Swischuk L E, L'Heureux P
AJR Am J Roentgenol. 1980 Oct;135(4):667-72. doi: 10.2214/ajr.135.4.667.
Three cases of unilateral pulmonary vein atresia in children are described. The findings in all three cases were similar and consisted of hemoptysis, recurrent pulmonary infections, and a normal size or small lung with reticular markings. In combination, these findings should allow one to strongly suspect the diagnosis which then can be confirmed with radionuclide perfusion studies and pulmonary angiography. Both procedures demonstrate decreased blood flow to the involved lung, but the pulmonary arteriogram is the definitive study in that it shows a small pulmonary artery, pruned peripheral branches, stasis of contrast material, and nonvisualization of draining pulmonary veins. The condition generally is believed to be congenital and related to the somewhat more common, but still rare, focal congenital pulmonary vein stenosis. Treatment consists of surgical removal of the involved lung or lobe.
本文描述了3例儿童单侧肺静脉闭锁病例。所有3例的表现相似，包括咯血、反复肺部感染，以及肺大小正常或较小伴网状纹理。综合这些表现应能强烈怀疑该诊断，随后可通过放射性核素灌注研究和肺血管造影加以证实。这两种检查均显示受累肺的血流减少，但肺动脉造影是确诊检查，因为它显示肺动脉细小、外周分支变细、造影剂滞留以及引流肺静脉不显影。一般认为该病为先天性，与相对更常见但仍属罕见的局灶性先天性肺静脉狭窄有关。治疗方法为手术切除受累的肺或肺叶。
