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伴有大型肺动静脉畸形的肝肺综合征:CT表现,重点关注其与肺实质马赛克征的关联

Hepatopulmonary syndrome with large pulmonary arteriovenous malformations: CT findings with emphasis on its association with a mosaic pattern of the lung parenchyma.

作者信息

Gorospe Sarasúa Luis, Olavarría-Delgado Andreína, Farfán-Leal Frank Eric, Pérez-Templado Ladrón de Guevara Josefa

机构信息

Radiodiagnóstico, Hospital Universitario Ramón y Cajal. Madrid, ESPAÑA.

Hospital Universitario Ramón y Cajal.

出版信息

Rev Esp Enferm Dig. 2017 May;109(5):369.

Abstract

We present a case of a 54-year-old patient with cirrhosis, progressive dyspnea, and platypnea. Thoracic computed tomography (CT) showed multiple pulmonary arteriovenous malformations (PAVM), confirming the diagnosis of hepatopulmonary syndrome (HPS). Besides precisely identifying the number and location of PAVM, CT also demonstrated a striking mosaic pattern of the lung parenchyma, characterized by the presence of alternating geographic areas of low attenuation (showing pulmonary vessels with a decreased diameter) with regions of relatively increased attenuation (showing pulmonary vessels with a normal diameter). This mosaic pattern of the lung parenchyma has scarcely been described in patients with HPS since it is not always present and usually requires a post-processing of the CT images in order to increase the contrast between the low attenuation areas (representing hypoperfused regions) and the areas with a relatively increased attenuation (representing better perfused regions). The decision was made to embolize the major PAVM, achieving an improvement of both the oxygen partial pressure and the patient's symptoms. This improvement allowed the patient to become an acceptable candidate for liver transplantation. We believe that, unlike other radiological signs of HPS, the mosaic pattern has not been sufficiently described in the scientific literature. If the association of the mosaic pattern on CT with HPS is confirmed in larger studies, it could become a useful sign for detecting hypoperfused pulmonary areas related to small nonvisible PAVM.

摘要

我们报告一例54岁患有肝硬化、进行性呼吸困难和平卧呼吸的患者。胸部计算机断层扫描(CT)显示多处肺动静脉畸形(PAVM),确诊为肝肺综合征(HPS)。除了精确识别PAVM的数量和位置外,CT还显示出肺实质明显的马赛克样表现,其特征为交替出现的低衰减地理区域(显示肺血管直径减小)与相对高衰减区域(显示肺血管直径正常)。肺实质的这种马赛克样表现很少在HPS患者中被描述,因为它并非总是存在,通常需要对CT图像进行后处理,以增加低衰减区域(代表灌注不足区域)与相对高衰减区域(代表灌注较好区域)之间的对比度。决定对主要的PAVM进行栓塞,使氧分压和患者症状均得到改善。这种改善使患者成为肝移植的合适候选者。我们认为,与HPS的其他放射学征象不同,马赛克样表现尚未在科学文献中得到充分描述。如果在更大规模的研究中证实CT上的马赛克样表现与HPS之间的关联,它可能成为检测与小的不可见PAVM相关的灌注不足肺区域的有用征象。

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