小脑扁桃体下疝(Cjoari malformation,CM),是后颅凹中线结构在胚胎期的异常发育致使小脑扁桃体疝入枕大孔内而引起延髓、上颈髓受压,颅内压增高所表现的一组综合征。属于先天性疾病,除常伴发脊髓空洞外,尚有25％～50％的病例合并其他颅颈部畸形,如颈椎管狭窄,颅底凹陷,扁平颅底,寰椎和/或颈椎融合畸形,寰椎半脱位等。该类畸形可单发,也可二种或多种并发。本病症状多样,特征性体征不明显,不易诊断。近年来,随着影像学技术的不断进展,对该病有了进一步认识。本文就近年的进展,对其诊断、治疗作一综述。1 分型 Cjoari malformation (CM), which is caused by the abnormal development of the posterior fossa midline structure during the embryonic period, causes the tonsillar herniation into the macropore of the occipital lobe and causes medulla oblongata, compression of the upper cervical cord, and increased intracranial pressure Group syndrome. Belong to congenital diseases, in addition to often associated with syringomyelia, there are 25% to 50% of cases with other craniocerebral deformities, such as cervical stenosis, skull base depression, flat skull base, atlas and / or cervical fusion deformity , Atlas subluxation and so on. This type of deformity can be single, but also two or more concurrent. Symptoms of the disease varied, characteristic signs are not obvious, not easy to diagnose. In recent years, as the imaging technology continues to progress, the disease has been further understanding. This article on the progress in recent years, its diagnosis and treatment are reviewed. 1 type