First Author: I.Contreras SPAIN
Co Author(s): 0 0 0 0 0 0 0 0 0
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To report the results of intravitreal Ozurdex for the treatment of refractory macular edema in a patient with retinitis pigmentosa
A 24-year old woman was first seen in our center in April 2012. She was diagnosed with Usher syndrome. Visual acuity was 0.1 in her right eye (RE) and 0.4 in her left eye (LE). Fundus examination showed the typical signs of retinitis pigmentosa (bone-spicule-type intraretinal pigmentation in the mid-periphery with attenuated retinal vessels) as well as macular edema and optic nerve head drusen. Visual fields showed bilateral concentric defects. Optical coherence tomography (OCT) showed severe macular edema, with a central retinal thickness (CRT) of 775 µm in the RE and 640 µm in the LE. Treatment was started with oral acetazolamide and topical NSAIDS. However, the patient did not tolerate acetazolamide. Treatment with intravitreal anti-VEGF lead only to a slight reduction in macular edema. Therefore, treatment was stopped. In September 2013 the patient reported a decrease in visual acuity: it had dropped to 0.05 in the RE and 0.16 in the LE. Macular edema had increased, with a CRT of 874 µm in the RE and 878 µm in the LE. The patient was offered treatment with intravitreal Ozurdex. The LE was treated in October 2013.
One week after Ozurdex injection in the LE, macular edema had improved dramatically, with a CRT of 377 µm. Subjectively, the patien reported better contrast sensitivity. These results lead to the decision to also treat the RE with intravitreal Ozurdex. One week after the injection, CRT in the RE was 649 µm. In December 2013, 9 weeks after the injection in the LE and 6 weeks after the injection in the RE, visual acuity had improved to 0.1 in the right eye and remained stable at 0.16 in the left eye. Macular edema was almost resolved in both eyes: in the RE, CRT was 317 µm and a thin epiretinal membrane could be seen in OCT. In the LE, CRT was 210 µm. Intraocular pressure (IOP) was 36 mmHg in both eyes. Treatment with a fixed combination of timolol and dorzolamide twice daily was started and one week later IOP was 16 mmHg RE and 17 mmHg LE. In February 2014, 15 and 18 weeks after treatment, macular edema had reappeared in both eyes, with a CRT of 632 µm RE and 728 µm LE. The patient has been scheduled to receive a new injection in both eyes.
The pathogenesis of macular edema in retinitis pigmentosa is not clearly understood. Although some patients may respond to oral acetazolamide, our patient was unable to tolerate treatment. Several authors have suggested that in many types of tapetoretinal degeneration there may be an inflammatory response against actively degenerating photoreceptors and retinal pigment epithelium. Multiple studies have reported an improvement in macular edema with intravitreal triamcinolone (IVT). However, the effect of IVT is short and adverse effects are relatively high. Recently, several case reports have reported good results with intravitreal Ozurdex. In our case, intravitreal Ozurdex lead to the resolution of severe macular edema, although visual acuity did not improve probably due to the long-standing nature of the edema. However, macular edema reappeared three months after treatment. Further studies should be performed to better understand the pathogenesis of macular edema in retinitis pigmentosa and the possible role of intravitreal dexamethasone for its treatment.