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June 1965

Mottled Fundus Oculi in Pseudoxanthoma ElasticumA Report on Two Siblings

Author Affiliations

Both from the Wilmer Ophthalmological Institute Baltimore. Training Grant No. ERG N 2204 A 64 (Dr. Gills).

Arch Ophthalmol. 1965;73(6):792-795. doi:10.1001/archopht.1965.00970030794007

Introduction  This paper reports two cases of pseudoxanthoma elasticum with diffuse mottling of the fundus oculi. The occurrence of a mottled fundus in this disease is rare, but of a characteristic appearance. Pseudoxanthoma elasticum (PXE) with mottled fundi has been reported in the foreign literature2,3,7,10,13 but has not been emphasized in papers in this country. Not infrequently, this characteristic fundus mottling is noted in eyes with angioid streaks; but it is rarely the predominant change, as it is in the cases presented here.

Report of Cases 

Case 1.  —A boy, age 12, was admitted to the Wilmer Institute on Oct 22, 1962, after having been struck in the right eye with a rock and sustaining traumatic hyphema. It is of note that at age 7 he had a well-documented case of typical rheumatic fever, followed by recurrent attacks. About the time of onset of rheumatic fever, the parents first

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