Microscopia confocal no auxílio diagnóstico de Distrofia Corneana de Schnyder / In vivo confocal microscopy as a diagnostic tool in Schnyder Corneal Dystrophy's case

Resumo Neste relato, descrevemos um caso de Distrofia corneana de Schnyder que apresentou o desfecho de seu diagnóstico baseado em achados característicos na microscopia confocal, ferramenta que se aponta em destaque no universo oftalmológico.

Abstract Schnyder's corneal dystrophy (SCD) is a rare corneal condition characterized by cholesterol and phospholipids deposition in the stroma and Bowman's layer. We present a case report of a patient who had a progressive corneal stromal haze in both eyes since he was 15 years old. Etiological diagnosis of SCD was well established by In Vivo Confocal Microscopy (IVCM).

Ranibizumab for choroidal neovascular membrane in a rare case of Bietti's crystalline dystrophy: A case report.

We report a rare case of Bietti's crystalline dystrophy presenting with choroidal neovascular membrane (CNVM) which was treated with three injections of intravitreal ranibizumab. The CNVM underwent scarring after the injections with stabilization of visual acuity at a follow-up period of 12 months suggesting that intravitreal ranibizumab may have a role in the management of CNVM in these rare cases.

Subretinal fibrosis in stargardt's disease: case report / Fibrose sub-retiniana na doença de Stargardt: relato de caso

The authors describe the clinical characteristics of a 14-year-old female patient with ophthalmoscopic and angiographic aspects of Stargardt's disease in both eyes who developed subretinal fibrosis and localized retinal pigment epithelial hyperplasia in the posterior pole of the right eye 6 months after a mild blunt trauma to the right globe. The left fundus remains with the typical presentation of Stargardt's disease. Our findings support the theory that ocular trauma occurring in patients with Stargardt's disease can precipitate a sequence of events leading to the development of subretinal fibrosis and significant visual loss.

Os autores descrevem as características clínicas de uma paciente de 14 anos com aspectos na oftalmocospia e angiofluoresceinografia de doença de Stargardt em ambos olhos que desenvolveu fibrose sub-retiniana e hiperplasia localizada do epitélio pigmentado da retina no polo posterior do olho direito seis meses após um trauma contuso leve neste globo. A fundoscopia do olho esquerdo permanence com a apresentação típica de doença de Stargardt. Nossos achados suportam a teoria de que o trauma ocular em pacientes com doença de Stargardt pode precipitar uma sequência de eventos que pode levar ao desenvolvimento de fibrose sub-retiniana e acentuada baixa da acuidade visual.

Fungal keratitis in Lattice dystrophy.

We report a case of fungal keratitis occurring in a patient with lattice dystrophy. A 57-year-old farmer presented with a corneal ulcer following probable entry of paddy husk in the right eye, of one month duration. Corneal scraping revealed pigmented fungal filaments while culture grew Alternaria alternata. Treatment with 5% natamycin eye drops and 1% atropine healed the infection in four weeks. We would like to draw attention to the fact that the cornea in lattice dystrophy is prone to frequent erosions and is a compromised epithelial barrier to invasion by microorganisms. Patients must be made aware of this fact and should seek attention at the earliest following any trivial trauma. Management of minor corneal abrasions in them should be directed at healing the epithelium with adequate lubricants and preventing infection with topical antibiotic prophylaxis.

Queratotoxon o anillos cornéales / Corneal rings

Los anillos cornéales habitualmente no son completos, pueden representar desde cambios fisiológicos (anillo limbar ) hasta enfermedades que, si no son diagnosticadas y tratadas oportunamente, pueden terminar con la visión, el ojo e, incluso, con la vida del paciente (anillo limbar, embriotoxon anterior, embriotoxon posterior), de la segunda a cuarta década de la vida (arco juvenil, anillo de Fleisher, anillo de Kayser-Fleisher, queratoconjutivitis alérgica, distrofia corneal anular, desgeneración marginal de Terrien) y seniles (gerotoxon). La distrofia corneal de Schnyder puede presentarse tan precozmente como al año y medio de edad. El anillo por anestésico local, lo hemos visto desde la adolescencia en adelante. Los anillos cornéales intraestromales se implantan, en general, a partir de la tercera o cuarta década de la vida. El anillo de Wessely, el anillo por queratoplastía y el anillo blanco de Cotas pueden presentarse en cualquier etapa de la vida. Respecto a su ubicación pueden ser paracentrales, paralimbares y perilesionales.

Lattice corneal dystrophy type III with corneal fistula. A case report.

Lattice corneal dystrophy is a distinct clinical entity characterised by amyloid deposits in the corneal stroma. We report a patient who presented with a corneal fistula in the right eye and thick lattice lines involving the peripheral cornea in both eyes suggestive of type III lattice dystrophy. The association of corneal fistula with lattice corneal dystrophy type III makes this a unique case.

Unilateral pellucid marginal degeneration.

Pellucid marginal degeneration (PMD) is a rare and usually a bilateral condition. We report a case of typical PMD with a unilateral presentation.

Senior-Loken syndrome with unusual manifestations.

The Senior-Loken syndrome, also called Hereditary Renal Retinal Dystrophy is a rare disorder that combines a juvenile nephronophthisis-like disease with tapetoretinal degeneration. We are reporting a family in which two, possibly three siblings are affected by this disorder. In addition to these two documented components of the disease, our patients also have unusual manifestations: short stature; skeletal abnormalities in the form of kyphoscoliosis, short metacarpals; and cutis laxa-unreported so far.