Fundus findings in Leber's hereditary optic neuroretinopathy. III. Fluorescein angiographic studies
E. Nikoskelainen, W. F. Hoyt, K. Nummelin and H. Schatz
Fluorescein angiography was performed in nine members of families with
Leber's disease. Serial studies were obtained in four men observed from
asymptomatic to atrophic stages. Peripapillary microangiopathy was observed
in six of nine asymptomatic eyes. Arteriovenous shunting occurred in the
telangiectatic vascular bed. These changes remained stable in some eyes and
progressed in others. In the acute stage, arteries and telangiectatic
vessels were maximally dilated and flow was rapid. Angiography showed
florid shunting in lower and upper vascular arcades and reduced filling of
papillomacular capillaries. Vessels of the shunting vascular bed gradually
narrowed irregularly. In the atrophic stage, disc vascularity diminished
and arteriovenous circulation time increased markedly. Arterioles narrowed
and peripapillary microangiopathy disappeared. These findings support our
contention that Leber's disease is a hereditary vascular neuroretinopathy.
