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Photographic montages of the ocular fundus of patients of various ages with gyrate atrophy. In these black-and-white photographs, the normally reddishbrown retina appears dark gray while the atrophic areas are light gray or white. The area covered by the montage, and thus themagnification of these
reproductions, is different in each patient and can be judged roughly by the diameter of the optic disc. The optic disc, with its accompanying retinal vessels,
is indicated in each montage with a “d.” A, The right eye of a 6-year-old girl. There are isolated lesions in the mid- and far-periphery and circumferential
diffuse thinning of the pigment in the midperiphery.258 B, The left eye of a 14-year-old girl. The atrophic lesions are more prominent than in A and have
coalesced. The posterior pole remains normal in appearance. C, The left eye of a 34-year-old female with much more extensive atrophy than B. The
Source: The Hyperornithinemias, The Online Metabolic and Molecular Bases of Inherited Disease
circumferential zone of coalesced atrophic lesions now involves most of the retina. In addition, there is a central area of atrophy surrounding the optic disc,
Valle D,
Beaudet
Vogelstein B, Kinzler
Antonarakis
Ballabio A, Gibson
K, Mitchell
G.chorioretinal
The Online Metabolic
andscattered
Molecular
leaving only Citation:
a thin circular
remnant
of AL,
normal-appearing
retina. KW,
D, The
left eye ofSE,
a 62-year-old
male with
complete
atrophy with
Bases
of
Inherited
Disease;
2014
Available
at:
http://mhmedical.com/
Accessed:
May
05,
2017
clumps of pigment. (All montages provided by Muriel Kaiser-Kupfer, M.D., National Eye Institute, Bethesda, MD.)
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