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Purpose: To describe the outcomes of a novel technique for the repair of peripheral corneal perforations using autologous limbal tissue. Methods: Two patients with peripheral corneal perforations with contraindications to other corneal procedures underwent limbal advancements. This technique involves creating a pedicle of the limbus, sclera and conjunctiva to cover the perforation. Results: The tissue had optimal integration in both patients; no aqueous leaks or flat anterior chambers were noted. None of the patients had recurrence of perforation or ocular discomfort. Conclusion: In conclusion, this technique is a cost-effective and straightforward alternative for the repair of impending acute peripheral perforations.

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Peripheral ulcerative keratitis (PUK) is a progressive peripheral thinning of the corneal stroma caused by proinflammatory mediators' release from corneal limbal vasculitis. The clinical presentation is an epithelial defect with a crescent-shaped stromal inflammation. Its exact pathophysiologic mechanisms of PUK remain partially understood, but the overall understanding of the fundamental processes that mediate and effect corneal immunity has continued to expand over the past 25 years. The unique anatomical and physiological characteristics of the periphery in relation to collagen bundles and peripheral corneal vascular arch contribute to the occurrence of this type of ulcer in this region, in addition to the concentration of complement and immunoglobulins. There is a relevant participation of the adjacent conjunctiva. Both cell-mediated immunity and humoral immunity are implicated in the pathogenesis of PUK, and the postulated mechanisms are autoimmune reactions to corneal antigens, deposition of circulating immune complexes and hypersensitivity reactions to foreign antigens. These immunocomplexes are deposited in limbic vessels resulting in the activation of the classical pathway of the complement system and, consequently, in the chemotaxis of inflammatory cells and in the release of several pro-inflammatory cytokines, which allow the production and release of matrix metalloproteinases. The release of inflammatory cytokines by infiltrating cells may induce keratocyte activation, which could then generate more release of a variety of cytokines, such as the neutrophil calgranulin C, thus facilitating an autoimmune response to the protein and precipitating an antibody- and cell-mediated hyperimmune reaction in the peripheral cornea.

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RESUMEN Mujer de 31 años con cuadro de poliartralgias, poliartritis asimétrica, fiebre, lesiones vasculíticas en piel, asociado a disminución de agudeza visual en ojo derecho, el cual presentaba compromiso corneal con adelgazamiento periférico severo, queratitis ulcerativa periférica, hipopión de fibrina y sinequias posteriores. Ante el cuadro clínico y paraclínicos con C-ANCA positivos, se realiza el diagnóstico de granulomatosis con poliangeítis con compromiso ocular (adelgazamiento corneal, queratitis ulcerativa periférica, uveítis) y sistémico. Se inicia tratamiento con metilprednisolona y ciclofosfamida con poco control, por lo que se inicia rituximab sistémico, evolucionando hacia la mejoría.

ABSTRACT A 31-year-old female, presented with polyarthralgia, asymmetrical polyarthritis, fever, vasculitis skin lesions, together with decreased visual acuity in the right eye with severe peripheral corneal melting, peripheral ulcerative keratitis, fibrin hypopyon and posterior synechiae. Given the clinical and paraclinical findings with positive C-ANCA (anti-neutrophil cytoplasmic antibodies), the patient was diagnosed with granulomatosis with polyangiitis with ocular (corneal melting, peripheral ulcerative keratitis and uveitis), and systemic involvement. Treatment was started with methylprednisolone and cyclophosphamide, but due to poor control, Rituximab was subsequently administered, with medical improvement.

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Objetivo: reportar un caso de perforación corneana manejado con injerto-parche de esclera. Diseño: reporte de caso. Discusión: las perforaciones corneanas requieren tratamiento quirúrgico urgente para reducir las complicaciones. Contamos con varias opciones de acuerdo al tamaño de la perforación incluyendo adhesivo tisular, transplante de membrana amniótica y queratoplastia penetrante. El injerto-parche de esclera es otra alternativa útil para el tratamiento de esta condición. Conclusiones: describimos el caso de una perforación corneana secundaria a queratitis ulcerativa periférica (QPU) asociada a Síndrome de Sjögren que fue manejada con un aloinjerto de esclera.

Objective: to report a case in which scleral patch graft was used to treat a corneal perforation. Design: case report. Discussion: corneal perforations require urgent management to reduce subsequent ocular morbidity. Depending on its size and location, treatment options include corneal gluing, amniotic membrane transplantation and corneal transplantation. Scleral patch grafting is another feasible alternative for treating this condition.Conclusions: We described a case of scleral allograft use in the management of corneal perforation due to peripheral ulcerative keratitis associated with Sjögren's syndrome.

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Objetivo. Informar las manifestaciones oftalmológicas en pacientes con granulomatosis de Wegener (GW). Método. Se revisó la base de datos del Instituto de Oftalmología Conde de Valenciana. Se recolectó la exploración oftalmológica completa, los exámenes de laboratorio y de gabinete, el tratamiento y la evolución. Resultados. Se incluyeron 11 pacientes con GW (18 ojos). Siete pacientes masculinos y 4 femeninos con edad promedio de 43.7 años (28-55). Tres habían tenido diagnóstico previo de GW y los 8 restantes se diagnosticaron en nuestro departamento. Siete presentaron cuadros bilaterales y 4 unilaterales. Las formas de presentación clínica fueron escleritis necrosante con queratitis ulcerativa periférica (QUP) (7/18), escleritis difusa (3/18), escleritis nodular (1/18), uveítis anterior no granulomatosa (1/18), neuropatía óptica isquémica (1/18), neuropatía óptica retrobulbar (1/18), desprendimiento de retina seroso (2/18) y dacriocistitis (2/18). De los 18 ojos, la capacidad visual final fue mejor o igual a 20/40 en 13, 20/400 en 3, cuenta dedos a 30cm o no-percepción de luz en 1. Actualmente 7 pacientes se encuentran en fase inactiva. Conclusiones. Las manifestaciones oftalmológicas más frecuentes en pacientes con GW fueron: escleritis necrosante y QUP. En la mayoría, la GW se diagnosticó después de las manifestaciones oftalmológicas, sin embargo, todos presentaron síntomas sistémicos u oftalmológicos previos.

OBJECTIVE: Report the ophthalmologic manifestations among patients with Wegener 's Granulomatosis (WG). METHOD: We reviewed the database of the Instituto de Oftalmologia Fundación Conde de Valenciana in order to collect information regarding complete ophthalmic examination, laboratory and cabinet tests, treatment, and disease progression. RESULTS: We included 11 patients with WG (18 eyes). Seven men and four women, mean age 43.7 years (range = 28-55). Three patients had a prior diagnosis of WG and the remaining eight patients were diagnosed by our study team. Seven subjects developed a bilateral affection and four had unilateral involvement. The clinical presentation was necrotizing scleritis with peripheral ulcerative keratitis (PUK) (7/18), diffuse scleritis (3/18), nodular scleritis (1/18), non-granulomatous uveitis (1/18), optic ischemic neuropathy (1/18), retrobulbar neuritis (1/18), serous retinal detachment (2/18), and dacryocystitis (2/18). Final visual acuity was better or equal to 20/40 (13/18), 20/400 (3/18), finger-counting or no-perception of light in 1/18. Currently, seven patients are symptom free. CONCLUSIONS: The most frequent ophthalmic manifestations among our patients with WG were: necrotizing scleritis and PUK. In most cases, WG was diagnosed after ophthalmic manifestations; however, all patients displayed prior systemic or ocular symptoms.

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