Prevalence of ocular manifestations in hereditary hemorrhagic telangiectasia.

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.