Sindhar Sampat, O'Bryhim Bliss E, Licata Jordan, Piccirillo Jay F, Apte Rajendra S
Department of Otolaryngology-Head and Neck Surgery, Washington University in St. Louis, St. Louis, Missouri.
Department of Ophthalmology and Vision Science, Washington University in St. Louis, St. Louis, Missouri.
Ophthalmol Retina. 2019 Jun;3(6):510-515. doi: 10.1016/j.oret.2019.02.004. Epub 2019 Feb 15.
To determine the presence and to characterize location of retinal vascular lesions in patients with hereditary hemorrhagic telangiectasia (HHT).
Prospective cross-sectional pilot descriptive study.
Eighteen patients (age range, 22-65 years) with a clinical diagnosis of HHT.
Patients completed the 25-item National Eye Institute Visual Function Questionnaire and underwent a single study visit with dilated ophthalmic examination, OCT angiography (OCTA), and fluorescein angiography (FA) with widefield imaging.
Presence of retinal vascular abnormalities in 1 or more quadrants identified on widefield FA, Visual Function Questionnaire scores, retinal vessel architecture on FA and OCTA, and dilated ophthalmic examination findings.
Of the 18 patients recruited, fine telangiectatic vessels with capillary dilation and tortuosity were identified in 78% by FA imaging.
In the first FA and OCTA analysis of the retina of unrelated HHT patients, we found a high rate of temporal and nasal telangiectasias. These telangiectasias were more apparent in older patients, suggesting that they may appear in later stages of HHT development. No abnormalities of the macular vasculature and architecture were identified, explaining the generally well-preserved visual acuity. Temporal and nasal telangiectasias may have clinical significance in a patient's risk for retinal hemorrhage and likely warrant periodic surveillance by annual FA imaging.
确定遗传性出血性毛细血管扩张症（HHT）患者视网膜血管病变的存在情况并对其位置进行特征描述。
前瞻性横断面试点描述性研究。
18例临床诊断为HHT的患者（年龄范围22 - 65岁）。
患者完成25项美国国立眼科研究所视觉功能问卷，并接受一次扩瞳眼科检查、光学相干断层扫描血管造影（OCTA）以及宽视野成像荧光素血管造影（FA）的研究性检查。
宽视野FA检查发现1个或更多象限存在视网膜血管异常情况、视觉功能问卷得分、FA和OCTA检查的视网膜血管结构以及扩瞳眼科检查结果。
在招募的18例患者中，78%的患者通过FA成像发现有伴有毛细血管扩张和迂曲的细微毛细血管扩张性血管。
在对非亲属HHT患者视网膜进行的首次FA和OCTA分析中，我们发现颞侧和鼻侧毛细血管扩张的发生率很高。这些毛细血管扩张在老年患者中更明显，表明它们可能出现在HHT发展的后期阶段。未发现黄斑部血管系统和结构异常，这解释了患者视力通常保存良好的原因。颞侧和鼻侧毛细血管扩张可能对患者发生视网膜出血的风险具有临床意义，可能需要通过每年的FA成像进行定期监测。
