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May 15, 2026Eye and Vision0 citationsOpen Access

Ultra-widefield optical coherence tomography angiography plus colour fundus photography in von Hippel‒Lindau disease: detection, classification, and correlations with findings on fluorescein angiography

XZXiaonan ZhuangYZYao ZhouFGFengjuan Gao

Key Points

  • The aim is to compare the effectiveness of UWF-OCTA combined with UWF-CFP versus UWF-FA in detecting retinal capillary haemangiomas in von Hippel–Lindau disease.
  • Observational cross-sectional design with 39 eyes from 21 patients with VHL.
  • Used single-capture UWF-OCTA and UWF-CFP for detecting suspected retinal capillary haemangiomas, comparing with UWF-FA.
  • Performed detection and classification of RCHs using different imaging modalities and statistical analyses.
  • UWF-OCTA plus UWF-CFP detected RCH involvement at 87.2%, comparable to UWF-FA at 89.7% (P = 0.319).
  • Single-capture UWF-OCTA detected RCHs at 51.7%, significantly lower than UWF-FA at 93.4% (P < 0.001).
  • RCH size > 0.5 mm showed a strong association with leakage (OR: 10.987; 95% CI: 1.747 to 69.090; P = 0.011).

Abstract

Abstract Background To compare the utility of single-capture ultra-widefield optical coherence tomography angiography (UWF-OCTA) and UWF-OCTA plus UWF colour fundus photography (UWF-CFP) versus UWF fluorescein angiography (UWF-FA) in detecting retinal capillary haemangiomas (RCHs) in von Hippel‒Lindau disease (VHL) and to explore the associations of RCH multimodal imaging features. Methods In this observational cross-sectional study, all enrolled eyes underwent single-capture UWF-OCTA (29 × 24 mm). RCHs suspected on UWF-CFP with eye-steering were further checked using regional UWF-OCTA scans. UWF-FA was used for comparison. Independent observers performed the RCH detection and characterisation using different imaging methods. The detection performance was compared, and logistic regression was used to identify the factors associated with leakage. Results Thirty-nine eyes of 21 patients with VHL were included in this study. At the eye level, UWF-OCTA plus UWF-CFP exhibited a similar performance to UWF-FA in detecting RCH involvement (87.2% vs. 89.7%, P = 0.319) and the median number of RCHs per eye (2 vs. 2, P = 0.252). However, the RCH involvement rate (61.5% vs. 89.7%, P 0.5 mm was associated with hyperfluorescence with leakage (odds ratio OR: 10.987; 95% confidence interval CI: 1.747 to 69.090; P = 0.011), whereas type 3 RCH was associated with lower odds of leakage than type 1 (OR: 0.083; 95% CI: 0.026 to 0.267; P < 0.001). Conclusions A screening strategy integrating UWF-OCTA and UWF-CFP, instead of 150° single-capture UWF-OCTA alone, is reliable for non-invasive detection of RCHs in patients with VHL. OCTA-derived features, particularly the morphological subtype, may replace FA in assessing RCH activity and guiding the management of ocular VHL.

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Cite This Study

Zhuang et al. (2026) studied this question.

synapsesocial.com/papers/6a06b940e7dec685947abda1https://doi.org/10.1186/s40662-026-00489-x
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