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本文报告３３例Ａｒｎｏｌｄ－Ｃｈｉａｒｉ畸形（ＡＣＭ），均经ＭＲＩ证实，其中合并脊髓空洞症２４例，颅颈交界处骨质畸形７例，脑积水６例．临床主要征象为枕大孔区压迫综合征。根据其临床表现、Ｘ线平片、ＣＴ和ＭＲＩ检查明确诊断。本组病人２例行保守治疗，２例行侧脑室－腹腔分流术，２９例行后颅窝开颅、上颈椎椎板切除术。结果好转２５例，无变化７例，恶化１例．本文就ＡＣＭ的发病机理、分型、诊断及治疗进行了简要讨论．
This article reports 33 cases of Arnold-Chiari deformity (ACM), confirmed by MRI, including 24 cases of syringomyelia, craniocervical junctional bone deformity in 7 cases, 6 cases of hydrocephalus. The main clinical signs of occipitocentrum oppression syndrome. According to their clinical manifestations, X-ray, CT and MRI examination confirm the diagnosis. Two patients in our group were treated conservatively. Two patients underwent lateral ventricle-peritoneal shunt. Twenty-nine patients underwent cranial skull craniotomy and upper cervical spondylotomy. Results improved in 25 cases, no change in 7 cases, 1 case of deterioration. This article briefly discusses the pathogenesis, classification, diagnosis and treatment of ACM.