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摘要: 肝门静脉肝血窦疾病(porto-sinusoidal vascular disease，PSVD)作为近年新命名的一种特殊类型非肝硬化性门静脉高压症，越来越受到关注。PSVD具有门静脉高压的相关症状，但其病理特征不同于常见的肝炎后肝硬化，临床易误诊和漏诊。由于PSVD的发病机制不明确，临床影像学认识不足，诊治中存在诸多挑战。本文综述了PSVD的病因病理学、临床影像学诊断研究进展，以期提高对该疾病的认识和诊断水平。Abstract: Porto-sinusoidal vascular disease(PSVD), a recently designated subtype of non-cirrhotic portal hypertension, has been receiving growing attention. PSVD is characterized by symptoms of portal hypertension, but its pathology is distinct from the more common post-hepatitic cirrhosis, often leading to misdiagnosis in clinical practice. Given the unclear pathogenesis of PSVD and the limited knowledge of its imaging characteristics, there are significant challenges in its diagnosis and management. This review summarizes the current understanding of the pathogenesis, pathology, and imaging advancements in the diagnosis of PSVD, with the aim of improving recognition and diagnostic accuracy for this condition.
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表 1 PSVD与肝硬化在病理特征上的对比
特征 PSVD 肝硬化 门静脉病变 门静脉壁增厚，内皮细胞增生，血栓形成 门静脉纤维化，血栓形成常见 肝小叶结构 肝小叶结构紊乱，局灶性轻度纤维化 小叶结构严重破坏 纤维化 局灶性，轻度 广泛，弥漫性 再生结节 结节性再生结节(无显著纤维化) 显著的再生结节伴随纤维化 血管异常 门静脉和肝血窦的微血管病变 严重的血管结构重塑 
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