3页
Beijing Medical Journal
