Carbonic anhydrase inhibitor with topical NSAID therapy to manage cystoid macular edema in a case of gyrate atrophy



Gyrate atrophy of the choroid and retina (GACR) is a rare chorioretinal dystrophy characterized by a deficiency of the enzyme ornithine aminotransferase, inherited in an autosomal recessive pattern.

Case report

We report a case of a 17-year-old girl with GACR, for whom the level of serum ornithine had been reduced by an arginine-restricted diet. The patient was responsive to an association of topical nonsteroidal anti-inflammatory drugs (NSAIDs) and a carbonic anhydrase inhibitor (CAI) to reduce cystoid macular edema (CME).


The efficacy of topical NSAIDs and systemic CAI association indicates that the imbalance in the distribution of retinal pigment epithelium membrane-bound carbonic anhydrase could play a major role in CME pathogenesis in GACR. To our knowledge, this is the first case of therapy with CAI treatment for GACR-related CME.

Eur J Ophthalmol 2017; 27(6): e179 - e183

Article Type: CASE REPORT



Elena Piozzi, Salvatore Alessi, Silvia Santambrogio, Giovanni Cillino, Marco Mazza, Aissa Iggui, Salvatore Cillino

Article History


Financial support: No financial support was received for this submission.
Conflict of interest: None of the authors has conflict of interest with this submission.

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  • Pediatric Ophthalmology Unit, Niguarda Ca’Granda Metropolitan Hospital, Milan - Italy
  • Ophthalmology Section, Department of Experimental Biomedicine and Clinical Neuroscience, University of Palermo, Palermo - Italy

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