Sickle cell disease has varied manifestations in all systems of the body, ocular manifestations can be severe and sudden blindness may result amidst other complications. The study aims at determining the ocular findings in patients with sickle cell anaemia (HbSS) and sickle cell haemoglobin C disease (HbSC). 99 subjects were recruited into the study carried out at the Lagos University Teaching Hospital over a 2 year period. A structured interview questionnaire was administered and physical examination was carried out at the Medical Out Patients' Sickle Cell Clinics. Subsequently in the (Guinness) Eye centre, examination for ocular manifestations was carried out using Snellen's chart, Slit light microscopy, direct and indirect ophthalmoscopy under mydriasis (with Gutt Tropicamide 1% , epinephrine 10% ) as well as tonometry and gonioscopy. The age of the 99 (51 males, 48 females) patients ranged from 13 to 43 years with a mean +/-SD of 20.42 +/- 6.13. There were 88 (88.9% ) SS and 11 (11.1% ) SC patients. The male to female ratio was 1.1: 1. Normal vision was present in 95 (96% ) of the patients [86 (97.8% ) SS and 9 (81.8% ) SC] while 4 (4.0% ) of the patients [2 (2.2% ) SS and 2 (18.2% ) SC] had impaired vision. Of the complaints, blurred vision was found in 12 (13.6% ) SS and one (9.0% ) SC patients. Itching of the eyes was found in 15 (17.0% ) SS and 3 (27.3% ) SC patients. Icterus was found in 50 (50.5% ), conjunctival signs were found in 49 (49.5% ), retinal vascular tortuosity was in 12 (12 .10% ), peripheral retinal degeneration was in 5 (5% ), iridescent spots was in 3 (3% ) and optic atrophy in 2 (2% ) patients. The tortuosity of major fundus vessels were graded into 4 according to Hook and Cooper's criteria 87 (87.9% ) of the patients were in grade 0. The conjunctival vessels anomalies were classified into 5 grades (criteria of Sergeant). 37 (37.5% ) were in grade 0, 35 (35.3% ) in grade 1, 8 (8.1% ) in grade 2, 7 (7.1% ) in grade 3 and 12 (12.1% ) in grade 4. Conjunctival abnormalities occur commonly while proliferative retinopathy and optic atrophy were uncommon in sickle cell disease patients in Lagos. Further studies are required with a larger number of subjects to investigate the low incidence of proliferative retinopathy and optic atrophy in Lagos.
[Show abstract][Hide abstract] ABSTRACT: Citation: Oluleye TS (2012) Pattern of Presentation of Sickle Cell Retinopathy in Ibadan. J Clin Exp Ophthalmol 3:257. Introduction The prevalence of the hemoglobin S gene in Nigeria is between 20 and 25% . About 25% of adult's population in Nigeria have the sickle cell trait, AS, while the Hb C trait is largely confined to the Yoruba people of southwestern Nigeria in whom it occurs in about 6%. Other variant hemoglobin including beta thalassemia is rare, but alpha thalassemia occurs in 39% (32% with 3 alpha-globin genes; 7% with 2 alpha-globin genes . In Nigeria, HbSC produces most of the retinopathic changes with previous studies showing patient's presentation at the late stages of the disease [3-13]. In the presence of oxidative stress, the red blood cells of sickle cell patients take on a sickle shape instead of the normal bi-concave disc. They become trapped in the small vessels leading to ischemia, hypoxia, and tissue necrosis. The hypoxia leads to more sickling and a cycle is created. SS patients have the worst systemic complications while SC and Sthal patients have the most severe ocular problems. This is because the blood is more viscous in the latter group as small retinal arterioles occlude more easily. Pre-proliferative retinal findings include salmon-patch hemorrhages and black sunburst pigments. These are retinal pigment epithelial layer reaction to hemorrhage and choroidal infarction . Others are intraretinal refractile bodies, and silvering of peripheral arterioles. Proliferative findings include sea-fan neovascularization, vitreous hemorrhages, and retinal detachments. Other associated findings include venous tortuosity, retinal holes, Central retina artery occlusion, and angioid streaks. Treatment consists of pan retina laser photocoagulation (PRP) or cryotherapy applied to the peripheral areas of retinal ischemia, usually anterior to the sea fans. Treatment will lead to involution of the new vessels due to blockage of their feeder vessels. Recently, antiVEGF agents have proven to be useful in the treatment of sickle retinopathy. They block VEGF thereby causing involution of sea fans, helped Abstract Background: Sickle cell retinopathy is not uncommon in Nigeria. Most cases of retinopathy occur in patients with the hemoglobin SC genotype. A significant proportion present late.
[Show abstract][Hide abstract] ABSTRACT: 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.
Annals of Tropical Paediatrics International Child Health 03/2010; 30(1):19-26. DOI:10.1179/146532810X12637745451870 · 1.17 Impact Factor
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