RESUMEN
ABSTRACT A 39-year-old policeman complained of decreased bilateral central vision over the last two years. On examination, visual acuity was 20/40 and 20/400 in the right (OD) and left eye (OS), respectively, and fundoscopy revealed a bilateral hypopigmented macular lesion. Fluorescein and indocyanine green angiography demonstrated leakage and hyperintense spots, respectively, within the macular areas. At baseline, optical coherence tomography showed subretinal fluid in the OD and a conforming focal choroidal excavation in the OS. Focal choroidal excavation converted from conforming to nonconforming type at 4-month follow-up and then reversed to conforming type at 12-month follow-up, and was associated with incomplete retinal pigment epithelium and outer retina atrophy over the area of excavation. Pachyvessels were also evidenced in the choroid, without neovascularization. We report for the first time a case of focal choroidal excavation that progressed from conforming to nonconforming type and then reverted to its primary configuration (conforming type) in a patient with concurrent bilateral central serous chorioretinopathy.
RESUMO Um policial de 39 anos se queixava de diminuição bilateral da visão central nos últimos 2 anos. A acuidade visual era 20/40 e 20/400 no olho direito (OD) e esquerdo (OE) e a fundoscopia revelou lesão macular hipopigmentada bilateral. A angiografia fluoresceínica e com indocianina verde revelaram, respectivamente, vazamento do corante e áreas hiperintensas nas regiões maculares. A tomografia de coerência óptica evi denciou fluido sub-retiniano no OD e escavação focal de coroide do tipo conformacional no OE. Após 4 meses, a escavação focal de coroide mudou de conformacional para não conformacional e, aos 12 meses, reverteu para conformacional associado a atrofia incompleta do epitélio pigmentar da retina e da retina externa na região da escavação. Também foi evidenciado paquicoroide, sem neovascularização. Relatamos pela primeira vez uma escavação focal de coroide que evoluiu de conformacional para não conformacional e, em seguida, retornou à configuração primária (conformacional) em um paciente com coriorretinopatia serosa central bilateral.
RESUMEN
A 39-year-old policeman complained of decreased bilateral central vision over the last two years. On examination, visual acuity was 20/40 and 20/400 in the right (OD) and left eye (OS), respectively, and fundoscopy revealed a bilateral hypopigmented macular lesion. Fluorescein and indocyanine green angiography demonstrated leakage and hyperintense spots, respectively, within the macular areas. At baseline, optical coherence tomography showed subretinal fluid in the OD and a conforming focal choroidal excavation in the OS. Focal choroidal excavation converted from conforming to nonconforming type at 4-month follow-up and then reversed to conforming type at 12-month follow-up, and was associated with incomplete retinal pigment epithelium and outer retina atrophy over the area of excavation. Pachyvessels were also evidenced in the choroid, without neovascularization. We report for the first time a case of focal choroidal excavation that progressed from conforming to nonconforming type and then reverted to its primary configuration (conforming type) in a patient with concurrent bilateral central serous chorioretinopathy.
Asunto(s)
Coriorretinopatía Serosa Central , Enfermedades de la Coroides , Adulto , Coriorretinopatía Serosa Central/complicaciones , Coriorretinopatía Serosa Central/diagnóstico por imagen , Coroides/diagnóstico por imagen , Enfermedades de la Coroides/diagnóstico por imagen , Angiografía con Fluoresceína , Humanos , Verde de Indocianina , Tomografía de Coherencia ÓpticaRESUMEN
Colorectal cancer may yield metastasis to the choroid. Its management may be challenging, since there is no consensus about treatment. We describe a case of a 70-year-old male with colon cancer who complained of worsening visual acuity of his better-seeing eye to 20/40 secondary to a nonpigmented choroidal mass of medium reflectivity under the inferior temporal arcade and neurosensory foveal detachment. Besides systemic chemotherapy, local treatment with verteporfin photodynamic therapy (vPDT) was performed. After one month, visual acuity improved to 20/25 and subretinal fluid faded. In conclusion, vPDT may be a useful adjuvant treatment modality for choroidal metastasis secondary to colorectal cancer.
RESUMEN
INTRODUCTION: There are few real-life studies on the intravitreal 0.7-mg dexamethasone implant for the treatment of diabetic macular edema (DME) conducted in Latin America. We aimed to assess the effectiveness and safety of this implant in clinical practice. METHODS: Twenty-seven centers from Brazil and one from Argentina provided information on patients with DME treated with Ozurdex. The efficacy outcome variables were best-corrected visual acuity (BCVA) in Snellen and central retinal thickness (CRT). Safety was assessed by the elevation in intraocular pressure (IOP), occurrence of cataracts, and adverse events. RESULTS: A total of 329 eyes (both treated cases and naïve eyes) from 282 patients underwent treatment. The time since diagnosis of DME ranged from 1 to 156 months. The median BCVA was 0.7 logMAR/50 letters at baseline and 0.3 logMAR/70 letters after treatment (both p < 0.001). Median CRT values decreased from 425 µm at baseline to 270 µm after treatment (p < 0.001). Increases in IOP of at least 10 mm Hg were observed in 7.4% of eyes, and 4% of eyes had cataract evolution. No cases of endophthalmitis were reported. CONCLUSION: These real-life results suggest that the intravitreal dexamethasone implant is effective and safe for eyes with DME.
Asunto(s)
Dexametasona/administración & dosificación , Retinopatía Diabética/complicaciones , Edema Macular/tratamiento farmacológico , Agudeza Visual , Adulto , Anciano , Anciano de 80 o más Años , Retinopatía Diabética/diagnóstico , Retinopatía Diabética/tratamiento farmacológico , Implantes de Medicamentos , Femenino , Estudios de Seguimiento , Glucocorticoides/administración & dosificación , Humanos , Inyecciones Intravítreas , Edema Macular/diagnóstico , Edema Macular/etiología , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
BACKGROUND: Sudden visual loss and optic disc edema caused by optic neuritis (ON) is usually followed by significant visual recovery. However, little or no recovery occurs when the loss is caused by atypical ON, especially in patients with neuromyelitis optica (NMO). Optic disc drusen (ODD) is a cause of pseudo optic disc edema and may be a predisposing factor for non-arteritic anterior ischemic optic neuropathy (NAION), thereby mimicking atypical ON. In such cases, if globular concretions are seen protruding from the disc substance, ODD may be suspected. The purpose of this paper is to describe two patients with acute visual loss followed by optic disc atrophy initially labeled as atypical ON. Though not suspected on clinical examination, optical coherence tomography (OCT) revealed deeply buried ODD as a predisposing factor for NAION. CASE PRESENTATIONS: Case 1: A 48-year-old woman had bilateral sequential visual loss associated with optic disc edema. Despite treatment, vision did not improve and severe disc pallor ensued. Atypical ON was suspected. Eventually, she was started on immunosuppressant therapy based on a tentative diagnosis of NMO-spectrum disorder. On examination 5 years later, only severe optic disc pallor was observed, but OCT radial B-scans showed ovoid hyporeflective areas in the retrolaminar region of both eyes, compatible with ODD; this led to a diagnosis of NAION and deeply buried ODD. Case 2. A 35-year-old woman with suspicion of ON in the left eye and a history of previous atypical ON in the right eye was referred for neuro-ophthalmic examination which revealed diffuse optic disc pallor and a dense arcuate visual field defect in the right eye. OCT B-scans passing through the disc showed large ovoid areas of reduced reflectivity in the retrolaminar region of the optic disc in the right eye. These findings helped confirm the diagnosis of NAION in one eye, with deeply buried ODD as predisposing factor. CONCLUSIONS: Deeply buried ODD may be associated with NAION causing irreversible visual loss and optic disc pallor, a condition easily mistaken for atypical ON. Awareness of such occurrence is important to avoid unnecessary testing and minimize the risk of mismanagement.
Asunto(s)
Ceguera/etiología , Atrofia Óptica/complicaciones , Drusas del Disco Óptico/complicaciones , Papiledema/etiología , Tomografía de Coherencia Óptica/métodos , Agudeza Visual , Adulto , Ceguera/diagnóstico , Ceguera/fisiopatología , Diagnóstico Diferencial , Femenino , Estudios de Seguimiento , Humanos , Persona de Mediana Edad , Atrofia Óptica/diagnóstico , Disco Óptico , Drusas del Disco Óptico/diagnóstico , Neuritis Óptica/diagnóstico , Papiledema/diagnósticoRESUMEN
OBJECTIVE: To evaluate the quality of life (QoL) and potential QoL determinants in patients with clinically isolated syndrome (CIS). METHODS: Eighteen CIS patients and eighteen controls were submitted to QoL evaluation with Functional Assessment of Multiple Sclerosis QoL instrument (FAMS). Cognition was evaluated with specific battery tests; Anxiety and depression with Beck Anxiety (BAI) and Depression (BDI) Inventories and Neurological disability with Guy's Neurological Disability Scale (GNDS). RESULTS: There was a significant difference in QoL between CIS patients and controls. CIS patients had worse performance in Paced Auditory Serial Addition 2 seconds (p=0.009) and fluency tests (p=0.0038). There was a significant difference in BAI (p=0.003), but no significant difference in BDI between patients and controls. There were significant correlations between QoL measure and verbal fluency and Stroop's test. CONCLUSIONS: Cognition, but not anxiety, depression and disability, was associated with reduced quality of life.
OBJETIVO: Avaliar a qualidade de vida (QoL) e seus potenciais determinantes em pacientes com síndrome clinicamente isolada (SCI). MÉTODOS: Dezoito pacientes com SCI e 18 controles realizaram avaliação da QoL com Escala de Determinação da QoL na Esclerose Múltipla; cognição foi avaliada com bateria de testes específica; ansiedade e depressão com os Inventários de Beck de ansiedade (BAI) e de depressão (BDI) e a incapacidade neurológica com a Guy's Neurological Disability Scale. RESULTADOS: Houve diferença significativa na QoL avaliada entre pacientes com SCI e controles. Pacientes com SCI apresentaram pior desempenho no Pased Auditory Serial Addition 2 segundos (p=0,009) e na fluência verbal (p=0,0038). Houve diferença no BAI (p=0,003), entretanto sem diferença do BDI entre pacientes e controles. Houve correlações significativas entre QoL, fluência verbal e Stroop. CONCLUSÕES: Alterações cognitivas tiveram correlação com diminuição da QoL, o que não ocorreu com a depressão, ansiedade e incapacidade neurológica.
Asunto(s)
Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Adulto Joven , Trastornos del Conocimiento/psicología , Cognición/fisiología , Enfermedades Desmielinizantes/psicología , Calidad de Vida/psicología , Ansiedad/psicología , Estudios de Casos y Controles , Depresión/psicología , Esclerosis Múltiple/psicología , Pruebas Neuropsicológicas , Estadísticas no ParamétricasRESUMEN
OBJECTIVE: To evaluate the quality of life (QoL) and potential QoL determinants in patients with clinically isolated syndrome (CIS). METHODS: Eighteen CIS patients and eighteen controls were submitted to QoL evaluation with Functional Assessment of Multiple Sclerosis QoL instrument (FAMS). Cognition was evaluated with specific battery tests; Anxiety and depression with Beck Anxiety (BAI) and Depression (BDI) Inventories and Neurological disability with Guy's Neurological Disability Scale (GNDS). RESULTS: There was a significant difference in QoL between CIS patients and controls. CIS patients had worse performance in Paced Auditory Serial Addition 2 seconds (p=0.009) and fluency tests (p=0.0038). There was a significant difference in BAI (p=0.003), but no significant difference in BDI between patients and controls. There were significant correlations between QoL measure and verbal fluency and Stroop's test. CONCLUSIONS: Cognition, but not anxiety, depression and disability, was associated with reduced quality of life.
Asunto(s)
Trastornos del Conocimiento/psicología , Cognición/fisiología , Enfermedades Desmielinizantes/psicología , Calidad de Vida/psicología , Adulto , Ansiedad/psicología , Estudios de Casos y Controles , Depresión/psicología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Esclerosis Múltiple/psicología , Pruebas Neuropsicológicas , Estadísticas no Paramétricas , Adulto JovenRESUMEN
Cognitive abnormalities have been extensively studied in Multiple Sclerosis (MS). However, little is known aboutthe cognitive involvement in patients with Clinically Isolated Syndrome (CIS). Objective: This study aimed to investigatecognitive impairment in patients with CIS compared with healthy subjects. Methods: 18 CIS patients and 18 controlswere subjected to the Wechsler memory scale, Rey Auditory Verbal Learning, Rey Complex Figure, Paced Auditory SerialAddition, Digit Span, verbal fluency, Stroop color card test, D2, and Digit Symbol tests. Results: CIS patients had significantlyworse performance on the Paced Auditory Serial Addition Test (PASAT) 2 seconds (P=0.009) and on verbal fluency tests(P=0.0038) than controls. Conclusion: CIS patients had worse cognitive performance than controls on neuropsychologicaltests evaluating executive functioning.
As alterações cognitivas na Esclerose Múltipla (EM) têm sido bastante estudadas. No entanto, ainda são poucosos estudos acerca do comprometimento cognitivo em pacientes com Síndrome Clinicamente Isolada (SCI). Objetivo:O objetivo deste estudo foi o de investigar funções cognitivas em pacientes com SCI em relação a um grupo controle.Métodos: Dezoito pacientes com SCI e 18 controles saudáveis foram submetidos à avaliação neuropsicológica, incluindoos seguintes testes: Escala Wechsler de Memória, ?Rey Auditory Verbal Learning Test?, Figura Complexa de Rey, ?PacedAuditory Serial Addition (PASAT) 2 e 3 segundos, ?Digit Span?, fluência verbal, teste de Stroop, D2 e ?Digit Symbol Test?.Resultados: Pacientes com SCI tiveram desempenho significativamente inferior nos testes PASAT 2 segundos (P=0.009)e fluência verbal (P=0.0038) quando comparados ao grupo controle. Conclusão: Pacientes com síndrome clinicamenteisolada apresentaram pior desempenho cognitivo em testes relacionados a funções executivas.
Asunto(s)
Humanos , Cognición , Pruebas NeuropsicológicasRESUMEN
Cognitive abnormalities have been extensively studied in Multiple Sclerosis (MS). However, little is known about the cognitive involvement in patients with Clinically Isolated Syndrome (CIS). OBJECTIVE: This study aimed to investigate cognitive impairment in patients with CIS compared with healthy subjects. METHODS: 18 CIS patients and 18 controls were subjected to the Wechsler memory scale, Rey Auditory Verbal Learning, Rey Complex Figure, Paced Auditory Serial Addition, Digit Span, verbal fluency, Stroop color card test, D2, and Digit Symbol tests. RESULTS: CIS patients had significantly worse performance on the Paced Auditory Serial Addition Test (PASAT) 2 seconds (P=0.009) and on verbal fluency tests (P=0.0038) than controls. CONCLUSION: CIS patients had worse cognitive performance than controls on neuropsychological tests evaluating executive functioning.
As alterações cognitivas na Esclerose Múltipla (EM) têm sido bastante estudadas. No entanto, ainda são poucos os estudos acerca do comprometimento cognitivo em pacientes com Síndrome Clinicamente Isolada (SCI). OBJETIVO: O objetivo deste estudo foi o de investigar funções cognitivas em pacientes com SCI em relação a um grupo controle. MÉTODOS: Dezoito pacientes com SCI e 18 controles saudáveis foram submetidos à avaliação neuropsicológica, incluindo os seguintes testes: Escala Wechsler de Memória, "Rey Auditory Verbal Learning Test", Figura Complexa de Rey, "Paced Auditory Serial Addition (PASAT) 2 e 3 segundos, "Digit Span", fluência verbal, teste de Stroop, D2 e "Digit Symbol Test". RESULTADOS: Pacientes com SCI tiveram desempenho significativamente inferior nos testes PASAT 2 segundos (P=0.009) e fluência verbal (P=0.0038) quando comparados ao grupo controle. CONCLUSÃO: Pacientes com síndrome clinicamente isolada apresentaram pior desempenho cognitivo em testes relacionados a funções executivas.
RESUMEN
Optical coherence tomography (OCT) has proved to be a very valuable tool in the assessment of patients with glaucoma. In this report, intracranial tumors were discovered in two glaucoma suspects referred for diagnostic confirmation by OCT - a clivus chordoma and a craniopharyngeoma. Optical coherence tomography findings - marked asymmetrical diffuse attenuation of the peripapillary nerve fiber layer in nasal and temporal sectors - raised concerns about lesions in chiasmatic region and permitted the timely diagnosis of these intraocular tumors.
Asunto(s)
Hipertensión Ocular/diagnóstico , Quiasma Óptico , Neoplasias del Nervio Óptico/diagnóstico , Neoplasias Hipofisarias/diagnóstico , Cordoma/diagnóstico , Craneofaringioma/diagnóstico , Diagnóstico Diferencial , Femenino , Humanos , Persona de Mediana Edad , Tomografía de Coherencia ÓpticaRESUMEN
Antimalarial drugs, such as chloroquine, are useful in the management of rheumatic diseases, but may cause a potentially blinding condition known as toxic maculopathy. This report describes the findings on standard and on high-resolution spectral domain ocular coherence tomography performed in a CirrusTM HD-OCTdevice in two patients with chloroquine maculopathy. In one case, a very similar aspect of retinal pigment epithelium (RPE) plaque atrophy occured in both angiographic and HD-OCT images obtained by 'en-face' (coronal) segmentation. In another patient with clinical signs of maculopathy and no angiographic abnormalities, signs of RPE atrophy could also be observed in HD-OCT scans, raising the possibility that this technique may allow the early detection of the disease.
Asunto(s)
Antimaláricos/efectos adversos , Cloroquina/efectos adversos , Enfermedades de la Retina/inducido químicamente , Epitelio Pigmentado de la Retina/efectos de los fármacos , Femenino , Humanos , Persona de Mediana Edad , Epitelio Pigmentado de la Retina/patología , Tomografía de Coherencia Óptica/métodosRESUMEN
A tomografia de coerência óptica (OCT) tem se mostrado muito útil na avaliação de pacientes com glaucoma. São relatadas duas pacientes referidas com a suspeita de glaucoma sem hipertensão para avaliação por tomografia de coerência óptica que, na verdade, eram portadoras de tumores intracranianos - um cordoma de clivo no primeiro caso e um craniofaringeoma no segundo. Os achados à tomografia de coerência óptica - diminuição difusa da espessura da camada de fibras nervosas circumdiscais desproporcionalmente acentuada nos setores nasal e temporal - levantaram a suspeita de acometimento na região do quiasma e permitiram o diagnóstico destes importantes tumores intracranianos.
Optical coherence tomography (OCT) has proved to be a very valuable tool in the assessment of patients with glaucoma. In this report, intracranial tumors were discovered in two glaucoma suspects referred for diagnostic confirmation by OCT - a clivus chordoma and a craniopharyngeoma. Optical coherence tomography findings - marked asymmetrical diffuse attenuation of the peripapillary nerve fiber layer in nasal and temporal sectors - raised concerns about lesions in chiasmatic region and permitted the timely diagnosis of these intraocular tumors.
Asunto(s)
Femenino , Humanos , Persona de Mediana Edad , Quiasma Óptico , Hipertensión Ocular/diagnóstico , Neoplasias del Nervio Óptico/diagnóstico , Neoplasias Hipofisarias/diagnóstico , Cordoma/diagnóstico , Craneofaringioma/diagnóstico , Diagnóstico Diferencial , Tomografía de Coherencia ÓpticaRESUMEN
As drogas antimaláricas, como a cloroquina, são úteis no controle de doenças reumáticas, mas podem causar lesões oculares potencialmente graves como a retinopatia cloroquínica (RC). Neste relato, são descritos os achados à tomografia de coerência óptica convencional e na espectral de alta resolução (HD-OCT) em aparelho CirrusTM HD-OCT de duas pacientes com RC. Em um dos casos, chama a atenção à similaridade entre a imagem de atrofia do epitélio pigmentado retiniano (EPR) na angiofluoresceinografia e a obtida na HD-OCT por segmentação "en -face" (reconstruções coronais). Em outra paciente, com sinais clínicos de maculopatia, mas sem anormalidades na angiofluoresceinografia, os sinais de atrofia do EPR foram também observados nos "scans" da HD-OCT, levantando a possibilidade de que esta técnica possa permitir a detecção precoce desta doença.
Antimalarial drugs, such as chloroquine, are useful in the management of rheumatic diseases, but may cause a potentially blinding condition known as toxic maculopathy. This report describes the findings on standard and on high-resolution spectral domain ocular coherence tomography performed in a CirrusTM HD-OCTdevice in two patients with chloroquine maculopathy. In one case, a very similar aspect of retinal pigment epithelium (RPE) plaque atrophy occured in both angiographic and HD-OCT images obtained by "en-face" (coronal) segmentation. In another patient with clinical signs of maculopathy and no angiographic abnormalities, signs of RPE atrophy could also be observed in HD-OCT scans, raising the possibility that this technique may allow the early detection of the disease.
Asunto(s)
Femenino , Humanos , Persona de Mediana Edad , Antimaláricos/efectos adversos , Cloroquina/efectos adversos , Enfermedades de la Retina/inducido químicamente , Epitelio Pigmentado de la Retina/efectos de los fármacos , Epitelio Pigmentado de la Retina/patología , Tomografía de Coherencia Óptica/métodosRESUMEN
Familial exudative vitreoretinopathy (FEVR) is an inherited blinding condition characterized by abnormal development of the retinal vasculature. The authors describe a rare case of the disease associated with severe infantile osteoporosis in a young female patient. The patient was submitted to multiple vitreoretinal procedures in both eyes due to tractional macular detachments. The case was complicated by diffuse uveitis of difficult control in one eye, which stimulated proliferative vitreoretinopathy and retinal redetachment. The inflammatory potential of drugs used in the control of the osteoporosis, in contrast with the inherent inflammatory activity the disease, are discussed.
Asunto(s)
Osteoporosis/complicaciones , Desprendimiento de Retina/etiología , Vitreorretinopatía Proliferativa/etiología , Niño , Femenino , Humanos , Recurrencia , Desprendimiento de Retina/cirugía , Índice de Severidad de la Enfermedad , Vitrectomía , Vitreorretinopatía Proliferativa/diagnóstico , Vitreorretinopatía Proliferativa/cirugíaRESUMEN
Familial exudative vitreoretinopathy (FEVR) is an inherited blinding condition characterized by abnormal development of the retinal vasculature. The authors describe a rare case of the disease associated with severe infantile osteoporosis in a young female patient. The patient was submitted to multiple vitreoretinal procedures in both eyes due to tractional macular detachments. The case was complicated by diffuse uveitis of difficult control in one eye, which stimulated proliferative vitreoretinopathy and retinal redetachment. The inflammatory potential of drugs used in the control of the osteoporosis, in contrast with the inherent inflammatory activity the disease, are discussed.
Relato de um caso incomum de vitreorretinopatia exsudativa familiar (FEVR) bilateral em criança do sexo feminino portadora de grave osteoporose infantil. A paciente foi submetida a vitrectomias sucessivas para correção de descolamentos tracionais maculares em ambos os olhos. O quadro foi complicado em um olho por uveíte difusa de difícil controle, que estimulava o aparecimento de proliferação vitreorretiniana de difícil abordagem, responsável por redescolamentos sucessivos da retina. Discute-se sobre o potencial pró-inflamatório de drogas usadas no controle da osteoporose, em contraste com a atividade inflamatória inerente à doença.
Asunto(s)
Niño , Femenino , Humanos , Osteoporosis/complicaciones , Desprendimiento de Retina/etiología , Vitreorretinopatía Proliferativa/etiología , Recurrencia , Desprendimiento de Retina/cirugía , Índice de Severidad de la Enfermedad , Vitrectomía , Vitreorretinopatía Proliferativa/diagnóstico , Vitreorretinopatía Proliferativa/cirugíaRESUMEN
Erdheim-Chester disease (ECD) is a granulomatous and infiltrative disorder of unknown etiology with proliferation of cholesterol-containing histiocytes and peculiar bone involvement. It is very similar to Langerhans cell histiocytosis (LCH) on histology but with a different immunohistochemical profile. This is the first report of intraocular involvement in this disease. MPSG, a 46 y.o. woman, presented with proptosis of the OD. She referred ulcerated lesions on the hard palate, symmetrical and bilateral osteosclerosis of the fibulae and tibiae and a nodule in the right breast (biopsy: xantomatous histiocytic infiltrate CD68+, S-100 and CD1a negative on immunohistochemistry compatible with ECD). MRI studies demonstrated an extraconal tumor in the juxta-bulbar temporal portion of the right orbit close to the lacrimal gland and hyperintense on T1. Vision was 20/20 OU, with numerous drusen in the posterior pole, similar to basal laminar drusen. Two regions of orange subretinal infiltrates that showed progressive staining on the angiogram were seen in the peripapillary region and also close to the fovea in the OD. Choroidal neovascular membranes were seen 2 years later in OU leading to severe visual loss in the OS and to a slight visual field loss in the OD, which retained 20/20 vision. This pioneer report depicts in vivo characteristics of histiocytic granulomas in ECD. Caution should be taken with patients with ECD as potentially blinding intraocular complications may arise.
Asunto(s)
Enfermedad de Erdheim-Chester/complicaciones , Exoftalmia/etiología , Brasil/epidemiología , Neovascularización Coroidal/etiología , Neovascularización Coroidal/patología , Enfermedad de Erdheim-Chester/epidemiología , Exoftalmia/patología , Femenino , Angiografía con Fluoresceína , Humanos , Persona de Mediana Edad , Neoplasias Orbitales/etiología , Neoplasias Orbitales/patología , Drusas Retinianas/etiología , Drusas Retinianas/patología , Neovascularización Retiniana/etiología , Neovascularización Retiniana/patología , Tomografía de Coherencia Óptica , Agudeza Visual/fisiologíaRESUMEN
A doença de Erdheim-Chester (DEC) tem causa desconhecida e se caracteriza por ser granulomatosa e infiltrativa, com proliferação de histiócitos contendo colesterol e particular acometimento ósseo. À histologia, é semelhante à histiocitose de células Langerhans, salvo na análise imuno-histoquímica. Pela primeira vez é descrito o acometimento intra-ocular nesta doença. MPSG, 46 anos, sexo feminino, apresentou-se com proptose do olho direto. Referia úlceras no palato duro, osteoesclerose bilateral e simétrica na diáfise tibial e fibular e nódulo na mama direita (biópsia: infiltrado rico em histiócitos xantomatosos e perfil imuno-histoquímico com CD68 +, S-100 e CD1a negativos, compatível com DEC). À RNM, tumoração extraconal justa-bulbar temporal superior na órbita direita, hiperintensa em T1 e próxima da glândula lacrimal. Em cada olho, visão 20/20, com numerosas drusas nas arcadas e na região macular similares às drusas laminares basais, além de duas regiões placóides elevadas com infiltrado alaranjado sub-retiniano e hiperfluorescentes na angiografia na região peridiscal inferior e justamacular temporal do olho direito. Dois anos depois, surgiram membranas neovasculares sub-retinianas em ambos os olhos. O OD manteve visão 20/20 com pequenas alterações campimétricas e o OE evoluiu com grande cicatriz disciforme e visão de vultos. Esta descrição pioneira demonstra características in vivo dos granulomas histiocíticos da DEC, e alerta para possíveis complicações intra-oculares.
Erdheim-Chester disease (ECD) is a granulomatous and infiltrative disorder of unknown etiology with proliferation of cholesterol-containing histiocytes and peculiar bone involvement. It is very similar to Langerhans cell histiocytosis (LCH) on histology but with a different immunohistochemical profile. This is the first report of intraocular involvement in this disease. MPSG, a 46 y.o. woman, presented with proptosis of the OD. She referred ulcerated lesions on the hard palate, symmetrical and bilateral osteosclerosis of the fibulae and tibiae and a nodule in the right breast (biopsy: xantomatous histiocytic infiltrate CD68+, S-100 and CD1a negative on immunohistochemistry compatible with ECD). MRI studies demonstrated an extraconal tumor in the juxta-bulbar temporal portion of the right orbit close to the lacrimal gland and hyperintense on T1. Vision was 20/20 OU, with numerous drusen in the posterior pole, similar to basal laminar drusen. Two regions of orange subretinal infiltrates that showed progressive staining on the angiogram were seen in the peripapillary region and also close to the fovea in the OD. Choroidal neovascular membranes were seen 2 years later in OU leading to severe visual loss in the OS and to a slight visual field loss in the OD, which retained 20/20 vision. This pioneer report depicts in vivo characteristics of histiocytic granulomas in ECD. Caution should be taken with patients with ECD as potentially blinding intraocular complications may arise.
Asunto(s)
Femenino , Humanos , Persona de Mediana Edad , Enfermedad de Erdheim-Chester/complicaciones , Exoftalmia/etiología , Brasil/epidemiología , Neovascularización Coroidal/etiología , Neovascularización Coroidal/patología , Enfermedad de Erdheim-Chester/epidemiología , Exoftalmia/patología , Angiografía con Fluoresceína , Neoplasias Orbitales/etiología , Neoplasias Orbitales/patología , Drusas Retinianas/etiología , Drusas Retinianas/patología , Neovascularización Retiniana/etiología , Neovascularización Retiniana/patología , Tomografía de Coherencia Óptica , Agudeza Visual/fisiologíaRESUMEN
A tomografia de coerência óptica (TCO) é um novo recurso diagnóstico que revolucionou a retinologia, desafiando conceitos e criando novos paradigmas de tratamento e de conduta. Neste artigo, revisam-se aspectos importantes desta tecnologia aplicada às doenças adquiridas da interface vítreo-macular, somando-se aos relatos da literatura aexperiência pessoal do autor (LBN).
Optical coherence tomography is a new and powerful diagnostic tool that has challenged traditional concepts in retinal diseases, bringing up new paradigms in diagnosis and treatment. This article revises paramount aspects of this technology applied to the study of acquired maculopathies, enriched with the authors (LBN) personal experience.