Karimi Saeed, Arabi Amir, Shahraki Toktam, Safi Sare
Ophthalmic Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Department of Ophthalmology, Torfeh Medical Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
J Ophthalmic Vis Res. 2020 Feb 2;15(1):78-94. doi: 10.18502/jovr.v15i1.5950. eCollection 2020 Jan-Mar.
Retinal hemangioblastoma (also referred to as retinal capillary hemangioma) is a benign lesion originating from the endothelial and glial components of the neurosensory retina and optic nerve head. Historically known as a manifestation of the von Hippel-Lindau (VHL) disease, it can be seen as an isolated finding or in association with some rare ocular conditions. In addition to characteristic ophthalmoscopic features, results of numerous ancillary tests including angiography, ultrasound, optical coherence tomography, and genetic tests may support the diagnosis and differentiate it from similar conditions. Because of serious life-threatening complications of VHL disease, every ocular approach to retinal hemangioblastomas should be in relationship with additional multidisciplinary diagnostic and therapeutic efforts. In addition, any patient with actual or probable diagnosis of VHL disease should be screened for ocular involvement. Unfavorable visual loss can occur early, and ocular complications of VHL range from exudative retinopathy to tractional retinal detachment, neovascular glaucoma, and phthisis bulbi. Accordingly, various treatment methods have been tested with overall acceptable responses, including photocoagulation, cryotherapy, photodynamic therapy, plaque radiotherapy, vitrectomy, and more novel intravitreal injections of anti-vascular endothelial growth factors and propranolol.
视网膜血管瘤（也称为视网膜毛细血管瘤）是一种起源于神经感觉视网膜和视神经乳头内皮及神经胶质成分的良性病变。它在历史上被认为是冯·希佩尔-林道（VHL）病的一种表现，可表现为孤立性病变，或与某些罕见的眼部疾病相关。除了典型的检眼镜特征外，包括血管造影、超声、光学相干断层扫描和基因检测在内的众多辅助检查结果，可能有助于诊断并将其与类似疾病相鉴别。由于VHL病会引发严重的危及生命的并发症，因此对于视网膜血管瘤的每一种眼科治疗方法，都应结合多学科的诊断和治疗措施。此外，任何实际或可能诊断为VHL病的患者都应接受眼部受累情况的筛查。早期可能出现严重的视力丧失，VHL病的眼部并发症包括渗出性视网膜病变、牵拉性视网膜脱离、新生血管性青光眼和眼球痨。因此，人们已经尝试了各种治疗方法，总体反应尚可，包括光凝、冷冻疗法、光动力疗法、敷贴放射疗法、玻璃体切除术，以及更新颖的玻璃体内注射抗血管内皮生长因子和普萘洛尔。
