Kuo Chih-Hsi, Wang Chun-Hua, Kuo Han-Pin
Department of Thoracic Medicine, Chang Gung Memorial Hospital, Taipei, Taiwan.
Respirology. 2007 Mar;12(2):295-8. doi: 10.1111/j.1440-1843.2006.01025.x.
Osler-Weber-Rendu syndrome is a disease of hereditary telangiectasia with involvement of multiple visceral organs. Pulmonary arteriovenous malformation (AVM) is a frequent clinical manifestation, that may mimic pulmonary mass. In this report, endobronchial ultrasound (EBUS) was used in a 64-year-old woman with Osler-Weber-Rendu syndrome who had a solitary pulmonary mass associated with clinical evidence of a right-to-left shunt and was able to detect a pulsating hypoechoic mass connected to the right pulmonary artery suggesting of pulmonary AVM. Following magnetic resonance angiogram and chest CT as well as noting multiple telangiectasis on her skin and considering a strong family history, Osler-Weber-Rendu syndrome was confirmed. Exercise intolerance and desaturation improved after surgical resection of the pulmonary AVM. The echoic findings were correlated to the pathological features. EBUS can be used as a good diagnostic tool for determining the nature of peripheral lung lesions such as in this case, and EBUS may be of practical tool to investigate a lung mass when a vascular component is suspected.
奥-韦-伦综合征是一种遗传性毛细血管扩张症,累及多个内脏器官。肺动静脉畸形(AVM)是其常见临床表现,可能类似肺部肿块。在本报告中,对一名64岁患有奥-韦-伦综合征的女性使用了支气管内超声(EBUS),该患者有一个孤立性肺部肿块,并伴有右向左分流的临床证据,EBUS能够检测到一个与右肺动脉相连的搏动性低回声肿块,提示为肺AVM。经磁共振血管造影和胸部CT检查,并注意到其皮肤上有多处毛细血管扩张,且考虑到家族病史强烈,确诊为奥-韦-伦综合征。肺AVM手术切除后,运动不耐受和血氧饱和度下降情况有所改善。回声表现与病理特征相关。EBUS可作为确定此类外周肺病变性质的良好诊断工具,当怀疑肺部肿块有血管成分时,EBUS可能是研究肺部肿块的实用工具。