Investigating the role of vortex vein anastomoses in central serous chorioretinopathy (CSC)
Final Report Abstract
Central serous chorioretinopathy (CSC) is the fourth most common maculopathy worldwide and can lead to irreversible vision loss. Because CSC primarily affects young men in the working age range of 20 to 60 years and can lead to severe visual impairment if left untreated, the disease has a significant socioeconomic impact. Its hallmark feature is an accumulation of subretinal fluid (SRF) underneath the retina. In most cases, the SRF resolves without any treatment during the first 3 to 6 months after disease onset (“acute CSC”). In about 20% of cases, however, SRF persists for more than 6 months and about 50% of cases experience recurrences of SRF, both of which can lead to irreversible damage to photoreceptors and thus a loss of visual acuity (“chronic CSC”). The pathogenesis of the disease is, however, still not fully understood. When studying ultra-widefield indocyanine green (UWF ICGA) angiographies of patients suffering from CSC, several authors observed large choroidal vessels in general and large anastomoses between the eye’s vortex vein drainage systems in particular that were previously not noted in healthy patients. One previous report on UWF ICGA imaging reported dilated choroidal vessels and enlarged vortex vein ampullae in 83% of eyes with CSC. Two further studies found larger choroidal anastomoses between the eye’s vortex veins in CSC patients compared to healthy controls based on swept source-optical coherence tomography imaging. It was also hypothesised that large subfoveal choroidal vascular anastomoses may explain the thickening of the choroid seen in CSC. This led to the introduction of the idea of a “venous overload choroidopathy”, which proposes a venous stasis and the consequent increase in venous vessel diameter as one of causes of CSC. As part of this project, we collected the largest cohort of UWF ICGA imaging on CSC patients so far, which we refer to as the CERTAIN (Central sErous choRioreTinopAthy wIde-field aNgiography) study. In the first study report on 178 eyes of 91 patients, we found that signs of venous overload choroidopathy are present in the vast majority of CSC patients. Signs of venous overload were found both in affected as well as in clinically unaffected, fellow eyes. The only sign that was observed in all CSC patients, however, was choroidal vascular hyperpermeability (CVH). In a subsequent study report we found that CVH patterns, which we termed focal indistinct signs of choroidal hyperpermeability (FISH) and diffuse indistinct signs of choroidal hyperpermeability (DISH) correlated with many known signs of CSC disease chronicity. We are currently investigating how these patterns relate to (treatment) outcomes of CSC patients and how they could improve disease management by a more tailored, individualized treatment approach.
Publications
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SINGLE-SESSION BILATERAL REDUCED-SETTINGS PHOTODYNAMIC THERAPY FOR BILATERAL CHRONIC CENTRAL SEROUS CHORIORETINOPATHY. Retina, 43(8), 1356-1363.
Pauleikhoff, Laurenz J.B.; Diederen, Roselie M.H.; Feenstra, Helena M.A.; Schlingemann, Reinier O.; van Dijk Elon, H.C. & Boon, Camiel J.F.
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Choroidal Vascular Changes on Ultrawidefield Indocyanine Green Angiography in Central Serous Chorioretinopathy. Ophthalmology Retina, 8(3), 254-263.
Pauleikhoff, Laurenz J.B.; Diederen, Roselie M.H.; Chang-Wolf, Jennifer M.; Moll, Annette C.; Schlingemann, Reinier O.; van Dijk Elon, H.C. & Boon, Camiel J.F.
