The following is the summary of “Non-vasogenic cystoid maculopathies” published in the November 2022 issue of Progress in retinal and eye research by Gaudric, et al.


Non-vasogenic cystoid maculopathies (NVCM) are another type of macular cystoid spaces that can be observed on optical coherence tomography but not on fluorescein angiography, similar to cystoid macular edema caused by a breakdown of the blood-retinal barrier. Cystoid gaps can occur in the retina due to a breakdown in retinal cell cohesion or impairment in the functions of the retinal pigment epithelium (RPE) and Müller cells involved in maintaining retinal dryness. Tractional causes include vitreomacular traction, epiretinal membranes, and myopic foveoschisis. Resorption of the cystoid space is not guaranteed even after surgery. 

Cystoid spaces may be present throughout the disease process, as in X-linked retinoschisis or increased S-cone syndrome, or seldom occur, as in bestrophinopathies, retinitis pigmentosa, and allied diseases, congenital microphthalmia, choroideremia, gyrate atrophy, and Bietti crystalline dystrophy. Cavitations and cystoid gaps in macular telangiectasia type 2 are independent of telangiectasia-related fluid leaking. Chronic central serous chorioretinopathy and paraneoplastic syndromes are two examples of conditions that can disrupt RPE function and lead to NVCM. The absence of fluorescein leaking does not rule out the possibility of intraretinal cystoid spaces in patients with non-exudative age-related macular degeneration. 

Cystoid spaces develop in the retina due to cell death caused by these conditions. In addition, microcystoid gaps in the inner nuclear layer occur from retrograde transsynaptic degeneration, seen in many forms of optic atrophy, such as open-angle glaucoma. Lastly, cystoid maculopathy can be brought on by medication poisoning. When accessible, guiding the diagnosis of the underlying condition and selecting the most appropriate treatment is made possible by identifying NVCM on multimodal imaging, including fluorescein angiography if necessary.

Source: sciencedirect.com/science/article/pii/S1350946222000520