Dorzolamide in a management of cystoid macular edema in a patient with retinitis pigmentosa sine pigmento

Abstract

Introduction. Retinitis pigmentosa (RP) is a group of inherited retinal dystrophies caused by mutations in various genes. The disease leads to progressive photoreceptors loss (rods predominantly) and retinal pigment epithelium alteration. RP can lead to blindness in the advanced stages of the disease, when the central retina is involved, mostly due to the presence of cystoid macular edema (CME). Several therapeutic approaches for CME in RP patients have been attempted but responses have been variable. Case outline. A 51-year-old man was referred due to progressive six-month-long blurring of vision in both eyes. The patient underwent complete ophthalmological examination at baseline. Based on the clinical presentation of mottled mid periphery of the retina and characteristic tubular visual field loss, hence typical fluorescein angiography and optical coherence tomography (OCT) findings, the patient was diagnosed as bilateral retinitis pigmentosa sine pigmento with CME. In an attempt to control the edema, treatment was started with dorzolamide, instilled three times daily in each eye, which resulted in reduction of macular edema in a one-month-period, as documented by OCT. This effect was further monitored for five months and was stable. Conclusion. In the presented case, we investigate the six-month therapeutic efficacy of dorzolamide for dealing with the CME secondary to RP. Topical carbonic anhydrase inhibitors are considered as the first option for treatment of CME in RP patients, due to their high efficacy and safety.

Keywords

• Retinitis pigmentosa sine pigmento
• Cystoid macular edema
• Topical carbonic anhydrase inhibitors
• Dorzolamide