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Prevalence of ocular manifestations in hereditary hemorrhagic telangiectasia
Urban W Geisthoff1, Konrad Hille, Klaus W Ruprecht
1Department of Otorhinolaryngology, University of the Saarland, Homburg/Saar, Germany. urban.geisthoff@uniklinik-saarland.de
Background:
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.
Methods:
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.
Results:
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).
Conclusions:
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.
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