Mondini畸形伴脑脊液耳鼻漏的外科处理

Surgical management of Mondini dysplasia with cerebrospinal fluid leakage

目的 :探讨Mondini畸形伴脑脊液耳鼻漏的临床表现及外科手术方法。方法 :对 3例Mondini型内耳畸形伴自发性脑脊液耳鼻漏患儿 ,均采用经卵圆窗内耳填塞方法修补。结果 :3例皆一次手术修补成功 ,随访半年以上无脑脊液漏或脑膜炎复发。结论 :幼儿自发性脑脊液耳鼻漏应警惕Mondini型内耳畸形 ,颞骨薄层CT扫描可明确诊断 ,经鼓室进路手术修补为有效方法。

Objective:To evaluate the clinical manifestation and surgical technique of Mondini dysplasia with cerebrospinal fluid (CSF) leakage. Method:Three Mondini dysplasia with spontaneous cerebrospinal fluid leakage were treated by the authors. A transcanal tympanotomy were used to pack the vestibular cavity with muscle. Result:Three children were first manifested with recurrent meningitis and spontaneous cerebrospinal fluid rhinorrhea. Further examinations found that the leakages were otogenic. One side or both sides Mondini dysplasia were confirmed by CT scanning of temporal bones. The leakages were all stopped by the primary surgical closure. Conclusion:Mondini dysplasia should be considered in children with spantaneous cerebrospinal fluid otorhinorrhea. A temporal bone CT scan can confirmed the diagnosis. A transtympanic closure is very effective in the management.