青少年症状性Chiari畸形I型外科手术治疗的临床研究

Surgical treatments of adolescent symptomatic Chiari malformation type I

目的总结青少年症状性Chiari畸形I型患者临床特点，评价后颅窝小范同骨窗减压手术治疗青少年症状性ChiariI型畸形的临床疗效果。方法对自2006年12月至2011年12月期间在成都军区总医院神经外科接受手术治疗的12例青少年症状性Chiari畸形I型患者进行回顾性分析。所有患者均接受了标准的小骨窗后颅凹减压手术．骨性减压窗范围以枕骨大孔后缘为中心，大小约2cm-2cm，弱电流电凝缩小扁桃体，疏通中脑导水管流出道和第四脑室正中孔，硬脑膜直线切开并行自体筋膜扩大修补。对术前术后进行症状以及脊髓空洞改善情况进行比较。结果短期随访对照研究表明，患者临床症状术后改善明显，影像学特征显示小脑延髓池恢复良好，术前存在的脊髓空洞有不同程度的缩小。半年以上随访8例，手术效果稳定，症状得到持续改善。结论早期接受后颅窝小范围骨窗减压手术是缓解青少年症状性Chiari畸形I型症状发展的重要手段。

Objective To summarize the clinical characteristics of adolescents with symptomatic Chiari malformation type I, and evaluate the clinical results of small-range posterior fossa decompression on these patients. Methods Twelve adolescents with symptomatic Chiari malformation type I, admitted to and underwent a standard small-range posterior decompression surgery in our hospital from December 2006 to December 2011, were chosen; their clinical data were retrospectively analyzed. The standard surgical technique was composed of limited bone cut in 2 cmx2 cm, weak current coagulation in shrinking the tonsils, probe of aqueduct cerebrospinal fluid outflow tract and Magendie＇s foramen of the fourth ventricle, and suture of the dural grafting with patch ofautologous fascia. Preoperative and postoperative symptoms and sizes of syringomyelia were compared. Results Short-term follow-up showed that the clinical symptoms of patients improved significantly, and MRI confirmed that the syringomyelia disappeared or shrank in all the patients and the shape of cisterna magna recovered. Eight patients accepted follow-up for more than six months, showing continues improvement of clinical symptoms. Conclusion Small-range posterior fossa decompression surgery is important means to alleviate adolescent symptomatic Chiari malformation type I.