Ocular findings in sickle cell disease patients in Lagos.
ABSTRACT 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: The pathophysiology of sickle cell disease is not limited to abnormal red blood cells. The clinical manifestations of sickle cell disease include complex pathways and processes such as endothelial activation, inflammation, bioavailability of nitric oxide, oxidative stress, and the adhesiveness of a variety of blood cells. Increasingly, distinct subphenotypes and genetic modifiers of sickle cell disease are being recognized. We apply recent advances in sickle cell disease to ocular biology to highlight translational research in this field and encourage additional studies on the ocular manifestations of sickle cell disease.Survey of Ophthalmology 05/2010; 55(4):359-77. · 2.86 Impact Factor
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ABSTRACT: To identify retinal manifestations in patients with sickle cell disease referred to a reference eye hospital in Goiânia (GO). Ophthalmic evaluation was made in 50 patients (100 eyes) with sickle cell disease to evaluate the most common manifestations of this group. Hemoglobinopathy SS was the most commonly found, followed by hemoglobin SC, AS and Stahl. Twenty-two percent of the patients had retinal changes, of these 73% were male. Retinal changes observed were: "sea fan", "black sunburst", vitreous hemorrhage, and retinal detachment. In the classification of retinopathy, 73% had proliferative form, seen in the types AS and SC and 27% had non-proliferative retinopathy, seen in patients with SS type. We observed a large numbers of patients with retinal changes, most of them with hemoglobinopathy SC, followed by AS and SS groups. The proliferative changes were the most commonly observed. Vitreous hemorrhage and retinal detachment were the most prevalent manifestations in proliferative retinopathy and showed to be more common in patients with SC hemoglobinopathy in the studied population.Arquivos brasileiros de oftalmologia 10/2011; 74(5):335-7.
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ABSTRACT: BACKGROUND: Sickle cell disease (SCD) is characterised by occlusion of small blood vessels. This study aimed to assess retinal changes in patients with SCD and its correlation with time-averaged mean flow velocity (TAMV) in middle cerebral arteries (MCA) and ophthalmic arteries (OA). METHODS: Sixty SCD patients (aged 3-18 years) attending a paediatric hospital in Cairo, Egypt, during March 2010 to November 2011, were compared with 30 healthy controls. All underwent clinical and fundus examination by indirect ophthalmoscopy, and assessment of TAMV in MCAs and OAs by transcranial Doppler, repeated 1 year later for those with conditional velocities. RESULTS: HbS/β was diagnosed in 32 patients and HbSS in 28; 50 patients had normal fundus and 10 had bilateral non-proliferative retinopathy. Risk factors for retinopathy included HbSS, age, previous stroke, non-compliant hydroxyurea (HU) therapy, frequency of sickling crises and HbS level. TAMVs were increased in MCAs, but not in OAs, in sicklers. TAMVs in MCAs and OAs increased with non-compliant HU therapy, previous stroke, age, frequency of sickling crises and level of HbS. No significant interhemispheric difference was found. CONCLUSION: Sickle retinopathy was correlated with TAMV in MCAs but not in OAs. A significant difference was found between initial and follow-up TAMVs in the MCAs, after 1 year of regular HU and transfusion therapy, in those with conditional velocities.Transactions of the Royal Society of Tropical Medicine and Hygiene 02/2013; · 1.82 Impact Factor