To report a case of Bietti crystalline retinal dystrophy with cystoid macular edema (CME) that was successfully treated with topical carbonic anhydrase inhibitor.
A 35-year-old otherwise healthy woman, with a known case of Bietti crystalline retinal dystrophy, presented with progressive visual impairment in her right eye for 3 months. The best-corrected visual acuity was 20/50 in the right eye and 20/25 in the left eye. On the basis of the multimodal imaging findings, the patient was diagnosed with Bietti crystalline retinal dystrophy with unilateral CME. Carbonic anhydrase inhibitor therapy twice a day was initiated.
Three months later, visual acuity improved to 20/25 in the right eye, and CME had resolved based on spectral domain ocular coherence tomography findings, although the CME reoccurred after discontinuation of the drug. Three months after resuming the therapy, the best-corrected visual acuity improved back to 20/25.
Cystoid macular edema is one of the main causes of central visual worsening in patients with Bietti crystalline retinal dystrophy. This complication may be resolved with topical carbonic anhydrase inhibitor, resulting in improved anatomical and visual outcomes.