小脑扁桃体下疝是一种先天性发育畸形疾病,临床较为常见,症状不典型,常合并脊髓空洞症,对病患生活质量影响大。长期以来对本病的手术方式多样,疗效各异,各家说法不一。随着影像学诊断技术的提高,对本病的临床诊断并不困难。目前争论主要集中在手术方式的选择及各术式远期预后的随访。本文主要对小脑扁桃体下疝畸形并脊髓空洞的诊断及手术治疗的新进展进行综述。 Cerebellar tonsil hernia is a congenital malformations of development, clinical is more common, the symptoms are not typical, often associated with syringomyelia, the quality of life of patients with large impact. For a long time the operation of this disease varied, curative effects vary, different opinions. With the improvement of imaging diagnostic techniques, the clinical diagnosis of the disease is not difficult. The current controversy is mainly focused on the choice of surgical approach and follow-up of long-term prognosis of various surgical procedures. This article mainly reviews the diagnosis and surgical treatment of the inferior cerebellar tonsillar hernia deformity and syringomyelia.