摘要
目的探讨儿童颞骨朗格汉斯组织细胞增生症(langerhans cell histiocytosis,LCH)的临床特点。方法回顾性分析武汉儿童医院耳鼻咽喉科2011年6月~2016年12月期间收治并经病理确诊的14例颞骨LCH患儿的临床资料,分析临床表现、影像学检查、诊断、治疗及预后等特点。结果 14例颞骨LCH患儿中,女5例,男9例,年龄4个月~3岁10个月,2岁以下者12例(85.71%);8例为单系统病变(3例为颞骨单灶型,5例为多灶型),6例为多系统病变;病变大多累及颞骨鳞部、外耳道及乳突,其次为鼓室及鼓窦,较少累及听小骨及内耳;主要临床表现为耳颞部肿块、耳漏、听力下降、外耳道肉芽等。14例患儿中8例行外耳道深部病损切除术,3例行颞部肿块切除术,1例行额部病损切除活检术,2例行四肢骨病损清除活检术明确病理诊断,所有患儿确诊后均加用化疗[长春花碱(VBL)+泼尼松],随访0.5~5年,3例疾病消退,8例疾病稳定,3例疾病进展,其中1例合并败血症死亡,2例出现尿崩症。单系统病变患儿预后优于多系统病变患儿。结论颞骨LCH多累及单系统,多以耳漏及耳部包块为首发症状,影像学检查是颞骨LCH的主要检查手段;需与耳部感染性疾病或肿瘤相鉴别,确诊主要靠病理学检查;颞骨LCH单系统受累患儿预后好于多系统受累患儿。
ObjectiveTo study the clinical characteristics of the Langerhans cell histiocytosis(LCH)of the temporal bone in children.MethodsA total of14children with LCH of the temporal bone at Wuhan Children's Hospital were retrospectively reviewed from June2011to December2016.The clinical data,imaging findings,diagnosis,treatment and prognosis were studied.The correlation between clinical classifications and prognosis was also analyzed.Among the14patients,5were girls and9were boys.Eight cases were the single system group(3cases as the single temporal bone,5case as the multifocality),and6case as the multisystem group.ResultsThe most common clinical characteristics of LCH were temporal tumors,otorrhea,otalgia,hearing loss and the granulation of external auditory canals.The followed-up was for1~5years.Four cases were regressive,l case kept stable,while2cases showed progressive.The prognosis in the multisystem group was significantly worse than the single system group(P<0.05).Conclusion The most of temporal bone LCH involves the single system,while temporal tumor and otorrhea are common as the first signs of temporal bone LCH.The imaging examination remains as the major diagnosis method.The differential diagnosis focuses on the common ear infection and otic neoplasms.The diagnosis is based on histological and immunophenotypic examination of the lesion tissue.The prognosis of the single system group is much better than the multisystem group.
作者
周长璐
夏忠芳
Zhou Changlu;Xia Zhongfang(Department of Otolaryngology- Head and Neck Surgery, Wuhan Children’s Hospital,Tongji Medical College , Huazhong University of Science & Technology,Wuhan,430016,China)
出处
《听力学及言语疾病杂志》
CAS
CSCD
北大核心
2018年第1期16-20,共5页
Journal of Audiology and Speech Pathology
