Purtscher retinopathy is a unilateral or bilateral visual loss following acute injury to the thorax or head. It is characterized by large cotton-wool spots, hemorrhages, and retinal edema. Vision loss may be permanent due to isquemia of the retina, and optic atrophy. Is thought to be a result of injury-induced complement ativation causing granulocyte aggregation and leukoembolization. Other conditions may active complement and may produce similar fundus appearance including acute pancreatitis, collagen-vascular diagnosis of acute pancreatitis.Keywords: Retina. Pancreatitis. Optic atrophy. Complement activation. Optic nerve.Retinopatia de Purscher é caracterizada por perda visual uni ou bilateral seguida de trauma de tórax ou crânio. A retinopatia apresenta-se com manchas algodonosas, hemorragias e edema da retina. A perda da visão pode ser permanente devido a isquemia visão súbita e permanente após diagnostico de pancreatite aguda.
Objectives: Report a case of Tay-Sachs disease. Methods: A complete ophthalmologic examination, retinography and blood dosage of hexosaminidase A (EHA) activity were performed. Results: A one year and seven months old male with a history of generalized tonic-clonic seizures, with delayed neuropsychomotor development from the eighth month of life. At the ophthalmologic examination the patient did not fix or follow objects, with bilateral horizontal nystagmus. The fundoscopy revealed an image of “cherry-red spot” in the macula in both eyes. Dosage of EHA activity confirmed diagnosis of Tay-Sachs disease. Conclusion: Tay-Sachs Disease is a genetic disease with an autosomal recessive inheritance pattern characterized by deficiency of EHA activity, leading to the accumulation of sphingolipids in neuronal cells that results in progressive neurological dysfunction. In the retina, there is deposition in the ganglion cells, resulting in the appearance of cherry-red spot macula. There is no curative treatment, aiming at the therapy, only the clinical support in the patient.
Siderosis bulbi is a sight-threatening condition characterized by a pigmentary and degenerative change in the eye that follows the intra-ocular retention of a foreign body containing iron. The main cause of ocular siderosis is penetrating trauma mostly by hammering a steel object without or with poor eye protection. Herein, we report a case of siderosis bulbi after penetrating trauma that was lately treated with vitrectomy to remove the foreign body, and despite treatment resulted in ocular globe atrophy.Keywords: Retina. Vitrectomy. Wounds and injuries. Optic atrophy. Night blindness. RESUMOSiderose ocular é caracterizada por alterações pigmentares e degenerativas oculares causada por retenção de corpo estranho intraocular contendo ferro, que pode levar à perda da visão. A principal causa de siderose ocular é o trauma penetrante, causado principalmente por martelar objetos de metal com pouca ou nenhuma proteção ocular. Relatamos o caso de um paciente com siderose ocular causado por trauma penetrante, que foi tratado tardiamente com vitrectomia para remoção do corpo estranho e Palavras-chave:
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