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Therapeutic Effect of Prolonged Treatment with Topical Dorzolamide for Cystoid Macular Edema in Patients with Retinitis Pigmentosa

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TLDR
The prolonged (longer than 1 year) use of topical dorzolamide is effective for the treatment of CME in patients with RP and is proposed as a first choice treatment.
Abstract
Aim 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). Methods 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. Results 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. Conclusions 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.

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Citations
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Molecular genetics and emerging therapies for retinitis pigmentosa: Basic research and clinical perspectives

TL;DR: Development in gene editing technology and novel gene delivery carriers make gene therapy a promising therapeutic modality for RP and other hereditary retinal dystrophies in the future.
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Retinitis pigmentosa-associated cystoid macular oedema: pathogenesis and avenues of intervention

TL;DR: The evidence supporting treatment modalities for retinitis pigmentosa-CMO should aim to improve both quality and quantity of vision in the short term and may also slow central vision loss over time.
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Retinitis Pigmentosa: Progress and Perspective.

TL;DR: Investigation into the molecular pathways from mutation to rod cell death may reveal targets for developing new treatment and future trends in the study of retinitis pigmentosa are discussed.
Journal ArticleDOI

Efficacy and prognostic factors of response to carbonic anhydrase inhibitors in management of cystoid macular edema in retinitis pigmentosa.

TL;DR: Eyes that responded to topical dorzolamide were more likely to have autosomal recessive than autosomal dominant RP, and greater initial central retinal thickness predicted response to treatment with topical dorsolamide.
Journal ArticleDOI

Retinitis Pigmentosa: Review of Current Treatment.

TL;DR: The classic fundus appearance of typical retinitis pigmentosa (RP) includes optic disc pallor, attenuated retinal vessels, mottling of the retinal pigment epithelium (RPE), and peripheral bone spicule pigmentation.
References
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Journal ArticleDOI

A point mutation of the rhodopsin gene in one form of retinitis pigmentosa

TL;DR: A C→A transversion in codon 23 (corresponding to a proline →histidine substitution) in 17 of 148 unrelated patients and not in any of 102 unaffected individuals indicates that this mutation could be the cause of one form of autosomal dominant retinitis pigmentosa.
Journal ArticleDOI

Mutations in the human retinal degeneration slow gene in autosomal dominant retinitis pigmentosa.

TL;DR: The results indicate that some cases of autosomal dominant retinitis pigmentosa are due to mutations at the RDS locus, and three mutations of the human homologue of the rds gene (RDS) are reported here that cosegregate with autosomal dominates retinal degenerations in separate families.
Journal ArticleDOI

Treatment of Chronic Macular Edema With Acetazolamide

TL;DR: Sixteen patients showed a reproducible response to acetazolamide with partial or complete resolution of edema and improvement of visual acuity, and the therapeutic effect occurred in more than half of the patients with inherited outer retinal disease or uveitis, but in none with primary retinal vascular disorders.
Journal ArticleDOI

Molecular genetics and emerging therapies for retinitis pigmentosa: Basic research and clinical perspectives

TL;DR: Development in gene editing technology and novel gene delivery carriers make gene therapy a promising therapeutic modality for RP and other hereditary retinal dystrophies in the future.
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