Purpose:  

Review of the management of choroidal neovascularisation secondary to Best’s vitelliform macular dystrophy.

Materials and methods:

Eight eyes of 6 patients with Best’s disease complicated by choroidal neovascularization (CNV) were included. The visual acuity, angiographic and optical coherence tomography (OCT) findings were used to assess management outcome.  All patients presented with reduction in visual acuity.  Two patients were treated with intravitreal bevacizumab. Two patients received combination treatment with photodynamic therapy and intravitreal ranibizumab.  One patient with bilateral disease had one eye closely observed and the other eye received bevacizumab injections. The last patient had bilateral disease and one eye received no treatment other than observation and the other eye was treated with photodynamic therapy.

Results:

All patients noted visual loss at presentation with CNV with an average visual acuity of 0.76 LogMAR. Eyes that received treatment demonstrated improvement of vision with and had a final visual acuity of 0.3 or better (average visual acuity 0.21 LogMAR). Eyes that did not receive treatment had a poor visual outcome with a mean visual acuity of 1.00.

Conclusion:

The natural history of Best’s disease complicated by CNV has a very poor visual prognosis.  Using different treatment options available today, including anti VEGF and PDT, it is possible to improve the final visual outcome of these patients.