Ocular findings in sickle cell disease patients in Lagos

Department of Surgery, College of Medicine, University of Lagos, P. M. B. 12003, Idi-Araba, Lagos.
The Nigerian postgraduate medical journal 10/2004; 11(3):203-6.
Source: PubMed


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.

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    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% [1]. 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 [2]. 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 [14]. 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.
    Full-text · Article · Jan 2012
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    ABSTRACT: A cross-sectional and descriptive study was carried out to assess the ocular manifestations of drepanocytemia in 64 adult patients seen in Hemoglobinopathy of "Dr. Gustavo Aldereguía Lima" University Hospital. The aims of present paper were: to assess the ophthalmologic alterations frequency related to clinical-hematological parameters and to estimate its functional consequences. A basal total ophthalmologic examination was performed. For data analysis descriptive statistical models, mean comparisons and the Chi2 test. The decimal point sign was the more frequent alteration associated with SS genotype and with lower hemoglobin levels. Vessel tortuous was the commonest finding in retina in all genotypes, whereas the vitreous body opacities and the black cupping in sun shape showed a statistically significant association in some cases. A non-proliferative retinopathy was diagnosed in most of study patients and in some of them the findings of both types of retinopathy were showed. Despite the numerous lesions showed, visual acuity was qualified as good in the 94,6% of study eyes.
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