Turner综合征合并中耳胆脂瘤一例及文献复习

王素芳;韩富根;许莹;张杰;

1:郑州大学附属儿童医院(河南省儿童医院)耳鼻咽喉头颈外科

摘要

目的探讨Turner综合征(turner syndrome,TS)即先天性卵巢发育不全综合征并中耳胆脂瘤的临床诊治特点。方法对1例合并中耳胆脂瘤的TS患儿(女性,13岁)的临床资料及诊治过程进行总结,并复习相关文献。结果本例患儿身材矮小,双侧耳廓招风耳畸形,右侧耳道内大量肉芽样物及脓、血性分泌物,鼓膜未能窥及;右耳传导性聋(0.25～8 kHz气导阈值:70、75、75、70、60、50 dB HL,0.25～4 kHz骨导阈值:0、0、5、5、5 dB HL);颞骨CT显示脑膜低位,乳突气化不良,右侧耳道、中耳、乳突内软组织密度影,周围骨质破坏明显,听小骨骨质密度减低。于全身麻醉下行右侧乳突根治及鼓室成形术,术后病理检查显示胆脂瘤继发慢性化脓性炎症,异物肉芽肿形成。诊断为右侧中耳胆脂瘤,Turner综合征,核型45,X,O/46,X,X(99%/1%);术后复查纯音听阈,气导听阈:35、40、40、40、30、20 dB HL听阈,骨导听阈:20、20、20、25、25 dB HL,随访半年无感染及复发。结论中耳胆脂瘤在TS中具有较高的患病率及强侵袭性,应当及早关注、早期诊断、及时手术治疗和仔细随访,以尽量减少胆脂瘤进展的风险。

关键词

Turner综合征;胆脂瘤;中耳;儿童

基金项目(Foundation):

作者

王素芳;韩富根;许莹;张杰;

参考文献

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