Hereditary hemorrhagic telangiectasia with multiple fusiform retinal aneurysms

Uğurlu, Nagihan; Emre, Selma; Yülek, Fatma; Akçay, Emine; Şengün, Ahmet

doi:10.1007/s12177-013-9100-y

Cited by 1 publication

(1 citation statement)

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“…18 Prior studies have shown rates of retinal lesions varying from 0 to 10% [3][4][5] in all patients, with no consistent topographic location of these lesions. Two prior case series of patients with HHT also reported fine telangiectic capillaries and tortuous arteriovenous malformations, 19,20 although these were found in a minority of study subjects. The authors in one of these studies 3 acknowledged that subtle retinal vascular alterations may have been overlooked in the remaining subjects that did not have fundoscopic abnormalities and were consequently not examined by FA.…”

Section: Discussionmentioning

confidence: 78%

Identification of Retinal Vascular Lesions Using Ultra-Widefield Angiography in Hereditary Hemorrhagic Telangiectasia Patients

Sindhar

O’Bryhim

Licata

et al. 2019

Ophthalmology Retina

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Purpose: To determine the presence and characterize location of retinal vascular lesions in patients with hereditary hemorrhagic telangiectasia. Design: Prospective cross-sectional pilot descriptive study. Participants: Eighteen patients (age 22 to 65) with a clinical diagnosis of hereditary hemorrhagic telangiectasia. Methods: Patients completed the Visual Function Questionnaire-25 and underwent a single study visit with dilated ophthalmic examination, optical coherence tomography angiography (OCTA) and fluorescein angiography (FA) with widefield imaging. Main Outcome Measures: Presence of retinal vascular abnormalities in 1 or more quadrants identified on widefield FA, VFQ-25 scores, retinal vessel architecture on FA and OCTA, and dilated ophthalmic exam findings. Results: Of the 18 patients recruited, fine telangiectatic vessels with capillary dilation and tortuosity were identified in 78% by FA imaging. Conclusions: In the first FA and OCTA analysis of the retina of unrelated HHT subjects, we found a high rate of temporal and nasal telangiectasias. These telangiectasias were more apparent

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