Proliferative sickle cell retinopathy under age 20: a review

Of the 256 sickle patients with hemaglobinopathies SC, SS and S-thal referred to the Wills Eye Hospital Retina Vascular Service from 1966 through 1983, 64 (50%) of the 128 males and 71 (55%) of the 128 females were less than 20 years old. Proliferative sickle retinopathy (PSR) was documented by fluorescein angiography in five of the males (8%) and two of the females (3%) under age 20. Included in this series is a 13-year-old black girl with homozygous sickle cell anemia (HbSS), to our knowledge the youngest reported case of PSR in an SS patient. This report emphasizes the importance of ophthalmic examinations in teenage sickle patients to detect and treat proliferative disease.