Purpose: 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).Conclusions: 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.
Piozzi E., Alessi S., Santambrogio S., Cillino G., Mazza M., Iggui A., et al. (2017). Carbonic anhydrase inhibitor with topical NSAID therapy to manage cystoid macular edema in a case of gyrate atrophy. EUROPEAN JOURNAL OF OPHTHALMOLOGY, 27(6), e179-e183 [10.5301/ejo.5001010].
Carbonic anhydrase inhibitor with topical NSAID therapy to manage cystoid macular edema in a case of gyrate atrophy
Cillino G.;Mazza M.;Iggui A.;Cillino S.
2017-01-01
Abstract
Purpose: 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).Conclusions: 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.File | Dimensione | Formato | |
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