Sarcoid optic neuropathy.

A 45-year-old woman presented with sudden complete vision loss in her left eye and retroorbital pain worsened by eye movements. A previous milder episode of vision loss had occurred in the same eye 1 year before, with complete recovery after high-dose intravenous methylprednisolone. She had no light perception in the left eye with a swollen optic disc, but with a normal right optic disc. There were no systemic manifestations or infections. MR scan of the brain showed extensive enlargement and enhancement of the left optic nerve and optic chiasm. After excluding infections and autoimmune markers, a left optic nerve biopsy confirmed non-caseating granulomas, leading to a diagnosis of neurosarcoidosis.

SD-OCT assessment and visual field changes 1 year or more after a single episode of unilateral acute primary angle closure / Avaliação do SD-OCT e mudanças do campo visual 1 ano ou mais após episódio único de fechamento angular primário agudo unilateral

ABSTRACT Objective: To evaluate structural and visual field (VF) changes after ≥1 year of a single acute primary angle closure (APAC) attack using spectral-domain optical coherence tomography (SD-OCT) and standard automated perimetry (SAP). Methods: Patients with a single unilateral APAC crisis at least 1 year ago were included consecutively from 2013 to 2016. Contralateral eye was used as control. All patients underwent ophthalmic examination, RNFL imaging by SD-OCT, and SAP using Octopus 1-2-3. Results: 54 eyes (27 patients) were enrolled. Male-to-female ratio was 1:2. Mean time for the SD-OCT and SAP assessment after the crisis was 5.0±5.1 (1.0-23.5) years, and IOP was 52.5±9.8 mmHg. In APAC eyes, the thicknesses of all quadrants of peripapillary RNFL (36.3%; P<0.001) and some macular sections (from 2.1% to 4.7%; P<0.01) were reduced compared to contralateral eyes. Additionally, in APAC eyes, the mean defect on VF was negatively and statistically correlated with the reduction of all quadrants of peripapillary RNFL thickness. Conclusion: A single episode of APAC was associated with peripapillary RNFL and macular thickness and with VF defects after ≥1 year of the crisis in the affected eye. Statistically meaningful correlations were found between structural and functional damage.

RESUMO Objetivo: Avaliar alterações estruturais e do campo visual 1 ano ou mais após uma crise única de fechamento angular primário agudo utilizando tomografia de coerência óptica de domínio espectral e perimetria automatizada padronizada. Métodos: Pacientes que apresentaram crise unilateral única de fechamento angular primário agudo há pelo menos 1 ano foram consecutivamente incluídos entre 2013 e 2016. Os olhos contralaterais foram utilizados como controles. Todos os pacientes foram submetidos a exame oftalmológico, avaliação das camadas de fibras nervosas da retina utilizando tomografia de coerência óptica de domínio espectral e perimetria automatizada padronizada com o Octopus 1-2-3. Resultados: Foram incluídos 54 olhos (27 pacientes) com razão homem:mulher de 1:2. O tempo médio após a crise foi de 5,0±5,1 anos (1,0 a 23,5) e a pressão intraocular na crise foi 52,5±9,8mmHg. Nos olhos com fechamento angular primário agudo, todas as espessuras das camadas de fibras nervosas da retina peripapilares (36,3%; p<0,001) e de algumas seções maculares (de 2,1 a 4,7%; p<0,01) estavam reduzidas em comparação aos olhos contralaterais. Além do mais, nos olhos submetidos a fechamento angular primário agudo, o mean defect do campo visual foi estatisticamente e negativamente correlacionado com a redução da espessura de todos os quadrantes peripapilares da camada de fibras nervosas da retina. Conclusão: Um único episódio de fechamento angular primário agudo foi associado com redução na espessura da camada de fibras nervosas da retina peripapilar e da espessura macular e com defeitos de campo visual 1 ano ou mais após a crise no olho afetado. Correlações estatisticamente significativas foram identificadas entre danos estruturais e funcionais.

Diagnostic ability of confocal near-infrared reflectance fundus imaging to detect retrograde microcystic maculopathy from chiasm compression. A comparative study with OCT findings.

PURPOSE: To evaluate the ability of confocal near-infrared reflectance (NIR) to diagnose retrograde microcystic maculopathy (RMM) in eyes with temporal visual field (VF) loss and optic atrophy from chiasmal compression. To compare NIR findings with optical coherence tomography (OCT) findings in the same group of patients. METHODS: Thirty-four eyes (26 patients) with temporal VF loss from chiasmal compression and 41 healthy eyes (22 controls) underwent NIR fundus photography, and macular OCT scanning. VF loss was estimated and retinal layers thickness were measured on OCT. Two examiners blinded to the diagnosis randomly examined NIR images for the presence of hyporeflective abnormality (HA) and OCT scans for the presence of microcystic macular abnormalities (MMA). The total average and hemi-macular HA area and number of microcysts were determined. The groups were compared and the level of agreement was estimated. RESULTS: The OCT-measured macular retinal nerve fiber and ganglion cell layers were thinner and the inner nuclear layer was thicker in patients compared to controls. HA and MMA were detected in 22 and 12 patient eyes, respectively, and in 0 controls (p<0.001, both comparisons). HA was significantly more frequent than MMA in patients with optic atrophy, and agreement between HA and MMA (both total and hemi-macular) was fair (kappa range: 0.24-0.29). The mean HA area was significantly greater in the nasal than temporal hemi-macula. A re-analysis of the 14 eyes with discrepant findings allowed to confirm RMM in 20 eyes (20/34) indicating that OCT detected RMM in 12 and missed it in 8 eyes. On the other hand, NIR correctly detected 18 out of 20 eyes, overcalled 4 and missed 2. CONCLUSIONS: RMM is a frequent finding in eyes with severe VF loss from long-standing chiasmal compression. NIR photography appears to be more sensitive than OCT for detecting RMM and may be useful as screening method for its presence.

Coexistence of papilledema and pseudopapilledema after remission of idiopathic intracranial hypertension by bariatric surgery / Coexistência de papiledema e pseudopapiledema após remissão de hipertensão intracraniana idiopática por cirurgia bariátrica

ABSTRACT A 37-year-old woman complained of headaches following bilateral visual loss in the past two years. She was obese and had undergone bariatric surgery three months earlier, followed by a considerable weight loss. Neuro-ophthalmic examination revealed a bilateral swollen optic disk. After a computerized analysis of the visual fields and magnetic resonance imaging of the brain and orbits, a diagnosis of idiopathic intracranial hypertension was made. At six months after the bariatric surgery, the patient reported no further headaches and exhibited better findings on computerized analysis of visual fields. However, fundus examination revealed persistent mild papilledema in both eyes. Ocular B-scan ultrasonography showed bilateral optic disk drusen. This report highlights the coexistence of true papilledema and pseudopapilledema due to optic disk drusen, following remission of idiopathic intracranial hypertension after a bariatric surgery.

RESUMO Uma mulher de 37 anos queixou-se de cefaleia após perda visual bilateral nos últimos dois anos. Apresentava história de obesidade e havia sido submetida à cirurgia bariátrica três meses antes, seguida de considerável perda de peso. O exame neuro-oftálmico revelou um disco óptico inchado bilateral. Após uma análise computadorizada dos campos visuais e ressonância magnética do crânio e órbitas, foi feito um diagnóstico de hiper tensão intracraniana idiopática. Após seis meses da cirurgia bariátrica, a paciente não relatou mais cefaleia e foram descobertas melhoras na análise computadorizada dos campos visuais. No entanto, o exame de fundo de olho revelou papiledema leve persistente em ambos os olhos. A ultrassonografia ocular B-scan mostrou drusas do disco óptico bilateralmente. Este relato destaca a coexistência de papiledema verdadeiro e pseudopapiledema devido à drusa de disco óptico após remissão da hipertensão intracraniana idiopática após uma cirurgia bariátrica.

Coexistence of papilledema and pseudopapilledema after remission of idiopathic intracranial hypertension by bariatric surgery.

A 37-year-old woman complained of headaches following bilateral visual loss in the past two years. She was obese and had undergone bariatric surgery three months earlier, followed by a considerable weight loss. Neuro-ophthalmic examination revealed a bilateral swollen optic disk. After a computerized analysis of the visual fields and magnetic resonance imaging of the brain and orbits, a diagnosis of idiopathic intracranial hypertension was made. At six months after the bariatric surgery, the patient reported no further headaches and exhibited better findings on computerized analysis of visual fields. However, fundus examination revealed persistent mild papilledema in both eyes. Ocular B-scan ultrasonography showed bilateral optic disk drusen. This report highlights the coexistence of true papilledema and pseudopapilledema due to optic disk drusen, following remission of idiopathic intracranial hypertension after a bariatric surgery.

Human Versus Machine: Comparing a Deep Learning Algorithm to Human Gradings for Detecting Glaucoma on Fundus Photographs.

PURPOSE: To compare the diagnostic performance of human gradings vs predictions provided by a machine-to-machine (M2M) deep learning (DL) algorithm trained to quantify retinal nerve fiber layer (RNFL) damage on fundus photographs. DESIGN: Evaluation of a machine learning algorithm. METHODS: An M2M DL algorithm trained with RNFL thickness parameters from spectral-domain optical coherence tomography was applied to a subset of 490 fundus photos of 490 eyes of 370 subjects graded by 2 glaucoma specialists for the probability of glaucomatous optical neuropathy (GON), and estimates of cup-to-disc (C/D) ratios. Spearman correlations with standard automated perimetry (SAP) global indices were compared between the human gradings vs the M2M DL-predicted RNFL thickness values. The area under the receiver operating characteristic curves (AUC) and partial AUC for the region of clinically meaningful specificity (85%-100%) were used to compare the ability of each output to discriminate eyes with repeatable glaucomatous SAP defects vs eyes with normal fields. RESULTS: The M2M DL-predicted RNFL thickness had a significantly stronger absolute correlation with SAP mean deviation (rho=0.54) than the probability of GON given by human graders (rho=0.48; P < .001). The partial AUC for the M2M DL algorithm was significantly higher than that for the probability of GON by human graders (partial AUC = 0.529 vs 0.411, respectively; P = .016). CONCLUSION: An M2M DL algorithm performed as well as, if not better than, human graders at detecting eyes with repeatable glaucomatous visual field loss. This DL algorithm could potentially replace human graders in population screening efforts for glaucoma.

Evolution of optic nerve and retina alterations in a child with indirect traumatic neuropathy as assessed by optical coherence tomography.

Herein, we describe the case of a 4-year-old child with indirect traumatic optic neuropathy and serial changes of the optic nerve head and retinal nerve fiber layer (RNFL) documented using optical coherence tomography (OCT). Visual acuity improved despite progressive RNFL thinning and optic disc pallor. We concluded that OCT may be useful for monitoring axonal loss but may not predict the final visual outcome.

Evolution of optic nerve and retina alterations in a child with indirect traumatic neuropathy as assessed by optical coherence tomography / Evolução das alterações no nervo óptico e na retina em criança com neuropatia traumática indireta avaliadas com tomografia de coerência óptica

ABSTRACT Herein, we describe the case of a 4-year-old child with indirect traumatic optic neuropathy and serial changes of the optic nerve head and retinal nerve fiber layer (RNFL) documented using optical coherence tomography (OCT). Visual acuity improved despite progressive RNFL thinning and optic disc pallor. We concluded that OCT may be useful for monitoring axonal loss but may not predict the final visual outcome.

RESUMO Descrição do caso de uma criança de 4 anos de idade com neuropatia óptica traumática indireta, cujas alterações no nervo óptico e na camada de fibras nervosas da retina foram documentadas com tomografia de coerência óptica seriadas. A acuidade visual apresentou melhora apesar da diminuição progressiva da camada de fibras nervosas e da palidez do disco óptico. Em conclusão, a tomografia de coerência óptica pode ser útil para monitorar a perda axonal na neuropatia óptica traumática indireta, sem no entanto, predizer o desfecho visual.

Macroadenoma hipofisário: alterações campimétricas visuais / Pituitary macroadenoma: visual field defects

O macroadenoma de hipófise é um tumor de evolução lenta e que apresenta importantes distúrbios visuais como baixa acuidade visual e alterações campimétricas. Estes sintomas levam os pacientes a procurarem, muitas vezes, os oftalmologistas. Neste caso, analisaremos uma paciente portadora de glaucoma que apresentava, associadamente, um macroadenoma hipofisário.

The pituitary adenoma is a tumor of slow evolution and has important visual disturbances such as low visual acuity and field defects. These symptoms lead patients to seek often ophthalmologists. In this case, we will analyze a patient with glaucoma who had an associated pituitary macroadenoma.

Progressão atípica de perda visual em paciente com glaucoma primário de ângulo aberto / Atypical progression of visual loss in a patient with primary open-angle glaucoma

A dolicoectasia da artéria carótida interna (ACI) é uma condição rara que pode ser acompanhada de manifestações neuro-oftalmológicas, como perda da acuidade e alteração do campo visual decorrente da compressão do nervo óptico (NO). O objetivo é relatar um caso de paciente do sexo masculino, 67 anos, portador de glaucoma primário de ângulo aberto (GPAA) com evolução atípica, assimetria de escavação, palidez da rima do NO à esquerda, devido à neuropatia óptica compressiva à esquerda, por segmento dolicoectásico da ACI. O diagnóstico foi baseado na história clínica, aspecto do NO e exames de neuroimagem.

Dolichoectasia of the internal carotid artery (ICA) is a rare condition that may be associated with neuro-ophthalmic manifestations, such as loss of visual acuity and visual field resulting from compression of the optic nerve (ON). The aim is to report a 67-year-old male patient with primary open-angle glaucoma (POAG) with atypical evolution, asymmetry of cupping and increased pallor of the rim of the left ON, due to compressive optic neuropathy by the dolichoectatic segment. The diagnosis was based on clinical history, appearance of the ON and neuroimaging.

Predicting dysthyroid optic neuropathy using computed tomography volumetric analyses of orbital structures.

OBJECTIVE: To evaluate the ability of orbital apex crowding volume measurements calculated with multidetector-computed tomography to detect dysthyroid optic neuropathy. METHODS: Ninety-three patients with Graves' orbitopathy were studied prospectively. All of the patients underwent a complete neuro-ophthalmic examination and computed tomography scanning. Volumetric measurements were calculated from axial and coronal contiguous sections using a dedicated workstation. Orbital fat and muscle volume were estimated on the basis of their attenuation values (in Hounsfield units) using measurements from the anterior orbital rim to the optic foramen. Two indexes of orbital muscle crowding were calculated: i) the volumetric crowding index, which is the ratio between soft tissue (mainly extraocular muscles) and orbital fat volume and is based on axial scans of the entire orbit; and ii) the volumetric orbital apex crowding index, which is the ratio between the extraocular muscles and orbital fat volume and is based on coronal scans of the orbital apex. Two groups of orbits (with and without dysthyroid optic neuropathy) were compared. RESULTS: One hundred and two orbits of 61 patients with Graves' orbitopathy met the inclusion criteria and were analyzed. Forty-one orbits were diagnosed with Graves' orbitopathy, and 61 orbits did not have optic neuropathy. The two groups of orbits differed significantly with regard to both of the volumetric indexes (p<0.001). Although both indexes had good discrimination ability, the volumetric orbital apex crowding index yielded the best results with 92% sensitivity, 86% specificity, 81%/94% positive/negative predictive value and 88% accuracy at a cutoff of 4.14. CONCLUSION: This study found that the orbital volumetric crowding index was a more effective predictor of dysthyroid optic neuropathy than previously described computed tomography indexes were.

Quantification of orbital apex crowding for screening of dysthyroid optic neuropathy using multidetector CT.

BACKGROUND AND PURPOSE: DON, a serious complication of GO, is frequently difficult to diagnose clinically in its early stages because of confounding signs and symptoms of congestive orbitopathy. We evaluated the ability of square area measurements of orbital apex crowding, calculated with MDCT, to detect DON. MATERIALS AND METHODS: Fifty-six patients with GO were studied prospectively with complete neuro-ophthalmologic examination and MDCT scanning. Square measurements were taken from coronal sections 12 mm, 18 mm, and 24 mm from the interzygomatic line. The ratio between the extraocular muscle area and the orbital bone area was used as a CI. Intracranial fat prolapse through the superior orbital fissure was recorded as present or absent. Severity of optic nerve crowding was also subjectively graded on coronal images. Orbits were divided into 2 groups (with or without clinical evidence of DON) and compared. RESULTS: Ninety-five orbits (36 with and 59 without DON) were studied. The CIs at all 3 levels and the subjective crowding score were significantly greater in orbits with DON (P < .001). No significant difference was observed regarding intracranial fat prolapse (P = .105). The area under the ROC curves was 0.91, 0.93, and 0.87 for CIs at 12, 18, and 24 mm, respectively. The best performance was at 18 mm, where a cutoff value of 57.5% corresponded to 91.7% sensitivity, 89.8% specificity, and an odds ratio of 97.2 for detecting DON. A significant correlation (P < .001) between the CIs and VF defects was observed. CONCLUSIONS: Orbital CIs based on area measurements were found to predict DON more reliably than subjective grading of orbital crowding or intracranial fat prolapse.

Repeated peribulbar injections of triamcinolone acetonide: a successful and safe treatment for moderate to severe Graves' ophthalmopathy.

PURPOSE: In this study, we aimed to evaluate the efficacy of peribulbar triamcinolone injections to treat inflammatory signs of Graves' ophthalmopathy (GO) in patients with moderate to severe GO and associated optic neuropathy (ON). METHODS: Twenty-one patients with active GO [clinical activity score (CAS) > or = 4] and systemic thyroid disease under control were enrolled in this prospective pilot study. Peribulbar triamcinolone acetonide was injected in each orbit (42 eyes), in four doses of 20 mg at 2-week intervals. Ophthalmological examination including CAS evaluation, visual field, computerized tomography (CT) scan and digital photography were performed before and after treatment. RESULTS: Twenty-one patients (11 with moderate disease, 10 with ON) were enrolled in this study and followed for at least 14 months. Initial mean CAS was 6.38 +/- 1.49, which dropped to 1.8 +/- 1.12 after 6 months of treatment (P = 0.01; mean difference of 4.57 +/- 1.56; range 1-8 score points). ON was diagnosed in 10 patients. Of these, 66% improved with peribulbar triamcinolone exclusively. A transitory increase in intraocular pressure in two patients was controlled with topic medication. CONCLUSION: Peribulbar triamcinolone injections reduce the inflammatory signs of moderate GO, as measured by the CAS, and could also be used as an alternative treatment for ON. Randomized clinical trials are needed to compare the results of triamcinolone peribulbar injections to those of other treatment modalities.

Diagnostic ability of Barrett's index to detect dysthyroid optic neuropathy using multidetector computed tomography.

OBJECTIVES: The objective of this study was to evaluate the ability of a muscular index (Barrett's Index), calculated with multidetector computed tomography, to detect dysthyroid optic neuropathy in patients with Graves' orbitopathy. METHODS: Thirty-six patients with Graves' orbitopathy were prospectively studied and submitted to neuro-ophthalmic evaluation and multidetector computed tomography scans of the orbits. Orbits were divided into two groups: those with and without dysthyroid optic neuropathy. Barrett's index was calculated as the percentage of the orbit occupied by muscles. Sensitivity and specificity were determined for several index values. RESULTS: Sixty-four orbits (19 with and 45 without dysthyroid optic neuropathy) met the inclusion criteria for the study. The mean Barrett's index values (+/- SD) were 64.47% +/- 6.06% and 49.44% +/- 10.94%in the groups with and without dysthyroid optic neuropathy, respectively (p<0.001). Barrett's index sensitivity ranged from 32% to 100%, and Barrett's index specificity ranged from 24% to 100%. The best combination of sensitivity and specificity was 79%/72% for BI=60% (odds ratio: 9.2). CONCLUSIONS: Barrett's Index is a useful indicator of dysthyroid optic neuropathy and may contribute to early diagnosis and treatment. Patients with a Barrett's index >60% should be carefully examined and followed for the development of dysthyroid optic neuropathy.

Optic nerve infiltration by acute lymphoblastic leukemia: MRI contribution.

We describe the clinical presentation and imaging features of a patient with acute lymphoblastic leukemia (ALL) that was complicated by optic nerve infiltration. The clinical and diagnostic characteristics of this complication must be recognized so that optimal therapy can be started to prevent blindness. MR imaging is useful in early detection and should be performed in any leukemic patient with ocular complaints, even during remission.

Emergence of optic pathway gliomas in children with neurofibromatosis type 1 after normal neuroimaging results.

We report the appearance of gliomas of the optic nerve or chiasm in four young children with neurofibromatosis type 1 whose previous neuroimaging studies showed no abnormalities; the age range of the children was 1 year 8 months to 5 years 9 months at the time the tumors were detected. Normal neuroimaging findings in an infant or young child with neurofibromatosis type 1 does not provide assurance that the optic nerves and chiasm will remain healthy.