Morphology of Retinal Hemangioblastoma in von Hippel-Lindau Disease Revealed by Optical Coherence Tomography Angiography.

IF 0.8 4区医学 Q4 OPHTHALMOLOGY

Klinische Monatsblatter fur Augenheilkunde Pub Date : 2025-02-10 DOI:10.1055/a-2501-8628

Yannik Laich, Stefan J Lang, Andreas Glatz, Navid Farassat, Felicitas Bucher, Simone Nuessle, Daniel Böhringer, Wolf Alexander Lagrèze, Spyridon Sideris, Peter Maloca, Clemens Lange, Thomas Reinhard, Hansjürgen Agostini, Michael Reich

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引用次数: 0

Abstract

Background: To characterize different growth patterns of retinal hemangioblastomas (RHs) in patients with von Hippel-Lindau disease (VHLD) using swept-source optical coherence tomography angiography (SS-OCTA).

Methods: Single-center observational cross-sectional study. SS-OCTA B-scans were centered over primary treatment-naïve RHs to display the perfusion in the inner, middle, and outer areas (IA, MA, OA). Distinctive growth patterns were characterized using K-means cluster analysis of the flow signal.

Results: Annual screening of 201 patients with VHLD revealed 49 patients with 85 RHs (41 recurrent, 44 primary treatment-naïve RHs). High-quality SS-OCTA images were available for 24 primary RHs and were pooled with scans of the treatment-naïve form of 6 recurrent RHs, which were taken at a prior visit, for further analysis. Out of 30 primary RHs, 18 were located juxtapapillary (jRH) and 12 peripherally (pRH). K-means cluster analysis distinguished seven exophytic (23.3%, 2 jRH, 5 pRH), nine endophytic (30%, 6 jRH, 3 pRH), and fourteen sessile (46.7%, 10 jRH, 4 pRH) growth patterns. Fractions of the flow signal in the IA, MA, and OA were 7.3 ± 3.3%, 89.3 ± 5.0%, and 3.4 ± 3.5% for exophytic RHs, 22.2 ± 7.4%, 65.6 ± 7.3%, and 12.1 ± 8.2% for sessile RHs, and 65.4 ± 5.7%, 26.6 ± 4.9%, and 8.0 ± 6.1% for endophytic RH.

Conclusion: OCTA contributes to further elucidating the morphology of RHs as a useful complementary diagnostic tool that can help to provide customized therapeutic approaches in the future and thus improve the effectiveness of RH treatment.

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光学相干断层扫描血管造影显示von Hippel-Lindau病视网膜血管母细胞瘤的形态学。

背景：利用扫描源光学相干断层扫描血管造影（SS-OCTA）表征von Hippel-Lindau病（VHLD）患者视网膜血管母细胞瘤（RHs）的不同生长模式。方法：单中心横断面观察研究。SS-OCTA b扫描以主要treatment-naïve RHs为中心显示内、中、外区域的灌注情况（IA、MA、OA）。利用流量信号的k -均值聚类分析表征了不同的生长模式。结果：对201例VHLD患者进行年度筛查，发现49例患者有85例RHs（41例复发，44例原发treatment-naïve RHs）。24例原发性RHs可获得高质量SS-OCTA图像，并与先前就诊时拍摄的6例复发性RHs treatment-naïve形式的扫描结果合并，以作进一步分析。在30例原发性RHs中，18例位于乳头旁（jRH）， 12例位于周围（pRH）。K-means聚类分析区分出7种外生（23.3%,2 jRH, 5 pRH）、9种内生（30%,6 jRH, 3 pRH）和14种无根（46.7%,10 jRH, 4 pRH）生长模式。在IA、MA和OA中，外生RH的流量信号分别为7.3±3.3%、89.3±5.0%和3.4±3.5%，无根RH为22.2±7.4%、65.6±7.3%和12.1±8.2%，内生RH为65.4±5.7%、26.6±4.9%和8.0±6.1%。结论：OCTA有助于进一步阐明RH的形态，作为一种有用的辅助诊断工具，有助于在未来提供定制的治疗方法，从而提高RH治疗的有效性。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Klinische Monatsblatter fur Augenheilkunde 医学-眼科学

CiteScore

1.30

自引率

0.00%

发文量

235

审稿时长

4-8 weeks

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