Abstract
Purpose: :
To investigate the prevalence of cystoid macular edema (CME) in patients with cone–dystrophies (CD) and to monitor treatment effects of acetazolamide using OCT.
Methods: :
Prospective clinical study in 40 patients with CD. Diagnosis of CD was established using funduscopy, Ganzfeld–ERG (ISCEV protocol), and BCVA (Snellen and EDTRS). OCT recordings in the fovea was performed in all patients. Outcome measures were visual acuity (VA, ETDRS) and reduction of retinal thickness measured in OCT images. If CME was present, treatment with acetazolamide was started. Follow up exams with OCT and VA measurements were scheduled 2, 4, 6, 12, 24 and 36 weeks after initiation of therapy. Statistical significance was defined p<0.05.
Results: :
Out of 40 patients with CD, 11 were diagnosed having CME in both eyes in OCT recordings (27.5%). CME was not diagnosed in any of the patients in routine clinical examination. Six patients were treated with acetazolamide, 5 patients declined to be treated. A statistically significant increase in BCVA (average of 8.9 letters) was observed 12 weeks after initiation of therapy. Retinal thickness was reduced significantly by 25.6±12µm. In none of the treated patients BCVA worsened. No rebound effect after cessation of therapy was observed (9 months observation time). None of the untreated patients showed improvement in BCVA or reduction of retinal thickness. Treatment was well tolerated in all treated patients. No severe adverse events were observed.
Conclusions: :
Patients with CD show a high prevalence of CME which has not yet been reported in the literature. Treatment with azetazolamide results in significant increase of BCVA and reduction in macular thickness. OCT imaging of the fovea in patients suffering from CD is recommended.
Keywords: photoreceptors • imaging methods (CT, FA, ICG, MRI, OCT, RTA, SLO, ultrasound) • retinal degenerations: hereditary