Unilateral Retinal Pigment Epithelium Dysgenesis

Cohen, Salomon Y.; Fung, Anne E.; Tadayoni, Ramin; Massin, Pascale; Barbazetto, Irene; Berthout, A.; P, Gayet; Meunier, Isabelle; Yannuzzi, Lawrence A.

doi:10.1016/j.ajo.2009.06.033

Cited by 28 publications

(54 citation statements)

References 7 publications

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“…In their study, 2 of 4 cases were complicated by CNV. Finally, in 2009, they proposed naming this disorder ‘URPED’ and reported that 2 of 9 cases were affected by CNV [1]. We believe that this is the first follow-up report which can provide new clinical information about this disease because Cohen et al [1, 2] did not mention the course of CNV treatment.…”

Section: Discussionmentioning

confidence: 94%

“…In the previous report, Cohen et al [1] stated that the affected area is usually contiguous with the optic nerve, but they did not mention the condition of the optic nerve itself. It is possible that the inflammation of the optic nerve is one of the triggers in the development of this unique pattern of the fundus.…”

Section: Discussionmentioning

confidence: 99%

“…Unilateral retinal pigment epithelium dysgenesis (URPED) is a rare clinical entity [1, 2, 3]. It is characterized by a unilateral occurrence in young patients and consists of a round affected area with a distinct scalloped margin of reticular retinal pigment epithelium (RPE) hyperplasia, mid-lesion lacunae of RPE hyperplasia, central thinning and atrophy of RPE.…”

Section: Introductionmentioning

confidence: 99%

“…The findings of fundus autofluorescence (FAF) and fluorescein angiography (FA) are the most important when diagnosing URPED. The visual prognosis of URPED is still unclear, but it may depend on whether choroidal neovascularization (CNV) will develop [1]. …”

Section: Introductionmentioning

confidence: 99%

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A Case of Choroidal Neovascularization Secondary to Unilateral Retinal Pigment Epithelium Dysgenesis

Shimoyama

Imai

Honda

et al. 2014

Case Rep Ophthalmol

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Aim: 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). Case Report: 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. Conclusion: 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.

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Section: Discussionmentioning

confidence: 94%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

A Case of Choroidal Neovascularization Secondary to Unilateral Retinal Pigment Epithelium Dysgenesis

Shimoyama

Imai

Honda

et al. 2014

Case Rep Ophthalmol

View full text Add to dashboard Cite

show abstract

“…2 In URPED, a large area of the posterior pole, usually contiguous to the optic nerve, is characterized by a distinct scalloped margin of reticular RPE hyperplasia, midlesion lacunae of RPE hypoplasia, and central thinning and atrophy of the RPE. The results of FA showed a rounded lesion with a pattern of peripheral dark spots surrounded by localized hyperfluorescence, central atrophy, and retinal tortuosity.…”

Section: Discussionmentioning

confidence: 99%