To report the first case of cystoid macular edema in a retinitis pigmentosa patient with pars plana vitrectomy.

A 43-year-old female visited our hospital with visual disturbances of both eyes. Corrected visual acuity was 20/22 in the right eye and 20/25 in the left eye. Peripheral depigmentation and atrophy of the retinal pigment epithelium, pigmentary retinal degeneration, and attenuated arterioles were observed in both eyes. Cystoid macular edema was observed on optical coherence tomography which showed that the central macular thickness was 308 µm in the right eye and 422 µm in left eye. Intravitreal aflibercept was injected into the left eye. One month after injection, the central macular thickness showed no response with a thickness of 449 µm. An intravitreal dexamethasone implant was then injected, 1 month after injection, the central macular thickness was 367 µm. Six months after injection, the patient again complained of visual disturbance of the left eye with a corrected visual acuity of 20/70. Vitreous opacity was observed and the central macular thickness was 501 µm. The patient underwent pars plana vitrectomy. Three days after surgery, the central macular thickness was 320 µm. One year after surgery, the corrected visual acuity was 20/33 and the central macular thickness was 311 µm.

Pars plana vitrectomy due to cystoid macular edema in a retinitis pigmentosa patient has not been previously reported in the Republic of Korea. Pars plana vitrectomy can therefore be an effective treatment for cystoid macular edema in retinitis pigmentosa patients.