What is it about?

Intraretinal cystoid spaces (IRCS) are fluid-filled spaces seen in some retinal dystrophies and often treated with carbonic anhydrase inhibitors. We have shown that in 17% of our cohort, an unexpected bilateral improvement in the IRCS occurred after discontinuation of therapy.

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Why is it important?

We are still unsure of the physiological mechanism from which carbonic anhydrase inhibitors reduce the intraretinal cysts that tend to form in patients with inherited retinal dystrophies. These cysts distinguish themselves from the typical cystoid macular edema (that occurs in the natural course of other dystrophies, like retinitis pigmentosa) in that it does not leak in fluorescence angiography. Although the pathophysiology of these phenomena is unclear, stopping treatment may be an option in patients who cease to improve or get worse on treatment.


Although we are in the era of great advances in gene therapy, particularly for inherited retinal dystrophies, we are still not quite there in terms of treating these cysts. This study helps in the sense that it allows us to understand how carbonic anhydrase inhibitors might work and also increase our awareness of unexpected responses in patients under long-term treatments.

Thales Antonio Cabral de Guimaraes

Read the Original

This page is a summary of: Paradoxical response to carbonic anhydrase inhibitors in patients with intraretinal cystoid spaces, Ophthalmic Genetics, May 2019, Taylor & Francis,
DOI: 10.1080/13816810.2019.1622021.
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