Incidence and Natural History of Retinochoroidal Neovascularization in Enhanced S-Cone Syndrome.
Am J Ophthalmol
; 222: 174-184, 2021 02.
Article
em En
| MEDLINE
| ID: mdl-32941856
ABSTRACT
OBJECTIVE:
We examined the incidence and natural history of macular retinochoroidal neovascularization (RCN) in enhanced S-cone syndrome (ESCS).DESIGN:
Retrospective case series.METHODS:
This single-center study included 14 of 93 patients with ESCS who had signs of active or inactive RCN in ≥1 eye. We conducted multimodal retinal imaging, full-field electroretinography, and molecular genetic analysis of NR2E3 gene. Our main outcome measures included the cumulative incidence of RCN in ESCS, type of RCN, and mode of evolution of RCN.RESULTS:
Fourteen (15.1%) of 93 patients with ESCS had RCN in ≥1 eye at 2 to 27 years of age. All 22 RCNs (21 eyes of 14 patients) were macular. Twelve of the RCNs were active with exudates/hemorrhages. Of these, 5 appeared de novo in a subretinal location, with photographic evidence of no pre-existing lesions. The latter were compatible with type 3 neovascularization or retinal angiomatous proliferation and subsequently evolved into unifocal fibrotic nodules. The remaining active lesions all had some degree of pre-existing fibrosis and remained stable. Ten inactive fibrotic nodules, identical to end-stage de novo lesions, were found and were presumed to represent healed RCNs.CONCLUSIONS:
RCN, a treatable condition, may occur as early as 2 years of age and may be much more common in patients with ESCS than previously estimated. It may be the primary cause of the unifocal submacular fibrosis that is commonly observed in this condition. Additional research is needed to establish the pathogenesis of RCN in patients with ESCS and its optimal management.
Texto completo:
1
Coleções:
01-internacional
Temas:
Geral
Base de dados:
MEDLINE
Assunto principal:
Degeneração Retiniana
/
Transtornos da Visão
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Acuidade Visual
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Campos Visuais
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Oftalmopatias Hereditárias
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Neovascularização Retiniana
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Células Fotorreceptoras Retinianas Cones
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Neovascularização de Coroide
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Tomografia de Coerência Óptica
Tipo de estudo:
Diagnostic_studies
/
Etiology_studies
/
Incidence_studies
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Limite:
Adolescent
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Adult
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Child
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Child, preschool
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Female
/
Humans
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Infant
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Male
País/Região como assunto:
Asia
Idioma:
En
Revista:
Am J Ophthalmol
Ano de publicação:
2021
Tipo de documento:
Article
País de afiliação:
Arábia Saudita