Manal Hajali1, Gerald A Fishman. 1. From the Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, Illinois.
Abstract
PURPOSE: To determine the efficacy of dorzolamide ophthalmic solution, a carbonic anhydrase inhibitor, for treating macular schisis in enhanced S-cone syndrome. METHODS: A 12-year-old white boy was diagnosed with enhanced S-cone syndrome based on the patient's history of night blindness, fundus examination, and electroretinogram testing that showed nondetectible rod function and atypical cone function consistent with an overabundance of blue cones. Time domain optical coherence tomography (OCT) baseline scans confirmed the presence of macular cysts, and the patient was started on dorzolamide ophthalmic drops twice a day. A Fourier domain-OCT unit was used to monitor any changes in the macular cysts. RESULTS: After 8 weeks of starting the eye drops, the Fourier domain OCT showed an improvement in the macular cysts of the right eye with notable but more moderate changes in the left. Visual acuity improved one line in the right eye compared with baseline. Ten weeks later, the patient showed a further improvement in macular cysts by OCT testing in both eyes, with visual acuity improvement of one line in the left eye, and sustained one line improvement in the right. CONCLUSION: The treatment of macular cysts using a topical carbonic anhydrase inhibitor twice a day can potentially reduce foveal cystic changes and possibly improve visual acuity in at least some patients with enhanced S-cone syndrome.
PURPOSE: To determine the efficacy of dorzolamide ophthalmic solution, a carbonic anhydrase inhibitor, for treating macular schisis in enhanced S-cone syndrome. METHODS: A 12-year-old white boy was diagnosed with enhanced S-cone syndrome based on the patient's history of night blindness, fundus examination, and electroretinogram testing that showed nondetectible rod function and atypical cone function consistent with an overabundance of blue cones. Time domain optical coherence tomography (OCT) baseline scans confirmed the presence of macular cysts, and the patient was started on dorzolamide ophthalmic drops twice a day. A Fourier domain-OCT unit was used to monitor any changes in the macular cysts. RESULTS: After 8 weeks of starting the eye drops, the Fourier domain OCT showed an improvement in the macular cysts of the right eye with notable but more moderate changes in the left. Visual acuity improved one line in the right eye compared with baseline. Ten weeks later, the patient showed a further improvement in macular cysts by OCT testing in both eyes, with visual acuity improvement of one line in the left eye, and sustained one line improvement in the right. CONCLUSION: The treatment of macular cysts using a topical carbonic anhydrase inhibitor twice a day can potentially reduce foveal cystic changes and possibly improve visual acuity in at least some patients with enhanced S-cone syndrome.