Sight Threatening Retinopathy in an Eight Year Old Nigerian Male With Sickle Cell β° Thalassaemia

Sight threatening changes in the retina are a well-recognized complication of sickle cell disease (SCD). However they usually occur in older patients with Haemoglobin SC or Sβ⁺thal patterns. It is rarely found under the age of 20 years in patients who are Hb SS or Sβ°thal. This is a report of sight threatening retinopathy in an 8-year-old male Nigerian patient with Sβ°thal-one of the youngest reported cases to our knowledge. The patient had been diagnosed at birth and had his first ophthalmic examination done at 6 years of age when he developed an acute cerebral syndrome with transient blindness and hemiplegia. Retinal examination at that time was normal. In the subsequent years, he had several episodes of vaso-occlusive crisis including renal papillary necrosis. Two years later despite minimal visual symptoms, he had developed abnormal conjunctival vessels and bilateral retinopathy. He had high levels of Hb F and irreversibly sickled cells (ISC). Four years after treatment with Argon photocoagulation, he has developed no further neo-vascularization and his visual acuity remains normal with correction. As sight-threatening retinopathy could occur even in children, there is need for early detection and treatment in patients with SCD to prevent progression of lesions. Hence, a yearly examination is recommended for children, irrespective of age or electrophoretic pattern of patient. Another option would be to screen high-risk patients- those with frequent vaso-occlusive crisis, and high ISC counts.