Sun C C, Doyle T, Ringel R E
Department of Pediatrics, School of Medicine, University of Maryland, Baltimore, USA.
Hum Pathol. 1995 Aug;26(8):880-6. doi: 10.1016/0046-8177(95)90011-x.
Pulmonary vein stenosis (PVS) is a rare disorder. Accurate diagnosis often requires anatomical examination. We report four children with pulmonary vein stenosis. Autopsy showed bilateral lesions in two patients who were thought clinically to have unilateral disease. A diagnosis of PVS was made at autopsy in the third case. Intimal and medial fibromuscular proliferation was noted in extrapulmonary and intrapulmonary veins. Some of the fibromuscular proliferation were eccentric, resembling organized thrombi. In one case a focal organizing thrombus was found in a clinically unobstructed but anatomically narrowed veno-atrial junction. In another case injection of contrast medium into the stenotic pulmonary vein (PV) showed anastomosis between PV and bronchial vessels as well as small pulmonary arteries. Bilateral hypertensive arteriopathy was observed in unilateral and bilateral PVS. Our histological finding of intrapulmonary venous lesions in the lobes in which PVS was not detected clinically suggests that during surgical correction of unilateral PVS multiple biopsies of the opposite lung may help to evaluate possible bilateral disease. Our study also suggests that thrombosis in a stenotic pulmonary vein may further compromise the lumen and contribute to the progression of pulmonary vein obstruction. The possible pathogenesis of bilateral pulmonary hypertensive arteriopathy in unilateral PVS also is discussed.
肺静脉狭窄（PVS）是一种罕见的疾病。准确诊断通常需要进行解剖学检查。我们报告了4例肺静脉狭窄患儿。尸检显示，2例临床诊断为单侧病变的患者存在双侧病变。第3例在尸检时诊断为PVS。肺外和肺内静脉可见内膜和中膜纤维肌性增生。部分纤维肌性增生呈偏心性，类似机化血栓。1例患者在临床无梗阻但解剖学上狭窄的静脉心房连接处发现局灶性机化血栓。另1例患者向狭窄的肺静脉（PV）内注入造影剂后显示PV与支气管血管以及小肺动脉之间存在吻合。单侧和双侧PVS均观察到双侧高血压性动脉病。我们的组织学研究发现，在临床上未检测到PVS的肺叶中存在肺内静脉病变，这表明在单侧PVS的手术矫正过程中，对侧肺进行多次活检可能有助于评估是否存在双侧疾病。我们的研究还表明，狭窄肺静脉内的血栓可能会进一步使管腔狭窄，并导致肺静脉梗阻进展。本文还讨论了单侧PVS中双侧肺高血压性动脉病的可能发病机制。
