ANGIOID STREAKS ASSOCIATED WITH SICKLE-CELL ANEMIA

Judy Lo

Abstract

BACKGROUND: Angioid streaks are breaks in Bruch's membrane with secondary changes in the retinal-pigmentedepithelium and choriocapillaris. They are usually bilateral and the coloration of the streaks depends on the pigmentation of the fundus. A high risk of macular choroidal neovascularization may occur with the streaks, causing the streaks to appear gray/green in color. Angioid streaks tend to radiate out from the optic disc to the periphery, giving the appearance of a cracked eggshell. They are a rare disorder having the most prevalent systemic associations with pseudoxanthoma elasticum, Ehlers-Danlos syndrome, Marfan's syndrome and Paget''s disease of the bone. Angioid streaks have also been associated with sickle cell hemoglobinopathies with an incidence of one to two percent.

CASE REPORT(S). A 55-year old black female with sickle-cell anemia Hb SS) presented with a request for a second opinion on a "retinal tear," diagnosed nine months prior. She complained that she was still seeing flashes of light. Her history included joint pain and renal dialysis secondary to her sickle cell disease. Her best corrected visual acuities were 20/20 in the right eye and 20/20 in the left eye. A dilated fundus examination revealed angioid streaks in both eyes and an operculated retinal hole infero-temporally in the left eye. There was no choroidal neovascular membrane noted. No other signs of sickle-cell retinopathy were found. The patient was referred out for possible laser treatment of the retinal hole.

CONCLUSIONS. Although angioid streaks are rare sequelae of sickle cell disease, the clinician should be cognizant of the relationship between the two due to the potential severity of sickle cell disease. The clinician should be aware of the ocular appearance of angioid streaks due to the possible sight-threatening complication of subretinal choroidal neovascular membrane formation.

Details

Year: 2001

Program Number: Poster 84

Author Affiliation: State University of New York

Co-Authors: Purvi Shah, Richard Madonna

Co-Author Affiliation: State University of New York, State University of New York

Room: Exhibit Hall C