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INTRODUCTION: Central retinal artery occlusion is an ophthalmic emergency which typically causes acute, painless visual loss. Several conservative treatment options are practiced with little benefit. Thrombolysis as a therapeutic option has gathered interest as well as controversy. This paper aims at reviewing the relevant literature to assess the efficacy and safety of intra-arterial thrombolysis for acute central retinal artery occlusion. METHODS: A review of the literature was conducted. Keywords included "intra-arterial thrombolysis" or "intra-arterial fibrinolysis" in combination with "central retinal artery occlusion" or "CRAO". A Cochrane Database search was performed for randomised control trials, systematic reviews and meta-analyses using the same keywords. RESULTS: Twenty-eight studies were identified which included case reports, case series, case-control studies and 1 randomised control trial: the European Assessment Group for Lysis in the Eye Study. Improvement in vision was measured using different methods and at different time points. The findings of these studies generally favour an effect towards intra-arterial thrombolysis however there are many limitations. Additionally, the European Assessment Group for Lysis in the Eye Study showed lack of effect in intra-arterial thrombolysis vs conservative measures. CONCLUSION: Current evidence is not sufficient to recommend intra-arterial thrombolysis due to the variability of visual improvement within retrospective studies, heterogeneity in treatment regimens between studies and adverse effects. Intra-arterial thrombolysis may have a role in patients presenting early, particularly if they have monocular vision, after discussion of the risks and benefits. Further high-quality trials assessing the clinical efficacy of intra-arterial thrombolysis may shed more light on this topic.
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PURPOSE: Pigmentary maculopathy can occur in the context of various inherited and acquired diseases. Anterior segment dysgenesis arises due to developmental anomalies and may be associated with systemic disease, as in Rieger syndrome. CASE REPORT: A 49-year-old woman presented with longstanding reduction in vision, evidence of anterior segment dysgenesis, and multiple discrete pigmented lesions throughout the macula bilaterally. Electroretinographic findings were consistent with severe macular dysfunction. Gene array analysis did not reveal any chromosomal imbalances or other specific abnormalities. CONCLUSIONS: This is a unique case of bilateral pigmentary maculopathy and anterior segment dysgenesis, with clinical findings that are not characteristic of previously reported disease.
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Segmento Anterior do Olho/anormalidades , Anormalidades do Olho/complicações , Retinose Pigmentar/complicações , Eletrorretinografia , Potenciais Evocados Visuais/fisiologia , Anormalidades do Olho/diagnóstico por imagem , Anormalidades do Olho/fisiopatologia , Feminino , Humanos , Pessoa de Meia-Idade , Retina/fisiopatologia , Retinose Pigmentar/diagnóstico por imagem , Retinose Pigmentar/fisiopatologia , Tomografia de Coerência Óptica , Acuidade Visual/fisiologiaRESUMO
Walker-Warburg syndrome (WWS) is a disorder characterized by ocular and brain malformations, and congenital muscular dystrophy. Retinal malformations are common in WWS; however, bilateral retinal detachment is a rare occurrence. We present a case of a newborn baby delivered at 36+3 weeks, who was the first living child of consanguineous parents of Turkish origin. On antenatal anomaly scans, the fetus had hydrocephalus that had increased throughout pregnancy, and a diagnosis of hydrancephaly was made at 36 weeks of gestation. Hypotonia, cleft lip and palate, poor suck and absent gag reflex were noted at birth. Ophthalmic examination at the age of 2 days revealed bilateral funnel retinal detachment. B-scan ultrasonography confirmed these findings, and magnetic resonance imaging (MRI) of the brain was performed at the age of 13 days to establish a diagnosis. The MRI showed lissencephaly, hydrocephalus and thin rim of brain parenchyma, with a cobblestone appearance of the cortex and pontine and cerebellar hypoplasia, consistent with the diagnosis of WWS. The infant deteriorated and died at 39 days of age from complications associated with the brain anomalies. In summary, bilateral retinal detachment is extremely rare and in association with hydrocephalus and posterior fossa anomalies strongly suggests the diagnosis of WWS.