Isolated Macular Infarction in Sickle Cell (Ss) Disease

Abstract

A 10-year-old black girl complained of a painless decrease of vision in her left eye over a twoto three-week period. She had become icteric about the time that the vision decreased. Sickle cell disease was diagnosed when this patient was four years of age; thus it was probable that she was having a sickle cell crisis. Hemoglobin electrophoresis at San Francisco Children's Hospital when she was four years of age showed 99% Hb S, and 1% Hb A, therefore, the patient is homozygous S, (sickle cell SS disease). Past eye history included a strabismus operation on the right eye at the age of nine years for a right esotropia present since the age of three or four years. She had never been hospitalized for sickle cell crises. Best corrected vision prior to the present illness was RE: 20/60, LE: 20/20. Her present vision was RE: 20/60, LE: 20/100. There was a variable left esotropia of 1 to about 40 prism diopters. The bulbar conjunctiva appeared icteric, and the conjunctival vessels exhibited sludging and commas. The retina of the left eye revealed an atrophie, beaten silver appearance in the macular area, and the blood vessels appeared ophthalmoscopically normal without significant tortuosity. There was no other evidence of retinopathy, except for some slight distortion of the foveal light reflex in the right macula. The periphery of both retinas was checked with the indirect ophthalmoscope and showed no abnormality. The child was not coopera­ tive enough for a formal tangent screen examina­ tion, but she described a central scotoma.