摘要
目的了解肝肺综合征(HPS)Ⅱ型肺血管异常(肺动静脉畸形)合并血胸的临床特征和处理原则。方法对1例HPSⅡ型肺血管异常合并反复血胸患者的临床资料进行分析,并结合国内外相关文献进行复习。结果患者男性,72岁,以反复加重的喘憋和包裹性胸腔积液就诊。先后4次胸穿,总共抽取血样胸液2510mL,胸液常规红细胞计数在100×109/L以上,细胞学检查和肿瘤标记物均阴性。后经CTPA和肺动脉造影证实为HPSⅡ型肺血管异常合并血胸。患者立卧位PaO2分别为58.3mmHg和66.3mmHg。对血管畸形行栓塞治疗后,低氧血症改善,血胸得到控制。总结文献,HPSⅡ型肺血管异常合并血胸既往未见报道。HPS最突出的临床表现是呼吸困难和发绀。直立位低氧血症或卧位呼吸是HPS较为特异的表现。P(A-a)O2是筛查HPS低氧血症的最敏感指标。HPS胸部CT特点为被强化的团片状密度增高影或多发小结节影,以双下肺野多见。肺动脉造影可对肺血管异常予以分型。HPS肺血管病变Ⅱ型患者对吸氧反应不佳,血管畸形栓塞治疗有助于缓解症状,改善低氧血症。结论HPSⅡ型肺血管异常破裂是血胸的少见病因。肺血管畸形栓塞治疗可有效改善低氧血症,控制血胸。对于具有肝脏病史者,出现肺血管扩张和低氧血症应警惕HPS的存在。
Objective To investigate the clinical characteristics and treatment for Type Ⅱ pulmonary vascular anomaly （puhnonary arteriovenous malformations） of hepatopulmonary syndrome （HPS） presenting with hemothorax. Methods A case of Type Ⅱ pulmonary vascular anomaly of HPS presenting with recurrent hemothorax was described. The clinical data was analyzed and the related literature was reviewed. Results A 72-year-old male patient with Type Ⅱ pulmonary vascular dilatations of HPS was described to present with recurrent dyspnea and encapsulated pleural effusions. After 4 procedures of thoracentesis, a total of 2510 mL of bloody pleural effusions was drained. The routine analysis of pleural fluid showed the count of red cells exceeded 100 × 10^9/L, whereas cytologic examination and tumor biomarkers were negative. Then CTPA and pulmonary angiogram revealed a Type Ⅱ pulmonary vascular anomaly of HPS combined with hemothorax. The PaO2 of arterial blood in upright and supine position was 58.3 mm Hg and 66. 3 mm Hg, respectively. Hypoxemia was alleviated and hemothorax was controlled after embolization of malformed blood vessels. From literature review, similar cases of hemothorax resulted from rupture of Type Ⅱ pulmonary vascular anomaly of HPS were not reported. The primary clinical manifestations of HPS were dyspnea and cyanosis. Orthodeoxia and platypnea were most consistent with HPS. The best screening tool for hypoxemia in patients with HPS was P（A-a） O2· The characteristic findings of HPS on chest CT was a lesion or reticulonodular opacities occurring predominantly in the bases of the lungs, which could be enhanced by contrast medium. Pulmonary angiogram was necessary to identify the types of pulmonary vascular dilatations. Hepoxemia of patients with Type Ⅱ HPS often responded poorly to oxygen therapy, whereas embolization of the pulmonary arteriovenous fistulas was helpful to improve anoxia. Conclusions Rupture of Type Ⅱ pulmonary vascular malformations in HPS was a rare cause of hemothorax. Thrombosis of pulmonary arteriovenous malformations may result in significant improvement in oxygen saturations as well as control of hemothorax. In the setting of liver disease, intrapulmonary vascular dilatations and hypoxemia often suggest the existence of HPS.
出处
《中国呼吸与危重监护杂志》
CAS
2010年第1期61-65,共5页
Chinese Journal of Respiratory and Critical Care Medicine
关键词
肝肺综合征
肺动静脉畸形
血胸
肺动脉造影
Hepatopulmonary syndrome
Pulmonary arteriovenous malformations
Hemothorax
Pulmonary angiogram
