A Case of Choroidal Neovascularization Secondary to Unilateral Retinal Pigment Epithelium Dysgenesis
An 8-year-old boy was referred to us because of a unilateral unique funduscopic appearance in the left eye (OS). Funduscopic findings revealed a geometric lesion at the RPE level of the interpapillomacular area, contiguous with the slightly hyperemic optic nerve (fig. ). The lesion was composed of marginal RPE hyperplasia and atrophic RPE in its center. Findings from the right fundus examination were normal. Based on these characteristic findings, the patient was diagnosed with URPED. Best corrected Landolt ring chart visual acuity (BCVA) was 1.0 in both eyes. Twenty-three months after the first visit, he presented with visual disturbance OS. Funduscopic examination showed enlargement of the lesion and development of subfoveal CNV (fig. ). FA findings revealed hypofluorescence from RPE hyperplasia, hyperfluorescence from RPE atrophy and dye leakage from the optic nerve and CNV (fig. ). Optical coherence tomography (OCT) revealed type 2 CNV, but the exudative change was not obvious (fig. ). The patient's BCVA was 0.4 OS. Two-time STTA (40 mg/1 ml) was performed at the onset of CNV and 6 months later. Additional IVB (1.25 mg/0.05 ml) was performed 10 months later for the treatment of CNV. Both STTA and IVB were performed with the written informed consent from both the patient and his father as well as with an approval of the institutional review board of Kobe University of Medicine and the Tenets of the Declaration of Helsinki. Despite the treatment, the geometric lesion and CNV were resistant to the treatment and expanded gradually. Seven years after the first visit, the geometric lesion and the CNV kept expanding steadily, and new CNV developed at the inferior retina (fig. ).