Efficacy of Sustained Topical Dorzolamide Therapy for Cystic Macular Lesions in Patients With X-Linked Retinoschisis

Abstract

To determine the efficacy of sustained topical therapy with dorzolamide hydrochloride, 2%, on visual acuity and cystic macular lesions in patients with juvenile X-linked retinoschisis (XLRS). Design: Retrospective analysis. Setting: University hospital, tertiary care referral center. Patients: Twenty-nine eyes of 15 patients with XLRS receiving treatment with the topical dorzolamide formulation for 4 to 41 months were enrolled. Main Outcome Measures: Changes in visual acuity, cystic macular lesions, and central foveal zone thickness on optical coherence tomography during follow-up for the duration of treatment. Results: Among the 15 patients with XLRS, 20 eyes (69%) of 11 patients showed a positive response to

Efficacy of topical dorzolamide for treatment of cystic macular lesions in a patient with enhanced S-cone syndrome

“…This finding is similar to two previous studies that demonstrated the beneficial effect of CAIs in one patient with Enhanced S-cone syndrome in each study [6,7]. CAIs have been shown to be effective for the improvement of cystic macular changes in patients with other retinal diseases such as retinitis pigmentosa [8,9], uveitis [21], X-linked retinoschisis [10,22], and for patients with epiretinal membranes [23]. Improvement in our case included a marked decrease in central macular thickness on spectral-domain OCT exam and an increase in retinal sensitivity on microperimetry testing.…”

Efficacy of topical dorzolamide for treatment of cystic macular lesions in a patient with enhanced S-cone syndrome

“…This finding is similar to two previous studies that demonstrated the beneficial effect of CAIs in one patient with Enhanced S-cone syndrome in each study [6,7]. CAIs have been shown to be effective for the improvement of cystic macular changes in patients with other retinal diseases such as retinitis pigmentosa [8,9], uveitis [21], X-linked retinoschisis [10,22], and for patients with epiretinal membranes [23]. Improvement in our case included a marked decrease in central macular thickness on spectral-domain OCT exam and an increase in retinal sensitivity on microperimetry testing.…”

X-Linked Retinoschisis

“…Some studies have reported macular atrophy in patients in their fourth decade of life and clinical descriptions of macular schisis flattening with age without a clear mechanism for these changes. 14,22 In our study, we found the mean age of patients with macular atrophy was 46.5 years (range, 19e66 years). In contrast, patients with foveal schisis were younger (mean, 22.1 years; range, 3e56 years).…”

Molecular characterization of a rare phenotype of X-linked retinoschisis with angle-closure glaucoma

“…Topical carbonic anhydrase inhibitors like dorzolamide 2% have been shown to be beneficial in cases of XLRS, causing significant reduction in foveal cystic cavities. [13] Our proband, after 6 weeks of dorzolamide usage, showed significant reduction in the central macular thickness of both eyes (RE 39% and LE 29%), which sustained until last follow-up.…”

Efficacy of topical dorzolamide for treatment of cystic macular lesions in a patient with enhanced S-cone syndrome

“…This finding is similar to two previous studies that demonstrated the beneficial effect of CAIs in one patient with Enhanced S-cone syndrome in each study [6,7]. CAIs have been shown to be effective for the improvement of cystic macular changes in patients with other retinal diseases such as retinitis pigmentosa [8,9], uveitis [21], X-linked retinoschisis [10,22], and for patients with epiretinal membranes [23]. Improvement in our case included a marked decrease in central macular thickness on spectral-domain OCT exam and an increase in retinal sensitivity on microperimetry testing.…”

X-Linked Retinoschisis

“…Some studies have reported macular atrophy in patients in their fourth decade of life and clinical descriptions of macular schisis flattening with age without a clear mechanism for these changes. 14,22 In our study, we found the mean age of patients with macular atrophy was 46.5 years (range, 19e66 years). In contrast, patients with foveal schisis were younger (mean, 22.1 years; range, 3e56 years).…”

Molecular characterization of a rare phenotype of X-linked retinoschisis with angle-closure glaucoma

“…Topical carbonic anhydrase inhibitors like dorzolamide 2% have been shown to be beneficial in cases of XLRS, causing significant reduction in foveal cystic cavities. [13] Our proband, after 6 weeks of dorzolamide usage, showed significant reduction in the central macular thickness of both eyes (RE 39% and LE 29%), which sustained until last follow-up.…”

Efficacy of topical dorzolamide for treatment of cystic macular lesions in a patient with enhanced S-cone syndrome

“…This finding is similar to two previous studies that demonstrated the beneficial effect of CAIs in one patient with Enhanced S-cone syndrome in each study [6,7]. CAIs have been shown to be effective for the improvement of cystic macular changes in patients with other retinal diseases such as retinitis pigmentosa [8,9], uveitis [21], X-linked retinoschisis [10,22], and for patients with epiretinal membranes [23]. Improvement in our case included a marked decrease in central macular thickness on spectral-domain OCT exam and an increase in retinal sensitivity on microperimetry testing.…”

X-Linked Retinoschisis

“…Some studies have reported macular atrophy in patients in their fourth decade of life and clinical descriptions of macular schisis flattening with age without a clear mechanism for these changes. 14,22 In our study, we found the mean age of patients with macular atrophy was 46.5 years (range, 19e66 years). In contrast, patients with foveal schisis were younger (mean, 22.1 years; range, 3e56 years).…”

Molecular characterization of a rare phenotype of X-linked retinoschisis with angle-closure glaucoma

“…Topical carbonic anhydrase inhibitors like dorzolamide 2% have been shown to be beneficial in cases of XLRS, causing significant reduction in foveal cystic cavities. [13] Our proband, after 6 weeks of dorzolamide usage, showed significant reduction in the central macular thickness of both eyes (RE 39% and LE 29%), which sustained until last follow-up.…”