Fadugbagbe A O, Gurgel R Q, Mendonça C Q, Cipolotti R, dos Santos A M, Cuevas L E
Liverpool School of Tropical Medicine, UK.
Ann Trop Paediatr. 2010;30(1):19-26. doi: 10.1179/146532810X12637745451870.
Sickle cell disease (SCD) is the most common genetic disease worldwide. The increase in life expectancy of SCD patients in recent years has led to the emergence of more complications of the disease, e.g. ocular, which in the past were uncommon. This review describes current knowledge of the ocular manifestations of patients with SCD. SCD can affect virtually every vascular bed in the eye and can cause blindness in the advanced stages. The most significant ocular changes are those which occur in the fundus, which can be grouped into proliferative sickle retinopathy, and non-proliferative retinal changes based on the presence of vascular proliferation. This distinction is important because the formation of new vessels is the single most important precursor of potentially blinding complications. Although various systemic complications of SCD are known to be more common in patients with the Hb SS genotype, visual impairment secondary to proliferative sickle retinopathy is more common in patients with the Hb SC genotype. There is also an increase with age in the incidence and prevalence rates of all ocular complications of SCD. It is therefore recommended that all patients with SCD undergo periodic ophthalmological screening from the age of 10 years.
镰状细胞病（SCD）是全球最常见的遗传性疾病。近年来，SCD患者预期寿命的增加导致该疾病出现了更多并发症，例如眼部并发症，而这些并发症在过去并不常见。本综述阐述了目前对SCD患者眼部表现的认识。SCD几乎可影响眼部的每个血管床，并在疾病晚期可导致失明。最显著的眼部变化发生在眼底，根据血管增殖情况可分为增殖性镰状视网膜病变和非增殖性视网膜病变。这种区分很重要，因为新血管的形成是潜在致盲并发症的最重要单一先兆。虽然已知SCD的各种全身并发症在Hb SS基因型患者中更为常见，但增殖性镰状视网膜病变继发的视力损害在Hb SC基因型患者中更为常见。SCD所有眼部并发症的发病率和患病率也随年龄增长而增加。因此，建议所有SCD患者从10岁起定期进行眼科筛查。
