摘要肌纤维空泡是一种非特异性的骨骼肌病理改变，作为一个病理学术语，可以出现在多种罕见病中，其形成大多与糖、脂肪、蛋白和细胞器自噬有关，少部分形成于糖、脂肪贮积或肌质网腔空泡化。任何肌纤维空泡都可以破坏肌纤维的内部结构，形成各种疾病骨骼肌病理改变的一部分。文中介绍伴随肌纤维空泡疾病的临床和病理特征，重点阐述肌纤维空泡改变在诊断中的价值，要理解这些病理改变还需熟悉其产生的机制。

abstractsThe vacuole in muscle fibers is a non-specific myopathological change. As a myopathological term, it occurs in several rare disorders. The vacuoles are mostly related to the autophagy of glycogen, lipids, abnormal proteins, and organelles, and a few are caused by glycogen or lipids deposition or sarcoplasmic reticulum luminal vacuolization. The vacuoles impair the structure of muscle fibers, being one of the myopathological features in various diseases. In this review, clinical features and myopathological changes of various diseases with vacuolar muscle fibers were introduced and the diagnostic value of intrafibral vacuoles was focused on. Knowing the underlying pathogenesis is required to understand these myopathological changes.