RESUMEN
In a study involving 97 patients with hemoglobin SC sickle cell disease (Hb SC) and 87 subjects with hemoglobin SS sickle cell disease (Hb SS) on the island of Curaçao, we found a higher incidence of peripheral retinal vascular closure and proliferative retinopathy in the former as compared with the latter. Examination of composite fluorescein angiograms obtained from 15 Hb SS and 36 Hb SC patients, whose mean and median age were the same and in whom more advanced stages of sickle retinopathy were equally represented, revealed statistically significantly smaller measurements of perfused retina (indicative of larger areas of non-perfusion) in Hb SC patients as compared with Hb SS patients (P = 0.002, P = 0.014) as well as in subjects exhibiting neovascularization as compared with those who did not (P = 0.022, P = 0.004). This suggests that a vaso-occlusive tendency in the retina is greater in individuals with Hb SC than in those with Hb SS; the more extensive ischemic areas provide a stimulus for neovascularization, explaining the higher prevalence of proliferative retinopathy in the former patients as compared with the latter.
Asunto(s)
Anemia de Células Falciformes/complicaciones , Oclusión de la Arteria Retiniana/etiología , Enfermedades de la Retina/etiología , Adulto , Angiografía con Fluoresceína , Fondo de Ojo , Humanos , Incidencia , Antillas Holandesas , Prevalencia , Neovascularización Retiniana/etiologíaRESUMEN
Two choroidal occlusion syndromes are recognized: the triangular syndrome after ciliary artery occlusions, and the multifocal lesions associated with choroidal arteriolar occlusions. Both syndromes have been described in sickle cell patients. We compared early venous phase fluorescein angiograms of 51 Hb SS or Hb SC patients and 15 age and race matched controls, to test whether geographic early filling defects of the choriocapillaris might be more common in these rheologically impaired patients. We found no difference in occurrence of these filling defects between the two groups, and conclude that choriocapillaris filling defects in the early venous phase only are not pathological.
Asunto(s)
Anemia de Células Falciformes/complicaciones , Arteriopatías Oclusivas/diagnóstico , Coroides/irrigación sanguínea , Enfermedad de la Hemoglobina SC/complicaciones , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Anemia de Células Falciformes/epidemiología , Capilares , Niño , Preescolar , Angiografía con Fluoresceína , Fondo de Ojo , Enfermedad de la Hemoglobina SC/epidemiología , Humanos , Lactante , Recién Nacido , Persona de Mediana Edad , Antillas Holandesas/epidemiologíaRESUMEN
We examined about half the estimated number (based on gene frequency) of sickle cell patients on the island of Curaçao. This study may represent one of the most comprehensive and a selective cross-sectional surveys available. Studying 81 Hb SS and 97 Hb SC patients, we were able to confirm the correlation between iris atrophy and proliferative sickle retinopathy (PSR), the correlation between Hb SC and proliferative sickle retinopathy (PSR) and the major role of PSR in blindness in sickle cell patients. In Hb SC patients over the age of 18 PSR occurred in 50%, leading to vitreous hemorrhage in 18% and to retinal detachment in 8%. We found a frequency of blindness in one eye (fingers counting or less) of 4% in the entire sickle population, and 6% among Hb SC patients. Bilateral blindness was rare: one Hb SS patient, due to cortical blindness. The relatively frequent occurrence of severe complications in a narrowly defined population should stimulate our efforts to prevent these from occurring.
Asunto(s)
Anemia de Células Falciformes/complicaciones , Enfermedad de la Hemoglobina SC/complicaciones , Enfermedades de la Retina/etiología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Anemia de Células Falciformes/epidemiología , Ceguera/etiología , Niño , Preescolar , Estudios Transversales , Angiografía con Fluoresceína , Fondo de Ojo , Enfermedad de la Hemoglobina SC/epidemiología , Humanos , Lactante , Recién Nacido , Enfermedades del Iris/complicaciones , Persona de Mediana Edad , Antillas Holandesas/epidemiología , Desprendimiento de Retina/complicaciones , Enfermedades de la Retina/complicaciones , Enfermedades de la Retina/diagnóstico , Neovascularización Retiniana/complicaciones , Hemorragia Vítrea/complicacionesRESUMEN
Prospectively we performed an ophthalmological examination in 81 Hb SS and 97 Hb SC patients on the island of Curaçao, about half the estimated number of patients, based on gene frequency. These patients had not been selected on ophthalmological symptoms. Proliferative retinopathy was present in 2% of the Hb SS patients, and in 50% of the Hb SC patients (71% among those over 18 years old). The frequency of severe visual loss (counting fingers or less) in one eye due to sickle cell disease in Hb SS patients was 1% (one patient, both eyes), and in Hb SC patients 6% (6 patients, 6 eyes). Bilateral blindness was rare: one Hb SS patient had cortical blindness. Visual loss in Hb SC patients (of which four eyes with loss of light perception) was caused by proliferative retinopathy, with its complications like vitreous haemorrhage and retinal detachment. Although the effectiveness of prophylactic laser treatment remains to be established, it would appear prudent to have an eye examination performed regularly in Hb SC patients older than 20 years.