Hankins Mark, Murtagh Ryan, Margo Curtis E, Bajric Jasmina, Agazzi Siviero, Malafronte Patrick J, Drucker Mitch
Departments of Ophthalmology, the University of South Florida, USA.
Departments of Radiology, the University of South Florida, USA.
Am J Ophthalmol Case Rep. 2019 Jul 13;15:100517. doi: 10.1016/j.ajoc.2019.100517. eCollection 2019 Sep.
To familiarize clinicians with the clinical and magnetic resonance imaging (MRI) features of a small orbital apex lymphaticovenous malformation that resulted in blindness and evaded timely clinical diagnosis.
A 68-year-old man presented with severe vision loss due to a 9 mm mass at the apex of the orbit above the optic nerve. When surgically removed 4 years later, the lesion was characterized by vascular spaces of varying size. Larger ones were filled with fibrin and organized thrombi. Stromal septa of endothelial-lined cavernous spaces were partially necrotic and there was evidence of remote hemorrhage. Some endothelial cells expressed D2-40, a marker of lymphatic channels.
Unless a high index of suspicion is maintained for a lymphaticovenous malformation the clinical diagnosis of a small but vision-threatening lesion can be overlooked.
使临床医生熟悉一种导致失明且未得到及时临床诊断的小眼眶尖部淋巴管静脉畸形的临床及磁共振成像（MRI）特征。
一名68岁男性因视神经上方眼眶尖部的一个9毫米肿物导致严重视力丧失。4年后手术切除时，病变的特征为大小不一的血管腔隙。较大的腔隙内充满纤维蛋白和机化血栓。内衬内皮细胞的海绵状腔隙的间质间隔部分坏死，并有陈旧性出血的迹象。一些内皮细胞表达D2-40，这是淋巴管的标志物。
除非对淋巴管静脉畸形保持高度怀疑指数，否则一个小但威胁视力的病变的临床诊断可能会被忽视。
