本文選題：兒童 + 顳骨��； 參考：《聽力學及言語疾病雜志》2018年01期

【摘要】：目的探討兒童顳骨朗格漢斯組織細胞增生癥(langerhans cell histiocytosis,LCH)的臨床特點。方法回顧性分析武漢兒童醫(yī)院耳鼻咽喉科2011年6月~2016年12月期間收治并經(jīng)病理確診的14例顳骨LCH患兒的臨床資料,分析臨床表現(xiàn)、影像學檢查、診斷、治療及預后等特點。結(jié)果 14例顳骨LCH患兒中,女5例,男9例,年齡4個月~3歲10個月,2歲以下者12例(85.71%);8例為單系統(tǒng)病變(3例為顳骨單灶型,5例為多灶型),6例為多系統(tǒng)病變;病變大多累及顳骨鱗部、外耳道及乳突,其次為鼓室及鼓竇,較少累及聽小骨及內(nèi)耳;主要臨床表現(xiàn)為耳顳部腫塊、耳漏、聽力下降、外耳道肉芽等。14例患兒中8例行外耳道深部病損切除術(shù),3例行顳部腫塊切除術(shù),1例行額部病損切除活檢術(shù),2例行四肢骨病損清除活檢術(shù)明確病理診斷,所有患兒確診后均加用化療[長春花堿(VBL)+潑尼松],隨訪0.5~5年,3例疾病消退,8例疾病穩(wěn)定,3例疾病進展,其中1例合并敗血癥死亡,2例出現(xiàn)尿崩癥。單系統(tǒng)病變患兒預后優(yōu)于多系統(tǒng)病變患兒。結(jié)論顳骨LCH多累及單系統(tǒng),多以耳漏及耳部包塊為首發(fā)癥狀,影像學檢查是顳骨LCH的主要檢查手段;需與耳部感染性疾病或腫瘤相鑒別,確診主要靠病理學檢查;顳骨LCH單系統(tǒng)受累患兒預后好于多系統(tǒng)受累患兒。
[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.
【作者單位】： 華中科技大學同濟醫(yī)學院附屬武漢兒童醫(yī)院耳鼻咽喉頭頸外科;
【分類號】：R764
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本文編號：2006557