Nickel Miriam, Löbel Ulrike, Holst Brigitte, Kammler Gertrud, Matschke Jakob, Schulz Angela, Kohlschütter Alfried
Department of Pediatrics, Clinic for Degenerative Brain Diseases, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Department of Diagnostic and Interventional Neuroradiology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Neuropediatrics. 2014 Oct;45(5):321-4. doi: 10.1055/s-0034-1372303. Epub 2014 Apr 3.
A 4-year-old girl gradually lost her vision to become practically blind at the age of 10 years. Examinations at several medical centers had been unable to establish an etiology. Traditional investigation using cerebral magnetic resonance imaging (MRI) initially showed normal results; however, later on it showed progressive atrophy of both optical nerves without recognizable cause. Subsequently, MRI including adequate orbital sequences, contrast-enhanced sequences, and fat suppression demonstrated bilateral primary optic nerve sheath meningioma, a rare but treatable tumor of childhood. The patient underwent neurosurgery and to date retains minimal vision. Adequate neuroradiological investigation of unexplained optic atrophy is advocated.
一名4岁女孩的视力逐渐丧失，到10岁时几乎完全失明。多家医疗中心的检查均未能确定病因。最初使用脑磁共振成像（MRI）进行的传统检查结果正常；然而，后来显示双侧视神经进行性萎缩，原因不明。随后，包括适当眼眶序列、增强扫描序列和脂肪抑制的MRI显示为双侧原发性视神经鞘膜瘤，这是一种罕见但可治疗的儿童肿瘤。该患者接受了神经外科手术，至今仅保留了极少的视力。提倡对不明原因的视神经萎缩进行充分的神经放射学检查。
