Purpose: To document by optical coherence tomography angiography the presence of a choroidal neovascularization (CNV) secondary to unilateral retinal pigment epithelium dysgenesis and describe its changes after treatment with intravitreal bevacizumab.
Methods: Case report.
Results: A 51-year-old woman referred to our ocular oncology service for a possible choroidal osteoma in her left eye was found to have a unilateral retinal pigment epithelium dysgenesis complicated by CNV. Best-corrected visual acuity at baseline was 20/200. A complete ophthalmological evaluation including color fundus photography, fundus autofluorescence, fluorescein angiography, indocyanine green angiography, optical coherence tomography, and optical coherence tomography angiography was performed. The CNV appeared as a tangled vascular network on optical coherence tomography angiography. After 2 intravitreal injections of bevacizumab, the best-corrected visual acuity improved to 20/50, and optical coherence tomography angiography showed a progressive contraction of the CNV. Unilateral retinal pigment epithelium dysgenesis margins remained stable at consecutive follow-up.
Conclusion: Optical coherence tomography angiography is a useful imaging technique for the diagnosis and follow-up of CNVs secondary to unilateral retinal pigment epithelium dysgenesis. Anti-vascular endothelial growth factor agents represent an effective therapy for the treatment of CNV in patients with this disease.