Sickle cell retinopathy can be defined as retinal changes due to blood vessel damage in the eye of a person with a background of sickle cell disease. It can likely progress to loss of vision in late stages due to vitreous hemorrhage or retinal detachment. Sickle cell disease is a structural red blood cell disorder leading to consequences in multiple systems. It is characterized by chronic red blood cell destruction, vascular injury, and tissue ischemia causing damage to the brain, eyes, heart, lungs, kidneys, spleen, and musculoskeletal system.People affected by sickle cell disease are commonly of African or Asian descent. Emigration patterns towards the Western Hemisphere have led to increased numbers of persons affected by sickle cell disease in regions where it was previously uncommon. Knowledge and understanding of sickle cell disease and its management are now increasingly relevant in areas such as the European Union. At a young age, a great proportion of people living with sickle cell disease can develop retinal changes. Sickle cell disease consists of several subtypes; however, the Haemoglobin type C (HbSC) subtype carries the gravest prognosis for sickle cell retinopathy and vision changes.Regular retinal examinations can aid in early detection and treatment, thus reducing the impact of the condition and the risk of vision loss. Development and progression of sickle cell retinopathy can be favorably modified through management of the underling sickle cell disease. Treatment of the general disease can ameliorate its systemic effects. Classification
Sickle cell retinopathy can be classified based on retinal changes into non-proliferative and proliferative subtypes.
Visual acuity and intraocular pressure assessments can also yield results that are diagnostic of sickle cell retinopathy. Differential diagnosis
Sickle cell retinopathy should be differentiated from other retinal conditions that arise due to similar pathologies. The following differentials are classified by those pathologies. Additional causes of macular ischemiaDiabetic retinopathy
Retinal embolization (talc retinopathy, internal carotid artery embolism)
Retinal vascular occlusion
Infectious retinopathyInflammatory diseases leading to ischemiaSarcoidosis
Retinal vasculitis
Intermediate uveitis
Acute retinal necrosisAlternative causes of ischemic vascular diseaseProliferative diabetic retinopathy
Central retinal vein occlusion
Branch retinal vein occlusion
Ocular ischemic syndromE
Radiation retinopathy
Retinopathy of prematurityUnclassified causesIncontinentia pigmenti
Autosomal dominant vitreoretinochoroidopathy
Chronic rhegmatogenous retinal detachment
Scleral buckle
Retinitis pigmentosa

Prevention
Screening
Screening is an important pillar of prevention of sickle cell retinopathy as it provides the benefit of early detection and treatment thus reducing complications. Screening also provides an opportunity for education of families predisposed to retinopathy as a result of a family history of sickle cell disease. Screening can take the form of DNA screening at birth and Ophthalmology-related examinations later in life. DNA screening in newborns at risk of having sickle cell disease allows the early diagnosis of the disease. Families can then be educated on the possible effects of sickle cell disease at each stage of life. Ophthalmology-related examinations - dilated fundoscopic examination is recommended from the age of 10 and biannually thereafter in patients with no abnormal retinal findings. Fluorescein angiography and angioscopy is recommended for patients with abnormal findings that may be indicative of proliferative sickle cell retinopathy. The frequency of follow up appointments is determined by the ophthalmologist based on the severity of the findings.