Chiari畸形合并脊髓空洞症的诊疗进展

Advances in Diagnosis and Treatment of Chiari Malformation Associated with Syringomyelia

Chiari畸形(Chiari malformation)是一种以小脑扁桃体下疝入枕骨大孔为特征的先天性畸形,脊髓空洞症(syringomyelia)是该病最常见的并发症,其诊断主要依赖于MRI。目前,对于该病的病因、监测手段、随访条件以及治疗方法等,专家们还未能达成共识。根据Chiari畸形解剖上的异常将其分为四型,分型有利于术式的选择,尽管关于分型的争议较多,但Hans Chiari分型仍最普遍被临床广为接受和应用。Chiari畸形临床表现多变,根据常见主要症状及体征大体可分为四种类型。手术是治疗Chiari畸形合并脊髓空洞症的唯一有效方法,针对不同病情采用后颅窝减压术、小脑扁桃体切除及枕大池重建术、脊髓空洞分流术等,大部分患者的症状和体征可以得到改善。随着手术方法的不断改进,Chiari畸形合并脊髓空洞的治疗会得到比较理想的效果。

Chiari malformation is a congenital malformation characterized with hindbrain hernia, syringomyelia is the most common complications of the disease. It is generally accepted that it was caused by underdevelopment of the occipital somite originating from the paraxial mesoderm. The diagnosis of Chiari malformation is mainly based on MRI. The experts have not been able to reach a consensus on the cause of the disease, monitoring means, follow-up conditions or treatments. Chiad malformation can be classified into four types according to anatomic features. The various clinical classification can guide the selection of surgical treatment. The clinical manifestations varied, according to the common symptoms and signs earl be roughly divided into four syndrome group. Surgery is the only cure-intended therapy to Chiari malformation. Majority of the patients experience improvement in their symptoms and signs after accordingly treatment, such as posterior fossa decompression, cerebellar tonsillectomy, cistern magna reconstruction and shunt. With the continuous improvement of surgical techniques, the treatment of Chiari malformation with syringomyelia will get more satisfactory effect.