Black Sunburst Research Articles

Sickle cell disease retinopathy: characterization among pediatric and teenage patients from northeastern Brazil

ObjectiveThe aim of the present study was to characterize sickle cell disease retinopathy in children and teenagers from Bahia, the state in northeastern Brazil with the highest incidence and prevalence of sickle cell disease.MethodsA group of 51 sickle cell disease patients (36 hemoglobin SS and 15 hemoglobin SC) with ages ranging from 4 to 18 years was studied. Ophthalmological examinations were performed in all patients. Moreover, a fluorescein angiography was also performed in over 10-year-old patients.ResultsThe most common ocular lesions were vascular tortuosity, which was found in nine (25%) hemoglobin SS patients, and black sunburst, in three (20%) hemoglobin SC patients. Peripheral arterial closure was observed in five (13.9%) hemoglobin SS patients and in three (13.3%) hemoglobin SC patients. Arteriovenous anastomoses were present in six (16.5%) hemoglobin SS patients and six (37.5%) hemoglobin SC patients. Neovascularization was not identified in any of the patients.ConclusionsThis study supports the use of early ophthalmological examinations in young sickle cell disease patients to prevent the progression of retinopathy to severe disease and further blindness.

Alterações retinianas em jovens portadores de anemia falciforme (hemoglobinopatias) em hospital universitário no nordeste do Brasil

To describe and categorize retinal vascular changes in patients with sickle cell anemia, as well as to compare diagnostic methods (indirect ophthalmoscopy and fluorescein angiography). Patients with sickle cell anemia over the age of seven were examined. Complete ophthalmologic examination with indirect ophthalmoscopy and angiography was performed in each patient. The fundoscopy results were grouped in 3 classes: normal; non-proliferative retinopathy, which includes vascular tortuosity, black sunburst, salmon-patch and peripheral closure/anastomoses; and proliferative retinopathy, related to neovascular proliferation. Angiography results were classified according to Goldberg classifications from stage I to V. Retinopathy related to sickle cell anemia was seen in 62.3% (38/61) of the eyes checked. Neovascularization was observed in one eye. The frequency of bilateral changes in angiography was high. Non-proliferative retinopathy was more common, especially vascular tortuosities (17/61), followed by arteriovenous anastomoses in the retinal periphery (15/61) and arterial occlusions (5/61). The mean age of retinopathy group was 14.4 years old, significantly lower than the mean age of non-retinopathy group, which was 17.4. The result was normal in 16.4% (10/61) of the eyes in the fundoscopy exam, while angiography showed alterations. All the results pointed to the conclusion that the non-proliferative retinal vascular changes are frequent and precocious in patients with sickle cell anemia (SS genotype). Fluorescein angiography is more sensitive in the diagnosis of retinopathy when compared to indirect ophthalmoscopy.

New optical coherence tomography fundus findings in a case of beta-thalassemia

Patients with beta-thalassemia may present with an acquired diffuse elastic tissue defect due to degeneration of elastic tissue along with vaso-occlusive findings in the retinal microvasculature. Here we report the case of a patient with granular-like accumulation presenting as black sunburst lesions detected by optical coherence tomography (OCT). A 38-year-old man with beta-thalassemia intermedia associated with angioid streaks complained of deterioration of vision in both eyes. Funduscopic examination revealed small, round, hyperpigmented lesions bilaterally. During the early and late phases of fluorescein angiography, granular hyperfluoresence was present, associated with pigment decompensation and mottled-like hypofluorescence. The main OCT finding was the presence of granuloid-like accumulations at the retinal pigment epithelium level. Granule penetration was also noticed at the photoreceptor layer, while isolated granuloid-like accumulations were found in the inner layers of the macula and choroid. In this case, the new OCT finding was the granular-like hyperpigmented accumulations in the macula located at the level of the retinal pigment epithelium. To the best of our knowledge, our OCT findings show for the first time granuloid-like accumulations representing black sunburst lesions.

Alterações retinianas apresentadas em pacientes portadores de hemoglobinopatia falciforme atendidos em um Serviço Universitário de Oftalmologia

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.

Alterações oculares e eletrorretinográficas na doença falciforme

The primary objective of this case series was to get electroretinographic data in subjects with sickle cell disease. The secondary purpose was to identify ocular changes, their frequency and stage of evolution. 90 patients aged between 8 and 75 years were evaluated. They were subjected to complete ophthalmological examination, indirect ophthalmoscopy, retinography, angiography and electroretinography. Electroretinograms with amplitude of wave b less than 400 mW were considered subnormal. Prevalence study was conducted using the Chi-square test, P<0.001. The electroretinogram was subnormal in 27 (30%) of 90 patients, but of these, only 4 (4.4%) had proliferative retinopathy. The sign of comma in 95% of individuals, the retinal vascular tortuosity in 36.6% and black sunburst in 24.4% were the most frequent changes. No correlation between sickle cell disease and electroretinographics changes, as described in other studies, were noted. On the other hand, the ocular findings corroborate the previous researches.

Ocular lesions in sickle cell disease patients from Bahia, Brazil

OBJETIVO: O presente estudo teve como objetivo descrever as alterações oculares em pacientes portadores de doença falciforme, na Bahia, um estado do Nordeste, que tem a maior prevalência da doença no Brasil. MÉTODOS: Nós conduzimos um estudo de corte transversal em um grupo de 146 (292 olhos) pacientes com Doença Falciforme (90 HBSS e 56 HBSC). Para exame oftalmológico foi realizada oftalmoscopia binocular indireta complementada pela retinografia fluorescente para detecção de lesões retinianas decorrentes da Doença Falciforme. RESULTADOS: As lesões [...]

Achados fundoscópicos em crianças portadoras de anemia falciforme no estado do Rio Grande do Norte

Objetivos: Detectar os principais achados fundoscopicos em criancas portadoras de hemoglobinopatias falciformes. Metodos: Foram estudados 26 pacientes com hemoglobinopatias falciformes, no Servico de Oftalmologia do Hospital Universitario Onofre Lopes, Natal, RN, que foram submetidos a protocolo de pesquisa pre-estabelecido. Os resultados foram avaliados estatisticamente pelo teste qui-quadrado. Resultados: A idade media foi de 10,6 anos, com acuidade visual igual ou melhor que 20/25 na maioria, excetuando-se 3 olhos, que apresentavam outras doencas associadas. O tipo mais frequente foi o SS com 57,7% (15/26) dos casos, seguido pelos SC e SA com 15,4% (4/26) cada, e pelo S-Thal com 11,5% (3/26). A frequencia da retinopatia por celulas falciformes foi maior apos os 10 anos de idade, sendo mais frequente, em valores relativos, no tipo S-Thal (100% dos casos) e, em valores absolutos, no tipo SS (9 casos). Os dois achados mais comuns foram tortuosidade venosa (12/26) e black sunburst (7/26). Conclusoes: Observamos que a incidencia de retinopatia por celulas falciformes aumentou apos os 10 anos de idade e nao evidenciamos achados da doenca proliferativa. Portanto, enfatizamos a necessidade do exame oftalmologico precoce nos portadores de anemia falciforme, como forma de prevenir futuras complicacoes oculares.

Influência do genótipo da hemoglobinopatia falciforme nas manifestações retinianas em pacientes de um hospital universitário

Objetivo: Identificar as alterações retinianas em pacientes com hemoglobinopatia falciforme e comparar sua prevalência nos diferentes genótipos da doença em uma população de pacientes de hospital universitário de Porto Alegre - Brasil. Métodos: Realizou-se exame oftalmológico com avaliação fundoscópica e estudo do genótipo de pacientes portadores de hemoglobinopatia falciforme. Resultados: Foram examinados 94 olhos de 47 pacientes com doença falciforme: 17 pacientes do sexo masculino e 30 feminino, com média de idades de 22,4 anos (± 16,8 anos). 20 pacientes possuíam genótipo SS, 10 possuíam SC, 10 possuíam AS, e 7 SThal. As alterações retinianas encontradas foram: hemorragia tipo "salmon patch" em 1 olho, hiperpigmentação tipo "black sunburst" em 12 olhos, neovascularização tipo "sea fan" em 3 olhos, hemorragia vítrea em 1 olho e descolamento de retina em 1 olho. O grupo SC foi o que demonstrou ter maior freqüência de alterações (60%), e quando comparado com o grupo SS mostrou 4,0 vezes mais chance de apresentar retinopatia (p<0,05).Todos os 5 olhos com retinopatia proliferativa possuíam genótipo SC. Conclusões: A freqüência de alterações fundoscópicas no grupo estudado bem como a gravidade das alterações (retinopatia proliferativa) são maiores nos pacientes heterozigotos de genótipo SC, quando comparados com os demais genótipos.

Retinabefunde bei hereditärer Persistenz fetalen Hämoglobins (HPFH) mit Glucose-6-phosphat-Dehydrogenase-Mangel (G-6-PD-Mangel)

Peripheral arteriolar occlusions of the retinal vessels and the 'black sunburst sign' as the first stage of sickle-cell retinopathy (Goldberg 1976) were found in a 34-year-old black patient from West Africa with hereditary persistence of fetal hemoglobin (HPFH) and glucose-6-phosphate dehydrogenase deficiency (G-6-PD-D). The patient's son was found to have HPFH without G-6-PD-D, his daughter HPFH as well as G-6-PD-D. Ophthalmoscopic findings in the two children were normal. In the mother - a white German without clinical symptoms - neither HPFH nor G-6-PD-D were found. This is the first description of the typical picture of 'sickle-cell retinopathy' in a patient with HPFH combined with G-6-PD-D. HPFH is not associated with any retinal symptoms or hematological abnormalities. Hemolytic crises induced by fava beans or drugs in G-6-PD-D alone lead to different ocular complications from those found in this patient.

Posterior Pole Neovascularization in a Patient with Hemoglobin SC Disease

A 33-year-old black woman with hemoglobin SC disease and a history of photocoagulation for peripherally located retinal neovascularization had a neovascular frond at the temporal border of her right macula. Multiple zones of black sunburst hyperpigmentation were located nearby. Fluorescein angiography showed that the lesions were closely related to an extensive zone of avascular retina in the posterior pole. The perifoveal vasculature was uninvolved, and the patient was unaware of any visual deficit. Although sickle cell maculopathy is a well-recognized entity, there have been no published reports, to the best of our knowledge, of posterior pole neovascularization in patients with sickle cell hemoglobinopathy. The fluorescein angiographic findings in our case provide further support for the hypothesis that retinal hypoxia is an important stimulus for retinal neovascularization.