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肝窦状隙障碍。

Hepatic Sinusoidal Disorders.

机构信息

From the Department of Radiology, Abdominal Imaging Division, Mayo Clinic, 200 First St SW, Rochester, MN 55905 (S.K.V., K.C.H., S.M.T., S.H., P.J.N., C.N.G., C.L.W.); Department of Medical Imaging, University of Ottawa, Ottawa, Ontario, Canada (K.C.H.); Department of Abdominal Imaging, Northwestern Memorial Hospital, Chicago, Ill (A.A.B.); Department of Abdominal Imaging, University of Pittsburgh, Pittsburgh, Pa (A.F., F.H.M.); Department of Anatomic Pathology, Mayo Clinic, Rochester, Minn (E.Z.M.C., M.S.T.); and Department of Radiology, Hacettepe University, Ankara, Turkey (I.S.I.).

出版信息

Radiographics. 2024 Sep;44(9):e240006. doi: 10.1148/rg.240006.

Abstract

Hepatic sinusoids are highly specialized microcirculatory conduits within the hepatic lobules that facilitate liver functions. The sinusoids can be affected by various disorders, including sinusoidal dilatation, sinusoidal obstruction syndrome (SOS), sinusoidal cellular infiltration, perisinusoidal infiltration, and endothelial neoplasms, such as hemangioendothelioma and angiosarcoma. While these disorders, particularly SOS and neoplasms, can be life threatening, their clinical manifestation is often nonspecific. Patients may present with right upper quadrant pain, jaundice, hepatomegaly, ascites, splenomegaly, and unexplained weight gain, although the exact manifestation depends on the cause, severity, and duration of the disease. Ultimately, invasive tests may be necessary to establish the diagnosis. A comprehensive understanding of imaging manifestations of various sinusoidal disorders contributes to early diagnosis and can help radiologists detect subclinical disease. Additionally, specific imaging features may assist in identifying the cause of the disorder, leading to a more focused and quicker workup. For example, a mosaic pattern of enhancement of the liver parenchyma is suggestive of sinusoidal dilatation; peripheral and patchy reticular hypointensity of the liver parenchyma on hepatobiliary MR images is characteristic of SOS; and associated diffuse multiple hyperintensities on diffusion-weighted images may be specific for malignant sinusoidal cellular infiltration. The authors provide an overview of the pathogenesis, clinical features, and imaging appearances of various hepatic sinusoidal disorders, with a special emphasis on SOS. RSNA, 2024 Supplemental material is available for this article.

摘要

肝窦是肝小叶内高度特化的微循环管道,有助于肝脏功能。各种疾病可影响肝窦,包括窦扩张、窦阻塞综合征(SOS)、窦细胞浸润、窦周浸润和内皮肿瘤,如血管内皮细胞瘤和血管肉瘤。尽管这些疾病,特别是 SOS 和肿瘤,可能危及生命,但它们的临床表现通常是非特异性的。患者可能出现右上腹疼痛、黄疸、肝肿大、腹水、脾肿大和不明原因的体重增加,尽管确切的表现取决于病因、严重程度和疾病持续时间。最终可能需要进行有创检查以确立诊断。全面了解各种肝窦疾病的影像学表现有助于早期诊断,并能帮助放射科医生发现亚临床疾病。此外,特定的影像学特征可能有助于确定疾病的病因,从而进行更有针对性和更快速的检查。例如,肝脏实质马赛克样增强提示窦扩张;肝胆磁共振成像上肝脏实质周边和斑片状网状低信号提示 SOS;弥散加权图像上弥漫性多发高信号可能是恶性窦细胞浸润的特征。作者概述了各种肝窦疾病的发病机制、临床特征和影像学表现,特别强调了 SOS。RSNA,2024 补充材料可用于本文。

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