Mano Yui, Kumabe Toshihiro, Saito Ryuta, Watanabe Mika, Tominaga Teiji
Department of Neurosurgery, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan.
Childs Nerv Syst. 2014 Oct;30(10):1753-8. doi: 10.1007/s00381-014-2465-9. Epub 2014 Jul 3.
Optic pathway cavernous malformations (CMs) are extremely rare, accounting for less than 1 % of all intracranial CMs. We report a case of optic pathway CM mimicking optic glioma because the initial magnetic resonance (MR) images did not disclose hemorrhagic findings such as popcorn-like lesion or hemosiderin ring.
A 20-year-old woman presented with subacute left visual acuity loss and visual field defect and was referred to our hospital. Initial MR imaging findings were suggestive of optic glioma. Second MR imaging demonstrated hemorrhagic findings, but the hemorrhage was considered to be intratumoral hemorrhage from left optic pilocytic astrocytoma. She underwent radiochemotherapy and intravenous administration of corticosteroids but her symptoms deteriorated. Third and fourth MR imaging revealed enlargement of the hematoma. Therefore, the radiation therapy was interrupted at a delivered dose of 16.2 Gy, and craniotomy was performed to preserve residual right visual field. The lesion was totally removed and the histological diagnosis was CM. Her right visual field was preserved, but not improved.
The present case and previous cases suggest that optic pathway CMs sometimes do not initially manifest with signs of hemorrhage, so CMs should be included in the differential diagnosis of optic pathway lesions.
Histological confirmation with preparation for total resection should be considered for rapidly progressive cases even if the neuroimaging findings are compatible with optic glioma.
视路海绵状畸形(CMs)极为罕见,占所有颅内CMs的比例不到1%。我们报告一例视路CMs,其最初的磁共振(MR)图像未显示出如爆米花样病变或含铁血黄素环等出血表现,从而误诊为视神经胶质瘤。
一名20岁女性因亚急性左眼视力丧失和视野缺损转诊至我院。最初的MR成像结果提示为视神经胶质瘤。第二次MR成像显示有出血表现,但出血被认为是来自左侧视神经毛细胞型星形细胞瘤的瘤内出血。她接受了放化疗及静脉注射皮质类固醇,但症状仍恶化。第三次和第四次MR成像显示血肿增大。因此,放疗在给予16.2 Gy剂量时中断,并行开颅手术以保留残余的右侧视野。病变被完全切除,组织学诊断为CMs。她的右侧视野得以保留,但未改善。
本病例及之前的病例提示,视路CMs有时最初并不表现出出血迹象,因此在视路病变的鉴别诊断中应考虑到CMs。
即使神经影像学结果与视神经胶质瘤相符,对于快速进展的病例,也应考虑进行组织学确认并准备行全切除手术。