title: A case of choroidal neovascularization secondary to unilateral retinal pigment epithelium dysgenesis.
abstract: OBJECTIVE
  To report a case of choroidal neovascularization secondary to unilateral retinal pigment epithelium dysgenesis (URPED), which was resistant to posterior subtenon injection of triamcinolone acetonide (STTA) and intravitreal bevacizumab injection (IVB).
  
  
  METHODS
  An 8-year-old boy was referred to us because of a unilateral unique clinical appearance on funduscopic examination in his left eye (OS). A geometric lesion at the retinal pigment epithelium level of the interpapillomacular area was disclosed OS. The optic nerve was slightly hyperemic OS. Findings from the right fundus examination were normal. Based on these characteristic findings, he was diagnosed as having URPED. Best corrected Landolt ring chart visual acuity (BCVA) was 1.0 in both eyes. Twenty-three months after the first visit, the patient presented with visual disturbance OS. Funduscopic examination showed an expansion of the geometric lesion and the development of a subfoveal choroidal neovascularization (CNV). BCVA was 0.4 OS. Two-time STTA (40 mg/1 ml) was performed at the onset of CNV and 6 months later, and additional IVB (1.25 mg/0.05 ml) was done 10 months later for the treatment of CNV, but the geometric lesion and CNV were resistant to the treatment and continued to expand. Seven years after the first visit, the geometric lesion and the CNV kept expanding steadily.
  
  
  CONCLUSIONS
  URPED is a rare clinical entity, and the prognosis of this disease is still unclear. The visual prognosis may depend on whether CNV fully develops.
authors.affiliation: Division of Ophthalmology, Department of Organ Therapeutics, Kobe University Graduate School of Medicine, Kobe, Japan ; Department of Ophthalmology, Kobe Kaisei Hospital, Kobe, Japan.
authors.forename: Tsuyoshi
authors.initials: T
authors.lastname: Shimoyama
authors.affiliation: Division of Ophthalmology, Department of Organ Therapeutics, Kobe University Graduate School of Medicine, Kobe, Japan ; Department of Ophthalmology, Kobe Kaisei Hospital, Kobe, Japan.
authors.forename: Hisanori
authors.initials: H
authors.lastname: Imai
authors.affiliation: Division of Ophthalmology, Department of Organ Therapeutics, Kobe University Graduate School of Medicine, Kobe, Japan.
authors.forename: Shigeru
authors.initials: S
authors.lastname: Honda
authors.affiliation: Division of Ophthalmology, Department of Organ Therapeutics, Kobe University Graduate School of Medicine, Kobe, Japan.
authors.forename: Akira
authors.initials: A
authors.lastname: Negi
keywords: Anti-VEGF; Bevacizumab; Choroidal neovascularization; Fundus autofluorescence; Triamcinolone; Unilateral retinal pigment epithelium dysgenesis