第四脑室输出道疏通术治疗Chiari畸形并脊髓空洞

Microsurgical treatment of Chiari malformation associated with syringomyelia by the tonsillectomy and the cutting of the dissepiment

目的探讨显微外科治疗Chiari畸形并脊髓空洞的手术方法。方法Chiari畸形Ⅰ型并脊髓空洞经MRI确诊。小范围后颅窝减压+C1后弓切除,纵形切开硬膜,显微镜下分离小脑扁桃体与脑干的粘连,切除小脑扁桃体下疝,探察脊髓中央管开口,发现有隔膜或活瓣形成并切开,探察第四脑室侧孔及中脑导水管,使第四脑室脑脊液循环通畅。结果第四脑室输出道疏通术共计97例,术中显微镜下发现脊髓中央管开口有隔膜或活瓣形成,手术切除脊髓中央管口隔膜或活瓣,并探察第四脑室正中孔及两侧外侧孔,恢复第四脑室脑脊液循环,原位缝合硬膜。术后随访48例,平均1.3年,MRI检查示所有患者下疝的小脑扁桃体均消失,并存脊髓空洞明显缩小和消失。结论第四脑室输出道疏通术能够改善Chiari畸形Ⅰ型并脊髓空洞患者的术后临床症状。

Objective To explore the microsurgical treatment of the Chiari malformation associated with syringomyelia. Methods Ninety-seven patients diagnosed by MRI as Chiari malformation type I with syringomyelia underwent such a surgery. The surgical procedures were carried out as the decompression of posterior cranial fossa, removal of posterior arch of the atlas, longitudinal incision of dura, separation of adherence of cerebellar tonsils and brain stem, resection of hernia below cerebellar tonsil, cutting of the thin membrane over the opening of central canal, and finally keeping normal cerebrospinal fluid （CSF） circulation of the fourth ventricle. Results This kind of dissepiment over the opening of central canal was found in all patients. By cutting ofdissepiment and overflowing of CSF, the CSF circulation recovered. Dura was sutured in situ. Postoperative MRI confirmed that the syringomyelia disappeared or shrank within follow-up averaging 1.3 years in 48 cases. Conclusion The tonsillectomy and the cutting of the dissepiment over the opening of the central canal are the ideal treatment for Chiari malformation associated with syringomyelia.