Department of Ophthalmology, Feinberg School of Medicine, Northwestern University, Chicago, IL 60208, USA.
School of Medicine, Vita-Salute San Raffaele University, 20132 Milan, Italy.
Medicina (Kaunas). 2021 Feb 27;57(3):213. doi: 10.3390/medicina57030213.
Best macular dystrophy (BMD) is an autosomal dominant macular dystrophy of childhood onset characterized by bilateral and symmetric vitelliform lesions. Several stages of disease have been well-described in the literature. Choroidal neovascularization (CNV) has traditionally been considered a hallmark of end-stage disease, and anti-vascular endothelial growth factor (anti-VEGF) agents have been used to improve visual prognosis. While CNV was historically detected with fluorescein angiography, optical coherence tomography angiography (OCTA) has recently been employed as a novel mechanism for identifying CNV in BMD. In this case series, we discuss our institutional experience with using OCTA to detect CNV in BMD and contextualize this experience within the broader emerging literature. While OCTA allows for the identification of CNV in less severe stages of BMD, the management of this CNV remains uncertain.
最佳型黄斑营养不良(Best macular dystrophy,BMD)是一种常染色体显性遗传的儿童黄斑营养不良,其特征为双侧对称的玻璃膜疣病变。该疾病的几个阶段在文献中已有详细描述。脉络膜新生血管(choroidal neovascularization,CNV)传统上被认为是疾病晚期的标志,抗血管内皮生长因子(vascular endothelial growth factor,VEGF)药物已被用于改善视觉预后。虽然 CNV 过去是通过荧光素血管造影术检测到的,但光学相干断层扫描血管造影术(optical coherence tomography angiography,OCTA)最近已被用作一种新的机制来识别 BMD 中的 CNV。在本病例系列中,我们讨论了我们机构使用 OCTA 检测 BMD 中 CNV 的经验,并将这一经验置于更广泛的新兴文献背景下进行讨论。虽然 OCTA 允许在 BMD 的较不严重阶段识别 CNV,但对这种 CNV 的处理仍然存在不确定性。