Université Paris Cité, France; Department of Ophthalmology, Hôpital Lariboisière, AP-HP, Paris, France; Ophthalmic Center of Imaging and Laser. Paris, France.
Sorbonne Université, Institut de la vision, INSERM, CNRS, Paris, France; Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts, National Rare Disease Center REFERET, Paris, France.
Prog Retin Eye Res. 2022 Nov;91:101092. doi: 10.1016/j.preteyeres.2022.101092. Epub 2022 Aug 1.
Besides cystoid macular edema due to a blood-retinal barrier breakdown, another type of macular cystoid spaces referred to as non-vasogenic cystoid maculopathies (NVCM) may be detected on optical coherence tomography but not on fluorescein angiography. Various causes may disrupt retinal cell cohesion or impair retinal pigment epithelium (RPE) and Müller cell functions in the maintenance of retinal dehydration, resulting in cystoid spaces formation. Tractional causes include vitreomacular traction, epiretinal membranes and myopic foveoschisis. Surgical treatment does not always allow cystoid space resorption. In inherited retinal dystrophies, cystoid spaces may be part of the disease as in X-linked retinoschisis or enhanced S-cone syndrome, or occur occasionally as in bestrophinopathies, retinitis pigmentosa and allied diseases, congenital microphthalmia, choroideremia, gyrate atrophy and Bietti crystalline dystrophy. In macular telangiectasia type 2, cystoid spaces and cavitations do not depend on the fluid leakage from telangiectasia. Various causes affecting RPE function may result in NVCM such as chronic central serous chorioretinopathy and paraneoplastic syndromes. Non-exudative age macular degeneration may also be complicated by intraretinal cystoid spaces in the absence of fluorescein leakage. In these diseases, cystoid spaces occur in a context of retinal cell loss. Various causes of optic atrophy, including open-angle glaucoma, result in microcystoid spaces in the inner nuclear layer due to a retrograde transsynaptic degeneration. Lastly, drug toxicity may also induce cystoid maculopathy. Identifying NVCM on multimodal imaging, including fluorescein angiography if needed, allows guiding the diagnosis of the causative disease and choosing adequate treatment when available.
除了血视网膜屏障破裂引起的囊样黄斑水肿外，另一种类型的黄斑囊样空间，称为非血管源性囊样黄斑病变 (NVCM)，可能在光学相干断层扫描 (OCT) 上检测到，但在荧光素血管造影 (FA) 上检测不到。各种原因可破坏视网膜细胞的黏附性，或损害视网膜色素上皮 (RPE) 和 Müller 细胞在维持视网膜脱水过程中的功能，导致囊样空间的形成。牵拉性原因包括玻璃体内黄斑牵引、视网膜内膜和近视性黄斑劈裂。手术治疗并不总是能使囊样空间吸收。在遗传性视网膜营养不良中，囊样空间可能是疾病的一部分，如 X 连锁性视网膜劈裂症或增强 S 锥细胞综合征，或偶尔发生，如 Bestrophinopathy、色素性视网膜炎和相关疾病、先天性小眼球症、脉络膜视网膜变性和回旋性萎缩等。在 2 型黄斑毛细血管扩张症中，囊样空间和空洞不依赖于毛细血管扩张的液体渗漏。各种影响 RPE 功能的原因都可能导致 NVCM，如慢性中心性浆液性脉络膜视网膜病变和副肿瘤综合征。非渗出性年龄相关性黄斑变性也可能伴有视网膜内囊样空间，而没有 FA 渗漏。在这些疾病中，囊样空间发生在视网膜细胞丢失的背景下。各种原因引起的视神经萎缩，包括开角型青光眼，由于逆行性跨突触变性，在内核层出现微囊样空间。最后，药物毒性也可能导致囊样黄斑病变。通过多模态成像（包括必要时的 FA）识别 NVCM，有助于指导病因诊断，并在有条件时选择适当的治疗方法。
