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隐匿性莓状血管瘤:血管之谜——两例眼眶海绵状静脉畸形(OCVM)病例比较剖析

The Hidden Berry, a Vascular Mystery: Unveiling a Comparison Between Two Cases of Orbital Cavernous Venous Malformation (OCVM).

作者信息

Kong Xin Yi, Abdul Rahim Ummi Faradiana, W Md Kasim Wan Mariny

机构信息

Ophthalmology, Hospital Raja Permaisuri Bainun, Ipoh, MYS.

Ophthalmology, Hospital Serdang, Kajang, MYS.

出版信息

Cureus. 2025 Jun 11;17(6):e85765. doi: 10.7759/cureus.85765. eCollection 2025 Jun.

Abstract

Orbital cavernous venous malformations (OCVMs) are rare, benign vascular lesions found within the orbit, characterized by a cluster of dilated venous spaces surrounded by a fibrous capsule. These malformations can pose significant challenges in diagnosis and management due to their varied clinical presentations, ranging from subtle proptosis and visual disturbances to more severe complications such as orbital hemorrhage and nerve compression. The etiology of OCVMs remains largely unknown, though they are believed to be congenital in nature. Case 1 was a 31-year-old male patient who had experienced painless swelling and blurred vision in his left eye for 11 years, which gradually worsened after a stone struck his left eye. He presented with a painful, red left eye for five days. On presentation, there was a significant left eye exophthalmos with lagophthalmos. He was also treated for exogenous endophthalmitis due to a corneal ulcer. Computed tomography (CT) demonstrated a large multilobulated intraorbital soft tissue mass. Tumor debulking via a transconjunctival approach was done. Histopathology confirmed orbital cavernous venous malformation. His left eye vision remained poor due to the past infection and optic nerve compression. One month post operation, it was noted that his left eye had developed recurrent ocular cavernous venous malformation. Case 2 was a 42-year-old female patient who presented with painless swelling for three months, with insidious onset and double vision. There were no thyroid or constitutional symptoms. She had diplopia on left lateral gaze, levoelevation, and levoversion, with restriction of the extraocular muscles in the superotemporal, superonasal, and nasal directions. On examination, axial proptosis was noted, but there was no lagophthalmos. Anterior and posterior chamber findings were unremarkable. CT showed a well-defined extraconal soft tissue lesion at the medial aspect measuring 2.1cm x 1.4cm x 1.6cm. The patient underwent excisional biopsy via anterior orbitotomy through vertical lid split. Histopathology reported as orbital cavernous venous malformation. The patient is recovering well. The vital and functional prognosis with complete excision of OCVMs is generally excellent, but the complications from untreated proptosis can be devastating.

摘要

眼眶海绵状静脉畸形(OCVMs)是一种罕见的眼眶内良性血管病变,其特征是一簇扩张的静脉间隙被纤维性包膜包裹。由于其临床表现多样,从轻微的眼球突出和视觉障碍到更严重的并发症,如眼眶出血和神经压迫,这些畸形在诊断和治疗上可能带来重大挑战。尽管OCVMs被认为本质上是先天性的,但其病因在很大程度上仍不清楚。病例1是一名31岁男性患者,其左眼无痛性肿胀和视力模糊已有11年,在左眼被石块击中后逐渐加重。他因左眼疼痛、发红就诊5天。就诊时,左眼明显眼球突出伴眼睑闭合不全。他还因角膜溃疡接受了外源性眼内炎治疗。计算机断层扫描(CT)显示眼眶内有一个大的多叶状软组织肿块。通过经结膜入路进行了肿瘤减容手术。组织病理学证实为眼眶海绵状静脉畸形。由于既往感染和视神经受压,他的左眼视力仍然很差。术后1个月,发现他的左眼出现了复发性眼眶海绵状静脉畸形。病例2是一名42岁女性患者,出现无痛性肿胀3个月,起病隐匿,伴有复视。无甲状腺或全身性症状。她在向左外侧凝视、左眼上抬和左旋时出现复视,眼外肌在颞上、鼻上和鼻侧方向活动受限。检查时,发现有轴向眼球突出,但无眼睑闭合不全。前房和后房检查结果无异常。CT显示在内侧有一个边界清晰的眶锥外软组织病变。患者通过垂直睑裂经前路眼眶切开术接受了切除活检。组织病理学报告为眼眶海绵状静脉畸形。患者恢复良好。OCVMs完全切除后的生命和功能预后通常良好,但未经治疗的眼球突出引起的并发症可能是灾难性的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/96c1/12247629/31b8e7ab1278/cureus-0017-00000085765-i01.jpg

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