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Abstract：
Intraorbital meningiomas are rare tumors, making up less than 4% of all intraorbital tumors. Intraorbital meningiomas of childhood are curiosities with only few documented cases. We present the case of an 8‑month-old male infant, presenting with strabismus and nystagmus. Magnetic resonance imaging showed a long segment thickening of the optical nerve and an intraocular tumor. The tumor was suspicious for retinal dysplasia and enucleation of the eye was performed to exclude malignancy. Histological examination revealed a meningothelial meningioma (WHO grade I), extending along the optical nerve and into the eye accompanied by retinal dysplasia and epiretinal membranes. Meningiomas of childhood, retinal dysplasia, and epiretinal membranes are regularly associated with neurofibromatosis type 2. Subsequent genetic analysis led to the final diagnosis. This case documents a very unusual early beginning of a neurofibromatosis type 2.
摘要：
眶内脑膜瘤是罕见的肿瘤，占所有眶内肿瘤的不到4%。儿童的眶内脑膜瘤是好奇心，只有很少的文献记载。我们介绍了一个8个月大的男婴的案例，表现为斜视和眼球震颤。磁共振成像显示视神经长段增厚和眼内肿瘤。肿瘤怀疑为视网膜发育不良，并进行了眼球摘除以排除恶性肿瘤。组织学检查显示脑膜上皮脑膜瘤（WHOI级），沿着视神经延伸并进入眼睛，伴有视网膜发育不良和视网膜前膜。儿童脑膜瘤，视网膜发育不良,视网膜前膜经常与2型神经纤维瘤病相关。随后的遗传分析导致最终诊断。该病例记录了2型神经纤维瘤病的非常不寻常的早期开始。
