Stanley N N, Williams A J, Dewar C A, Blendis L M, Reid L
Thorax. 1977 Aug;32(4):457-71. doi: 10.1136/thx.32.4.457.
, , 457-471. A case is reported of liver cirrhosis complicated by cyanosis and recurrent right hydrothorax. A diagnostic pneumoperitoneum demonstrated that direct movement of ascites through a diaphragmatic defect was responsible for the hydrothoraces. Pulmonary function tests between episodes of hydrothorax showed severe arterial hypoxaemia, a 23% right-to-left shunt, and a reduction in the carbon monoxide transfer factor to less than half of the predicted value. Evidence of abnormal intrapulmonary arteriovenous communications was obtained by perfusion scanning. At necropsy the central tendon of the diaphragm showed numerous areas of thinning which were easily ruptured. Injection of the pulmonary arterial tree demonstrated precapillary arteriovenous anastomoses and pleural spider naevi. A morphometric analysis provided quantitative evidence of pulmonary vasodilatation limited to the intra-acinar arteries, consistent with the effect of a circulating vasodilator. The scintigraphic and pathological findings suggested that shunting had been greater in the right than the left lung. Examination of thin lung sections by light microscopy showed that the walls of small veins were thickened, and electron microscopy showed that this was due to a layer of collagen. The walls of capillaries were similarly thickened, which caused an approximately two-fold increase in the minimum blood-gas distance and contributed to the reduction in transfer factor.
报道了一例肝硬化合并发绀和复发性右侧胸腔积液的病例。诊断性气腹显示腹水通过膈肌缺损直接移动是胸腔积液的原因。胸腔积液发作期间的肺功能测试显示严重的动脉低氧血症、23%的右向左分流以及一氧化碳转运因子降低至预测值的一半以下。通过灌注扫描获得了肺内动静脉异常交通的证据。尸检时,膈肌中心腱显示出许多变薄区域,这些区域容易破裂。肺动脉树注射显示毛细血管前动静脉吻合和胸膜蜘蛛痣。形态计量分析提供了仅限于腺泡内动脉的肺血管扩张的定量证据,这与循环血管扩张剂的作用一致。闪烁扫描和病理结果表明,右肺的分流比左肺更大。光镜检查薄肺切片显示小静脉壁增厚,电镜检查表明这是由于一层胶原蛋白所致。毛细血管壁同样增厚,这导致最小血气距离增加约两倍,并导致转运因子降低。