Japanese journal of ophthalmology · 2026
Branch retinal vein occlusion in Leber hereditary optic neuropathy with onset at age 50 years or older: frequency and clinical characteristics.
СтатьяНаблюдательное исследованиеНазвание и аннотация: Перевод в очереди
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Takai Yasuyuki, Yamagami Akiko, Inoue Kenji, Ishikawa Hitoshi, Wakakura Masato
Аннотация
PURPOSE: To describe branch retinal vein occlusion (BRVO) observed in patients with Leber hereditary optic neuropathy (LHON) with onset at age ≥50 years and to explore whether BRVO prevalence may be higher than age- and sex-matched general population estimates.
STUDY DESIGN: Retrospective observational study.
METHODS: We reviewed 187 genetically confirmed LHON patients seen at a single center (1990-2024) and analyzed 39 patients with LHON onset at age ≥50 years. We summarized fundus findings, management, and longitudinal visual function. We also performed indirect standardization using age- and sex-specific BRVO point prevalence from the Hisayama Study to calculate the standardized prevalence ratio (SPR) for BRVO diagnosed before LHON onset; a sensitivity analysis used an ever-BRVO definition that also included post-onset cases.
RESULTS: Four patients had BRVO. Three cases were diagnosed before LHON onset (SPR 3.88; 95% CI 0.80-11.34), and inclusion of one post-onset case increased the SPR to 5.17 (95% CI 1.41-13.25). Macular atrophy was present in two patients. All patients underwent laser photocoagulation and none received intravitreal anti-vascular endothelial growth factor (VEGF) therapy. Longitudinal visual function generally showed persistent central visual field loss consistent with LHON, with some BRVO-affected eyes demonstrating additional nasal or inferonasal components at follow-up.
CONCLUSION: In this older-onset LHON cohort, BRVO was observed in four patients and detailed case-level characteristics are presented. The indirect standardized comparison suggests a tendency toward higher standardized BRVO prevalence; however, this prevalence-based analysis is not adjusted for vascular risk profiles and should be interpreted as hypothesis-generating.
STUDY DESIGN: Retrospective observational study.
METHODS: We reviewed 187 genetically confirmed LHON patients seen at a single center (1990-2024) and analyzed 39 patients with LHON onset at age ≥50 years. We summarized fundus findings, management, and longitudinal visual function. We also performed indirect standardization using age- and sex-specific BRVO point prevalence from the Hisayama Study to calculate the standardized prevalence ratio (SPR) for BRVO diagnosed before LHON onset; a sensitivity analysis used an ever-BRVO definition that also included post-onset cases.
RESULTS: Four patients had BRVO. Three cases were diagnosed before LHON onset (SPR 3.88; 95% CI 0.80-11.34), and inclusion of one post-onset case increased the SPR to 5.17 (95% CI 1.41-13.25). Macular atrophy was present in two patients. All patients underwent laser photocoagulation and none received intravitreal anti-vascular endothelial growth factor (VEGF) therapy. Longitudinal visual function generally showed persistent central visual field loss consistent with LHON, with some BRVO-affected eyes demonstrating additional nasal or inferonasal components at follow-up.
CONCLUSION: In this older-onset LHON cohort, BRVO was observed in four patients and detailed case-level characteristics are presented. The indirect standardized comparison suggests a tendency toward higher standardized BRVO prevalence; however, this prevalence-based analysis is not adjusted for vascular risk profiles and should be interpreted as hypothesis-generating.