ABSTRACT
Caso clínico: Varón de 30 años, diagnosticado de desprendimiento viteliforme adquirido (DVA) secundario a drusas cuticulares que presentaba metamorfopsias en su OD. Se trató con inyecciones intravítreas de bevacizumab (Avastin), respondiendo favorablemente. Discusión: Enfermedad independiente, de fenotipo genético aún desconocido, debida a una disfunción generalizada del epitelio pigmentario retiniano (EPR). Evoluciona en un 50% a DVA; con la ayuda de nuevas pruebas complementarias llegamos a un diagnóstico certero. Sin tratamiento efectivo hasta el momento. Dada la frecuencia con la que se desarrolla neovascularización coroidea (NVC), creemos que el tratamiento con fármacos anti-VEGF podría ayudarnos en la estabilización o mejoría funcional y/o anatómica del cuadro (AU)
Case report: We report a case of a 30-year-old male with acquired vitelliform detachment (AVD) secondary to cuticular drusen and suffering from metamorphopsia in his right eye. Intravitreal bevacizumab (Avastin) was administered, achieving successful results. Discussion: An independent disease, of unknown genetic phenotype, caused by a generalized dysfunction of the retinal pigment epithelium (RPE). About 50% of patients develop AVD, and a correct diagnosis can be made with the help of new complementary tests. With no effective treatment currently available, and because of the incidence of developing choroidal neovascularization (NVC), treatment with anti-VEGF could help stabilize or improve the disease functionally and/or anatomically (AU)
Subject(s)
Humans , Male , Adult , Vitelliform Macular Dystrophy/drug therapy , Retinal Drusen/complications , Antibodies, Monoclonal/therapeutic use , Intravitreal InjectionsABSTRACT
CASE REPORT: We report a case of a 30-year-old male with acquired vitelliform detachment (AVD) secondary to cuticular drusen and suffering from metamorphopsia in his right eye. Intravitreal bevacizumab (Avastin) was administered, achieving successful results. DISCUSSION: An independent disease, of unknown genetic phenotype, caused by a generalized dysfunction of the retinal pigment epithelium (RPE). About 50% of patients develop AVD, and a correct diagnosis can be made with the help of new complementary tests. With no effective treatment currently available, and because of the incidence of developing choroidal neovascularization (NVC), treatment with anti-VEGF could help stabilize or improve the disease functionally and/or anatomically.
Subject(s)
Angiogenesis Inhibitors/therapeutic use , Bevacizumab/therapeutic use , Bruch Membrane/pathology , Choroidal Neovascularization/drug therapy , Eye Diseases, Hereditary/complications , Retinal Detachment/drug therapy , Retinal Drusen/complications , Adult , Angiogenesis Inhibitors/administration & dosage , Bevacizumab/administration & dosage , Choroidal Neovascularization/etiology , Emergencies , Fluorescein Angiography , Humans , Intravitreal Injections , Male , Retinal Detachment/etiology , Tomography, Optical Coherence , Vision Disorders/etiologyABSTRACT
CASE REPORT: We report 2 cases of pseudoxanthoma elasticum with angioid streaks and choroidal neovascularization (CNV) in both eyes. Intravitreal ranibizumab (Lucentis) was administered with successful results in both cases. DISCUSSION: CNV has been reported to occur in 72% to 86% of patients with angioid streaks. Although uncommon, the impact of CNV is important because it tends to affect people of working age. Based on the effectiveness of ranibizumab in other secondary CNVs, we decided to use it in our patients, observing the functional and anatomical improvement.
Subject(s)
Angiogenesis Inhibitors/therapeutic use , Antibodies, Monoclonal, Humanized/therapeutic use , Choroidal Neovascularization/drug therapy , Pseudoxanthoma Elasticum/complications , Angiogenesis Inhibitors/administration & dosage , Antibodies, Monoclonal, Humanized/administration & dosage , Choroidal Neovascularization/etiology , Female , Fluorescein Angiography , Humans , Intravitreal Injections/methods , Male , Middle Aged , Ranibizumab , Tomography, Optical CoherenceABSTRACT
Caso clínico: Se presentan dos pacientes diagnosticados de seudoxantoma elástico (PXE), que presentaban estrías angioides (EA) en ambos ojos y neovascularización coroidea (NVC) secundaria de manera unilateral. Ambos fueron tratados con inyecciones intravítreas de ranibizumab (Lucentis), obteniendo una respuesta favorable en ambos casos. Discusión: La NVC afecta al 72-86% de los pacientes con estrías angioides. Es una enfermedad poco frecuente pero importante, ya que se presenta en pacientes con vida laboral activa. Se decidió emplear este tratamiento teniendo en cuenta la efectividad del ranibizumab en NVC secundarias a otros procesos patológicos, observando la mejoría anatómica y funcional del cuadro(AU)
Case report: We report 2 cases of pseudoxanthoma elasticum with angioid streaks andchoroidal neovascularization (CNV) in both eyes. Intravitreal ranibizumab (Lucentis) was administered with successful results in both cases. Discussion: CNV has been reported to occur in 72% to 86% of patients with angioid streaks. Although uncommon, the impact of CNV is important because it tends to affect people of working age. Based on the effectiveness of ranibizumab in other secondary CNVs, we decided to use it in our patients, observing the functional and anatomical improvement(AU)
Subject(s)
Humans , Male , Female , Middle Aged , Choroidal Neovascularization/etiology , Pseudoxanthoma Elasticum/drug therapy , Antibodies, Monoclonal/therapeutic use , Pseudoxanthoma Elasticum/complications , Intravitreal InjectionsABSTRACT
PURPOSE: To evaluate the autofluorescence findings in patients diagnosed with pseudoxanthoma elasticum. MATERIAL AND METHODS: A prospective study was conducted on 18 eyes of 9 patients who had ocular pathology andfollowed up in the pseudoxanthoma elasticum (PSX) unit of our hospital. We evaluated the best corrected visual acuity (BCVA), colour and autofluorescence photography (AF), and fluorescein angiography (FA) in patients with choroidal neovascularitation. RESULTS: Of the 9 patients, 7 were women and 2 were men. The mean age was 40 +/- 14 years. The BCVA ranged from 1 to 0.01 (Mean 0.65 +/- 0.4). All patients showed PSX injuries. Angioid streaks (AS) 18 (100%), peau d'orange 16 (87.5%) and pigmented fibrotic plates 5 (31,5%). We observed different hypoautofluorescence patterns (RPE atrophy), of which 2 of them were AS patterns (irregular lines with hyperautofluorescence speckled in its interior and edges, or bands with lobulated lesions inside and hyperautofluorescence at the edges), and finally widespread areas of hypoautofluorescence, larger than observed by ophthalmoscope. CONCLUSIONS: Autofluorescence in patients with PSX is an easy method to evaluate the initial level of ophthalmoscopic involvement and its subsequent progression. The extensive changes in the retinal pigment epithelium (RPE) suggests the important role of this in the physiopathology of the disease.
Subject(s)
Fluorescence , Fluorometry , Lipofuscin/analysis , Ophthalmoscopy/methods , Pseudoxanthoma Elasticum/diagnosis , Retinal Pigment Epithelium/pathology , Adult , Angioid Streaks/etiology , Angioid Streaks/pathology , Atrophy , Choroidal Neovascularization/etiology , Female , Fibrosis , Fluorescein Angiography , Humans , Lipofuscin/chemistry , Lysosomes/chemistry , Lysosomes/ultrastructure , Male , Middle Aged , Prospective Studies , Pseudoxanthoma Elasticum/diagnostic imaging , Pseudoxanthoma Elasticum/pathology , Radiography , Retinal Pigment Epithelium/blood supply , Visual Acuity , Young AdultABSTRACT
PropósitoEvaluar las lesiones funduscópicas con autofluorescencia en pacientes diagnosticados de pseudoxantoma elástico (PSX).Material y métodoSe realizó un estudio prospectivo de 9 pacientes (18 ojos), en seguimiento por la unidad de PSX de nuestro hospital, que presentaban dolencia ocular. Evaluamos mejor agudeza visual corregida (MAVC), fotografía en color y con autofluorescencia (FA), así como angiografía fluoresceínica (AFG) en los casos que presentaban neovascularización coroidea (NVC).ResultadosDe los 9 pacientes, 7 eran mujeres y 2 hombres. Edad media de 40,5 +/- 14, MAVC desde la 1 a 0,01 (media=0,65 +/- 0,4). Todos los pacientes (18 ojos) presentaban lesiones del PSX: estrías angioides (EA) en 18 (100%), fondo en «piel de naranja» en 16 (87,5%) y placas fibrogliales pigmentadas en 5 ojos (31,5%). Observamos diferentes patrones de hipoautofluorescencias (atrofia de epitelio pigmentario), dos que se corresponderían a EA (líneas anfractuosas con moteado hiperautofluorescente en su seno y bordes, o bien bandas con lesiones lobuladas en su seno e hiperautofluorescencia en sus bordes) y por último grandes placas hipoautofluorescentes, de mayor tamaño que las observadas funduscópicamente.ConclusionesLa autofluorescencia en los pacientes con PSX es un método fácil para valorar el grado de afectación funduscópica inicial y su posterior evolución. La gran afectación del epitelio pigmentario retiniano (EPR), que hemos observado, nos sugiere el importante papel que juega este, en la fisiopatología de esta enfermedad(AU)
PurposeTo evaluate the autofluorescence findings in patients diagnosed with pseudoxanthoma elasticum.Material and methodsA prospective study was conducted on 18 eyes of 9 patients who had ocular pathology andfollowed up in the pseudoxanthoma elasticum (PSX) unit of our hospital. We evaluated the best corrected visual acuity (BCVA), colour and autofluorescence photography (AF), and fluorescein angiography (FA) in patients with choroidal neovascularitation.ResultsOf the 9 patients, 7 were women and 2 were men. The mean age was 40 +/- 14 years. The BCVA ranged from 1 to 0.01 (Mean 0.65 +/- 0.4).All patients showed PSX injuries. Angioid streaks (AS) 18 (100%), peau dorange 16 (87.5%) and pigmented fibrotic plates 5 (31,5%). We observed different hypoautofluorescence patterns (RPE atrophy), of which 2 of them were AS patterns (irregular lines with hyperautofluorescence speckled in its interior and edges, or bands with lobulated lesions inside and hyperautofluorescence at the edges), and finally widespread areas of hypoautofluorescence, larger than observed by ophthalmoscope.ConclusionsAutofluorescence in patients with PSX is an easy method to evaluate the initial level of ophthalmoscopic involvement and its subsequent progression. The extensive changes in the retinal pigment epithelium (RPE) suggests the important role of this in the physiopathology of the disease(AU)
Subject(s)
Humans , Pseudoxanthoma Elasticum/diagnosis , Angioid Streaks/diagnosis , Spectrometry, Fluorescence , Diagnosis, Differential , Prospective StudiesABSTRACT
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Subject(s)
Male , Aged , Humans , Carcinoma, Squamous Cell/complications , Cellulite/etiology , Urinary Bladder Neoplasms/complications , Urinary Diversion , Cystectomy , Fasciitis, NecrotizingABSTRACT
The effect of the enucleation of one eye on anterograde and retrograde labelling in geniculo-cortical, cortico-geniculate and commissural projections was investigated in adult cats by means of horseradish peroxidase (HRP) and tritiated aminoacids. It was found that in addition to the immediate decrease of retrograde labelling with HRP in the cortical projections from the deafferented A-laminae of the dorsal part of the lateral geniculate nucleus (Singer et al. 1977) there is a further reduction which lasts up to 75 days after enucleation. At 146 and 363 days after enucleation a slight increase in the number of labelled neurones was noted in the deafferented lamina. Qualitative assessment did not reveal any changes of anterograde labelling with tritiated amino acids in geniculo-cortical, cortico-geniculate and commissural axones. In addition, the retrograde labelling with HRP in cortico-geniculate and commissural projections seemed to be unaffected by eye enucleation.
