Iris atrophy in sickle cell disease.

Abstract

Iris atrophy, of unknown origin and believed to be secondary to the vaso-occlusive process of sickle cell disease, has been observed in 25 eyes of 22 patients (two SS disease, 20 SC disease). The crude prevalence was highest in males with SC disease, in whom 14.7% of patients were affected. Iris atrophy was closely associated with proliferative sickle retinopathy in the same eye. Analysis of haematological indices failed to reveal any significant differences between patients with and without iris atrophy. The characteristics and distribution of iris atrophy are described as well as the histopathology in one 68-year-old male patient with SS disease.

Ocular findings in sickle cell patients on Curaçao

“…The percentage of patients with iris atrophy we found was somewhat higher than the one found by Acheson et al [10]: 8% and 4% respectively. Our results confirm a strong correlation between iris atrophy and proliferative retinopathy, mostly in Hb SC patients.…”

An African American Man With Progressive Loss of Iris Pigmentation in Both Eyes

“…4 Iris atrophy is seen in only approximately 2% of eyes without proliferative sickle cell retinopathy. 5 Anterior segment fluorescein angiography (choice A) would be reasonable if the patient had confirmed sickle cell disease or another systemic disease that would predispose him to anterior segment ischemia. However, sickle cell testing (choice C) would be a better initial next step because a negative test result renders choice A unnecessary.…”

“…Iris atrophy is most common in the sickle cell blood type and highly associated with proliferative sickle cell retinopathy . Iris atrophy is seen in only approximately 2% of eyes without proliferative sickle cell retinopathy . Anterior segment fluorescein angiography (choice A) would be reasonable if the patient had confirmed sickle cell disease or another systemic disease that would predispose him to anterior segment ischemia.…”

Sickle cell retinopathy: diagnosis and treatment

“…Ocular findings are represented by orbital, conjunctival (1517) , uveal (18) , papillary and retinal (19) alterations. The most relevant anterior segment abnormalities are the conjunctival sign and iris atrophy, both of which are diagnostic indicators of sicklecell disease, and hy phema, for which there exists a particular therapeutic approach in these patients (17,20,21) .…”