儿童颞骨朗格汉斯组织细胞增生症临床特点分析

发布时间：2018-06-11 20:15

  本文选题：儿童 + 颞骨　； 参考：《听力学及言语疾病杂志》2018年01期

【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。
[Abstract]:Objective to investigate the clinical features of Langerhans cell histiocytosissis (LCHs) in children with temporal bone Langerhans histiocytosis. Methods the clinical data of 14 children with LCH of temporal bone admitted from June 2011 to December 2016 in Wuhan Children's Hospital were retrospectively analyzed. The clinical manifestations, imaging examination, diagnosis, treatment and prognosis were analyzed. Results among the 14 cases of temporal bone LCH, 5 cases were female, 9 cases were male, 12 cases were under 2 years old, aged 4 months and 10 months old, 8 cases were single system lesion, 3 cases were single focus type of temporal bone, 5 cases were multi-focal type and 6 cases were multi-system lesions. Most of the lesions involved the scales of temporal bone, external auditory meatus and mastoid process, followed by tympanic chamber and tympanic sinus, and less involvement of small auditory bone and inner ear. Of 14 children with external auditory canal granulation and so on, 8 cases underwent excision of deep lesion of external auditory canal and 3 cases underwent temporal mass resection. One case underwent frontal lesion excision and 2 cases underwent excision of bone lesions of extremities. The pathological diagnosis was confirmed by excision of bone lesions of extremities. All the children were treated with chemotherapy (VBL) prednisone after diagnosis. Follow up for 0.5 ~ 5 years included 3 cases with receding disease and 8 cases with stable disease progression, of which 1 case died of septicemia and 2 cases had diabetes insipidus. The prognosis of single system disease children is better than that of multi-system disease children. Conclusion LCH of temporal bone involves a single system, and the first symptom of LCH is ear leakage and periauricular mass. Imaging examination is the main examination method of LCH in temporal bone, and it is necessary to differentiate LCH from infectious diseases or tumors of the ear, and the diagnosis depends mainly on pathological examination. The prognosis of patients with single system involvement of temporal LCH was better than that of patients with multiple system involvement.
【作者单位】： 华中科技大学同济医学院附属武汉儿童医院耳鼻咽喉头颈外科;
【分类号】：R764
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本文编号：2006557