儿童内耳畸形相关脑脊液耳漏的影像学特点  

Imaging evaluation of cerebrospinal fluid otorrhea associated with inner ear malformation in children

作　　者：陈敏[1] 高军[2] 刘薇[1] 韩忠龙[2] 杨扬[1] 邵剑波 郑军[3] 张杰[1] Chen Min;Gao Jun;Liu Wei;Han Zhonglong;Yang Yang;Shao Jianbo;Zheng Jun;Zhang Jie(Department of Otorhinolaryngology Head and Neck Surgery,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing Key Laboratory for Pediatric Diseases of otolaryngology Head and Neck Surgery,Beijing 100045,China;Department of Radiology,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China;Department of Otorhinolaryngology Head and Neck Surgery,Tongren Hospital,Capital Medical University,Beijing 100730,China)

机构地区：[1]国家儿童医学中心、首都医科大学附属北京儿童医院耳鼻咽喉头颈外科、儿童耳鼻咽喉头颈外科疾病北京市重点实验室,北京100045 [2]国家儿童医学中心、首都医科大学附属北京儿童医院影像科,北京100045 [3]首都医科大学附属北京同仁医院耳鼻咽喉头颈外科,北京100730

出　　处：《中华耳鼻咽喉头颈外科杂志》2023年第5期452-459,共8页Chinese Journal of Otorhinolaryngology Head and Neck Surgery

基　　金：北京市医院管理中心儿科学科协同发展中心专项经费(XTYB201828);北京市医院管理中心“登峰”计划专项经费(DFL20191201)。

摘　　要：目的探讨儿童内耳畸形相关脑脊液耳漏的影像学特点。方法回顾分析2016年11月至2021年1月年在北京儿童医院经手术探查确诊的28例内耳畸形相关脑脊液耳漏患儿的临床资料,其中男16例、女12例,年龄8个月~15岁8个月,中位年龄4岁。探查术中观察患儿镫骨形态,分析术前、术后颞骨CT和内耳MRI的影像学特点。结果28例脑脊液耳漏患儿中89.3%(25/28)术中可见镫骨底板骨质缺损。术前颞骨CT显示内耳畸形、鼓膜膨隆、鼓室及乳突软组织影等间接征象;内耳畸形涉及四种耳蜗畸形:不完全分隔Ⅰ型(incomplete partition typeⅠ,IP-Ⅰ)、共同腔畸形、不完全分隔Ⅱ型(incomplete partition typeⅡ,IP-Ⅱ)和耳蜗未发育;100%(28/28)可见前庭扩大畸形;85.7%(24/28)内听道底板骨质缺损。术前内耳MRI检查T2加权像,73.9%(17/23)可见脑脊液耳漏的直接征象:内耳前庭与中耳鼓室两个T2片状高信号之间由点状略低或混杂T2信号相连,冠状位明显;100%(23/23)可见鼓室高T2信号与前、下方咽鼓管相延续的间接征象。填塞有效者,复查高分辨率CT:鼓室、乳突软组织影早至术后1周可减少或消失;复查核磁:术后3 d~1个月鼓室、乳突T2高信号影减少或消失,术后1~4个月前庭内可见中等T2信号软组织填塞影。结论伴前庭扩大的IP-Ⅰ、共同腔畸形、IP-Ⅱ和耳蜗未发育四类内耳畸形可能会导致脑脊液耳漏。结合特殊病史,内耳MRI的T2加权像可为多数内耳畸形伴脑脊液耳漏患儿提供直接诊断依据。颞骨CT及内耳MRI也可用于脑脊液耳漏填塞手术的疗效评估。Objective To explore the imaging evaluation of cerebrospinal fluid(CSF)otorrhea associated with inner ear malformation(IEM)in children.Methods The clinical data of 28 children with CSF otorrhea associated with IEM confirmed by surgical exploration in Beijing Children′s Hospital,from Nov,2016 to Jan,2021,were analyzed retrospectively,including 16 boys and 12 girls,aged from 8-month to 15-year and 8-month old,with a median age of 4-year old.The shapes of stapes were observed during the exploration surgery,and the imaging features of temporal bone high resolution CT(HRCT)and inner ear MRI pre-and post-operation were analyzed.Results In 28 children with CSF otorrhea,89.3%(25/28)had stapes footplates defect during exploration.Preoperative CT showed indirect signs such as IEM,tympanic membrane bulging,soft tissue in the tympanum and mastoid cavity.IEM included four kinds:incomplete partition type I(IP-Ⅰ),common cavity(CC),incomplete partition typeⅡ(IP-Ⅱ),and cochlear aplasia(CA);100%(28/28)presented with vestibule dilation;85.7%(24/28)with a defect in the lamina cribrosa of the internal auditory canal.The direct diagnostic sign of CSF otorrrhea could be seen in 73.9%(17/23)pre-operative MRI:two T2-weighted hyperintense signals between vestibule and middle ear cavity were connected by slightly lower or mixed intense T2-weighted signals,and obvious in the coronal-plane;100%(23/23)hyperintense T2-weighted signals in the tympanum connected with those in the Eustachian tube.In post-operative CT,the soft tissues in the tympanum and mastoid cavity decreased or disappeared as early as one week.In post-operative MRI,the hyperintense T2-weighted signals of tympanum and mastoid decreased or disappeared in 3 days to 1 month,soft tissues tamponade with moderate intense T2-weighted signal were seen in the vestibule in 1-4 months.Conclusions IP-Ⅰ,CC,IP-Ⅱand CA with dilated vestibule can lead to CSF otorrhea.Combined with special medical history,T2-weighted signal of inner ear MRI can provide diagnostic basie for most chil

关 键 词：脑脊液耳漏 内耳畸形 体层摄影术 X线计算机 磁共振成像 儿童 

分 类 号：R764.73[医药卫生—耳鼻咽喉科]