Fenómenos vasculares retinianos en esclerosis múltiple. Reporte de caso y revisión narrativa de la literatura / Retinal vascular phenomena in multiple sclerosis. Case report and narrative review of the literatura

Introducción: La esclerosis múltiple (EM) es la enfermedad inflamatoria desmielinizante del sistema nervioso central (SNC) más prevalente en el mundo. Puede presentar afectación a nivel ocular a través del compromiso inflamatorio de distinto tejidos. Objetivo: Dar a conocer la importancia del enfoque temprano de la esclerosis múltiple por parte del médico oftalmólogo. Diseño del estudio: Reporte de caso y revisión narrativa de la literatura. Resumen del caso: Se reporta el caso de una paciente joven con signos de vasculitis retiniana (VR) asociada a neuritis óptica bilateral y uveítis anterior como debut de inusual de la EM. Para la revisión narrativa se llevó a cabo la búsqueda avanzada en tres bases de datos electrónicas: PUBMED, LILACS y OVID. Por medio del gestor de referencias Zotero se realizó la eliminación de duplicados y lectura crítica de título y resumen de 162 artículos por parte de dos investigadores. Conclusión: La EM es una enfermedad crónica inflamatoria del SNC. Puede afectar las estructuras oculares de distintas maneras, una de ellas es la VR, que aunque se caracteriza por el compromiso de vasos venosos, hay que tener en cuenta presentaciones atípicas como hemorragias periarteriolares, aneurismas de grandes vasos, cierre capilar periférico, oclusión de arterial central de la retina, neovascularización del nervio óptico o aparición en etapas tempranas de la enfermedad. La fuerte asociación entre la VR y la actividad de la EM destaca la importancia del reconocimiento de patrones atípicos de una enfermedad que requiere del manejo y seguimiento oportuno por parte de neurología y oftalmología.

Background: Multiple sclerosis (MS) is the most prevalent infl ammatory demyelinating disease of the central nervous system (CNS) in the world. It may present involvement at the ocular level through the inflammatory compromise of different tissues. Objective: To provide knowledge on the importance of the early approach of multiple sclerosis by the ophthalmologist. Study design: Case report and narrative review of the literature. Case summary: We report the case of a young patient with signs of retinal vasculitis (RV) associated with bilateral optic neuritis and anterior uveitis as an unusual debut of MS. For the narrative review, the advanced search was carried out in three electronic databases: PUBMED, LILACS and OVID. Through the Zotero reference manager, the elimination of duplicates and critical reading of the title and summary of 162 articles by two researchers was carried out. Conclusion: MS is a chronic inflammatory disease of the CNS. It can affect the ocular structures in different ways, one of them is RV, which although it is characterized by the involvement of venous vessels, we must take into account atypical presentations such as periarteriolar hemorrhages, large vessel aneurysms,peripheral capillary closure, retinal artery occlusion, neovascularization of the optic nerve or appearance in the early stages of the disease. The strong association between RV and MS activity highlights the importance of recognizing atypical patterns of a disease that requires early management and follow-up by neurology and ophthalmology.

Vasculitis retinal / Retinal vasculitis

La vasculitis retinal es una enfermedad inflamatoria que amenaza la visión y afecta los vasos retinales (capilares, venas y arterias). Puede ocurrir como una condición idiopática o como complicación de enfermedades infecciosas y neoplásicas o en asociación con enfermedades sistémicas inflamatorias. Sus manifestaciones clínicas típicas incluyen revestimiento perivascular, envainamiento, filltración vascular y oclusión. Puede estar asociada con signos de isquemia retinal (puntos algodonosos y hemorragias intraretinales). Es clasificada en diferentes estadíos: inflamación, isquemia, neovascularización y complicaciones. En su diagnóstico es importante la biomicroscopia, la oftalmoscopia del segmento posterior y la prueba de angiografía fluoresceínica. Se presenta un paciente de 30 años con buena historia de salud anterior. El paciente comenzó con enrojecimiento del ojo izquierdo y la observación de flotadores en ambos ojos. Fue examinado y los signos clínicos fueron descritos en el examen oftalmológico. El examen del fondo de ojo con lente de 90 dioptrias y el oftalmoscopio binocular indirecto fue realizado para un cuidadoso examen. La retinografía del fondo y las imágenes del test de angiografía y otros estudios complementarios indicados permitieron conocer que presentaba una vasculopatía obliterativa retinal ideopática (Enfermedad de Eales), y el diagnóstico diferencial fue discutido. El tratamiento indicado fue la fotocoagulación con láser y evaluar la indicación de los estudios bioquímicos del cultivo del vítreo, por antígenos de leucocito humano, factor de crecimiento endotelial vascular e interleukinas que han sido observados en este examen(AU)

Retinal vasculitis is an inflammatory disease that threatens vision and affects retinal vessels (capillaries, veins and arteries). It may occur as an isolated idiopathic condition and as a complication of infective or neoplastic disorders, in association with systemic inflammatory diseases. Typical clinical manifestations including perivascular sheathing or cuffing, vascular leakage and occlusion. It may be associated with signs of retinal ischemia (cotton-wool spots and intra-retinal hemorrhage). It is classified into different stages: lnflamation, ischemia, neovascularization and complications. In the diagnosis, biomicroscopy and ophtalmoscopic of the posterior segment and fluorescein angiography test are important. A case report is presented of a 30-year-old male patient with a history of good health. In October 2015 the patient had started out with reddening of his left eye and the viewing of floaters with both eyes. Funduscopy was performed with a 90-dioptric lens and a binocular indirect ophthalmoscope. Fundus retinography, angiographic imaging and other complementary studies revealed an idiopathic obliterative retinal vasculopathy (Eales disease). The treatment indicated was photocoagulation, as well as a recommendation to evaluate the biochemical studies of the vitreous culture, since the test had found human leukocyte antigens, vascular endothelial growth factor and interleukins(AU)

Bilateral retinal vasculitis in a patient with lichen planus / Vasculite retiniana bilateral em um paciente com líquen plano

ABSTRACT Lichen planus (LP) is an autoimmune, inflammatory disease of unknown etiology that commonly affects the skin and mucous membranes. Retinal vasculitis is a group of vision-threatening disorders, in which autoimmunity is thought to play a role in pathogenesis. We present the case of a patient who was diagnosed with retinal vasculitis and who was followed up for mucosal LP. LP has not been reported as a cause of retinal vasculitis in the literature. We believe that the retinal vasculitis in this case was related to LP because cellular immunity plays a role in the pathogenesis of both entities.

RESUMO Líquen plano (LP) é uma doença auto-imune, inflamatória de etiologia desconhecida que normalmente afeta a pele e membranas mucosas. Vasculite retiniana engloba um grupo de doenças que ameaçam a visão em que a autoimunidade parece desempenhar um papel na sua patogênese. Apresentamos um caso que foi diagnosticado com vasculite da retina e que tinha sido acompanhado com LP de mucosa. LP não consta entre as causas de vasculite retiniana na literatura. Acreditamos que a vasculite da retina no nosso caso, foi relacionado com o LP considerando que a imunidade celular desempenha um papel na patogênese de ambas as entidades.

Correlation between Fluorescein Angiographic Findings and Visual Acuity in Behcet Retinal Vasculitis

PURPOSE: To identify significant fluorescein angiographic (FA) characteristics associated with visual acuity (VA) in Behcet retinal vasculitis. MATERIALS AND METHODS: Retrospective review of 86 eyes of 48 patients (age: 35.6+/-10.2 years) with Behcet retinal vasculitis were performed. VA and FA findings as well as correlation between them were assessed. RESULTS: The mean initial VA of eyes with posterior pole-involved vasculitis (63 eyes; 73.3%) was significantly worse than that of those with peripheral vasculitis (23 eye; 26.7%) (logarithm of the minimum angle of resolution VA: 0.554+/-0.572 vs. 0.078+/-0.148; p<0.0001). Subgroup analysis revealed a more severe and diffuse pattern of vascular leakage in posterior pole-involved vasculitis compared to peripheral vasculitis (p<0.0001). Retinal vascular leakage (beta=0.345; p<0.0001), optic disc hyperfluorescence (beta=0.147; p=0.032), and macular leakage (beta=0.107; p=0.047) were significantly associated with worse initial VA. During the follow up (mean: 33.3+/-17.9 months), the change of leakage showed no significant correlation with change of VA in posterior pole-involved vasculitis (tau=0.199, p=0.092). CONCLUSION: Posterior pole involvement, the degree of retinal vascular leakage, optic disc hyperfluorescence, and macular leakage are significantly associated with VA in Behcet retinal vasculitis.

Sickle cell retinopathy: diagnosis and treatment / Retinopatia falciforme: diagnóstico e tratamento

Hemoglobinopathies are a group of inherited disorders characterized by quantitative or qualitative malformations of hemoglobin (Hb). Some of these diseases present vaso-occlusive phenomena that are responsible for high morbidity in clinical and/or ophthalmologic terms. Diagnosis of hemoglobinopathies is performed exclusively through hemoglobin electrophoresis. From the ophthalmologic perspective, the most important representative of this group of diseases is sickle cell retinopathy, which presents a wide spectrum of fundus manifestations and may even lead to irreversible vision loss if not properly diagnosed and treated. The aim of this review is to present the classification of sickle cell retinopathy and to describe current management and future perspectives for its treatment, taking into consideration the clinical management of these patients.

As hemoglobinopatias são um grupo de doenças hereditárias caracterizadas por mal-formações quantitativas ou qualitativas da hemoglobina (Hb). Algumas destas doenças podem apresentar fenômenos vaso-oclusivos, responsáveis por alta morbidade do ponto de vista clínico e/ou oftalmológico. O diagnóstico das hemoglobinopatias é feito exclusivamente através da eletroforese de hemoglobinas. Do ponto de vista oftalmológico, a representante mais importante deste grupo de doenças é a retinopatia falciforme, que pode apresentar um amplo espectro de manifestações fundoscópicas, podendo, inclusive, levar à perda visual irreversível se não for corretamente diagnosticada e tratada. O objetivo desta revisão é apresentar a classificação desta doença, a conduta no tratamento atual, bem como suas perspectivas futuras de tratamento, considerando-se as particularidades no manejo clínico destes pacientes.

Frosted Branch Angiitis as Ocular Manifestation of Behcet's Disease: Unusual Case Report and Literature Review

We report an unusual case of unilateral frosted branch angiitis associated with Behcet's disease, including a review of previously reported cases. A 39-year-old male with history of recurrent oral and genital ulcers presented with visual loss in his left eye. Fundus findings demonstrated occlusive retinal vasculitis resembling acute frosted branch angiitis. Laboratory examinations including viral markers revealed no abnormal findings except positive HLA-B51. The patient was treated with systemic steroid and cyclosporine. Six months after presentation, new oral ulcers and pseudofolliculitis appeared, and he was diagnosed with Behcet's disease following rheumatology consultation. During follow-up, there was no change in visual acuity of hand movement, and disc neovascularization developed even after complete panretinal photocoagulation. Ocular manifestations of Behcet's disease can present as unilateral frosted branch angiitis, and may consecutively involve in both eyes. Early immunosuppressive treatment is recommended.

Retinite idiopática, vasculite, aneurismas e neurorretinite (IRVAN): relato de caso / Idiopathic retinal, vasculitis, aneurysms and neuroretinitis (IRVAN): case report

Relatamos caso de um paciente de 14 anos, sexo masculino, que foi admitido com queixa de embaçamento visual bilateral há dois anos. Ao exame oftalmológico observou-se leve hiperemia e edema de disco óptico bilateral, exsudação retiniana, poucas hemorragias retinianas, múltiplos aneurismas, assim como sinais de vasculite. A angiofluoresceinografia demonstrou isquemia periférica extensa, dilatações e hiperfluorescência das paredes dos vasos, e vazamento tardio do disco óptico nas fases finais do exame em ambos os olhos. Este caso representa uma rara entidade caracterizada por oclusão retiniana vascular periférica, vasculite retiniana, múltiplos aneurismas retinianos e neurorretinite (IRVAN). Avaliação sistêmica e laboratorial não revelaram nenhuma anormalidade. O paciente foi submetido à panfotocoagulação de retina com laser de argônio em ambos os olhos, e iniciado tratamento com prednisona via oral, com manutenção da acuidade visual de 20/25 depois de um ano de acompanhamento. O tratamento com laser deve ser considerado quando houver qualquer evidência angiográfica de má perfusão retiniana, e antes do desenvolvimento de qualquer sinal de neovascularização de retina.

A case of a 14 year-old boy that was admitted complaining of bilateral ocular visual blurring for 2 years is reported. The ophthalmological examination disclosed bilateral mild optic disc hyperemia and swelling, retinal exudation, few retinal hemorrhages, multiple aneurysms, as well as vasculitis. Fluorescein angiography showed extensive peripheral retinal ischemia, dilatations and hyperfluorescence of the vessels walls, and leakage of the optic disc in the late phases in both eyes. This rare case represents an entity characterized by peripheral retinal vascular occlusion, retinal vasculitis, multiple posterior retinal aneurysms, and neuroretinitis (IRVAN). Systemic evaluation and laboratory work-up did not suggest any systemic abnormality. Panretinal laser photocoagulation was performed in both eyes, and the patient was treated with oral prednisone with maintenance of the visual acuity after 1 year of follow-up. Laser treatment should be considered when angiographic evidence of widespread retinal no perfusion is present, and before the development of signs of retinal neovascularization.

Long term follow-up of acute multifocal hemorrhagic retinal vasculitis (Blumenkranz syndrome): case report / Síndrome de vasculite retiniana hemorrágica multifocal aguda (síndrome de Blumenkranz) com seguimento a longo prazo: relato de caso

PURPOSE: To report a 16-year long-term follow-up of a patient with acute multifocal hemorrhagic retinal vasculitis (Blumenkranz syndrome). A 21-year old male was seen in 1994 with acute multifocal hemorrhagic retinal vasculitis (Blumenkranz syndrome), first in the left eye, and later in the right eye. He was treated with retinal photocoagulation in areas of retinal ischemia and oral steroids, followed by sequential annual fundus examination and photography for 16 years. Vision improved to 20/25 in both eyes after retinal ischemic areas photocoagulation and oral steroids, and his vision has been maintained for 16 years. Photocoagulation of retinal ischemia and oral steroids are effective for the treatment of acute multifocal hemorrhagic retinal vasculitis (Blumenkranz syndrome).

Relato de caso com acompanhamento por 16 anos de um paciente com a vasculite hemorrágica multifocal aguda (síndrome de Blumenkranz). Um paciente de 21 anos de idade foi diagnosticado em 1994 com a vasculite hemorrágica multifocal aguda (síndrome de Blumenkranz), primeiro no olho esquerdo e depois no olho direito. Foi tratado com fotocoagulação retiniana nas áreas retinianas isquêmicas e corticosteroide oral e seguido por exames complementares da retina por 16 anos. A visão melhorou para 20/25 em ambos os olhos após a fotocoagulação retiniana nas áreas isquêmicas da retina e corticosteroide oral permanecendo assim até o momento por 16 anos. A fotocoagulação retiniana nas áreas isquêmicas e o uso de corticosteróide oral são tratamentos efetivos para a vasculite hemorrágica multifocal aguda (síndrome de Blumenkranz).

Retinal vasculitis in Susac syndrome: case report / Vasculite retiniana na síndrome de Susac: relato de caso

A 29 year old male was admitted at the emergency room suffering from gradually worsening headache followed by nausea. In the hospital patient presented with lethargy, reduction of consciousness level and bilateral hypoacusis. Ophthalmic examination and fluorescein angiography showed retinal vasculitis. This finding was crucial to the diagnosis of Susac syndrome, a rare disease characterized by vasculopathy of retina, encephalic tissues and cochlea. Patient was treated with immunosuppressors to prevent him from developing severe sequelae of this disease.

Paciente masculino de 29 anos foi admitido em pronto-socorro com quadro de cefaléia progressiva há 10 dias seguida de náuseas. Durante internação evoluiu com letargia, redução do nível de consciência e hipoacusia bilateral. Exame oftalmológico e angiofluoresceinografia evidenciaram vasculite retiniana. Este achado foi essencial para o diagnóstico de síndrome de Susac, uma patologia rara caracterizada por vasculopatia de retina, encéfalo e cóclea. Tratamento imunossupressor foi instituído, prevenindo sequelas graves da doença.

Ocular Sarcoidosis in a Korean Population

The aim of current study was to evaluate the incidence and characteristics of ocular sarcoidosis in a Korean population. We conducted a retrospective study of 104 consecutive patients with biopsy-proven sarcoidosis seen at Asan Medical Center in Seoul, Korea, from 1993 to 2007. Medical records, photographs, and fluorescein angiograms were reviewed. Of 104 patients, 22 (21%) had intraocular involvement with female predominance (86%, M:F=3:19). Of the 39 eyes with ocular involvement, 16 (41%) eyes had isolated anterior uveitis, 12 (31%) eyes had intermediate uveitis, 6 eyes (15%) had panuveitis with retinal vasculitis, and 5 (13%) eyes had panuveitis with punched multifocal choroiditis. Mean duration of ophthalmologic follow-up was 62 months. All ocular inflammation was well managed with topical steroid and/or systemic steroid with relatively good final visual outcomes. Ocular complications such as cataract (12 eyes, 30%), glaucoma (6 eyes, 15%), vitreous opacity (1 eye, 3%), cystoid macular edema (3 eyes, 7%), neovascularization (2 eye, 5%), and epiretinal membrane (4 eye, 10%) were related to ocular sarcoidosis. In Korea, where sarcoidosis is very rare, our study indicates relatively low ocular and predominantly non posterior segment involvement with relatively good visual prognosis.

Ocular Sarcoidosis in a Korean Population

The aim of current study was to evaluate the incidence and characteristics of ocular sarcoidosis in a Korean population. We conducted a retrospective study of 104 consecutive patients with biopsy-proven sarcoidosis seen at Asan Medical Center in Seoul, Korea, from 1993 to 2007. Medical records, photographs, and fluorescein angiograms were reviewed. Of 104 patients, 22 (21%) had intraocular involvement with female predominance (86%, M:F=3:19). Of the 39 eyes with ocular involvement, 16 (41%) eyes had isolated anterior uveitis, 12 (31%) eyes had intermediate uveitis, 6 eyes (15%) had panuveitis with retinal vasculitis, and 5 (13%) eyes had panuveitis with punched multifocal choroiditis. Mean duration of ophthalmologic follow-up was 62 months. All ocular inflammation was well managed with topical steroid and/or systemic steroid with relatively good final visual outcomes. Ocular complications such as cataract (12 eyes, 30%), glaucoma (6 eyes, 15%), vitreous opacity (1 eye, 3%), cystoid macular edema (3 eyes, 7%), neovascularization (2 eye, 5%), and epiretinal membrane (4 eye, 10%) were related to ocular sarcoidosis. In Korea, where sarcoidosis is very rare, our study indicates relatively low ocular and predominantly non posterior segment involvement with relatively good visual prognosis.

[Incontinentia pigmenti: a rare cause of retinal vasculitis in children]

Incontinentia pigmenti is a genetic disease with cutaneous, dental, neurologic and ophthalmologic manifestations. Ocular changes are very varied. Retinal vasculitis is uncommon, but can be observed in this disease. To report a case of a patient with retinal vasculitis associated to Incontinentia pigmenti. A seven year old girl was followed for cutaneous lesions with the diagnosis of incontinentia pigmenti confirmed by a skin biopsy. She had delayed eruption of her teeth some of which were conical. She had no neurologic nor developmental abnormalities. Ophthalmological examination and fluorescein angiography was performed and showed retinal neovascularization in the left eye, secondary to retinal ischemia and retinal sequelae vasculitis without inflammation or complications in the right eye. Laser photocoagulation was indicated to the left and a strict surveillance to the right eye. After one month of the beginning of treatment, we noticed an involution of the retinal neovascularisation. Early diagnosis and management of retinal changes in incontinentia pigmenti is essential because they may be serious leading to blindness

Síndrome de Vogt-Koyanagi-Harada incompleta associada a HLA DRB1*01 em criança de quatro anos de idade: relato de caso / Incomplete Vogt-Koyanagi-Harada syndrome associated with HLA DRB1*01 in a 4-year-old child: case report

A síndrome de Vogt-Koyanagi-Harada é doença crônica, sistêmica e auto-imune, com manifestações oculares, nervosas, auditivas e tegumentares. Descrevemos aqui o caso de uma criança com início dos sintomas aos quatro anos e dois meses de idade, com positividade para o HLA DRB1*01.

Vogt-Koyanagi-Harada syndrome is chronic systemic autoimmune disease with ocular, nervous, auditory and tegumental manifestations. We report here the case of a child with onset of symptoms at four years and two months of age, with positive HLA DRB1*01.

Retinal vasculitis--an initial presentation of systemic lupus erythematosus.

Retinal vasculitis is an intra-ocular inflammatory condition with diverse aetiology and a rare manifestation of systemic lupus erythematosus, often associated with antiphospholipid antibodies. A 13 years male presented with fever for 20 days associated with photosensitive skin rashes. Three years back he had suffered painless, progressive dimness of vision. Then a clinical diagnosis of retinal vascular occlusion was made. Examination revealed his visual acuity was reduced to finger counting at 3 metres distance and extensive retinal vasculitis in the right eye. His serum antinuclear factor was positive in high titre. A final diagnosis was made as systemic lupus erythematosus. He was treated with methylprednisolone. His fever subsided, skin rashes healed and vision did not deteriorate further.

Síndrome de Cogan: relato de caso / Cogan's Syndrome: case report

Ceratite intersticial não luética, surdez e vertigem caracterizam a síndrome de Cogan. Os achados oculares mais comuns no início da síndrome são opacidades corneanas numulares periféricas bilaterais. O tratamento desta rara doença utiliza imunossupressão sistêmica com agentes citotóxicos e corticosteróides. O relato de caso apresenta uma paciente com a evolução da forma clássica da síndrome de Cogan.

The successful use of mycophenolate mofetil in a patient with active birdshot chorio-retinopathy refractory to azathioprine therapy: case report

Relato da eficácia da terapia com micofenolato mofetil (MMF) no tratamento de paciente com coriorretinopatia do tipo "birdshot" (CB), no qual o processo inflamatório intra-ocular foi refratário ao uso de azatioprina (AZA) apos um ano de terapia. Paciente de 62 anos de idade e com CB desenvolveu edema macular cistóide e vasculite retiniana em ambos os olhos. No intuito de controlar a inflamação intra-ocular, a terapia com azatioprina oral (2 mg/kg) foi iniciada. Como a terapia com AZA não se mostrou eficaz a médio prazo, esse medicamento foi substituido pelo MMF, o qual controlou controlou o processo inflamatório com conseqüente melhora da acuidade visual do paciente. Após 3 anos de tratamento com MMF, nenhuma recorrência foi observada. Neste paciente com CB refratária ao tratamento com AZA, o MMF foi eficaz em debelar a inflamação intra-ocular.

Recurrent anterior uveitis and healed retinal vasculitis associated with multiple sclerosis.

We describe the occurrence of anterior uveitis with healed retinal vasculitis in an Asian-Indian woman. She had features of anterior uveitis and healed retinal vasculitis. This rare disease in India may be associated with intraocular inflammation.

Frosted retinal branch angiitis in an immunocompetent adult due to herpes simplex virus.

A case of acute severe loss of vision due to perivasculitis typical of frosted retinal branch angiitis due to Herpes simplex virus in an otherwise healthy young Omani lady is presented.

Retinal vasculitis

This work envolves the study of 34 cases of different fundus conditions associated with angiographically proved vaculitis. An extensive medical evaluation was done for all cases. In 8 cases a definite aetiological diagnosis has been reached e.g., toxoplasmosis. Twenty two cases could be categorized into known clinical enteties although their exact aetiology is still unkown e.g. Behcet's disease. In the remining 4 cases no aetilogical or clinical diagnosis could be reached