RESUMEN
BACKGROUND: Multiple sclerosis (MS) is an autoimmune demyelinating disease of the central nervous system with neuroaxonal damage. It is the principal driver of non-traumatic disability in young adults. Visual symptoms are common and optic neuritis (ON) may be the revealing feature in up to 30% of cases. Structural optical coherence tomography (OCT) represents a biomarker of central nervous system neurodegeneration in MS. OCT-angiography (OCT-A) is a noninvasive tool allowing the study of retinal vasculature and the detection of microvascular damage in neuro-retinal diseases. In this study, we aimed to assess structural and microvascular retinal changes in patients with MS with and without ON and to correlate the findings with visual function and MS disability. METHODS: We conducted a cross-sectional study including patients diagnosed with MS according to the 2017 McDonald criteria. All patients underwent complete neurological examination with evaluation of the Expanded Disability Status Scale (EDSS) and the Multiple Sclerosis Severity Score (MSSS) and an ophthalmological examination including OCT and OCT-A. Patients were compared with age- and sex-matched healthy subjects. The primary endpoints were assessment of retinal nerve fiber layer (RNFL) thickness, ganglion cell layer (GCL+), and ganglion cell complex (GCL++) thicknesses on OCT. Vascular densities in the superficial capillary plexus (SCP), deep capillary plexus (DCP), and choriocapillaris (CC) were assessed on OCT-A, as well as central avascular zone (CAZ) parameters, lacunarity and fractal dimension. RESULTS: A total of 160 MS eyes with and without a previous history of ON and 64 age- and gender-matched healthy eyes were analyzed. Among 160 eyes with MS, 69 had a history of ON. We observed a decrease in RNFL and GCL++ thickness in all 12 quadrants in MS patients when compared to healthy controls. Multivariate analysis by linear regression noted a significant correlation for temporal GCL++ and inferonasal RNFL thickness that were decreased in the MS group. A greater decrease in retinal layers thickness was identified in MS patients with a history of ON. On OCT-A, vascular density in (SCP) was significantly reduced in the MS group (P<0.002). A significant correlation between RNFL thickness and retinal vascular density was found but only in less than half of the hourly quadrants. A significant correlation was noted between visual acuity and CC density (P<0.0001). We also noted an inverse correlation between EDSS scores and CC density (P=0.02 and r=-0.275) and between MSSS and RNFL/GCL++ thicknesses. CONCLUSIONS: RNFL and GCL++ layers were thinner in MS patients with a history of ON and were reversely correlated with disease severity. Moreover, retinal vascular changes were observed in MS even in eyes without ON, and CC was reversely correlated with visual function and current disability. Thus, structural OCT coupled with OCT-A could represent a noninvasive and dynamic biomarker of MS severity and progression.
Asunto(s)
Esclerosis Múltiple , Neuritis Óptica , Retina , Vasos Retinianos , Tomografía de Coherencia Óptica , Humanos , Tomografía de Coherencia Óptica/métodos , Femenino , Masculino , Estudios Transversales , Adulto , Esclerosis Múltiple/diagnóstico por imagen , Esclerosis Múltiple/complicaciones , Esclerosis Múltiple/patología , Retina/diagnóstico por imagen , Retina/patología , Persona de Mediana Edad , Vasos Retinianos/diagnóstico por imagen , Vasos Retinianos/patología , Neuritis Óptica/diagnóstico por imagen , Neuritis Óptica/etiología , Neuritis Óptica/diagnóstico , Neuritis Óptica/patología , Angiografía con Fluoresceína/métodos , Adulto Joven , Agudeza VisualAsunto(s)
Enfermedades de la Coroides , Granuloma , Sarcoidosis , Humanos , Sarcoidosis/diagnóstico , Sarcoidosis/complicaciones , Sarcoidosis/patología , Granuloma/diagnóstico , Granuloma/patología , Enfermedades de la Coroides/diagnóstico , Enfermedades de la Coroides/patología , Enfermedades de la Coroides/etiología , Femenino , Masculino , Persona de Mediana EdadRESUMEN
Systemic Lupus Erythematosus (SLE) is a chronic auto-immune condition with systemic and ocular involvement. Ophthalmological findings are diverse and can involve all layers of the globe. Posterior segment manifestations can include lupus retinopathy, retinal vascular occlusions and lupus choroidopathy. We report here a rare case of central retinal artery occlusion (CRAO) associated with anterior ischemic optic neuropathy (AION) in a young female patient as presenting signs of SLE. In this case report, we highlight a very uncommon initial presentation of this severe systemic vasculitis. A multidisciplinary approach is required in order to prevent life-threatening vaso-occlusive complications.
Asunto(s)
Enfermedad por Rasguño de Gato , Oclusión de la Arteria Retiniana , Animales , Enfermedad por Rasguño de Gato/diagnóstico , Enfermedad por Rasguño de Gato/diagnóstico por imagen , Gatos , Humanos , Imagen Multimodal , Retina/diagnóstico por imagen , Oclusión de la Arteria Retiniana/diagnóstico , Oclusión de la Arteria Retiniana/etiologíaRESUMEN
PURPOSE: To study the appearance of angioid streaks (AS) in swept source optical coherence tomography angiography. METHODS: Retrospective observational study of 16 patients (31 eyes) with various stages of AS. All included patients underwent complete ophthalmologic examinations including best-corrected visual acuity (BCVA), slit-lamp examination, indirect ophthalmoscopy and fundus photography. Swept source optical coherence tomography (SS-OCT), OCT angiography (OCT-A) and fluorescein angiography were also performed. RESULTS: En face OCT detected hyper-reflective points in 65% of cases, with a choriocapillaris (CC) shadow on the corresponding OCT-A. Diffuse CC rarefaction was detected in 94%. In eyes without neovascular complications, streaks were visible as a hyposignal in the outer retina. An irregular vascular network (IVN) was detected in 80% of eyes. It filled the spaces between the AS and corresponded to flat elevation of the retinal pigmentary epithelium. Twenty-four eyes had choroidal neovascularization (CNV). CNV was type 1 in 8%, type 2 in 43%, mixed in 20% and unclassified in 29% because of a large scar. We found multiple sites of CNV in 8% of cases. CNV shape was tangled in 66% and in 2 eyes with newly diagnosed CNV. OCT-A showed a perilesional halo around new CNV. The morphology and configuration of neovascular network follow the IVN and the path of the AS and arises in proximity to sites of BM disruption. CONCLUSION: OCT-A allows early detection and monitoring of AS and their neovascular complications. It shows CC rarefaction, IVN and a predominantly tangled shape of CNV. However, there are some limitations associated with difficulty in characterizing signs of CNV activity.
Asunto(s)
Estrías Angioides , Neovascularización Coroidal , Estrías Angioides/complicaciones , Estrías Angioides/diagnóstico , Coroides , Neovascularización Coroidal/diagnóstico por imagen , Angiografía con Fluoresceína , Humanos , Tomografía de Coherencia ÓpticaAsunto(s)
Neoplasias de la Conjuntiva/diagnóstico , Dirofilariasis/diagnóstico , Infecciones Parasitarias del Ojo/diagnóstico , Corticoesteroides/uso terapéutico , Animales , Antibacterianos/uso terapéutico , Neoplasias de la Conjuntiva/tratamiento farmacológico , Diagnóstico Diferencial , Dirofilaria repens/fisiología , Dirofilariasis/tratamiento farmacológico , Dirofilariasis/parasitología , Perros , Infecciones Parasitarias del Ojo/tratamiento farmacológico , Infecciones Parasitarias del Ojo/parasitología , Humanos , Londres , Masculino , Persona de Mediana Edad , Movimiento/fisiología , TúnezAsunto(s)
Fóvea Central/diagnóstico por imagen , Imagen Óptica , Enfermedades de la Retina/diagnóstico , Tomografía de Coherencia Óptica , Trastornos de la Visión/diagnóstico , Niño , Color , Consanguinidad , Fóvea Central/metabolismo , Fóvea Central/patología , Humanos , Masculino , Trastornos de la Pigmentación/diagnóstico , Trastornos de la Pigmentación/metabolismo , Trastornos de la Pigmentación/patología , Enfermedades de la Retina/metabolismo , Enfermedades de la Retina/patología , Túnez , Trastornos de la Visión/metabolismo , Trastornos de la Visión/patologíaAsunto(s)
Anomalías del Ojo/diagnóstico , Anomalías del Ojo/genética , Enfermedades Genéticas Ligadas al Cromosoma X/diagnóstico , Retina/anomalías , Síndrome de Aicardi/diagnóstico , Síndrome de Aicardi/genética , Niño , Aberraciones Cromosómicas , Trastornos de los Cromosomas/diagnóstico , Trastornos de los Cromosomas/genética , Cromosomas Humanos Par 8/genética , Cromosomas Humanos X/genética , Femenino , Enfermedades Genéticas Ligadas al Cromosoma X/genética , Humanos , Lactante , Recién Nacido , Masculino , Retina/diagnóstico por imagen , Retina/patología , Trisomía/diagnóstico , Trisomía/genética , Síndrome de Turner/diagnóstico , Síndrome de Turner/genéticaAsunto(s)
Enfermedades Hereditarias del Ojo/complicaciones , Enfermedades Hereditarias del Ojo/diagnóstico por imagen , Edema Macular/diagnóstico por imagen , Edema Macular/etiología , Degeneración Retiniana/complicaciones , Degeneración Retiniana/diagnóstico por imagen , Quistes/diagnóstico , Quistes/diagnóstico por imagen , Quistes/etiología , Enfermedades Hereditarias del Ojo/diagnóstico , Fondo de Ojo , Humanos , Edema Macular/diagnóstico , Masculino , Persona de Mediana Edad , Imagen Óptica , Degeneración Retiniana/diagnóstico , Tomografía de Coherencia ÓpticaRESUMEN
PURPOSE: to report a case of a woman who had a severe cranial trauma complicated by two ophalmologic potentially cecitating complications: right carotido-cavernous fistula and left traumatic optic neuropathy. OBSERVATION: A 56 years-old woman, without any medical history, referred, one month after fall from a height of 3 meters, for right exophthalmos. Ophthalmic examination of the right eye completed by retinal angiography suspected carotid-cavernous fistula which was confirmed by angio-MRI. In the left eye, the visual acuity was decreased to no light perception and fundus examination showed optic nerve head palor secondary to traumatic optic neuropathy. Arterial embolization was performed and allowed closing of the fistula. CONCLUSION: Optic neuropathy and carotido-cavernous fistula are two severe complications that can occur simultaneously in the same patient. The prognosis of the optic neuropathy may be compromised, and the treatment of carotido-cavernous fistula benefited from progress in interventional neuro-radiology.
Asunto(s)
Fístula del Seno Cavernoso de la Carótida/etiología , Traumatismos Craneocerebrales/complicaciones , Enfermedades del Nervio Óptico/etiología , Fístula del Seno Cavernoso de la Carótida/diagnóstico , Fístula del Seno Cavernoso de la Carótida/terapia , Seno Cavernoso/patología , Traumatismos Craneocerebrales/diagnóstico , Traumatismos Craneocerebrales/terapia , Embolización Terapéutica , Exoftalmia/diagnóstico , Exoftalmia/etiología , Exoftalmia/terapia , Femenino , Humanos , Persona de Mediana Edad , Enfermedades del Nervio Óptico/diagnóstico , Enfermedades del Nervio Óptico/terapia , Resultado del TratamientoAsunto(s)
Coristoma/diagnóstico , Neoplasias de la Coroides/diagnóstico , Neovascularización Coroidal/diagnóstico , Osteoma/diagnóstico , Tomografía de Coherencia Óptica , Bevacizumab/administración & dosificación , Coristoma/complicaciones , Coristoma/tratamiento farmacológico , Neoplasias de la Coroides/complicaciones , Neoplasias de la Coroides/tratamiento farmacológico , Neovascularización Coroidal/complicaciones , Neovascularización Coroidal/tratamiento farmacológico , Femenino , Estudios de Seguimiento , Humanos , Inyecciones Intravítreas , Persona de Mediana Edad , Osteoma/complicaciones , Osteoma/tratamiento farmacológico , Factores de Tiempo , Tomografía de Coherencia Óptica/métodosRESUMEN
PURPOSE: To describe the contribution of multimodal imaging in the various stages of Stargardt disease (STGD). PATIENTS AND METHODS: We retrospectively reviewed 46 eyes of 23 STGD patients with identified ABCA4 mutations. All patients underwent a complete ophthalmic examination, spectral-domain optical coherence tomography (SD-OCT), fundus autofluorescence (FAF), fluorescein angiography (FA) and Indocyanine green angiography (ICGA). RESULTS: The mean age of patients was 25.5 years (range 8-56). Fundus examination was normal in 2 patients (subclinical stage), where SD-OCT showed localized retrofoveolar retinal pigment epithelium (RPE) thickening. FAF was normal in 1 eye and showed mild heterogeneous hyper-FAF in 3 eyes. Twelve eyes had mild salt and pepper changes in the macula (early stage) with diffuse retinal atrophy on SD-OCT and mixed hyper and hypoautofluorescence on FAF. Nine patients showed central atrophy with white-yellow flecks distributed in the posterior pole and mid-periphery. This phenotype showed total foveal atrophy on SD-OCT and normal peripapillary area on FAF. Twelve eyes had a large demarcated area of RPE atrophy, pigment clumping and migration extending to the peripheral retina associated with peripapillary atrophy. These eyes showed diffuse retinochoroidal atrophy on OCT with diffuse alterations reaching the peripapillary area on FAF. On FA, it was difficult to analyze the choroidal silence sign in patients with advanced stages of the disease. A hyperfluorescent window defect pattern was also found in patients with white-yellow flecks and did not correspond exactly to them, or to the areas of peripheral autofluorescent lesions. ICGA showed hypocyanescent areas seen at intermediate and late phases with multiple cyanescent points adjacent to them. On ICGA, hypocyanescent areas were more extensive than lesions observed on FAF. CONCLUSIONS: Multimodal imaging is helpful for the diagnosis of early stages of STGD disease and to better understand its pathophysiology. FAF and mostly SD-OCT have supplanted FA in the early, especially subclinical, stages. Over all, ICGA shows more extensive damage, making this tool useful for better understanding STGD and suggesting possible direct damage to the choriocapillaris associated with RPE lesions. In advanced stages, only DNA testing can confirm the diagnosis of STGD.
Asunto(s)
Técnicas de Diagnóstico Oftalmológico , Degeneración Macular/congénito , Imagen Multimodal/métodos , Adolescente , Adulto , Niño , Progresión de la Enfermedad , Familia , Femenino , Genes Recesivos , Humanos , Degeneración Macular/diagnóstico , Degeneración Macular/genética , Degeneración Macular/patología , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Enfermedad de Stargardt , Adulto JovenAsunto(s)
Contusiones/complicaciones , Lesiones Oculares/complicaciones , Oclusión de la Arteria Retiniana/etiología , Oclusión de la Vena Retiniana/etiología , Adulto , Contusiones/patología , Lesiones Oculares/patología , Femenino , Humanos , Oclusión de la Arteria Retiniana/patología , Oclusión de la Vena Retiniana/patología , Trastornos de la Visión/etiología , Trastornos de la Visión/patologíaAsunto(s)
Lesiones Oculares/diagnóstico , Neoplasias del Ojo/diagnóstico , Subluxación del Cristalino/diagnóstico , Limbo de la Córnea/patología , Anciano de 80 o más Años , Diagnóstico Diferencial , Lesiones Oculares/complicaciones , Lesiones Oculares/patología , Neoplasias del Ojo/patología , Femenino , Humanos , Subluxación del Cristalino/etiología , Subluxación del Cristalino/patologíaRESUMEN
Best vitelliform macular dystrophy is the second most frequent hereditary maculopathy, with bilateral involvement and juvenile onset. It is clinically characterized by bilateral deposits of lipofuscin-like autofluorescent material in the subretinal space, with a typical phenotypic manifestation taking the form of a vitelliform macular lesion evolving gradually into more advanced stages. The purpose of our study was to describe fundus autofluorescence patterns and OCT findings in three patients (6 eyes) with several stages of Best vitelliform macular dystrophy. Optical coherence tomography (OCT) has become the first imaging technique to order when confronted with a hereditary maculopathy suggesting Best disease. Fundus autofluorescence combined with OCT allow for better diagnosis and management, which are necessary for any genetic analysis.