Chiari畸形I型(Chiari malformation type I, CMI)，是一種以小腦扁桃體向下病入枕骨大孔為特徵的先天性畸形，在臨床上常常伴有很多合併症，如syringomyelia(SM)，其脊髓内存在異常液性囊腔，是慢性進行性脊髓變性。此外，有文獻還報導過個別CMI患者伴發的一些更為特別的臨床症狀或體徵。這些CMI相關病變目前尚未被系統歸類，甚至不能說清其究竟是CMI的原因還是結果。本文綜述相關文獻，試圖根據器官系統、是否能促進CMI的發生發展等，釐清這些相關病變和CMI間的關係，這將有助於我們進一步理解CMI的病因學並革新CMI治療策略。

Chiari type I malformation (Chiari malformation type I, CMI), is a congenital malformation characterized by downward herniation of cerebellar tonsillar into foramen magnum. It often associated with many complications, such as syringomyelia (SM), a chronic progressive degeneration of spinal cord. In addition, literatures also report that some CMI patients are accompanied by other special clinical symptoms or signs. These CMI-related disorders have not yet been systematically classified. We even do not know they are the causes or results of CMI. In this paper, we reviewed related literatures according to organs or systems involved and whether they can induce the occurrence and development of CMI. These reports may clarify the relationship between these disorders and CMI, and help us further understand the etiology and treatment strategy of CMI.