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Background <p>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.</p> Methods <p>In this observational cross-sectional study, all enrolled eyes underwent single-capture UWF-OCTA (29 × 24&#xa0;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.</p> Results <p>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%, <i>P</i> = 0.319) and the median number of RCHs per eye (2 vs. 2, <i>P</i> = 0.252). However, the RCH involvement rate (61.5% vs. 89.7%, <i>P</i> &lt; 0.001) and number of RCHs per eye (1 vs. 2, <i>P</i> = 0.003) were lower with single-capture UWF-OCTA than with UWF-FA. At the RCH level, UWF-OCTA plus UWF-CFP showed slightly lower detection rates than did UWF-FA, albeit without statistical significance (86.8% vs. 93.4%, <i>P</i> = 0.057). Single-capture UWF-OCTA detected significantly fewer RCHs than did UWF-FA (51.7% vs. 93.4%, <i>P</i> &lt; 0.001). RCHs were classified according to OCTA B-scan characteristics. Types 1 (48.8%) and 2 (18.1%) RCHs exhibited a nodular appearance with protrusion into the vitreous cavity and compression of the outer retina, respectively. Type 3 RCHs (28.3%) displayed flat growth patterns, whereas type 4 RCHs (4.7%) breached the inner limiting membrane. Logistic regression revealed that RCH size &gt; 0.5&#xa0;mm was associated with hyperfluorescence with leakage (odds ratio [OR]: 10.987; 95% confidence interval [CI]: 1.747&#xa0;to&#xa0;69.090; <i>P</i> = 0.011), whereas type 3 RCH was associated with lower odds of leakage than type 1 (OR: 0.083; 95% CI: 0.026&#xa0;to&#xa0;0.267; <i>P</i> &lt; 0.001).</p> Conclusions <p>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.</p>

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

  • 期刊论文

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  • 发表日期：

    2026年5月14日

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  • Xiaonan Zhuang,
  • Yao Zhou,
  • Fengjuan Gao,
  • Yi Xuan,
  • Min Wang,
  • Qing Chang,
  • Xin Huang,
  • Gezhi Xu,
  • Wei Liu

摘要

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 < 0.001) and number of RCHs per eye (1 vs. 2, P = 0.003) were lower with single-capture UWF-OCTA than with UWF-FA. At the RCH level, UWF-OCTA plus UWF-CFP showed slightly lower detection rates than did UWF-FA, albeit without statistical significance (86.8% vs. 93.4%, P = 0.057). Single-capture UWF-OCTA detected significantly fewer RCHs than did UWF-FA (51.7% vs. 93.4%, P < 0.001). RCHs were classified according to OCTA B-scan characteristics. Types 1 (48.8%) and 2 (18.1%) RCHs exhibited a nodular appearance with protrusion into the vitreous cavity and compression of the outer retina, respectively. Type 3 RCHs (28.3%) displayed flat growth patterns, whereas type 4 RCHs (4.7%) breached the inner limiting membrane. Logistic regression revealed that RCH size > 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.