目的　探索小儿严重寰枢椎不稳的手术治疗方式。方法　回顾总结 2 2例小儿严重寰枢椎不稳的临床资料 ,男 16例 ,女 6例 ,年龄 3～ 14岁 ,平均 11.4岁。病因有齿突骨折、横韧带断裂、枕颈畸形、类风湿关节炎等。所有患儿均行后路融合术 ,其中寰枢椎融合 14例 ,枕颈融合术 8例。结果　 2 0例患儿经平均 2 0个月 (3个月～ 11年 )随访 ,均获骨性融合 ,取得满意效果。结论　上颈椎后路融合术是治疗小儿严重寰枢椎不稳的有效方法 ,对寰枢椎能复位者选择寰枢椎融合 ,脱位不能复位者应行枕颈融合术 Objective To explore the surgical treatment of severe atlantoaxial instability in children. Methods The clinical data of 22 severe pediatric atlantoaxial instability were retrospectively reviewed. There were 16 males and 6 females, aged from 3 to 14 years with an average of 11.4 years. Causes of odontoid fractures, transverse ligament rupture, occipital neck deformity, rheumatoid arthritis. All children underwent posterior fusion, including 14 cases of atlantoaxial fusion, occipitocervical fusion in 8 cases. Results Twenty cases were followed up for an average of 20 months (range, 3 months to 11 years). All achieved satisfactory results. Conclusions The posterior cervical fusion is an effective method for the treatment of severe atlantoaxial instability in children. Atlantoaxial vertebral fusion can be selected for atlantoaxial detorsion. If the dislocation can not be reset, the occipitocervical fusion should be performed