Geisthoff Urban W, Hille Konrad, Ruprecht Klaus W, Verse Thomas, Plinkert Peter K
Department of Otorhinolaryngology, University of the Saarland, Homburg/Saar, Germany.
Graefes Arch Clin Exp Ophthalmol. 2007 Aug;245(8):1141-4. doi: 10.1007/s00417-006-0520-2.
Hereditary hemorrhagic telangiectasia (HHT) is a systemic disorder of the fibrovascular tissue. Few data have been published on the frequency of ophthalmologic manifestations. The aim of this study was to assess this frequency.
A prospective observational cross-sectional study was conducted in a multitude of clinical practices and in a tertiary referral center. The main outcome measure was the number of vascular malformations detected by an ophthalmologic inspection of the retina and the conjunctivae in both eyes in a random sample of patients with HHT.
No retinal telangiectases were observed in any of the 75 patients (150 eyes) examined. Conjunctival telangiectases were detected in 28 of 74 patients (47 of 148 eyes).
The prevalence of retinal telangiectases seems to be lower than 1/75 (< 1.3%). This data does not justify screening procedures to detect retinal telangiectases. Ophthalmologists should be aware of the symptoms of HHT and its systemic character.
遗传性出血性毛细血管扩张症（HHT）是一种纤维血管组织的全身性疾病。关于眼科表现的发生率，已发表的数据很少。本研究的目的是评估这一发生率。
在多个临床机构和一家三级转诊中心进行了一项前瞻性观察性横断面研究。主要观察指标是在HHT患者随机样本中，通过对双眼视网膜和结膜进行眼科检查所发现的血管畸形数量。
在检查的75例患者（150只眼）中，未观察到任何视网膜毛细血管扩张。在74例患者中的28例（148只眼中的47只）检测到结膜毛细血管扩张。
视网膜毛细血管扩张的患病率似乎低于1/75（<1.3%）。该数据不足以支持进行检测视网膜毛细血管扩张的筛查程序。眼科医生应了解HHT的症状及其全身性特征。
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