Síndrome de Wolfram - Diagnóstico clínico de condição rara multissistêmica / Wolfram syndrome - Clinical diagnosis of rare multisystemic condition

Resumo A Síndrome de Wolfram consiste em uma patologia neurodegenerativa de caráter genético, também conhecida pela sigla DIDMOAD que traduz os principais achados dessa doença, Diabetes Insipidus, Diabetes Mellitus, Atrofia Óptica e Surdez. O artigo visa relatar ocaso de um paciente diagnosticado clinicamente com essa síndrome em um ambulatório geral de oftalmologia. Tendo em vistaque os pacientes portadores dessa alteração genética apresentam mais de um par craniano afetado e quadro clínico sem históricode meningite ou outras alterações neurológicas, tem-se que pensar em alterações raras, como é o caso dessa síndrome. A partir dodiagnóstico, aplicou-se o questionário WRUS em consulta, o qual permitiu a comparação do paciente abordado com dados obtidosinternacionalmente disponíveis na literatura.

Abstract Wolfram Syndrome consists of a neurodegenerative pathology of genetic character, also known by the acronym DIDMOAD that translates the main findings of this disease, Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy and Deafness. The article report the case of a patient diagnosed clinically with this syndrome in a general ophthalmology out patient clinic. Considering that patients with this genetic alteration have more than one cranial nerve affected by the disease and clinical history without meningitis or other neurological alterations, one has to think about rare alterations, as is the case with this syndrome. From the diagnosis, the WRUS questionnaire was applied in consultation, which all owed the comparation of the patient with concepts obtained internationally available in the literature.

Straatsma syndrome: two case reports / Síndrome de Straatsma: dois relatos de caso

Abstract This article reports two cases of Straatsma Syndrome, a rare disease, emphasizing its clinical features that inclued myopia, strabismus and amblyopia associated with persistent myelinated fibers in the retina. Ophthalmic examination, color retinography and optical coherence tomography were performed.

Resumo Este artigo relata dois casos de síndrome de Straatsma, uma doença rara, enfatizando suas características clínicas que incluem miopia, estrabismo e ambliopia associada a persistência de fibras de mielina na retina. Foram realizados exame oftalmológico, retinografia e tomografia de coerência óptica.

Comparison of the Progression of High- and Low-tension Glaucoma as Determined by Two Different Criteria

PURPOSE: To investigate and compare the progression of medically treated primary open angle glaucoma according to the baseline intraocular pressure (IOP). METHODS: This study included a total of 345 eyes from 345 patients (mean follow-up period, 4.5 years). Eyes were classified into either conventional normal tension glaucoma (cNTG, 21 mmHg) groups according to the conventional cut-off value of the IOP. Additionally, the median IOP (15 mmHg) was used to create two other groups (median NTG [mNTG] 15 mmHg). Using these values, 306, 39, 153, and 192 eyes were assigned to the cNTG, cHTG, mNTG, and mHTG groups, respectively. Glaucoma progression was determined either by optic disc/retinal nerve fiber layer photographs or serial visual field data. RESULTS: Mean reduction of IOP after medical treatment and of central corneal thickness was lower in the cNTG group, while the prevalence of disc hemorrhage and baseline visual field mean deviation did not differ between the cNTG and cHTG groups. A mean reduction in the IOP was observed after medical treatment, and central corneal thickness was lower in the mNTG group; disc hemorrhage was more frequent in the mNTG than in the mHTG group. Among the 345 analyzed eyes, 100 (29%) showed progression during the follow-up period. In the cHTG group, a higher baseline IOP (hazard ratio, 1.147; p = 0.024) was associated with glaucoma progression. Disc hemorrhage (hazard ratio, 15.533; p < 0.001) was also strongly associated with progression in the mNTG group. CONCLUSIONS: Baseline IOP was a significant risk factor for glaucoma progression in cHTG patients (10% of our total participants), while disc hemorrhage showed the strongest association with progression in the mNTG group, indicating that a cut-off value other than the conventional 21 mmHg is required to define true low-tension glaucoma in populations where NTG predominates among all glaucoma patients.

Características del nervio óptico y la retina peripapilar en glaucoma primario de ángulo abierto / Optic nerve and peripapillary retina characteristics in primary open angle glaucoma

Objetivo: determinar la prevalencia de las diferentes características morfológicas asociadas al glaucoma en el nervio óptico y retina peripapilar de pacientes con diagnóstico de glaucoma primario de ángulo abierto. Métodos: se analizaron 374 fotografías del polo posterior de pacientes con glaucoma primario de ángulo abierto, registrando presencia o ausencia de adelgazamiento del anillo neural, hemorragias peripapilares, atrofia peripapilar alfa o beta, exposición de lámina cribosa, vasos denudados, vasos en bayoneta, escotadura en la excavación, medición del diámetro vertical y horizontal de la papila y de la excavación, área del disco óptico y de la excavación; los cuales se correlacionaron con datos de la historia clínica como edad, sexo, comorbilidades, presión intraocular, tratamiento, número de medicamentos en uso, historia de cirugía intraocular. Resultados: los cambios más frecuentemente asociado a glaucoma fueron adelgazamiento del anillo neuroretinal (59.4%), exposición de la lámina cribosa, (67.6%), atrofia peripapilar y vasos en bayoneta (35.3%). El promedio del diámetro vertical del disco fue 1.9 mm ± 0.2 y el diámetro horizontal del disco fue 1.7 mm ± 0.1. Conclusiones: la valoración de los cambios asociados a glaucoma por medio de fotografía del disco óptico permite realizar un registro detallado y sistemático de cada una de las características y su documentación para seguimiento en el tiempo.

Objetive: to determine the prevalence of optic nerve and peripapillar retina characteristics in patients diagnosed with primary open-angle glaucoma. Methods: 374 posterior pole photographs of patients with primary open-angle glaucoma were analyzed recording presence or absence of neuroretinal rim thinning, peripapillary hemorrhages, alpha or beta parapapillary atrophy, baring of blood vessels, vessel bayoneting, neuroretinal rim notching, vertical and horizontal disc and cup diameter, disc and cup area. These date were correlated with medical records including age, sex, comorbidities, intraocular pressure, treatment and intraocular surgery. Results: the most common glaucoma associated changes were neuroretinal rim thinning (59.4 %), exposure of the lamina cribrosa ( 67.6 % ), peripapillary atrophy and vessels bayoneting ( 35.3%). The disc vertical diameter was 1.9 ± 0.2 mm and horizontal disc diameter 1.7 ± 0.1 mm. Conclusions: glaucoma associated changes assessment using optic disc photography allows a detailed and systematic recording on each characteristic and its follow up over the time.

Associations between Optic Cup-to-disc Ratio and Systemic Factors in the Healthy Korean Population

PURPOSE: To assess the relationships between optic cup-to-disc ratio (CDR) and age, sex, and other demographic and health characteristics in the healthy Korean population. METHODS: The study design was retrospective and population-based. A total of 28,377 subjects who participated in the Korea National Health and Nutrition Examination Survey between 2008 and 2011 were enrolled in this study. Participants underwent structured interviews as well as systemic and ophthalmic examinations. Patients with glaucoma who were diagnosed using the International Society of Geographical and Epidemiological Ophthalmology classification were excluded. Changes in vertical CDR were examined by age in relation to systemic variables on multiple regression analysis. RESULTS: The mean vertical CDR was 0.34 +/- 0.12. The vertical CDR increased with age from subjects in their 20s to those in their 80s (p < 0.001). The mean CDR in males was significantly higher than that of females (p < 0.001). On multiple regression analysis, the vertical CDR was positively associated with age (p < 0.001), male sex (p < 0.001), diastolic blood pressure (p = 0.009), and intraocular pressure (p < 0.001) but was negatively associated with body mass index (p < 0.001). CONCLUSIONS: Greater vertical CDR was related to age, male sex, higher diastolic blood pressure, higher intraocular pressure, and lower body mass index in healthy Koreans.

The role of pattern-reversal electroretinography in the diagnosis of glaucoma / Importância do eletroretinograma de padrão reverso no diagnóstico do glaucoma

Pattern electroretinography is used to assess the function of the inner retinal layers, particularly the retinal ganglion cell layer, using a reversing checkerboard or grating pattern that maintains a constant overall mean luminance over time. A normal transient response comprises a positive component of the wave (P50) followed by a longer negative component of the wave (N95). Glaucomatous optic neuropathy causes progressive loss of retinal ganglion cells, potentially detectable as abnormalities on examination, particularly in the N95 component. Therefore, pattern electroretinography may be useful in the diagnosis and evaluation of glaucoma. The present article is an updated review of published data regarding the use of pattern electroretinography for the detection of glaucoma-induced retinal changes.

O eletroretinograma de padrão reverso é utilizado para avaliar a função das camadas internas da retina, particularmente a camada de células ganglionares retinianas, utilizando um estímulo em xadrez ou barras alternantes, mantendo constante o nível de contraste total. A resposta transiente normal é constituída por uma onda positiva (P50) seguida de uma onda maior negativa (N95). A neuropatia óptica glaucomatosa causa perda progressiva das células ganglionares da retina, detectável como anormalidades no exame, especialmente na onda N95. Por isso, o eletroretinograma de padrão reverso pode ser útil no diagnóstico e seguimento de pacientes glaucomatosos. Este artigo é uma revisão atualizada dos dados publicados a respeito da capacidade do eletroretinograma de padrão reverso em detectar alterações retinianas induzidas pelo glaucoma.

Comparison of the Thickness of the Lamina Cribrosa and Vascular Factors in Early Normal-tension Glaucoma with Low and High Intraocular Pressures

PURPOSE: To compare the thickness of the lamina cribrosa (LC) and vascular factors of early normal-tension glaucoma (NTG) patients with high and low intraocular pressure (IOP) that are expected to be associated with the development of glaucoma. METHODS: Seventy-one Korean NTG patients with low IOP (the highest IOP 15 mmHg, 31 patients) were included in this study. The thickness of LC and vascular factors were compared. The thickness of the LC was measured using the enhanced depth imaging method with spectral domain optical coherence tomography (Heidelberg Spectralis). RESULTS: The mean thickness of the central LC was 190.0 +/- 19.2 microm in the low IOP group and 197.8 +/- 23.6 microm in the high IOP group, but there was no statistical significant difference between the two groups (p > 0.05). The prevalence of self-reported Raynaud phenomenon was significantly higher in the low IOP group (33.0%) than the high IOP group (10.3%, p = 0.04). CONCLUSIONS: The laminar thickness did not significantly differ between the high and low IOP groups. However, the prevalence of Raynaud phenomenon was higher in the low IOP groups. These results suggest that the development of glaucoma with low IOP patients may be more influenced by peripheral vasospasm, such as Raynaud phenomenon, rather than laminar thickness in NTG.

Correlación clínico-radiológica de las anomalías congénitas excavadas del disco óptico / Clinical and radiological correlation of excavated congenital anomalies of the optic disk

Introducción: el diagnóstico de las anomalías congénitas excavadas del nervio óptico se ha beneficiado con la introducción y desarrollo de las técnicas imagenológicas; particularmente en los casos atípicos o con alteraciones oculares asociadas, donde el examen clínico no puede ser concluyente. Caso clínico: se presenta un paciente con una anomalía congénita del nervio óptico y se discuten las características clínico-imagenológicas que sustentan su diagnóstico diferencial. Se recomienda la evaluación conjunta, por parte de oftalmólogos y radiólogos, de pacientes con estas anomalías; sobre todo en aquellos donde las presentaciones fundoscópicas no son específicas. Conclusiones: el diagnóstico nosológico correcto garantiza una adecuada orientación sobre alteraciones sistémicas relacionadas y patrones de herencia, si los hay

Introducción: the diagnosis of excavated congenital anomalies of the optic nerve has benefitted from the introduction and development of radiological techniques, mainly in atypical cases or those with others related ocular disorders, where the clinical exam is not conclusive. Clincal case: Here is a patient with a congenital optic nerve anomaly. The clinical and radiological characteristics that explain the differential diagnosis of this case were discussed. Emphasis was made on the fact that patients with these anomalies and no specific fundus features must be evaluated by both ophthalmologists and radiologists. Conclusions: the right nosological diagnosis assures the appropriate orientation towards others related systemic associations and inheritance patterns, if any

Imaging studies for diagnosing Graves' orbitopathy and dysthyroid optic neuropathy

Although the diagnosis of Graves' orbitopathy is primarily made clinically based on laboratory tests indicative of thyroid dysfunction and autoimmunity, imaging studies, such as computed tomography, magnetic resonance imaging, ultrasound and color Doppler imaging, play an important role both in the diagnosis and follow-up after clinical or surgical treatment of the disease. Imaging studies can be used to evaluate morphological abnormalities of the orbital structures during the diagnostic workup when a differential diagnosis versus other orbital diseases is needed. Imaging may also be useful to distinguish the inflammatory early stage from the inactive stage of the disease. Finally, imaging studies can be of great help in identifying patients prone to develop dysthyroid optic neuropathy and therefore enabling the timely diagnosis and treatment of the condition, avoiding permanent visual loss. In this paper, we review the imaging modalities that aid in the diagnosis and management of Graves' orbitopathy, with special emphasis on the diagnosis of optic nerve dysfunction in this condition.

Avaliação da camada de fibras nervosas da retina nas afecções neuroftalmológicas da via óptica anterior / Retinal nerve fiber evaluation in neuro-ophthalmic diseases of the anterior visual pathway

A avaliação da camada de fibras nervosas da retina tem grande importância no diagnóstico e acompanhamento de várias afecções da via óptica anterior. Nesta revisão, discutiremos os principais métodos de análise clínica e instrumental da camada de fibras nervosas da retina e revisamos os principais achados encontrados nas afecções da via óptica anterior incluindo lesões inflamatórias, isquêmicas, tóxicas, hereditárias, compressivas e traumáticas do nervo óptico, as lesões do quiasma óptico, as do trato óptico e aquelas do corpo geniculado lateral.

Retinal nerve fiber evaluation is important in the diagnosis and management of several diseases of the anterior visual pathway. In this report we review the clinical findings and the current techonologies avalilable to analyse the retinal nerve fiber layer. We furthermore review the main findings in several disease of the anterior visual pathways including inflammatory, ischemic, toxics, hereditary, compressive and traumatic optic neuropathies as well as lesion of the optic chiasm, optic tract and lateral geniculate body.

Autoimmune optic neuropathy as the first manifestation of systemic lupus erythematosus / Neuropatia óptica autoimune como primeira manifestação do lúpus eritematoso sistêmico

We described a 35 years old female patient with bilateral visual loss and pain on eye movement, mild papillary edema in acute phase, arcuate scotoma and complementary test positive for antinuclear antibodies that did not respond to corticosteroid therapy. The lack of clinical criteria for systemic lupus erythematosus (SLE) didn't prevent the institution of the specific treatment with corticosteroids and azathioprine. After seven months the diagnosis was made after a skin manifestation of the disease. This case shows the value of the ocular complaints in systemic diseases. And how the ophthalmologic exam can help the clinician elaborating a diagnosis. It is also very important for ophthalmologists and rheumatologists due to the fact that it calls the attention to another diagnostic hypothesis in patients with nonspecific optic neuritis, even with inconclusive laboratory tests. Maybe some ocular findings deserve to be included to the diagnostic criteria already established for SLE.

Descrevemos caso de um paciente de 35 anos do sexo feminino, com perda visual bilateral associada à dor à movimentação ocular, edema papilar moderado na fase aguda, escotoma arqueado e exame complementar positivo para anticorpos antinucleares, que não responderam à terapia com corticosteróides. A falta de critérios clínicos para o lúpus eritematoso sistêmico (LES) não impediu a instituição do tratamento específico com corticosteróides e azatioprina. Depois de sete meses, o diagnóstico foi feito após uma manifestação da doença de pele. Este caso mostra o valor das queixas oculares em doenças sistêmicas e como o exame oftalmológico pode ajudar o clínico na elaboração de um diagnóstico. Também é muito importante para oftalmologistas e reumatologistas, devido ao fato de que chama a atenção para outra hipótese diagnóstica em pacientes com neurite óptica não-específica, mesmo com os testes laboratoriais conclusivos. Talvez alguns achados oculares merecem ser incluídos com os critérios de diagnóstico já estabelecido para o LES.

Optic Disc Atrophy in Patient with Posner-Schlossman Syndrome

A 32-year-old man with blurred vision in the right eye and headache presented with anterior uveitis, an intraocular pressure (IOP) of 60 mmHg, an open angle, no visual field defects, and normal optic nerve. He had a history of five previous similar attacks. In each of the previous instances, his anterior uveitis and high IOP were controlled with antiglaucoma medications and topical steroids. However, at the fifth attack, his optic disc was pale and a superior paracentral visual field defect was shown. Brain magnetic resonance image studies were normal. This case represents that a recurrent Posner-Schlossman syndrome (PSS)-induced optic disc atrophy likely due to ocular ischemia caused by a recurrent, high IOP. Although PSS is a self-limiting syndrome, we should manage high IOP and prevent ischemia of the optic nerve head by treating with ocular antihypertensive medications.

Pattern-reversal electroretinograms for the diagnosis and management of disorders of the anterior visual pathway / Eletroretinograma de padrão reverso no diagnóstico e acompanhamento das afecções da via óptica anterior

The pattern electroretinogram is an electrophysiological test that assesses the function of inner retinal layers, particularly the ganglion cells layer of retina, using a reversing checkerboard or grating pattern that produces no change in average luminance over time. The normal pattern electroretinogram is composed of a proeminent positive component (P50) and a large later negative component (N95). Since structural damage that compromises the retinal ganglion cell layer can lead to pattern electroretinogram changes, particularly in the N95 amplitude, the test can be useful in the treatment of a number of anterior visual pathway diseases. In this article, we review the methods for recording pattern electroretinogram and its usefulness in the diagnosis and management of diseases including inflammatory, hereditary, ischemic and compressive lesions of the anterior visual pathway.

O eletroretinograma de padrão reverso é um teste eletrofisiológico que avalia a função das camadas internas da retina, especialmente a camada de células ganglionares, através de um estímulo em xadrez ou em barras que não apresenta variação na luminância do estímulo. É composto de um componente positivo (P50) e um componente negativo (N95) tardio. Uma vez que lesões estruturais às células ganglionares da reitna podem levar a alterações no eletroretinograma de padrão reverso, especialmente na amplitude da onda N95, o teste pode ser útil no tratamento de várias doenças da via óptica anterior. Neste artigo revisamos os métodos de obtenção do eletroretinograma de padrão reverso e a sua utilidade no diagnóstico e acompanhamento de doenças incluindo lesões inflamatórias, hereditárias, isquemicas e compressivas na via óptica anterior.

Inter-Device Agreement of Retinal Nerve Fiber Layer Thickness Measurements Using Spectral Domain Cirrus HD OCT

PURPOSE: To assess the inter-device agreement of peripapillary retinal nerve fiber layer (RNFL) thickness measurements by 2 spectral domain Cirrus HD optical coherence tomography (OCT) devices in healthy Korean subjects. METHODS: Eleven eyes of 11 healthy volunteers were enrolled in the present study. Each eye was scanned with the Optic Disc Cube 200 x 200 scan of 2 Cirrus HD OCT devices for peripapillary RNFL thickness calculation. The inter-device agreements of the 2 Cirrus HD OCTs for average, quadrant, and clock-hour RNFL thickness values were determined with Wilcoxon signed rank test, Friedman test, Cronbach's alpha (alpha), intraclass correlation coefficient (ICC), coefficient of variation (COV), and Bland-Altman plot. RESULTS: The mean age of the participants was 25.82 +/- 3.28 years and all had a 0.00 logarithm of the minimum angle of resolution of best-corrected visual acuity. The signal strengths of scans from the 2 Cirrus HD OCT were not significantly different (p = 0.317). The inter-device agreement of average RNFL thickness was excellent (alpha, 0.940; ICC, 0.945; COV, 2.45 +/- 1.52%). However, the agreement of nasal quadrant RNFL thickness was not very good (alpha, 0.715; ICC, 0.716; COV, 5.72 +/- 4.64%). Additionally, on the Bland-Atman plot, the extent of agreement of the 2 Cirrus HD OCTs for RNFL thickness was variable according to scanned sectors. CONCLUSIONS: The inter-device agreement of 2 spectral domain Cirrus HD OCT devices for peripapillary RNFL thickness measurements was generally excellent but variable according to the scanned area. Thus, physicians should consider this fact before judging a change of RNFL thicknesses if they were measured by different OCT devices.

Optic Disc Pit with Peripapillary Retinoschisis Presenting as a Localized Retinal Nerve Fiber Layer Defect

A 59-year-old woman was referred to our clinic for a glaucoma evaluation. The visual acuity and intraocular pressure were normal in both eyes. However, red-free fundus photography in the left eye showed a superotemporal wedge-shaped retinal nerve fiber layer defect, and visual field testing showed a corresponding partial arcuate scotoma. In an optical coherence tomography examination, the macula was flat, but an arcuate-shaped peripapillary retinoschisis was found. Further, the retinoschisis seemed to be connected with a superotemporal optic pit shown in a disc photograph. After 3 months of a topical prostaglandin analogue medication, the intraocular pressure in the retinoschisis eye was lowered from 14 to 10 mmHg and the peripapillary retinoschisis was almost resolved. We report a rare case of an optic disc pit with peripapillary retinoschisis presenting as a localized retinal nerve fiber layer defect.

Simulated cup/disc ratio: a tool for ophthalmologists / Razão escavação/disco simulada: uma ferramenta para oftalmologistas

PURPOSE: To evaluate and compare the observers' ability to measure simulations of cup/disc ratios (CDR) as concentric and non-concentric circles. METHODS: In a prospective, random, and masked setting, 43 images representing the CDR spectrum from 0.2 to 0.9 for vertical and horizontal CDR measurements were developed and presented on a computer screen to 171 participants. RESULTS: There were satisfactory agreements according to the kappa coefficient (0.755 and 0.730 for horizontal and vertical cup disc ratios, respectively) and Lin's concordance correlation (R=0.88 and R=0.86 for horizontal and vertical measurements, respectively). However, very poor agreement was found for intermediate CDR values. The worst agreement occurred when the CDR was between 0.4 and 0.6 for both the horizontal and vertical values. The kappa coefficient was 0.37 and 0.39 for 0.4 CDR (horizontal and vertical, respectively), 0.39 and 0.38 for 0.5 CDR (horizontal and vertical, respectively) and 0.45 and 0.41 for 0.6 CDR (horizontal and vertical, respectively). CONCLUSION: Despite a good general agreement between the gold standard and the participants' responses, the absolute agreement for intermediate CDR values was very poor for both horizontal and vertical values.

OBJETIVO: Avaliar e comparar a habilidade de observadores em medir a razão escavação/disco (CDR) por meio de figuras esquemáticas. MÉTODOS: Em um estudo prospectivo, randomizado e mascarado, 43 imagens representado CDR horizontais e verticais entre 0,2 e 0,9 foram desenvolvidas e apresentadas em uma tela de computador para 171 participantes. RESULTADOS: Para todos os intervalos de CDR a concordância foi satisfatória para análise kappa (0,755 e 0,730 para CDR horizontais e verticais, respectivamente) e para concordância de Lin (R=0,88 e R=0,86 para medidas horizontais e verticais respectivamente). No entanto, a concordância foi fraca para valores intermediários de CDR. A pior concordância ocorreu para CDR horizontais e verticais entre 0,4 e 0,6. CONCLUSÃO: Apesar da boa concordância geral entre as respostas corretas e as respostas dadas pelos participantes, a concordância absoluta para valores intermediários de CDR mostrou-se muito fraca tanto para figuras horizontais como verticais.

Factors affecting the variability of the Heidelberg Retina Tomograph III measurements in newly diagnosed glaucoma patients / Fatores associados com a variabilidade das medidas do Heidelberg Retina Tomograph III em pacientes com glaucoma recém-diagnosticados

PURPOSE: To determine factors associated with the test-retest variability of optic nerve head (ONH) topography measurements with confocal scanning laser ophthalmoscopy (CSLO) in newly diagnosed glaucomatous patients. METHODS: Consecutive patients with newly diagnosed primary open-angle glaucoma were prospectively enrolled. Patients presenting with any ocular disease other than glaucoma were excluded. All patients underwent CSLO using the Heidelberg Retina Tomograph III (HRT-III) in one randomly selected eye (three consecutive scans; performed by the same examiner). For each Heidelberg Retina Tomograph III parameter, repeatability was assessed using within subject standard deviation (Sw) and coefficient of variation (CVw), repeatability coefficient (RC) and intraclass correlation coefficient (ICC). Scatter plots and regression lines were constructed to identify which factors influenced test-retest measurement variability. RESULTS: A total of 32 patients were included (mean age, 65.4 ± 13.8 years). Most patients were female (65 percent) and white (50 percent). Among all Heidelberg Retina Tomograph III parameters evaluated, rim area and mean cup depth had the best measurement repeatability. Vertical cup-to-disc ratio (CDR, as determined by optic disc stereophotograph examination) was significantly associated (R²=0.21, p<0.01) with test-retest measurement variability. Eyes with larger CDR showed less variable measurements. Other factors, including age, disc area, central corneal thickness and intraocular pressure were not significant (p>0.14). CONCLUSION: Heidelberg Retina Tomograph III showed good test-retest repeatability for all ONH topographic measurements, mainly for rim area and mean cup depth. Test-retest repeatability seemed to improve with increasing CDR. These findings suggest that HRT-III topographic measurements should be cautiously interpreted when evaluating longitudinally glaucoma patients with early structural damage (small CDR).

OBJETIVO: Determinar os fatores associados à variabilidade (teste-reteste) das medidas topográficas da cabeça do nervo óptico (CNO) utilizando a oftalmoscopia confocal de varredura a laser (CSLO) em pacientes com glaucoma recém-diagnosticados. MÉTODOS: Neste estudo, pacientes com glaucoma primário de ângulo aberto recém-diagnosticados foram prospectivamente incluídos. Aqueles que apresentassem outras doenças oculares (exceto glaucoma) foram excluídos. Todos os pacientes incluídos no estudo foram submetidos à CSLO usando o aparelho Heidelberg Retina Tomograph III (HRT-III) em um olho aleatoriamente selecionado (três exames consecutivos realizados pelo mesmo examinador). Para cada parâmetro do Heidelberg Retina Tomograph III, a repetibilidade foi avaliada através dos seguintes indicadores: desvio padrão (DP) e coeficiente de variação (CV) individual, coeficiente de repetibilidade (CR) e coeficiente de correlação intraclasse (CCI). Diagramas de dispersão e linhas de regressão foram construídos para identificar quais fatores poderiam influenciar a variabilidade das medidas. RESULTADOS: Trinta e dois pacientes foram incluídos no estudo (idade média, 65,4 ± 13,8 anos). A maior parte era composta por mulheres (65 por cento) e pacientes brancos (50 por cento). Dentre os parâmetros de Heidelberg Retina Tomograph III avaliados, a área da rima e a profundidade média da escavação apresentaram os melhores valores de repetibilidade. A relação escavação/disco (E/D) vertical (baseada na análise de estereofotografia do disco óptico), foi significativamente associada (R²=0.21, p<0.01) com a variabilidade teste-reteste. Pacientes com relação E/D maiores apresentaram medidas menos variáveis. Outros fatores como idade, área do disco, espessura corneana central e pressão intraocular não foram significativas (p>0,14). CONCLUSÃO: O Heidelberg Retina Tomograph III mostrou boa repetibilidade (teste-reteste) para todos os parâmetros topográficos da CNO avaliados, principalmente em relação à área da rima e à profundidade média da escavação. A repetibilidade teste-reteste apresentou melhores resultados com o aumento da relação E/D. Esses achados sugerem que as medidas topográficas do Heidelberg Retina Tomograph III devem ser interpretadas com cautela quando avaliarmos longitudinalmente pacientes glaucomatosos com dano estrutural inicial (relação E/D menor).

Optic Neuropathy Associated with Castleman Disease

A 44-year-old woman with Castleman disease presented with acute visual loss in the left eye. A full ophthalmologic examination and imaging were performed. Visual acuity was 20/20 in the right eye and 20/100 in the left eye. Total dyschromatopsia, a relative afferent pupillary defect, and a cecocentral scotoma were observed in the left eye. Mild disc edema, without leaking during fluorescein angiography, was also observed. Magnetic resonance imaging revealed a small cystic epidermoid-like lesion in the right prepontine and suprasellar cistern. Her visual acuity did not improve and deteriorated to 20/200 in the left eye at 22 months after the initial visual loss. Optic neuropathy may rarely be associated with Castleman disease and suggests a poor prognosis.