这篇综述旨在通过结合人类和兽医的见解来丰富我们对Chiari样畸形(CLM)的理解,并提供详细的跨物种概述。CLM是一种发育异常,其特征是由于颅骨体积不足引起的整个脑实质移位,后脑的尾移位进入大孔。这种畸形导致颅颈交界处进行性阻塞,破坏正常的脑脊液流动,导致继发性脊髓空洞症.CLM和脊髓空洞症的临床症状包括幻影刮伤,头部倾斜,头部震颤,共济失调,四肢轻瘫,疼痛,肌肉萎缩,和脊柱侧弯或斜颈。磁共振成像仍是诊断CLM的金标准,因为它可以可视化异常的发现,如尾小脑疝,枕骨发育不良引起的尾小脑压迫,和减弱的脑脊液池。虽然各种医疗和手术干预,包括大孔减压,可以提供暂时的症状/临床症状缓解,目前的文献显示缺乏持续的长期疗效。因此,需要更多的研究来评估现有治疗策略的长期效果,并比较不同的技术与大孔减压联合使用.
This review aims to enrich our understanding of Chiari-like malformation (CLM) by combining human and veterinary insights, and providing a detailed cross-species overview. CLM is a developmental abnormality characterised by caudal displacement of the hindbrain into the foramen magnum due to an entire brain parenchymal shift caused by insufficient skull volume. This malformation leads to a progressive obstruction at the craniocervical junction, which disrupts the normal cerebrospinal fluid flow, leading to secondary
syringomyelia. The clinical signs of CLM and
syringomyelia include phantom scratching, head tilt, head tremor, ataxia, tetraparesis, pain, muscle atrophy, and scoliosis or torticollis. Magnetic resonance imaging remains the gold standard for diagnosing CLM, since it allows the visualisation of abnormal findings such as the caudal cerebellar herniation, caudal cerebellar compression from occipital dysplasia, and attenuated cerebrospinal fluid cisternae. Although various medical and surgical interventions, including foramen magnum decompression, can provide temporary symptomatic/clinical sign relief, current literature shows a lack of sustained long-term efficacy. Therefore, additional research is needed to evaluate the long-term effects of existing treatment strategies and to compare different techniques utilised in conjunction with foramen magnum decompression.