Significant macular edema in a patient with cone dystrophy and improvement with acetazolamide treatment.

PURPOSE: To describe a unique presentation and treatment course of a patient with an established diagnosis of cone dystrophy.

METHODS

Clinical examination, spectral domain optical coherence tomography, autofluorescence, fluorescein angiography, and electrophysiology testing were evaluated.

RESULTS

A patient with cone dystrophy diagnosed by clinical examination and electrophysiology testing displayed a large amount of macular intraretinal and subretinal fluid on spectral domain optical coherence tomography in both eyes, without obvious angiographic leakage. Intraretinal fluid resolved after 7 weeks treatment on oral acetazolamide therapy. The patient had subjective improvement in peripheral vision, though the visual acuity remained the same.

CONCLUSION

Patients with cone dystrophy can accumulate intraretinal edema in the macula, which may respond to carbonic anhydrase therapy.