dorzolamide and Macular-Edema

Carbonic anhydrase inhibitors (CAI) are often used in the treatment of cystoid macular edema (CME) in retinitis pigmentosa (RP) patients. The aim of this meta-analysis is to gain a better understanding of the overall efficacy of CAI treatment.. Databases including PubMed, EMBASE, and Cochrane Library were searched to identify relevant studies. Eligible studies were clinical trials of patients with RP assigned topical or oral CAIs such as dorzolamide and acetazolamide. Changes in central macular thickness (CMT) by OCT in μm and best-corrected visual acuity (BCVA) in log MAR equivalents were extracted and results compared between baseline and after treatment.. 11 clinical reports were identified which included a total of 194 patients (358 eyes) available for analysis, with 59 patients (115 eyes) assigned oral CAI treatment and 135 patients (243 eyes) assigned topical CAI treatment. The combined results showed a significant reduction of macular edema, as calculated by baseline and final central macular thickness (CMT) based on OCT examination (46.02μm, 95%CI: -60.96, -31.08, I2 = 65%). However, the effect on visual acuity was inconsistent across studies.. Based on non randomized controlled clinical studies, RP patients with CME who were treated with CAIs had better anatomical outcomes, but the effect on visual acuity was contradictory across studies. Multicenter prospective randomized controlled trials would be ideal to definitively test its clinical efficacy in RP patients.

Topics: Acetazolamide; Administration, Oral; Administration, Topical; Carbonic Anhydrase Inhibitors; Clinical Trials as Topic; Female; Humans; Macular Edema; Male; Prospective Studies; Retinitis Pigmentosa; Sulfonamides; Thiophenes; Treatment Outcome; Visual Acuity

To evaluate the efficacy of three intravitreal bevacizumab (IVB) injections versus the same combined with 2% of topical dorzolamide in the treatment of diabetic macular edema (DME).. In this randomized double-masked clinical trial, 32 eyes of 16 treatment-naive patients with bilateral DME were enrolled. The eyes were randomly assigned to receive three monthly injections of IVB (1.25 mg) plus topical dorzolamide 2% twice daily or IVB (1.25 mg) plus topical artificial tear twice daily. Best-corrected visual acuity (BCVA) was the primary outcome of the study followed by the central macular thickness (CMT) and central macular volume (CMV) as the secondary outcomes.. Mean BCVA changes were insignificant in both groups. It changed from 0.21 ± 0.08 logMAR at baseline to 0.23 ± 0.09 (. This randomized contralateral clinical trial demonstrated that adjuvant topical dorzolamide with IVB injection had no additional effects on IVB in the treatment of DME over a three-month course. This trial is registered with the Iranian Registry of Clinical Trials under the registration code IRCT20131229015975N5.

Topics: Administration, Topical; Aged; Bevacizumab; Diabetic Retinopathy; Female; Humans; Intravitreal Injections; Iran; Macular Edema; Male; Middle Aged; Sulfonamides; Thiophenes

To compare the efficacy of fixed combinations of dorzolamide-timolol (FCDT) and brimonidine-timolol (FCBT) in patients with intraocular pressure (IOP) elevations after intravitreal triamcinolone acetonide (IVTA) injections.. This was a prospective, randomized, open-label study. Patients who received IVTA injections due to diffuse diabetic macular edema and who had an IOP of 24 mm Hg or higher after IVTA treatment were included. They were randomized to receive either FCBT or FCDT twice daily. Follow-up visits were scheduled on week 4 and 12 weeks after starting the study medication. At all follow-up visits, IOP was measured with Goldmann applanation tonometry. The primary outcome measure was mean IOP, the secondary outcome was reduction in mean IOP at 4 and 12 weeks compared with postinjection values.. Sixty patients were randomized in 1:1 ratio. The FCBT and FCDT groups were similar in terms of age, sex, and preinjection IOP (P>0.05 for all). Mean postinjection IOP was 31.95±7.39 and 29.83±5.17 mm Hg in FCBT and FCDT groups, respectively (P=0.239). After 4 weeks, mean IOP was 17.05±3.61 mm Hg in FCBT and 18.93±3.30 mm Hg in FCDT groups (P=0.063). After 12 weeks, mean IOP in the FCBT and FCDT study groups was 16.35±2.70 and 18.43±2.82 mm Hg, respectively (P=0.012). Both fixed combinations significantly reduced IOP in comparison with the postinjection values (P<0.05). Mean reduction in IOP after 4 weeks were 14.90±7.28 mm Hg in FCBT and 10.90±4.83 mm Hg in FCDT groups (P=0.024); after 12 weeks, these values were 15.60±7.77 and 11.40±5.89 mm Hg in FCBT and FCDT groups, respectively (P=0.035).. Both FCBT and FCDT are effective in controlling IOP elevations after IVTA injections. The results of this study suggest that FCBT is superior to FCDT in reducing IOP and provides better IOP control after IVTA injections.

Topics: Aged; Antihypertensive Agents; Brimonidine Tartrate; Diabetic Retinopathy; Drug Combinations; Female; Glucocorticoids; Humans; Intraocular Pressure; Intravitreal Injections; Macular Edema; Male; Middle Aged; Ocular Hypertension; Prospective Studies; Quinoxalines; Sulfonamides; Thiophenes; Timolol; Tonometry, Ocular; Triamcinolone Acetonide

To investigate the topical effect of dorzolamide versus ketorolac on retinitis pigmentosa (RP) and Usher's syndrome (US) macular edema.. Prospective, randomized and interventional study. A total of 28 eyes of 18 patients were included. Five eyes had US, 23 had RP. Fifteen eyes were allocated to ketorolac tromethamine 0.5% (4 drops daily regimen) and 13 eyes to dorzolamide hydrochloride 2% (3 drops daily regimen) treatment groups. Snellen's best-corrected visual acuity (BCVA), foveal thickness (FT) and foveal zone thickness (FZT) measured by Stratus® optical coherence tomography (OCT) were evaluated at baseline, 1, 3, 6 and 12 months after treatment.. Patients assigned to ketorolac had a baseline BCVA of 0.37 ± 0.17 logMAR which improved at the end of 1 year to 0.28 ± 0.16 (p = 0.02). Three eyes (20%) of 2 patients improved by 7 letters or more. Mean FT and FZT did not change significantly during the study follow-up. After 1 year of treatment, 4 eyes (27%) of 3 patients showed an improvement of at least 16% of FT and 11% of FZT. Patients assigned to dorzolamide had a baseline BCVA of 0.48 ± 0.34 logMAR which improved in the first 6 months (0.40 ± 0.30; p = 0.01), with a decrease at 1 year (0.42 ± 0.27; p = 0.20). Seven eyes (54%) of 5 patients had an improvement of 7 letters or more. Mean FT and FZT did not change significantly either. After 1 year of treatment, 3 eyes (23%) of 2 patients showed an improvement of at least 16% on FT and 11% on FZT.. RESULTS suggest that dorzolamide and ketorolac might improve visual acuity and therefore be of interest in selected cases. No relationship between retinal thickness fluctuation and visual acuity was found. Sample size was a limitation to the study.

Topics: Administration, Topical; Adult; Aged; Anti-Inflammatory Agents, Non-Steroidal; Carbonic Anhydrase Inhibitors; Female; Fovea Centralis; Humans; Ketorolac Tromethamine; Macular Edema; Male; Middle Aged; Ophthalmic Solutions; Prospective Studies; Retinitis Pigmentosa; Sulfonamides; Thiophenes; Tomography, Optical Coherence; Usher Syndromes; Visual Acuity; Visual Field Tests; Visual Fields; Young Adult

To evaluate the efficacy of timolol-dorzolamide drops used to decrease aqueous outflow from the eye on the prolongation of the biological activity of intravitreal bevacizumab.. Thirty-eight eyes of 38 patients with macular edema (ME) following retinal vein obstruction (RVO) were enrolled. These patients were randomly assigned into 2 groups: timolol-dorzolamide drops twice daily (n = 19) or no eyedrops (control; n = 19). All patients received 1.25 mg (in 0.05 mL) of bevacizumab intravitreally and were examined before treatment, 1 week after injection, and then every 4 weeks after injection. For each patient, visual acuity (VA), intraocular pressure (IOP), and central retinal thickness (CRT) by optical coherence tomography (OCT) were recorded before injection and at each visit after injection. The mean CRT at each timepoint was considered to directly reflect the biological activities of bevacizumab at those times.. The mean CRT measured by OCT showed a significant decrease 1 week after treatment in both groups, and this difference was maintained for a total of 9 weeks (paired t-test, <0.001). The mean CRT at 1 week post-injection showed no significant between-group difference (p = 0.781). At 5 weeks post-injection, the timolol-dorzolamide group showed a significantly smaller mean CRT than the control group (p = 0.032). The difference in mean CRT between the 2 groups disappeared, however, at 9 weeks post-injection (p = 0.462). In both groups, VA improved significantly 1 week after injection, and these changes were maintained to 9 weeks post-injection in both groups. The mean VA of the 2 groups showed no difference at any time.. Timolol-dorzolamide aqueous depressant drops may delay the elimination of intravitreal bevacizumab but clinical efficacy of the eyedrops have not been proven in this study.

Topics: Administration, Topical; Adult; Aged; Aged, 80 and over; Angiogenesis Inhibitors; Antibodies, Monoclonal; Antibodies, Monoclonal, Humanized; Antihypertensive Agents; Aqueous Humor; Bevacizumab; Diabetic Retinopathy; Drug Synergism; Drug Therapy, Combination; Female; Humans; Injections; Intraocular Pressure; Macular Edema; Male; Middle Aged; Retinal Vein Occlusion; Sulfonamides; Thiophenes; Timolol; Treatment Outcome; Visual Acuity; Vitreous Body

To determine the value of a topical carbonic anhydrase inhibitor for extended treatment of cystoid macular oedema (CME) in patients with retinitis pigmentosa (RP).. Eight patients with RP and foveal cystic-appearing lesions observed on fundus examination and by optical coherence tomography (OCT) testing were treated with a topical form of carbonic anhydrase inhibitor.. Foveal cystic-like spaces were documented by OCT testing in all eight patients before treatment. All patients had a significant reduction in their foveal thickness (FT) and foveal zone thickness (FZT) in at least one eye after using 2% dorzolamide three times a day for 1 or 2 months. Six patients had an improvement in both eyes. After an additional 6-13 months of the same treatment regimen, out of six patients who had a sustained reduction in FT and FZT in at least one eye, four had this reduction in both eyes. While they were still taking Trusopt, a recurrence (rebound) of CME in both eyes was observed in two patients, whereas one patient had a sustained improvement in one eye and rebound of CME in the other eye. Out of 8 patients, 3 showed an improvement in their visual acuity by > or =7 letters, in at least one eye, on Snellen acuity charts, which was determined as clinically significant.. Results from this study suggest that patients with RP could potentially sustain a beneficial effect from continued treatment with a topical form of carbonic anhydrase inhibitor.

Topics: Carbonic Anhydrase Inhibitors; Drug Administration Schedule; Follow-Up Studies; Fovea Centralis; Humans; Macular Edema; Prospective Studies; Retinitis Pigmentosa; Sulfonamides; Thiophenes; Tomography, Optical Coherence; Treatment Outcome; Visual Acuity

To determine if topical dorzolamide, as observed with the use of systemic acetazolamide and methazolamide, would be effective in treating cystoid macular edema (CME) in patients with retinitis pigmentosa (RP).. Prospective, nonrandomized clinical trial.. setting: Institutional. patients: Fifteen patients with CME and RP. intervention: A baseline visual acuity and optical coherence tomography (OCT) measurements were obtained in all patients. Each one of them was then treated with topical dorzolamide, three times a day, for at least four weeks in both eyes. main outcome measures: Significant decrease in "foveal thickness" (more than 16%) and "foveal zone thickness" (more than 11%), as measured by OCT.. Thirteen (87%) of 15 patients showed a significant decrease in retinal thickness in at least one eye after use of topical dorzolamide for at least four weeks. Five patients (33%) demonstrated improvement in both eyes. All patients, except one, who responded showed the effect within four weeks, but were monitored for a period of two to nine months (average 4.5 months). Four patients (31%) who showed an initial improvement in macular edema showed worsening with continued treatment.. The present study documents the potential efficacy of topical dorzolamide for treating CME in patients with RP. We observed that some patients may show a "rebound phenomenon" with continued use of the medication; hence, there is a need for careful follow-up in patients being treated.

Topics: Administration, Topical; Adolescent; Adult; Carbonic Anhydrase Inhibitors; Diagnostic Techniques, Ophthalmological; Female; Humans; Macular Edema; Male; Middle Aged; Prospective Studies; Retinitis Pigmentosa; Sulfonamides; Thiophenes; Tomography, Optical Coherence; Visual Acuity

To compare the effectiveness of topical dorzolamide hydrochloride (Trusopt, Merck and Co., Inc., West Point, PA), a carbonic anhydrase inhibitor, with that of oral acetazolamide (Diamox; Lederle Laboratories, Pearl River, NY) for the management of chronic cystoid macular edema in patients with retinitis pigmentosa.. A prospective, double-masked, crossover study was conducted in five patients with retinitis pigmentosa who had chronic cystoid macular edema. After baseline visual acuity was measured and a fluorescein angiogram was obtained, each patient was randomly assigned to receive either topical dorzolamide or a placebo for 4 weeks, followed by a crossover for the same period. Oral acetazolamide then was given separately to each patient for 2 weeks. Each phase of the study was followed by a washout period of 4 weeks, during which the patient was taken off all medications. At each visit, best corrected visual acuity was measured, a fluorescein angiogram was obtained, a subjective assessment of the effects on visual function, and any side effects of the medication or placebo were recorded in the form of a questionnaire by an independent observer.. Compared with baseline or placebo values, there was no measurable improvement in visual acuity on the Early Treatment Diabetic Retinopathy Study charts with dorzolamide in any of the patients. The visual acuity in three of five patients, however, improved by seven letters or more with acetazolamide. Compared again with baseline or placebo values, fluorescein angiograms of two of five patients showed improvement in macular edema in both eyes with the use of dorzolamide, whereas all five showed improvement with acetazolamide. The improvement in macular edema was more marked with acetazolamide than with dorzolamide. The effect of dorzolamide given three times a day was the same as that when it was given five times a day. One patient indicated that dorzolamide was more effective than acetazolamide in improving visual function, three of five patients believed that acetazolamide was more effective, and one felt that both were equally effective.. Dorzolamide provided improvement in cases of macular edema on fluorescein angiograms and subjective improvement of visual function in some patients with retinitis pigmentosa with cystoid macular edema. However, there was no measurable improvement in visual acuity with the topical use of this drug. Oral acetazolamide was found to be more effective than dorzolamide in managing macular edema and improving visual acuity.

Topics: Acetazolamide; Administration, Oral; Administration, Topical; Adolescent; Adult; Aged; Carbonic Anhydrase Inhibitors; Chronic Disease; Cross-Over Studies; Double-Blind Method; Female; Fluorescein Angiography; Follow-Up Studies; Fundus Oculi; Humans; Macular Edema; Male; Middle Aged; Ophthalmic Solutions; Prospective Studies; Retinitis Pigmentosa; Sulfonamides; Thiophenes; Visual Acuity

To investigate the rate of the recurrence of cystoid macular edema (CME) secondary to retinitis pigmentosa (RP) after the initiation of topical dorzolamide and the recurrence risk factors.. We retrospectively analyzed the data of RP patients at Kyushu University Hospital. We included patients who showed a treatment response to 1.0% topical dorzolamide. The day of treatment initiation was set as the baseline. Topical dorzolamide treatment was continued during the follow-up. The recurrence of CME (defined as a >20% increase in central subfield thickness compared to previous visit, or a central subfield thickness value that exceed baseline value) was evaluated at each follow-up visit. Risk factors for RP-CME recurrence were analyzed by Cox proportional hazards modeling. A Kaplan-Meier survival analysis was used to evaluate the time to recurrent RP-CME.. Forty RP-CME patients showed a treatment response to topical dorzolamide. During the mean 3.9-year follow-up, 14 patients exhibited recurrence; its rate was 15.6%, 34.7%, and 48.7% at 1, 3, and 5 years, respectively. A high baseline central subfield thickness was significantly associated with recurrent (hazard ratio 1.11, 95% CI: 1.05-1.18, P = 0.0004).. The recurrence rate of RP-CME increased with time. A high baseline central subfield thickness value was a risk factor for recurrence.

Topics: Administration, Topical; Carbonic Anhydrase Inhibitors; Female; Follow-Up Studies; Humans; Incidence; Japan; Macula Lutea; Macular Edema; Male; Middle Aged; Prospective Studies; Recurrence; Retinitis Pigmentosa; Risk Assessment; Risk Factors; Sulfonamides; Thiophenes; Time Factors; Tomography, Optical Coherence; Visual Acuity

Cystoid macular edema is a rare, vision-threatening side effect of the taxane family of anticancer agents. There is no established treatment or standard treatment protocol for taxane-related cystoid macular edema. Here, we report two cases of taxane-related cystoid macular edema that were treated with topical dorzolamide.. In case 1, a 72-year-old Japanese woman with bilateral geographic choroiditis reported for a follow-up visit with a complaint of blurred vision in both eyes for 2 months after starting nanoparticle albumin-bound paclitaxel chemotherapy for multiple metastases of her breast cancer. Her best-corrected visual acuity had dropped from 1.2 to 0.9 in the right eye and from 1.0 to 0.4 in the left eye. Fundus examination showed no newly active geographic choroiditis lesion, but optical coherence tomography exhibited cystoid macular edema. We suspected taxane-related cystoid macular edema and terminated nanoparticle albumin-bound paclitaxel, and started topical dorzolamide treatment. Cystoid macular edema nearly resolved within 6 weeks in the right eye and within 10 weeks in the left eye after starting topical dorzolamide treatment. The resolution of cystoid macular edema without leaving a chorioretinal scar after discontinuation of paclitaxel confirmed our initial diagnosis of taxane-related cystoid macular edema. A few inconspicuous cystoid spaces persisted at the parafovea for a year after dorzolamide treatment ended, but regressed after restarting dorzolamide treatment without any side effects. Best-corrected visual acuity improved to 1.2 in the right eye and 1.0 in the left eye. In case 2, a 70-year-old Japanese man, who received nanoparticle albumin-bound paclitaxel for pancreatic cancer with multiple metastases, developed bilateral cystoid macular edema. Best-corrected visual acuity was 0.3 bilaterally. Cystoid macular edema resolved within 5 weeks after stopping nanoparticle albumin-bound paclitaxel and starting topical dorzolamide treatment confirming the diagnosis of taxane-related cystoid macular edema. Nine weeks later, best-corrected visual acuity improved to 0.8 in the right eye and 1.0 in the left eye.. Cystoid macular edema in each case resolved within a few months without any side effects using topical dorzolamide and terminating taxane-based chemotherapy. Topical dorzolamide appears to be a safe and effective treatment option for patients with taxane-related cystoid macular edema whose quality of life is threatened by visual disturbances.

Topics: Aged; Female; Humans; Macular Edema; Male; Quality of Life; Sulfonamides; Taxoids; Thiophenes; Tomography, Optical Coherence

To report a case of Bietti crystalline retinal dystrophy with cystoid macular edema (CME) that was successfully treated with topical carbonic anhydrase inhibitor.. A 35-year-old otherwise healthy woman, with a known case of Bietti crystalline retinal dystrophy, presented with progressive visual impairment in her right eye for 3 months. The best-corrected visual acuity was 20/50 in the right eye and 20/25 in the left eye. On the basis of the multimodal imaging findings, the patient was diagnosed with Bietti crystalline retinal dystrophy with unilateral CME. Carbonic anhydrase inhibitor therapy twice a day was initiated.. Three months later, visual acuity improved to 20/25 in the right eye, and CME had resolved based on spectral domain ocular coherence tomography findings, although the CME reoccurred after discontinuation of the drug. Three months after resuming the therapy, the best-corrected visual acuity improved back to 20/25.. Cystoid macular edema is one of the main causes of central visual worsening in patients with Bietti crystalline retinal dystrophy. This complication may be resolved with topical carbonic anhydrase inhibitor, resulting in improved anatomical and visual outcomes.

Topics: Administration, Ophthalmic; Adult; Carbonic Anhydrase Inhibitors; Corneal Dystrophies, Hereditary; Female; Humans; Macular Edema; Multimodal Imaging; Ophthalmic Solutions; Retinal Diseases; Sulfonamides; Thiophenes; Visual Acuity

Topics: Carbonic Anhydrase Inhibitors; Dose-Response Relationship, Drug; Female; Humans; Macula Lutea; Macular Edema; Male; Ophthalmic Solutions; Retinitis Pigmentosa; Retrospective Studies; Sulfonamides; Thiophenes; Tomography, Optical Coherence; Treatment Outcome; Visual Acuity

A 65-year-old man was referred to our department with complaints of blurred vision in the left eye. Funduscopic examination revealed areas of retinochoroidal atrophy along the retinal veins bilaterally and bone spicule pigmentation along the nasal and superior temporal venous branches, as well as macular oedema in the left eye. Fluorescein angiography, visual field test, optical coherence tomography and electrophysiological examination were performed, and results were compatible with the diagnosis of pigmented paravenous retinochoroidal atrophy (PPRCA). Treatment with topical dorzolamide and intravitreal bevacizumab in the left eye resulted in poor anatomical and visual response. There is scarce documentation of macular involvement with non-inflammatory unilateral cystoid macular oedema in PPRCA in the literature. Further investigation is required to elucidate the pathogenesis of PPRCA and to properly manage these patients.

Topics: Aged; Angiogenesis Inhibitors; Bevacizumab; Carbonic Anhydrase Inhibitors; Eye Diseases, Hereditary; Fluorescein Angiography; Humans; Injections, Intraocular; Macular Edema; Male; Retinal Degeneration; Sulfonamides; Thiophenes; Tomography, Optical Coherence

Carbonic anhydrase inhibitors (CAIs) are frequently used as an initial step to treat retinitis pigmentosa-associated cystoid macular oedema (RP-CMO). Interestingly, it has been postulated that CAIs might reduce outer nuclear layer (ONL) fluid more effectively than inner nuclear layer (INL) fluid due to better access to retinal pigment epithelium basolateral membrane than neurosensory retina. This retrospective cohort study explores if an association between spatial distribution of cystoid spaces in RP-CMO and CAI response exists.. Two independent graders reviewed pretreatment and post-treatment optical coherence tomography (OCT) images of 25 patients (43 eyes) initiated on topical and/or oral CAIs between January 2013 and December 2014. Documentation included the presence/absence of fluid (and layer(s) involved), external limiting membrane, epiretinal membrane (ERM), vitreomacular adhesion/traction, lamellar/full-thickness macular hole and central macular thickness (CMT)/volume.. INL fluid was found in all study eyes. All 13 'responders' (at least 11% reduction of CMT after treatment) demonstrated pretreatment ONL fluid. In seven patients (four responders and three non-responders), complete clearance of ONL fluid was achieved despite persistence of INL fluid. ERM presence was similar in responders and non-responders.. In this study, INL fluid was found to be the most common spatial distribution of RP-CMO. However, patients who were classed as a 'responder' to CAI treatment all demonstrated coexisting ONL fluid on their pretreatment OCT scans. This may be explained by CAIs having better access to retinal pigment epithelium basolateral membrane than neurosensory retina. Our study also suggests a minimal impact on response to CAIs by ERM.

Topics: Acetazolamide; Adolescent; Adult; Aged; Carbonic Anhydrase Inhibitors; Female; Humans; Macular Edema; Male; Middle Aged; Retinitis Pigmentosa; Retrospective Studies; Sulfonamides; Thiazines; Thiophenes; Tomography, Optical Coherence; Visual Acuity; Visual Field Tests; Young Adult

Monozygotic twins with glutathione synthetase deficiency, progressive retinal dystrophy and cystoid macular edema were followed for foveal changes on optical coherence tomography under different treatment modalities. The purpose of the study is to show the effect of topical dorzolamide in conjunction with systemic acetazolamide in terms of decreasing macular edema in this specific disease. The results showed that systemic acetazolamide alone or in combination with topical dorzolamide decreased CME in both patients for a certain period of time. The result can be temporary sustained after treatment discontinuation. In conclusion, topical dorzolamide, in conjunction with systemic acetazolamide, could reduce cystoid macular edema in GSSD.

Topics: Acetazolamide; Administration, Oral; Amino Acid Metabolism, Inborn Errors; Child; Combined Modality Therapy; Diseases in Twins; Drug Therapy, Combination; Glutathione Synthase; Humans; Macular Edema; Male; Ophthalmic Solutions; Retinal Dystrophies; Sulfonamides; Thiophenes

Topics: Administration, Topical; Adult; Aged; Antirheumatic Agents; Carbonic Anhydrase Inhibitors; Female; Fluorescein Angiography; Fundus Oculi; Humans; Hydroxychloroquine; Lupus Erythematosus, Systemic; Macular Edema; Retinal Diseases; Sulfonamides; Thiophenes; Visual Acuity

To report the variability of clinical findings, rapid concentric progression, and successful treatment of macular edema in autosomal dominant vitreoretinochoroidopathy (ADVIRC) associated with a heterozygous c.256G > A missense mutation in the bestrophin-1 (BEST1) gene.. Three affected members of a four-generation ADVIRC family were examined with fundus autofluorescence (FAF), near-infrared autofluorescence (NIA) and spectral domain optical coherence tomography (SD-OCT). Direct sequence analysis of coding and flanking intronic regions of the BEST1 gene was performed.. Disease manifestations presented with high variability with visual problems manifesting between 10 and 40 years of age. Two probands showed marked signs of peripheral degeneration, while this retinal area was not noticeably affected in the third. Cystoid macular edema was present in one proband, which responded to long-term treatment with topic dorzolamide with improved visual acuity. FAF and NIA revealed mid-peripheral retinal degeneration in areas that appeared normal on ophthalmoscopy. The full-field ERG was markedly reduced in two probands. Within a 5-year period a marked increase in concentric progression of degeneration including the posterior pole was documented with FAF, NIA and SD-OCT in one proband after the age of 63 years. Direct sequence analysis of the BEST1 gene revealed a heterozygous c.256G > A missense mutation in the three affected probands.. The findings in this family emphasize the previously noted variability of clinical manifestations in BEST1-associated ADVIRC and the relevance of FAF and NIA imaging. Cystoid macular edema and vascular leakage can be successfully treated using dorzolamide.

Topics: Adult; Bestrophins; Carbonic Anhydrase Inhibitors; Child; Chloride Channels; Choroid Diseases; Disease Progression; Electroretinography; Eye Diseases, Hereditary; Eye Proteins; Female; Fluorescein Angiography; Follow-Up Studies; Fundus Oculi; Genes, Dominant; Humans; Macular Edema; Male; Middle Aged; Mutation, Missense; Optical Imaging; Pedigree; Polymerase Chain Reaction; Retinal Degeneration; Sequence Analysis, DNA; Sulfonamides; Thiophenes; Tomography, Optical Coherence; Visual Acuity

To evaluate whether cystoid macular lesions respond to treatment with dorzolamide 2% drops in the enhanced S-cone syndrome (ESCS) child, as several case reports document favorable efficacy in adults.. Seven-year-old boy with ESCS and cystoid macular lesions was treated with dorzolamide 2% in both eyes three times a day for a period of 7 months. The efficacy of treatment was analyzed by visual acuity assessment (ETDRS), multifocal electroretinography and SD-OCT central foveal thickness (CFT) measurement.. Baseline RE CFT was 540 and 453 µm in the LE, with amplitude of P1-wave density 39.8 and 50.4 nV/deg(2), respectively. Best corrected visual acuity (BCVA) was 0.3 logMAR RE and 0.3 logMAR LE at distance. At 7-month follow-up examination, CFT showed no reduction in thickness (RE 599 µm, LE 521 µm). P1-wave density increased (RE 49.1 nV/deg(2), LE 84.9 nV/deg(2)), with BCVA 0.3 logMAR RE and 0.2 logMAR LE.. To the best of our knowledge, this is the youngest ESCS patient treated with dorzolamide drops and the first report recording that cystoid macular lesions are resistant to topical dorzolamide treatment. Furthermore, these data are in favor of the hypothesis that microcystoid changes in ESCS appear due to defects in cell-to-cell adhesion rather than the disintegration of the retinal barrier. The marked differences in treatment response to carbonic anhydrase inhibitors between the adults and the child here presented suggest that the breakdown of the blood-retinal barrier may play a more important role later in life.

Topics: Administration, Topical; Carbonic Anhydrase Inhibitors; Child; Electroretinography; Eye Diseases, Hereditary; Fluorescein Angiography; Humans; Macular Edema; Male; Ophthalmic Solutions; Retina; Retinal Degeneration; Sulfonamides; Thiophenes; Tomography, Optical Coherence; Vision Disorders; Visual Acuity

Alström syndrome is a rare genetic ciliopathy caused by a mutation in the ALMS1 gene. The syndrome is characterized by cone-rod dystrophy, dilated myocardiopathy, childhood obesity and sensorineural hearing loss. To date, cystoid macular edema has not been reported.. A female affected by Alström syndrome developed bilateral cystoid macular edema evidenced by optical coherence tomography. A topical carbonic anhydrase inhibitor was prescribed.. Complete resolution of the cystoid macular edema was achieved, though visual acuity did not improve.. Topical carbonic anhydrase inhibitors may have a role in the treatment of macular edema in syndromic retinal dystrophies such as Alström syndrome.

Topics: Administration, Topical; Alstrom Syndrome; Carbonic Anhydrase Inhibitors; Cell Cycle Proteins; Female; Humans; Infant; Macular Edema; Ophthalmic Solutions; Proteins; Sulfonamides; Thiophenes; Tomography, Optical Coherence; Visual Acuity

Non leaking macular cystoid spaces (MCS) are seen in some retinal dystrophies. Carbonic anhydrase inhibitor (CAI) treatment may reduce the size of MSC and improve vision.. A retrospective study of patients with retinal dystrophy with MCS seen between 2009 and 2013 at two sites. Patients had ophthalmic examination, optical coherence tomography (OCT) and genetic testing. Patients with vision worse than 20/30 were treated with CAI. Post treatment visual acuity (VA), central foveal zone (CFZ) thickness, and qualitative estimation of MCS size were assessed.. Eighteen patients, 6-47 years old, were included. IVFA was performed in 15 (83%) patients. Of the 26 eyes in 13 patients who were treated and followed, VA improved in 15 eyes (58%) of 10 patients. Ten of these 15 eyes had decreased CFZ thickness and 9/10 showed qualitative MCS improvement. Regression analysis showed that change in CFZ thickness was not significantly predictive of change in final visual acuity (p = 0.405). Five of 15 eyes with improved VA had paradoxically increased CFZ thickness and 2/5 had enlarged MCS. Three of the treated eyes (11%) in two patients had decreased VA with decreased CFZ thickness and improved MCS in 2/3 eyes. Eight eyes (31%) in six patients showed no change in VA with decreased CFZ thickness in 6/8 eyes with improved MCS. Genetic testing showed mutations of NR2E3, XLRS, CRB1, GPR98 and CNGB1.. Non-leaking MCS occur in a variety of retinal dystrophies. Therapy with topical or systemic CAI has variable efficacy and may result in VA improvement with or without qualitative improvement in MCS and CFZ thickness.

Topics: Acetazolamide; Administration, Oral; Administration, Topical; Adolescent; Adult; Carbonic Anhydrase Inhibitors; Child; DNA Mutational Analysis; Eye Proteins; Female; Humans; Macular Edema; Male; Middle Aged; Retinal Dystrophies; Retrospective Studies; Sulfonamides; Thiophenes; Visual Acuity

Taxanes are a class of microtubule stabilising agents used to treat a wide range of malignancies. Taxane drug-induced cystoid macula oedema (TDICMO) is a known but rare complication of therapy. First reported with Docetaxel in 2003 and Paclitaxel in 2007, there are currently less than 20 cases of TDICMO in the literature. Although most cases resolve following taxane cessation, several authors have tried using carbonic anhydrase inhibitors or intravitreal bevacizumab to accelerate resolution or when taxane therapy cannot be discontinued. We report the first published case of TDICMO treated with a single-eye trial of topical dorzolamide versus intravitreal bevacizumab.

Topics: Administration, Topical; Aged; Angiogenesis Inhibitors; Bevacizumab; Bridged-Ring Compounds; Carbonic Anhydrase Inhibitors; Humans; Intravitreal Injections; Macular Edema; Male; Sulfonamides; Taxoids; Thiophenes

Wyburn-Mason syndrome is a rare phacomatosis characterized by unilateral arteriovenous malformations (AVMs) involving the brain, retina, and (rarely) the skin. The diagnosis is concluded with dilated fundus examination and markedly dilated tortuous vascular loops with arteriovenous communications on fluorescent angiography. We present a 14-year-old male patient with Wyburn-Mason syndrome who developed serous macular neuroretinal detachment, cystoid macular edema (CME), and consequent visual deterioration in the left eye. To the best of our knowledge, this is the first report of a patient with Wyburn-Mason syndrome who developed serous retinal detachment and CME.

Topics: Adolescent; Anti-Inflammatory Agents, Non-Steroidal; Antihypertensive Agents; Arteriovenous Fistula; Drug Therapy, Combination; Fluorescein Angiography; Glucocorticoids; Humans; Ketorolac Tromethamine; Macular Edema; Magnetic Resonance Imaging; Male; Neurocutaneous Syndromes; Prednisolone; Retinal Detachment; Subretinal Fluid; Sulfonamides; Thiophenes; Tomography, Optical Coherence; Triamcinolone Acetonide

To report the occurrence of intraretinal cystoid spaces presumably due to retinal degeneration caused by CRB1 mutations, and the response to treatment with carbonic anhydrase inhibitors.. Retrospective case series.. We report four patients with retinal degeneration and intraretinal cystoid spaces due to CRB1 mutation. Of these patients, three were treated with topical carbonic anhydrase inhibitors. One of these three patients was changed to oral carbonic anhydrase inhibitor. Best corrected visual acuity and quantitative and qualitative macular optical coherence tomography results were recorded.. Three patients were compound heterozygous for CRB1 mutations, and one had two mutations one of which was not found in the father. A total of seven different mutations were detected. All patients treated with carbonic anhydrase inhibitors experienced an improvement in visual acuity and decreased central retinal thickness, except in one eye in which retinal thickness paradoxically increased.. CRB1 mutations may be associated with intraretinal cystoid spaces. The use of carbonic anhydrase inhibitors can result in improved visual acuity in some patients.

Topics: Acetazolamide; Adolescent; Carbonic Anhydrase Inhibitors; Child; Child, Preschool; Eye Proteins; Female; Humans; Macular Edema; Male; Membrane Proteins; Mutation; Nerve Tissue Proteins; Pedigree; Polymerase Chain Reaction; Retinal Dystrophies; Retrospective Studies; Sulfonamides; Thiophenes; Tomography, Optical Coherence; Visual Acuity

Mutations in the MFRP (membrane-type frizzled-related protein) gene leads to an entity characterized by retinitis pigmentosa, nanophthalmos, optic disk drusen, and macular changes, originally described as foveoschisis. Despite the association of MFRP gene mutation and increase in macular thickness, no treatment modality has been described for cystoid macular edema related to this particular entity so far.. In this case report, a 52-year-old woman presented with nanophthalmos, optic disk drusen, retinitis pigmentosa, and increase in macular thickness. Genetic analysis revealed an MFRP gene mutation. The patient was treated with topical carbonic anhydrase inhibitors.. A progressive decrease in macular thickness and cystic changes was observed during the 2-month course of topical carbonic anhydrase inhibitor treatment, and best-corrected visual acuity improved from 20/100 to 20/50. Macular thickness remained stable after 6 months of follow-up.. Cystoid macular edema is part of the macular changes noted in the MFRP mutation-related nanophthalmos-retinitis pigmentosa-foveoschisis-optic disk drusen, syndrome. Taking into account that resolution of cystoid macular edema in patients with retinitis pigmentosa may delay an irreversible decrease in visual acuity, treatment should be considered when cystic changes are suspected. Topical carbonic anhydrase inhibitor was effective in decreasing macular thickness and cystic changes in the patient reported.

Topics: Administration, Topical; Carbonic Anhydrase Inhibitors; Female; Frameshift Mutation; Humans; Macular Edema; Membrane Proteins; Microphthalmos; Middle Aged; Optic Disk Drusen; Retinitis Pigmentosa; Sulfonamides; Syndrome; Thiophenes; Treatment Outcome

To determine the efficacy and prognostic factors associated with carbonic anhydrase inhibitors (CAI) in the treatment of cystoid macular edema (CME) in retinitis pigmentosa (RP).. This was a cohort study of 81 subjects who were assessed before and after treatment. Spectral-domain optical coherence tomography (SD-OCT) was used to quantify CME. A reduction of at least 11% in central subfield (CSF) thickness was defined as objective evidence of response.. In the 125 eyes that received topical dorzolamide, 40.0% demonstrated a response to treatment with a mean reduction in OCT CSF thickness of 105 μm (95% confidence interval [CI]: 82, 128). Mean starting visual acuity (VA) increased from 6/15 to 6/12 after a median time on treatment of 3.0 months. In patients prescribed oral acetazolamide, 28.1% of eyes (41.2% of patients) showed improvement in mean OCT CSF thickness of 115 μm (95% CI: 52, 177) over a median treatment interval of 4.0 months. Visual acuity improved from 6/15 to 6/12. Eyes that responded to topical dorzolamide were more likely to have autosomal recessive than autosomal dominant RP (44.6% vs. 23.3%, P = 0.02), and a higher mean baseline OCT CSF than eyes that did not respond (P = 0.02).. We report that 40.0% of eyes (53.1% of patients) showed an objective improvement in CME after treatment with topical dorzolamide and 28.1% of eyes (41.2% of patients) after treatment with oral acetazolamide. Autosomal recessive RP and greater initial central retinal thickness predicted response to treatment with topical dorzolamide.

Topics: Acetazolamide; Administration, Oral; Adult; Carbonic Anhydrase Inhibitors; Cohort Studies; Delayed-Action Preparations; Drug Administration Schedule; Female; Humans; Macular Edema; Male; Middle Aged; Ophthalmic Solutions; Retinitis Pigmentosa; Retrospective Studies; Sulfonamides; Thiophenes; Tomography, Optical Coherence; Treatment Outcome

Topics: Administration, Topical; Carbonic Anhydrase Inhibitors; Child; Diseases in Twins; DNA Mutational Analysis; Electroretinography; Eye Proteins; Female; Fluorescein Angiography; Humans; Macular Edema; Membrane Proteins; Mutation; Nerve Tissue Proteins; Ophthalmic Solutions; Retinal Dystrophies; Sulfonamides; Thiophenes; Tomography, Optical Coherence; Twins, Monozygotic; Visual Acuity

To evaluate the therapeutic effect of continuous treatment with topical dorzolamide (a carbonic anhydrase inhibitor) for cystoid macular oedema (CME) associated with retinitis pigmentosa (RP).. 18 eyes in 10 patients with CME secondary to RP were included. Baseline visual acuity, visual field and optical coherence tomography (OCT) measurements were obtained for all patients. All patients used 1% dorzolamide three times daily in each affected eye. Patients underwent follow-up examinations at 1, 3, 6, 12 and 18 months after treatment. The response to treatment was monitored by the Humphrey field analyser (HFA: the central 10-2 program); in addition, foveal thickness was measured by OCT. Evaluation of 'macular sensitivity' was calculated by HFA as the average of 12 central points.. The 'macular sensitivity' in 10 eyes in which CME was almost completely resolved was significantly improved (p<0.05). In eight of the nine eyes in which CME was almost completely resolved within 6 months, the therapeutic efficacy persisted through 18 months. Five eyes which were almost completely resolved or showed an initial response within 6 months experienced recurrence of CME.. The prolonged (longer than 1 year) use of topical dorzolamide is effective for the treatment of CME in patients with RP. Therefore, we propose topical dorzolamide treatment as a first choice.

Topics: Administration, Topical; Adult; Antihypertensive Agents; Carbonic Anhydrase Inhibitors; Female; Humans; Macular Edema; Male; Middle Aged; Prospective Studies; Retinitis Pigmentosa; Sulfonamides; Thiophenes; Time Factors; Tomography, Optical Coherence; Young Adult

Topics: Administration, Topical; Antihypertensive Agents; Antineoplastic Agents, Phytogenic; Female; Humans; Macular Edema; Middle Aged; Paclitaxel; Sulfonamides; Thiophenes; Treatment Outcome

The purpose of the study was to evaluate the efficacy of a topical form of a carbonic anhydrase inhibitor (dorzolamide) on the foveal function and thickness in the eye of a patient with enhanced S-cone syndrome (ESCS) associated with macular cysts.. Twenty-eight-year-old Polish man with ESCS and macular cysts appearance in the right eye was treated 3 times daily with 2.0 % dorzolamide drops for the period time equal to 6 months. Monthly controls included: best corrected distance visual acuity (BCDVA-logMAR), foveal thickness (optical coherence tomography, OCT) and foveal function (multi-focal electroretinography, mfERG).. Before treatment, BCDVA in the right eye was equal to 0.26 logMAR, improved to 0.1 logMAR during the first 3 months and remained stable for the next 3 months. After 6 months, foveal thickness decreased (from 482 to 224 μm) and foveal function improved (the amplitude of P1-wave density increased from 34.8 to 107.3 nV/deg(2)) and was between the ranges of normal values. Implicit time of P1-wave remained prolonged.. The results of our short-term study suggest potential efficacy of topical dorzolamide treatment in ESCS patients with macular cysts.

Topics: Administration, Topical; Adult; Carbonic Anhydrase Inhibitors; Cysts; Electroretinography; Eye Diseases, Hereditary; Follow-Up Studies; Humans; Macular Edema; Male; Ophthalmic Solutions; Retinal Degeneration; Sulfonamides; Thiophenes; Tomography, Optical Coherence; Vision Disorders; Visual Acuity

: To determine the value of a topical carbonic anhydrase inhibitor on the macular thickness and function in choroideremia patients with cystoid macular edema.. : Two choroideremia patients with cystoid macular edema, observed by spectral-domain optical coherence tomography, were treated with a topical form of carbonic anhydrase inhibitor. Examinations performed before and during treatment included best-corrected visual acuity by using the Early Treatment Diabetic Retinopathy Study charts and contrast sensitivity measured with briefly presented grating targets and the Pelli-Robson letter contrast sensitivity chart, microperimetry, and spectral-domain optical coherence tomography.. : The 2 choroideremia patients treated with dorzolamide 2% formulation had a noticeable reduction in macular thickness by spectral-domain optical coherence tomography. This reduction was found in both eyes after 2 months of treatment. After an additional 3 months of the same treatment regimen, a more noticeable reduction in macular thickness was observed. The two study patients had improvement of their visual acuity, in at least one eye, on Early Treatment Diabetic Retinopathy Study charts, but no clinically significant changes for the other measures of visual function.. : The present study shows the potential efficacy of topical dorzolamide for treating choroideremia patients with cystoid macular edema.

Topics: Carbonic Anhydrase Inhibitors; Choroideremia; Contrast Sensitivity; Humans; Macular Edema; Male; Middle Aged; Sulfonamides; Thiophenes; Tomography, Optical Coherence; Treatment Outcome; Visual Acuity; Visual Field Tests

Cystoid macular edema (CME) is one of the common complications of retinitis pigmentosa (RP), and is responsible for patient complications such as blurred and reduced visual acuity and for subsequent atrophic changes in the fovea. The objective of this work was to evaluate the clinical efficacy of a topical dorzolamide (a carbonic anhydrase inhibitor) in CME associated with RP.. Sixteen eyes of nine patients with CME secondary to typical forms of RP were included in the study. Baseline visual acuity, visual field, and optical coherence tomography (OCT) measurements were obtained for all patients. All patients used 1% dorzolamide three times daily in each eye. Patients underwent follow-up exams at 1, 3, and 6 months after treatment. The response to treatment was monitored by visual acuity and visual field measurement testing using the Humphrey Field Analyzer (HFA: the central 10-2 Program); in addition, foveal thickness was measured by OCT. Evaluation of macular sensitivity calculated by HFA as the average of 12 central points.. Thirteen (81.3%) of 16 eyes showed a clear decrease in retinal thickness after treatment. Evaluation of macular sensitivity, calculated by HFA as the average of 12 central points (with the exception of foveal point data, showed an improvement of more than 1.0 dB in nine (56.3%) of 16 eyes. Moreover, both the mean deviation value and macular sensitivity were significantly improved. No severe side-effects were seen in any of the patients examined.. The results demonstrated that a topical dorzolamide is effective for the treatment of CME in patients with RP, and that the positive treatment effects last for up to 6 months.

Topics: Administration, Topical; Adult; Carbonic Anhydrase Inhibitors; Female; Humans; Macular Edema; Male; Middle Aged; Ophthalmic Solutions; Retina; Retinitis Pigmentosa; Sulfonamides; Thiophenes; Tomography, Optical Coherence; Treatment Outcome; Visual Acuity; Visual Field Tests; Visual Fields; Young Adult

Topics: Acetazolamide; Adult; Electroretinography; Fluorescein Angiography; Fundus Oculi; Humans; Macular Edema; Male; Ophthalmic Solutions; Sulfonamides; Tablets; Thiophenes; Tomography, Optical Coherence; Vision Disorders; Visual Acuity; Visual Fields

The purpose of this study was to determine if carbonic anhydrase inhibitors can restore their efficacy after a period of discontinued use in patients with cystic foveal lesions who demonstrated subsequent worsening in the extent of their foveal cysts after initially exhibiting a favorable response to treatment.. Retrospective chart review was conducted on all patients with retinitis pigmentosa or X-linked retinoschisis who were either currently on treatment or had been treated with carbonic anhydrase inhibitors for cystic macular lesions. A total of three patients were included in the study.. All three patients exhibited a recurrence of their cystic macular lesions while on treatment with carbonic anhydrase inhibitors. After discontinuing treatment for a period of 1 month to 6 months, all patients showed a favorable response to retreatment as monitored with optical coherence tomography scans.. The present study shows that patients who show signs of recurring macular cysts while still on treatment can have a favorable response when treatment is reinstated after a period of discontinued use of a carbonic anhydrase inhibitor.

Topics: Acetazolamide; Adult; Aged; Carbonic Anhydrase Inhibitors; Female; Humans; Macular Edema; Male; Retinitis Pigmentosa; Retinoschisis; Retreatment; Retrospective Studies; Sulfonamides; Thiophenes; Tomography, Optical Coherence; Usher Syndromes; Visual Acuity

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).. Retrospective analysis.. University hospital, tertiary care referral center.. Twenty-nine eyes of 15 patients with XLRS receiving treatment with the topical dorzolamide formulation for 4 to 41 months were enrolled.. Changes in visual acuity, cystic macular lesions, and central foveal zone thickness on optical coherence tomography during follow-up for the duration of treatment.. Among the 15 patients with XLRS, 20 eyes (69%) of 11 patients showed a positive response to treatment. Five of the 20 eyes (25%) in 3 of the 11 patients showed an initial response and a subsequent rebound of macular cysts. In 4 eyes (14%) of 3 patients, there was no response to treatment, but the macular cysts did not worsen compared with the baseline level. In 5 additional eyes (17%) of 4 patients, there was no response to treatment, and the macular cysts worsened when compared with the baseline level. Sixteen eyes (55%) of 12 patients had improvement in best-corrected visual acuity by at least 7 letters in at least 1 eye at the most recent follow-up visit. Seventeen eyes (59%) of 10 patients showed a reduction in the central foveal zone thickness in at least 1 eye when compared with the pretreatment level.. Patients with XLRS have the potential to experience a beneficial effect from sustained treatment with dorzolamide, 2%.

Topics: Administration, Topical; Adolescent; Adult; Carbonic Anhydrase Inhibitors; Eye Proteins; Follow-Up Studies; Humans; Intraocular Pressure; Macular Edema; Middle Aged; Ophthalmic Solutions; Retinoschisis; Retrospective Studies; Sulfonamides; Thiophenes; Tomography, Optical Coherence; Treatment Outcome; Visual Acuity; Young Adult

To determine the efficacy of sustained topical therapy with dorzolamide hydrochloride, 2%, on visual acuity and cystic macular lesions in patients with retinitis pigmentosa and Usher syndrome.. In a retrospective case series at a university hospital, 64 eyes of 32 patients with retinitis pigmentosa or Usher syndrome receiving treatment with the topical dorzolamide formulation for 6 to 58 months were enrolled. Changes in visual acuity on the Early Treatment Diabetic Retinopathy Study chart and central foveal zone thickness on optical coherence tomography were measured during follow-up for the duration of treatment.. Among the study cohort, 20 of 32 patients (63%) showed a positive response to treatment in at least 1 eye and 13 patients (41%) showed a positive response in both eyes. Four patients (20%) showed an initial response and a subsequent rebound of macular cysts. In 8 patients (25%), there was no response to treatment and the macular cysts worsened when compared with the pretreatment level. Ten patients (31%) had improvement in visual acuity by 7 or more letters in at least 1 eye at the most recent follow-up visit. Sixteen patients (67%) showed a reduction of more than 11% in the central foveal zone thickness in at least 1 eye when compared with the pretreatment level.. Patients with either retinitis pigmentosa or Usher syndrome who received treatment of cystoid macular edema with topical dorzolamide followed by an optical coherence tomography-guided strategy showed a decrease in central foveal zone thickness in most cases. Visual acuity improved in almost one-third of the cases, suggesting a potential corresponding visual benefit.

Topics: Administration, Topical; Adult; Aged; Carbonic Anhydrase Inhibitors; Female; Follow-Up Studies; Humans; Macular Edema; Male; Middle Aged; Ophthalmic Solutions; Retina; Retinitis Pigmentosa; Retrospective Studies; Sulfonamides; Thiophenes; Tomography, Optical Coherence; Treatment Outcome; Usher Syndromes; Visual Acuity; Young Adult

The purpose of this study was to evaluate the efficacy of topical dorzolamide 2% eye drops on macular function and thickness in a case of enhanced S-cone syndrome (ESCS). A 24-year-old Asian man with enhanced S-cone syndrome treated with topical dorzolamide in the left eye participated in the study. Examinations performed before and during treatment were included visual acuity (VA), contrast sensitivity measured with briefly presented grating targets (grating CS) and the Pelli-Robson chart (P-R CS), microperimetry (MP), and spectral-domain optical coherence tomography (SD-OCT). Following 4 months of treatment, the mean thickness of the central 1-mm foveal subfield of the left eye, as measured by SD-OCT, decreased from 551 to 242 μm. Mean MP sensitivity within the central 12 degrees (28 points) increased from 9.4 dB at baseline to 11.2 dB. Although Pelli-Robson contrast sensitivity improved only minimally in the left eye, grating contrast sensitivity improved by more than a factor of two. Mean log MAR VA was 0.22 OD and 1.00 OS (at baseline), which improved to 0.10 OD and 0.66 OS after 4 months of treatment. The results indicate that in our patient with enhanced S-cone syndrome, treatment with topical dorzolamide was effective in improving macular thickness, VA, microperimetry sensitivity, and grating contrast sensitivity. These measures of retinal structure and function are sensitive tools for evaluating the effects of treatment in enhanced S-cone syndrome patients with cystoid macular edema. Further investigation is warranted to assess the relationships among visual performance for daily activities, visual sensitivity, and macular thickness.

Topics: Administration, Topical; Carbonic Anhydrase Inhibitors; Contrast Sensitivity; Humans; Macular Edema; Male; Retinal Cone Photoreceptor Cells; Retinal Diseases; Sulfonamides; Syndrome; Thiophenes; Tomography, Optical Coherence; Treatment Outcome; Visual Acuity; Visual Field Tests; Young Adult

Topics: Administration, Topical; Carbonic Anhydrase Inhibitors; Humans; Macular Edema; Retinitis Pigmentosa; Sulfonamides; Thiophenes; Tomography, Optical Coherence; Visual Acuity