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目的 探讨Arnold Chiari畸形 (ACM)合并脊柱侧凸、脊髓空洞等畸形的诊断和治疗方式。方法 通过对 5 8例ACM患者术前MRI及术中所见确定诊断 ,并选择手术方式。对单纯ACM及脊髓空洞较小者 (空洞与脊髓比 )小于 0 5者行枕大孔减压加硬脑膜扩大成形术 ,对空洞脊髓比大于 0 5者则加行脊髓空洞 蛛网膜下腔分流术。枕颈不稳定者及 15岁以下儿童 ,则加做颈枕融合手术。结果 5 8例中合并脊髓空洞 30例 ,脊柱侧凸 36例 ,颅底凹陷 5例 ,寰枕融合 6例。 5 8例均行枕大孔减压及硬脑膜扩大成形术 ,7例加行脊髓空洞 蛛网膜下腔分流术。 2 1例行一期枕颈融合术。结论 Chiari畸形系颅颈交界区畸形 ,常合并不同的脊柱、脊髓畸形 ,因此诊断时必须全面考虑 ,正确判断 ,便于手术方法的选择。
Objective To investigate the diagnosis and treatment of Arnold Chiari deformity (ACM) associated with scoliosis and syringomyelia. Methods Fifty-eight cases of ACM patients with preoperative MRI and intraoperative findings confirmed diagnosis, and select the surgical approach. For the simple ACM and the smaller syringomyelia (void vs spinal cord ratio) less than 0 5 underwent macroscopic decompression plus dura mater enlargement angioplasty, empty hole spinal cord ratio greater than 0 5 were treated with syringomyelia subarachnoid shunt Surgery. Occipital and neck instability and children under the age of 15, then add neck and neck fusion surgery. Results Fifty-eight cases had syringomyelia in 30 cases, scoliosis in 36 cases, skull base depression in 5 cases and atlanto-occipital fusion in 6 cases. Fifty-eight cases underwent both maxillofacial decompression and duraloplasty, and seven patients underwent syringomyelia shunting. 2 1 routine first occipitocervical fusion. Conclusions The Chiari deformity is a deformity of the craniocervical junction and is often associated with different spine and spinal cord deformities. Therefore, the diagnosis of Chiari deformity must be fully considered and correctly diagnosed to facilitate the selection of operative methods.