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Abstract：
Objective:To investigate the clinical features of craniofacial Langerhans cell histiocytosis（LCH） in children. Method:The clinical data of 11 children with craniofacial LCH confirmed by surgical histopathology were analyzed retrospectively, and their clinical characteristics were compared. Result:Eleven children with craniofacial LCH had a history of misdiagnosis and mistreatment. There were 10 cases aged 1-4 years. 9 cases of LCH occurred in the temporal bone with otorrhea and moderate to severe hearing loss, while 6 cases were binaural involvement. CT of the primary lesions in 7 children showed obvious extensive bone destruction. 2 cases died（both with multiple dangerous organs involved） and 8 cases survived. Conclusion:The craniofacial LCH in children mainly occurs in children under 4 years old, Most of the temporal bone LCH is involved in both ears with otorrhea and severe hearing loss. There is a high rate of misdiagnosis in clinical work due to lack of specific clinical symptoms. Imaging examinations and systemic examinations have important diagnostic value. The cases with dangerous organs involved have a higher mortality rate, while chemotherapy has better clinical effect for children with localized lesions.
目的：探讨儿童颅面部朗格汉斯细胞组织细胞增生症（LCH）的临床特点。 方法：回顾性分析11例经手术组织病理证实为颅面部LCH患儿的临床资料，分析其临床特点。 结果：11例颅面部LCH患儿均有门诊误诊误治病史。10例发病年龄为1～4岁。9例发生于颞骨的LCH均有耳漏及中度至极重度的听力损失，6例为双耳受累。7例患儿的原发灶部位的局部CT有明显的较大范围骨质破坏。死亡2例（均为多发危险器官受累患儿），存活8例，失访1例。 结论：儿童颅面部LCH主要发生于4岁以内儿童，颞骨LCH多为双耳受累，多有耳漏及严重的听力损失，由于局部的临床症状多不具有特异性，临床工作中有较高的误诊率，影像学检查及全身系统检查具有重要的诊断价值，危险器官受累患儿死亡率较高，化疗对病灶局限的患儿有较好的临床效果。.
目的：探讨儿童颅面部朗格汉斯细胞组织细胞增生症（LCH）的临床特点。 方法：回顾性分析11例经手术组织病理证实为颅面部LCH患儿的临床资料，分析其临床特点。 结果：11例颅面部LCH患儿均有门诊误诊误治病史。10例发病年龄为1～4岁。9例发生于颞骨的LCH均有耳漏及中度至极重度的听力损失，6例为双耳受累。7例患儿的原发灶部位的局部CT有明显的较大范围骨质破坏。死亡2例（均为多发危险器官受累患儿），存活8例，失访1例。 结论：儿童颅面部LCH主要发生于4岁以内儿童，颞骨LCH多为双耳受累，多有耳漏及严重的听力损失，由于局部的临床症状多不具有特异性，临床工作中有较高的误诊率，影像学检查及全身系统检查具有重要的诊断价值，危险器官受累患儿死亡率较高，化疗对病灶局限的患儿有较好的临床效果。.
摘要：
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