Azad Amee D, Sears Connie M, Hwang Peter H, Mohyeldin Ahmed, Fernandez-Miranda Juan, Kossler Andrea L
Stanford University School of Medicine, Stanford University, Palo Alto, CA, USA.
Department of Ophthalmology, Byers Eye Institute, Stanford University, Palo Alto, CA, USA.
Am J Ophthalmol Case Rep. 2021 Jan 21;21:101020. doi: 10.1016/j.ajoc.2021.101020. eCollection 2021 Mar.
We present a unique case of an orbital intraconal cavernous venous malformation that extended along the trigeminal nerve to the pterygopalatine and middle cranial fossa. Our aim is to describe an atypical presentation of this common orbital vascular mass.
A 57-year-old female presented with right eye proptosis. Orbital magnetic resonance imaging demonstrated a lobulated contrast-enhancing mass involving the right intraconal orbital space, pterygopalatine fossa, and right middle cranial fossa, radiographically presumed to be a schwannoma. Intraoperative and histopathologic evaluation confirmed a cavernous venous malformation that extended along the trigeminal nerve. The mass, including its attachments to the cranial nerves and dura, was successfully removed via a combined transorbital and endoscopic endonasal approach. The patient recovered well with 20/20 vision, full extraocular movements, and resolution of proptosis.
This a rare presentation of an orbital cavernous venous malformation not previously described. Cavernous venous malformations typically present as ovoid well-circumscribed lesions; however, they can also extend outside the orbit along the path of cranial nerves, as was observed in this case. These types of lesions should be included in the differential diagnosis of masses arising from or extending along cranial nerves, even when involving the orbit.
我们报告一例罕见的眶内圆锥海绵状静脉畸形病例，该畸形沿三叉神经延伸至翼腭窝和中颅窝。我们的目的是描述这种常见眶部血管性肿物的非典型表现。
一名57岁女性因右眼突出就诊。眼眶磁共振成像显示一个分叶状、有强化的肿物，累及右侧眶内圆锥间隙、翼腭窝和右侧中颅窝，影像学上推测为神经鞘瘤。术中及组织病理学评估证实为沿三叉神经延伸的海绵状静脉畸形。通过经眶和内镜鼻内联合入路成功切除了肿物，包括其与颅神经和硬脑膜的附着部分。患者术后恢复良好，视力20/20，眼球运动完全正常，突眼症状消失。
这是一例此前未被描述过的眶部海绵状静脉畸形的罕见表现。海绵状静脉畸形通常表现为卵圆形、边界清晰的病变；然而，它们也可如本例所示沿颅神经路径延伸至眶外。即使累及眼眶，这类病变也应列入源于颅神经或沿颅神经延伸的肿物的鉴别诊断中。
