分期双侧开胸手术治疗遗传性出血性毛细血管扩张症合并的多发性肺动静脉畸形。
Staged bilateral thoracotomies for multiple pulmonary arteriovenous malformations complicating hereditary hemorrhagic telangiectasia.
作者信息
Brown S E, Wright P W, Renner J W, Riker J B
出版信息
J Thorac Cardiovasc Surg. 1982 Feb;83(2):285-9.
PMID:7057669
Abstract
A patient with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) with multiple bilateral pulmonary arteriovenous malformations was hypoxic, cyanotic, polycythemic, and severely limited by dyspnea on exertion. Following staged bilateral thoracotomies, with removal of 23 major fistulas (12 from the right lung and 11 from the left), marked improvement in symptoms, blood gases, and objective measurements of exercise tolerance occurred.
摘要
一名患有遗传性出血性毛细血管扩张症（奥斯勒-韦伯-伦杜综合征）且伴有多处双侧肺动静脉畸形的患者出现缺氧、发绀、红细胞增多症，且因运动时呼吸困难而严重受限。在分期进行双侧开胸手术后，切除了23个主要瘘管（右肺12个，左肺11个），症状、血气以及运动耐量的客观测量指标均有显著改善。
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