Bilateral disc edema and unilateral macular hole in a patient with retinitis pigmentosa

A unique case of retinitis pigmentosa (RP) associated with bilateral disc edema and unilateral macular hole is presented.

A 49-year-old woman, a known RP patient, was found to have bilateral disc edema and a macular hole in the left eye during routine clinical examination. Fluorescein angiography revealed hyperfluorescent leakage of the optic nerve head significantly OD and minimally OS. There was staining in the macular regions which was consistent with retinal pigment epithelium atrophy OD and cystoid macular edema (CME) OS. Cerebrospinal fluid pressure and examination by lumbar puncture was normal. Disc edema spontaneously decreased bilaterally during follow-up.

Bilateral disc edema was thought to be secondary to inflammation caused by rapid degeneration of photoreceptors and retinal pigment epithelium and macular hole was secondary to CME.

Inflammatory response in the course of retinitis pigmentosa may result in disc edema and cystoid macular edema, which may further progresses to macular hole.