儿童耳颞部郎格罕斯组织细胞增生症临床分析

Analysis of children Langerhans cell histiocytosis of temporal bone

目的探讨儿童郎格罕斯组织细胞增生症在耳颞部的临床表现、影像学特点,总结分析本病的诊断和治疗方法,减少误诊。方法回顾性分析本科经手术证实的两例病例,结合文献进行讨论。结果本病CT表现为边界不规则的溶骨性破坏,边缘无硬化。MRI表现为T1加权呈等或低信号,增强后明显强化;T2加权均呈高信号。影像学检查有利于诊断。结论当临床遇见儿童耳颞部病变时,尤其是3岁以下儿童伴有血沉加快者,慢性中耳炎或胆脂瘤的诊断应慎重。对于病变局限于颞骨者,手术或手术加低剂量放疗即可;伴有全身其他器官系统受累者,应采取综合治疗措施。

Objective To explore the clinical characteristics, imageology features of children Langerhans cell histlocytosls of temporal bone, to conclude the right way for diagnosis and therapy. Methods Retrospective study of two children that with surgically confirmed Langerhans cell histlocyto- sis of temporal bone, discussions combined with literatures reviewed. Results CT showed massive de- struction associated with soft tissue masses in the region of temporal bone. The lesions had irregular bor- der and without osteosclerosis . On MRI imaging, the lesions were is intense or hypo intense in T1Wiand enhancement with gadolinium, while hypo intense in T2WI. Conclusions If a child（especially under 3 years old）suffers from temporal bone disease with high erythrocyte sedimentation rate, the diagnosis of chronic obits media or cholesteatoma would be made carefully. Treatment methods depend on the lesions range. Surgery or surgery combined with low dosage radiotherapy applies on local lesions of temporal bone is enough; comprehensive therapy methods apply on patients whose many organs or systems are concerned.