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August 1966

Angioid Streaks in Ehlers-Danlos Syndrome

Author Affiliations

Bethesda, Md
From the Ophthalmology Branch, National Institute of Neurological Diseases and Blindness (Dr. Green), and the Dermatology Branch (Dr. Friedman-Kien) and Laboratory of Pathology (Dr. Banfield), National Cancer Institute, National Institutes of Health, Public Health Service, US Department of Health, Education and Welfare, Bethesda, Md. Dr. Green is presently at the Armed Forces Institute of Pathology, Walter Reed Army Medical Center, Washington, DC, and Dr. Friedman-Kien is at the Skin and Cancer Unit, New York University Medical Center, New York.

Arch Ophthalmol. 1966;76(2):197-204. doi:10.1001/archopht.1966.03850010199009

Various ocular abnormalities have been observed in patients with the Ehlers-Danlos syndrome (ED). In a review of personal cases McKusick1 found that epicanthal folds were the most common associated ocular abnormality. Other less frequent ocular changes occurring in this disease were strabismus,2-6 blue sclerotics,6-12 microcornea,9,13 myopia,6,13 keratoconus,11,12,14 ectopia lentis,11,15 hypertelorism,16 and intraocular hemorrhage.17 Bonnet7 described depigmentation in the preequatorial region of the fundus. Retinal detachment and retinitis proliferans in one eye, and macular degeneration in the other eye, were observed in a 19-year-old girl with ED.8 Angioid streaks were seen in patients who were affected with pseudoxanthoma elasticum and ED,4,18 but the occurrence of angioid streaks in patients with ED alone has not been reported, to our knowledge.

In the present paper the ocular changes observed in the Ehlers-Danlos syndrome will be described in 15 members of two