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Chiari畸形表现为小脑尾端或/和延髓向下疝入到椎管内。根据疝的程度,这种畸形至少可分为两种不同的类型:Ⅰ型和Ⅱ型。Ⅰ型表现为小脑扁桃体下疝,Ⅱ型既表现为小脑尾端下疝又有四脑室和延髓下疝。从临床角度分类,Chiari畸形又可分为成人型和儿童型。成人型大都属于Ⅰ型,通常在20～30岁后发病,表现后颅窝过度拥挤或/和脊髓空洞症的症状和体征。这种畸形常伴有颅底异常(如颅底凹陷),除小脑扁桃体下疝外,很少有脑组织发育异常。儿童型主要属于Ⅱ型,典型表现为出生时患有脊髓裂和出生后早期出现脑干功能障碍,后颅窝狭小、脑于畸形和脑积水等。
Chiari deformity manifested as cerebellar caudal or / and medulla oblongata herniated into the spinal canal. According to the degree of hernia, this deformity can be divided into at least two different types: type I and type II. Ⅰ-type performance of the cerebellar tonsil hernia, Ⅱ-type performance of both the cerebellar tail hernia and four ventricle and medullary hernia. From the clinical point of view, Chiari deformity can be divided into adult and children. Adults mostly belong to type I, usually in the 20 to 30-year-old after the onset of the performance of the posterior fossa or / and syringomyelia symptoms and signs. This deformity often associated with skull base abnormalities (such as skull base depression), in addition to cerebellar tonsil hernia, very few brain dysplasia. Children mainly belong to type Ⅱ, the typical manifestations of birth at birth with spinal cord and brain stem early after birth dysfunction, posterior fossa narrow, brain deformity and hydrocephalus.