Reports of cases of choroideremia are still sufficiently rare to warrant the publication of an additional one. As the name implies, choroideremia is characterized by an almost total absence of choroid. The fundus picture is striking. A glaring white or greenish white sheen is reflected from the interior of the eye. One sees the almost naked sclera, made evident by the defection of the choroid and the pigment epithelium of the retina. In the macular region alone is the normal reddish coloration retained; here an island of choroidal circulation bravely withstands the surrounding dissolution, with the end that central vision is preserved. A few attenuated remnants of the choroidal vascular system are to be seen; these run a straight and lonely course. Here and there small stellate or cuneiform clumps of pigment are found in the retinal strata. The retinal circulation is unimpaired, although the vessels are somewhat thinner than
FRIEDMAN B. CHOROIDEREMIA. Arch Ophthalmol. 1940;23(6):1285–1287. doi:10.1001/archopht.1940.00860131445017
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