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We present a 6-year-old boy with unilateral retinoblastoma of the left eye. MRI showed an intraocular tumor that extended into the optic nerve beyond the lamina cribrosa. The affected eye was enucleated and the optic nerve resection margin proved to be free. Following protocol, this patient received six courses of adjuvant systemic chemotherapy. Unfortunately, after 5 months this patient returned with the leptomeningeal spread of the tumor and died quickly thereafter.Histopathologic analysis of the enucleated eye and distal optic nerve revealed that the postlaminar tumor cells occupied the entire width of the optic nerve, extending all the way up to the pia mater, whereas, more often the tumor invasion is restricted to the center of the optic nerve. This was also visible on the MR images where contrast enhancement occupied the entire nerve width. A resection margin with tumor cells is recognized as a risk factor for metastasis, but perhaps the proximity of tumor cells to the leptomeninges should also be judged with caution as a potential increased risk for metastatic spread.

Background/aim/aim: We aimed to evaluate the size/tortuosity of the optic nerve (ON) and the dilatation of the ON sheath (ONS) in neurofibromatosis type 1 (NF-1) patients with 3T-MRI, and to assess the usefulness of 3D-SPACE in imaging the optic pathway, ON, and ONS in NF-1 patients., Materials and Methods: Twenty consecutive NF-1 patients without optic pathway glioma (OPG) (Group 1), 16 consecutive NF-1 patients with OPG (Group 2), and 19 controls were included in this study. The thickness and tortuosity of the ON and the diameter of the ONS were measured on STIR and 3D-SPACE images., Results: The thickness of the ON was similar in all groups on STIR images (P>0.05). The mean ONS diameter was higher in Group 2 with this sequence (P=0.009). Controls had significantly lower grades of ON tortuosity than Groups 1 and 2 (P=0.001), and Group 1 had significantly lower ON tortuosity compared to Group 2 (P=0.001). Severe tortuosity was only detected in Group 2., Conclusion: ON tortuosity and ONS diameter were increased in NF-1 patients in the presence of OPG. High-resolution cranium imaging with the 3D-SPACE technique using 3T-MRI seems to be helpful for detection of the optic pathway morphology and pathologies in NF-1 patients., Competing Interests: The authors report no conflicts of interest., (This work is licensed under a Creative Commons Attribution 4.0 International License.)

Purpose: Treatment of optic pathway gliomas is prompted by neuroradiological evidence of tumor growth, usually associated with progressive visual loss. Despite therapy, approximately 40% will show visual deterioration. Treatment outcome is largely based on the preservation of vision. However, current visual function assessment is often unreliable in children with optic pathway gliomas who have limited collaboration. Thus, there is a need for new clinical tools to evaluate visual functions in these children. The aim of the study was to assess the value of steady-state visual evoked potentials as a tool to assess function in the central and peripheral visual fields of children with optic pathway gliomas., Method: Ten patients with optic pathway gliomas and 33 healthy controls (ages 3 to 18 years) were tested using steady-state visual evoked potentials. The dartboard stimulus consisted of one central circle alternating at 16 reversals/s and one peripheral hoop alternating at 14.4 reversals/s, separated by a hoop of gray space. It was presented monocularly at 30% and 96% contrasts., Results: Results indicated that central signal-to-noise ratios were significantly lower in children with optic pathway gliomas compared to controls. However, no significant group difference was detected in the peripheral visual field., Conclusion: Steady-state visual evoked potentials could eventually be implemented in the clinical assessment and follow-up of central visual field deficits in uncooperative or nonverbal children but seem to have limited usefulness for evaluation of peripheral visual field deficits. Additional studies are needed to identify testing parameters for full visual field assessment.

Background/aim: Fractionated conformal radiotherapy (FCRT) is now used to treat vision-threatening optic nerve sheath meningioma (ONSM), but long-term efficacy and safety data are lacking; the purpose of this study was to assess these key data., Methods: This is a retrospective chart review with prospective follow-up of adult patients treated with FCRT for primary ONSM at four academic medical centres between 1995 and 2007 with ≥10 years of follow-up after treatment., Results: 16 patients were identified with a mean post-treatment follow-up of 14.6 years (range: 10.5-20.7 years). The mean age at symptom onset was 47.6 years (range: 36-60 years). FCRT was performed at a mean of 2.3 years after symptom onset (range: 0.2-14.0 years). At last follow-up, visual acuity had improved or stabilised in 14 of the 16 (88%) patients, and 11 (69%) had retained or achieved ≥20/40. The mean deviation on automated perimetry remained stable (-14.5 dB pretreatment vs -12.2 dB at last follow-up; p=0.68, n=10). Two (11%) patients had persistent pain, proptosis or diplopia, compared with six (38%) pretreatment (p=0.11). Two (13%) patients developed radiation retinopathy more than 6 months after completion of therapy, one (50%) of whom had worse visual acuity compared with pretreatment. No patient developed tumour involvement or radiation damage in the fellow eye., Conclusion: FCRT stabilises or improves visual function in patients with primary ONSM and is associated with a low risk of significant ocular sequelae. This treatment should be considered instead of surgery in patients with primary ONSM who require intervention due to loss of visual sensory and/or ocular motor function., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: Asymmetric nystagmus can be an important presenting sign of optic pathway gliomas in young children. We investigated the causes of asymmetric nystagmus in children with chiasmal or suprasellar optic pathway gliomas compared with children with similar optic pathway gliomas and stable gaze., Methods: Longitudinal magnetic resonance imaging before and after treatment, age-corrected visual acuity, ocular examinations, video-oculography, visual evoked potentials, and retinal nerve fiber layer thickness were retrospectively reviewed., Results: Twenty-two children were included (eight with asymmetric nystagmus and 14 with stable gaze). Subjects with asymmetric nystagmus presented at a younger age than those with stable gaze (2.0 vs 5.6 years; P < 0.001). None had neurofibromatosis type 1. Visual acuity, visual evoked potentials, nerve fiber layer, severity of optic atrophy, hydrocephalus, tumor volume, and tumor locations did not differ between those with asymmetric nystagmus and stable gaze. Asymmetric nystagmus resolved shortly after treatment, even though the average visual acuity did not improve. Changes in visual acuity or tumor volume were not different between those with asymmetric nystagmus and stable gaze after treatment. Eye movement recording from two subjects with asymmetric nystagmus revealed an asymmetric pendular-oscillation with vertical components. One subject with stable gaze developed asymmetric nystagmus with tumor growth into the rostral midbrain and associated unilateral vision loss. Another subject with tumor growth into the rostral midbrain acquired vertical saccade dysmetria., Conclusion: We hypothesize that asymmetric nystagmus associated with optic pathway gliomas is caused by subclinical abnormalities to retinal axons that connect to gaze holding centers in the rostral midbrain. Direct compression of the rostral midbrain was a possible factor to asymmetric nystagmus in some subjects. However, many subjects with stable gaze also show midbrain compression., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Purpose: This study aims to examine the relationship between ocular circulation changes and visual field defects in optic disk melanocytoma (ODM)., Methods: Five eyes of five patients were enrolled in this study. All patients were diagnosed with ODM in the Department of Ophthalmology, Hokkaido University Hospital from March 2009 to November 2017. Ophthalmological data including optical coherence tomography angiography (OCTA) and laser speckle flowgraphy (LSFG) findings were retrospectively analyzed., Results: The five ODM cases consisted of two females and three males. Ages of the patients ranged from 47 to 82 years (mean 54 years). Follow-up periods were from 4 to 105 months. Fluorescein angiography showed hypo-fluorescence throughout the examination in all four eyes examined with this modality. OCTA detected dense blood vessel networks in the tumor in two out of the five eyes. Nasal visual field defects were found in two other eyes, which were correlated with locations of tumors free of vessel networks. One ODM eye without marked visual field defects and pigmentations showed lower mean blur rates determined by LSFG in optic disk vessels and tissue circulations than those in the contralateral eye. During follow-up, there was no tumor enlargement in any case., Conclusions: This study showed the relationship between the deficit of blood vessel networks and visual field defects in ODM patients. LSFG demonstrated reduced blood flow in the tumor, suggesting that circulatory disorder caused by the optic disk tumor might be correlated with visual field defect.

Aims: To report visual acuity (VA) outcomes following chemotherapy for optic pathway glioma (OPG) in children with or without neurofibromatosis type-1 (NF1) and to analyse associated risk factors., Methods: A prospective, multicentre, cohort study involving 155 children treated between September 2004 and December 2012. Initial and final VA was used for per-eye and per-subject analysis. Correlation tests were performed to determine whether initial VA predicted final VA. Logistic regression was used to determine whether age and tumour location were associated risk factors., Results: 90 children had complete ophthalmological data. At initiation of chemotherapy, 26% and 49% of eyes with NF1-OPG and sporadic OPG, respectively, had VA of ≥0.7 log of the minimum angle of resolution (logMAR). At final visit, per eye, 49% had ≤0.2, 23% had 0.30-0.60 and 28% had VA≥0.70 logMAR in the NF1-OPG group. In the sporadic OPG group, per eye, 32% had ≤0.2, 11% had VA 0.30-0.60 and 57% had ≥0.70 logMAR. Children with sporadic OPG, per eye, were significantly less likely to have VA outcomes ≤0.60 logMAR compared with children with NF1-OPG (OR=0.30; 95% CI 0.16 to 0.56; P<0.0001). Per subject, VA improved in 24%, remained stable in 35% and worsened in 41% of children with NF1-OPG and improved in 18%, remained stable in 43% and worsened in 39% of children with sporadic OPG., Conclusions: Children with and without NF1 demonstrated the same rate of VA improvement, stabilisation or worsening; however, children with sporadic OPG had a poorer VA outcome. Better initial VA, older age, absence of postchiasm tumour and presence of NF1 were associated with improved or stable VA outcomes., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

A 38-year-old woman presented with rapidly worsening, painless right monocular vision loss. An examination revealed a visual acuity of 1.4/10 and a central scotoma in the right eye. The orbital magnetic resonance imaging (MRI) showed a well-delineated ovoid intraconal mass of the right eye, hyperintense on T2-weighted MRI with homogenous enhancement after contrast injection. The mass abutted and displaced the optic nerve. A diagnosis of cavernous hemangioma was evoked, which is the most common benign adult orbital mass with these MRI features. A biopsy was performed, and the histopathologic examination yielded a diagnosis of optic nerve sheath meningioma based on a positive antiprogesterone receptor antibody immunostaining. Our case highlights the problem with establishing a specific pathologic diagnosis based on MRI alone, even though the morphologic aspect is evocative. It is recommended to always conduct a histopathologic examination before establishing a specific diagnosis as pathology remains the gold standard, especially when the course of action or treatment may change, as in our case., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Purpose: To report the successful treatment of a 78-year-old woman with bilateral mantle cell lymphoma involving the optic nerves. Chemotherapy initially was administered in the form of intravitreal methotrexate (MTX) monotherapy and was subsequently combined with systemic ibrutinib., Methods: Retrospective case report. The diagnosis of CD5-negative mantle cell lymphoma was confirmed via immunohistopathological analysis of an axillary lymph node. Serial ophthalmologic examinations in conjunction with fluorescein angiography, fundus photography, and spectral domain optical coherence tomography were used to assess the treatment response., Results: Prompt improvement in optic nerve infiltration, no significant side effects, and excellent tolerability were noted after two weekly injections of unilateral intravitreal MTX monotherapy. Combined systemic treatment with ibrutinib and bilateral weekly MTX intravitreal injections then resulted in continued regression of optic nerve infiltration bilaterally as confirmed by serial fundus photography and optical coherence tomography. After eight additional bilateral weekly injections, a mild MTX-associated keratopathy developed, which resolved promptly with cessation of injections and administration of topical lubrication. Six weeks after MTX cessation, but with continued ibrutinib treatment, the optic nerves revealed near-complete resolution of the lymphomatous infiltration and the visual acuity improved., Conclusion: Intravitreal MTX injections and systemic ibrutinib may represent effective treatment options for patients diagnosed with intraocular mantle cell lymphoma.

Background: Optic nerve sheath meningiomas account for only 2% of orbital lesions and 42% of optic nerve tumors. Diagnosis remains difficult because histologic confirmation carries a high risk of visual loss. Therefore, a less invasive and specific diagnostic method for differentiating optic nerve sheath meningiomas from other optic nerve lesions is needed to overcome the limitations of computed tomography and magnetic resonance imaging, and make the best individualized treatment decision. This case is a good illustration of the clinical and imaging difficulties inherent in this rare tumor, which may be hard to differentiate from other causes., Case Presentation: A 51-year-old Caucasian woman developed a central scotoma, visual loss, and abnormal visual evoked potentials. The first magnetic resonance imaging scan classified the optic nerve damage as retrobulbar optic neuritis. After magnetic resonance imaging follow-up at 3 months, a negative lumbar puncture and biological workup, and clinical worsening, an optic nerve sheath meningioma was suspected. We confirmed this diagnosis with 111In-pentetreotide single-photon emission computed tomography, which is able to bind with very high affinity to somatostatin receptor subtype 2 expressed on meningiomas., Conclusions: In the diagnosis of optic nerve sheath meningiomas, [111In]-pentetreotide single-photon emission computed tomography-fused magnetic resonance imaging is a valuable additional tool, optimizing the diagnosis and obviating the need for a more invasive procedure.

Objective: To analyze the flow of retrobulbar vessels in retinoblastoma by color Doppler imaging., Methods: A prospective study of monocular retinoblastoma treated by enucleation between 2010 and 2014. The examination comprised fundoscopy, magnetic resonance imaging, ultrasonography and color Doppler imaging. The peak blood velocities in the central retinal artery and central retinal vein of tumor-containing eyes (tuCRAv and tuCRVv, respectively) were assessed. The velocities were compared with those for normal eyes (nlCRAv and nlCRVv) and correlated with clinical and pathological findings. Tumor dimensions in the pathological sections were compared with those in magnetic resonance imaging and ultrasonography and were correlated with tuCRAv and tuCRVv. In tumor-containing eyes, the resistivity index in the central retinal artery and the pulse index in the central retinal vein were studied in relation to all variables., Results: Eighteen patients were included. Comparisons between tuCRAv and nlCRAv and between tuCRVv and nlCRVv revealed higher velocities in tumor-containing eyes (p < 0.001 for both), with a greater effect in the central retinal artery than in the central retinal vein (p = 0.024). Magnetic resonance imaging and ultrasonography measurements were as reliable as pathology assessments (p = 0.675 and p = 0.375, respectively). A positive relationship was found between tuCRAv and the tumor volume (p = 0.027). The pulse index in the central retinal vein was lower in male patients (p = 0.017) and in eyes with optic nerve invasion (p = 0.0088)., Conclusions: TuCRAv and tuCRVv are higher in tumor-containing eyes than in normal eyes. Magnetic resonance imaging and ultrasonography measurements are reliable. The tumor volume is correlated with a higher tuCRAv and a reduced pulse in the central retinal vein is correlated with male sex and optic nerve invasion.

Diffusion tensor tractography(DTT) can theoretically be used in assessing the optic chiasmatic glioma(OCGs),which are still in debate about optimal treatment. The purpose of this study was to investigate the role of this technology in offering more information about the tumor, assisting the debulking surgery, and helping to anticipate visual outcomes. As a prospective cohort study, the enrolled patients received routine pre- and postoperative neuro-ophthalmology, neuroimaging, and endocrine examinations. Fiber tractography was meanwhile performed based on diffusion tensor imaging examination. Identification of the position relationship between the lesions and residual optic path, and morphology analysis of them was done based on their DTT features. All the information was used for confirmation by the intraoperative findings. 11 pediatric patients were enrolled in this study. Most of them got subtotal resection of the tumors and stable postoperative visual outcomes. On the DTT imagings, the tumors were divided into infiltrative endophytic ones (TypeI) and inflated ones (TypeII), which can be subclassified as inferior and superior chiasmatic ones based on the positional relationships between the optic chiasm fibers and the tumors. These positional relationships were confirmed intraoperatively. The postoperative DTT images were quite different from preoperative ones. The application of DTT to children with OCGs is feasible, and valuable for getting more information about the disease, improving surgical techniques, and helping predict the overall and visual prognosis of the patients. The exact correlations of DTT features and visual outcomes need to be further verified.

Importance: Monitoring young children with optic pathway gliomas (OPGs) for visual deterioration can be difficult owing to age-related noncompliance. Optical coherence tomography (OCT) measures of retinal nerve fiber layer (RNFL) thickness have been proposed as a surrogate marker of vision but this technique is also limited by patient cooperation., Objective: To determine whether measures of circumpapillary RNFL thickness, acquired with handheld OCT (HH-OCT) during sedation, can differentiate between young children with and without vision loss from OPGs., Design, Setting, and Participants: This cross-sectional analysis of a prospective observational study was conducted at a tertiary-care children's hospital. Children with an OPG (sporadic or secondary to neurofibromatosis type 1) who were cooperative for visual acuity testing, but required sedation to complete magnetic resonance imaging, underwent HH-OCT imaging of the circumpapillary RNFL while sedated., Main Outcomes and Measures: Area under the curve of the receiver operating characteristic, sensitivity, specificity, positive predictive value, and negative predictive value of the average and quadrant-specific RNFL thicknesses., Results: Thirty-three children (64 eyes) met inclusion criteria (median age, 4.8 years; range, 1.8-12.6 years). In children with vision loss (abnormal visual acuity and/or visual field), RNFL thickness was decreased in all quadrants compared with the normal-vision group (P < .001 for all comparisons). Using abnormal criteria of less than 5% and less than 1%, the area under the curve was highest for the average RNFL thickness (0.96 and 0.97, respectively) compared with specific anatomic quadrants. The highest discrimination and predictive values were demonstrated for participants with 2 or more quadrants meeting less than 5% (sensitivity = 93.3; specificity = 97.9; positive predictive value = 93.3; and negative predictive value = 97.9) and less than 1% (sensitivity = 93.3; specificity = 100; positive predictive value = 100; and negative predictive value = 98.0) criteria., Conclusions and Relevance: Measures of RNFL thickness acquired with HH-OCT during sedation can differentiate between young children with and without vision loss from OPGs. For young children who do not cooperate with vision testing, HH-OCT measures may be a surrogate marker of vision. Longitudinal studies are needed to delineate the temporal relationship between RNFL decline and vision loss.

Importance: Children with optic pathway gliomas (OPGs) frequently experience vision loss from their tumors. Standard front-line treatment using carboplatin-based chemotherapy typically produces only a modest benefit (eg, stabilization or 0.2 logMAR improvement) in visual acuity (VA). Bevacizumab is a monoclonal antibody that targets vascular endothelial growth factor and acts primarily as an anti-angiogenic agent. Recent reports suggest a qualitative improvement in vision after bevacizumab-based treatment in children with OPGs., Observations: We report 4 cases of pediatric OPGs (2 neurofibromatosis type 1-related and 2 sporadic cases) that received treatment with bevacizumab due to progressive VA or visual field (VF) loss despite prior treatment with chemotherapy or proton-beam radiation. All 4 subjects demonstrated a marked improvement in their VA, VF, or both while receiving bevacizumab-based therapy. Three patients had complete resolution of their VA or VF loss in at least 1 eye-2 of whom had previously received bevacizumab therapy., Conclusions and Relevance: Given that most patients with OPG-related visual impairment will show modest or no visual improvement with standard treatment, the incorporation of bevacizumab in these cases may greatly improve visual outcomes and should be considered in appropriate clinical situations.

A 48-year-old woman noted progressive, painless visual loss in her left eye. She was diagnosed with bilateral optic nerve sheath meningiomas (ONSMs), extending across the planum sphenoidale. Radiation was offered, but the patient declined. She has been followed for more than 27 years with stable visual function and neuroimaging findings. Bilateral ONSMs, although usually described as aggressive in nature, may follow a stable clinical course.

Purpose: Longitudinal ophthalmologic clinical trials in young children require multiple visual acuity (VA) testing methods-especially when the subjects have cognitive and developmental delay. This study evaluated the success rate and comparability of two different VA testing methods in children with neurofibromatosis type 1 (NF1) and/or optic pathway gliomas (OPGs)., Methods: Two institutions prospectively enrolled children 10 years or younger with NF1 and/or an OPG. Both Teller grating acuity (TAC) and recognition acuity using the computerized version of the Amblyopia Treatment Study VA testing protocol that limits responses to four letters (H, O, T, or V) were attempted in all subjects. The association of age and diagnosis of NF1 on success rate was analyzed. Differences in grating and recognition acuity were compared., Results: One hundred twenty-seven children met inclusion criteria (median age = 5.58 years). Of 127 subjects, 11 (8.7%) could not complete monocular TAC testing in either eye; 39 (30.7%) could not complete HOTV testing and were younger than those able to complete HOTV testing (mean = 2.9 vs. 7.0 years, respectively; Z = -8.3, P < 0.01). Older age was associated with successful HOTV testing and remained significant in all regression analyses (P < 0.01). The within-subject logMAR values for TAC and HOTV testing results were significantly correlated (r = 0.69, P < 0.01)., Conclusions: Young children with NF1 and/or OPGs were frequently unable to complete recognition acuity testing. These factors are important to consider when designing a clinical trial for children with NF1 and/or OPGs.

Objectives: To analyze the differences and correlation between the ganglion cell complex (GCC), peripapillary retinal nerve fiber layer (pRNFL) and mean deviation (MD), mean sensitivity (MS) of saddle area tumor patients, and to evaluate the feasibility of using OCT to diagnose the visual pathway damage of saddle area tumor patients., Methods: It was a case-control study. One hundred and eighty-eight normal persons and 279 saddle area tumor patients treated in Beijing Tiantan hospital, from November 2010 to June 2011, were recruited; the saddle area tumor patients were diagnosed by surgical sample pathological analysis. The recruits were divided into four groups, including the normal group, the pituitary gland tumours group, the meningiomas group and the craniopharyngiomas group. All patients received the visual field (VF) test using the Octopus 900 automated perimeter with the central 30 degree program, and the mean thickness measurements of GCC and pRNFL were made by RTVue OCT. The Spearman rank correlation coefficient was used to assess the correlation between GCC, pRNFL and MD, MS. The differences between pRNFL and GCC among the four groups were compared., Results: The average thickness of the pRNFL (r(sOD) = 0.369 - 0.735, r(sOS) = 0.369 - 0.691) and GCC (r(sOD) = 0.357 - 0.797, r(sOS) = 0.375 - 0.681) were correlated with MD and MS (P < 0.01), and the measurements from the meningiomas was the most significant. Comparing with the normal group's thickness of pRNFL and GCC [OD: (113.60 ± 9.13) µm and (98.04 ± 6.85) µm; OS: (114.06 ± 8.99) µm and (97.70 ± 5.83) µm], the pituitary gland tumours group [OD: (101.25 ± 19.95) µm and (91.08 ± 13.19) µm; OS: (99.96 ± 20.95) µm and (89.82 ± 15.47) µm], meningiomas group [OD:(89.54 ± 19.19) µm and (80.77 ± 10.43) µm; OS: (92.79 ± 22.00) µm and (80.43 ± 10.09) µm] and craniopharyngiomas group [OD: (94.96 ± 16.59) µm and (86.46 ± 11.65) µm; OS: (94.92 ± 15.77) µm and (86.77 ± 9.56) µm] were thinner. There was statistically significant difference of pRNFL and GCC, among the three tumor groups, and the thickness of pRNFL and GCC of the meningiomas group was the thinnest (P < 0.05)., Conclusions: The average thickness of the pRNFL and GCC is correlated with vision field damage, which can be used to evaluate optic nerve damage of saddle area tumor patients quantitatively, where the meningiomas was the most significant. The thickness of the pRNFL and GCC was thinner, and the damage to visual functions was more serious. In the three tumor groups, the meningiomas group was the most serious. In the clinic, visual field test combined with OCT were helpful to find and assess the damage to visual pathway and prognosis.

Object: Magnetic resonance imaging is commonly used in diagnosis and surveillance for optic pathway glioma (OPG). The authors investigated the role of diffusion tensor (DT) tractography in assessing the location of visual pathway fibers in the presence of tumor., Methods: Data in 10 children with OPG were acquired using a 3T MRI generalized autocalibrating parallel acquisitions DT-echo planar imaging sequence (25 isotropic directions with a b value of 1000 seconds/mm(2), slice thickness 3 mm). Fiber tractography was performed, with seed regions placed within the optic chiasm and bilateral nerves on the coronal plane, including the tumor and surrounding normal-appearing tissue. Tracking was performed with a curvature threshold of 30°., Results: For prechiasmatic lesions, fibers either stopped abruptly at the tumor or traversed abnormally dilated nerve segments. Similar findings were seen with chiasmatic lesions, with an additional arrangement in which fibers diverged around the tumor. For each patient, DT tractography provided additional information about visual fiber arrangement in relation to the tumor that was not evident by using conventional MRI methods. Retrospective reconstruction of visual fibers in 1 patient with new postoperative hemianopia revealed an unexpected superior displacement of the optic tract that might have been helpful information had it been applied to preoperative planning or surgical navigation., Conclusions: Optic pathway DT tractography is feasible in patients with OPG and provides new information about the arrangement of visual fibers in relation to tumors that could be incorporated into surgical navigation for tumor biopsy or debulking procedures.

Background: Little is known about the visual outcome of children affected by an optic pathway glioma (OPG)., Procedures: We evaluated the long-term visual outcome of 32 consecutive children affected by OPG without neurofibromatosis type-1 referred to the Pediatric Department of Padua University and managed according to standardized strategies., Results: Eight children received chemotherapy, 10 radiotherapy, 7 both chemotherapy and radiotherapy, whereas 7 were untreated. At presentation, visual acuity (VA) was normal in 22 children (13 unilaterally and 9 bilaterally), and reduced in 10. At follow-up, VA had improved in 6 patients; it was stable in 8 and worse in 18. Visual field, assessed in 29 children, was normal in 9 and reduced in 20. The number of children with some grade of visual impairment increased from 7 to 10 during follow-up. Of the 17 children in whom the tumor became significantly smaller, VA improved in 6, was stable in 3, and worse in 8. Of the 6 children with improved VA, 5 received radiotherapy, and their papilla was normal or mildly pale. After a median follow-up of 6 years, 26 patients are alive with stable disease., Conclusions: The visual prognosis of children with OPG is unsatisfactory. Older children treated with radiotherapy seem to have a better visual outcome than younger children. Severe optic pallor at diagnosis or during follow-up may be indicative of a negative prognosis.

Purpose: The aim of this study was to test the correlation between Fourier-domain (FD) optical coherence tomography (OCT) macular and retinal nerve fibre layer (RNFL) thickness and visual field (VF) loss on standard automated perimetry (SAP) in chiasmal compression., Methods: A total of 35 eyes with permanent temporal VF defects and 35 controls underwent SAP and FD-OCT (3D OCT-1000; Topcon Corp.) examinations. Macular thickness measurements were averaged for the central area and for each quadrant and half of that area, whereas RNFL thickness was determined for six sectors around the optic disc. VF loss was estimated in six sectors of the VF and in the central 16 test points in the VF. The correlation between VF loss and OCT measurements was tested with Spearman's correlation coefficients and with linear regression analysis., Results: Macular and RNFL thickness parameters correlated strongly with SAP VF loss. Correlations were generally stronger between VF loss and quadrantic or hemianopic macular thickness than with sectoral RNFL thickness. For the macular parameters, we observed the strongest correlation between macular thickness in the inferonasal quadrant and VF loss in the superior temporal central quadrant (rho=0.78; P<0.001) whereas for the RNFL parameters the strongest correlation was observed between the superonasal optic disc sector and the central temporal VF defect (rho=0.60; P<0.001)., Conclusion: Although FD-OCT RNFL and macular thickness measurements were both correlated with VF loss, the correlation was stronger with quadrantic macular than with RNFL thickness measurements in patients with temporal hemianopia. Such measurements could potentially be used to quantify neuronal loss in patients with chiasmal compression.

Primary optic nerve sheath meningiomas (ONM) are rare. Most of these are World Health Organization Grade I meningiomas. Because of the intimate relationship to the optic nerve sheath from which they arise, radical excision is often not feasible. The chordoid variant of meningioma is an infrequent tumor and extremely uncommon among primary ONMs. We report a 36-year-old woman with painless proptosis and normal visual acuity who presented to us with an exophytic intraconal mass, which was excised. Histology revealed a chordoid meningioma. We could find only two previous reports, which are discussed., (Copyright 2009 Elsevier Ltd. All rights reserved.)

We analysed our long-term results after fractionated stereotactic radiotherapy (FSRT) in patients with primary optic nerve sheath (pONSM) meningioma, as a rare subtype of meningiomas. Between 01/1995 and 12/2007, 32 patients with pONSM were treated with FSRT. Fifteen patients received radiotherapy as primary treatment, four after biopsy, and six patients after surgical resection. Seven patients were irradiated for recurrent disease. Seventeen lesions were histologically proven and determined as WHO grade I pONSM. Median target volume was 15.7 cc, median total dose 54.9 Gy. Twenty-nine patients showed clinical symptoms before radiotherapy like reduced vision, unilateral loss of vision, or an exophthalmia. Median follow-up was 4.5 years. Overall local tumor control was 100%. Twenty-six patients had stable disease based on CT/MRI, while 6/32 had a partial remission of tumor volume. 97% of our patients showed stable vision or an improvement of visual acuity. Eleven patients (38%) showed an improvement of pre-existing clinical symptoms like double vision, exophthalmia and visual acuity. Only one patient showed an impairment of vision with progressive concentric decreasing of the visual field on the side of the irradiated ONSM. These data demonstrate that FSRT is an effective and safe treatment modality for local control in patients with pONSM with minimal risk of significant late toxicity.

Background: Though the pathology of optic pathway tumor is mostly pilocytic astrocytoma, the benign tumor behaves like malignant tumor because total resection is not feasible. Adjuvant chemotherapy might be a reasonable strategy for management of these low grade tumors which keep growing. We evaluate the responsiveness of optic pathway tumor to cisplatin-based chemotherapy., Methods: Patients with unresectable and progressive optic pathway tumors received conventional chemotherapy including cisplatin, etoposide, and vinblastine were enrolled in this study from 1992 to 2007. Patients treated with radiotherapy previously were excluded. Brain MRI was performed every 3 months to evaluate the objective response to chemotherapy., Results: There are seven girls and nine boys enrolled in this study. The median age at diagnosis was 30 months old (range from 3 months to 11 years old). The median follow-up duration was 81.5 months (range from 24 months to 14.5 years). The pathology showed pilocytic astrocytomas in 11 patients, astrocytoma in one patient, and anaplastic astrocytomas in two patients. The 6-month progression-free survival (PFS) is 100%, 12-month PFS is 81.3%, 3-year PFS is 71.4% and 5-year PFS is 55.5% respectively. The toxicity of the cisplatin-based chemotherapy showed mild bone marrow suppression in 13 patients (81.3%), infection in nine patients (56.3%), gastrointestinal discomfort in seven patients (43.8%), renal insufficiency in two patient (12.5%), cerebral salt wasting syndrome with hyponatremia in one patient (6.25%) and high pitch hearing loss in two patients (12.5%)., Conclusion: Cisplatin-based chemotherapy is an effective regimen for control of progressive optic pathway tumors.

Purpose: To report a case illustrating the value of pattern VEP and pattern ERG (PERG) in monitoring optic nerve gliomata (OG)., Case Report: A 15-year-old girl presented with a 3-year history of frontal headaches and a 5-month history of blurred vision in the right eye. MRI scanning revealed a thickened, right optic nerve extending to the cranial opening of the optic canal, consistent with an optic nerve glioma. Pattern VEP showed a mildly delayed major positive component consistent with optic nerve dysfunction. The PERG N95:P50 ratio was subnormal in keeping with retinal ganglion cell involvement. Visual acuity decreased over the following 2 years and repeat VEP objectively demonstrated marked deterioration in optic nerve function despite neuroradiology showing no significant change in the tumour., Conclusions: Pattern VEPs and pattern ERGs can provide early objective evidence of optic nerve/retinal ganglion cell dysfunction in optic nerve gliomata. Serial recordings can objectively demonstrate worsening function in the absence of significant neuroradiological change.

Objective: To report clinical findings of rare retrobulbar optic nerve hemangioblastomas associated with von Hippel-Lindau disease (VHL)., Design: Retrospective observational case series., Participants: Nine patients with VHL., Methods: The clinical course and magnetic resonance imaging findings of patients with VHL and hemangioblastomas affecting the anterior visual pathway from the intraorbital optic nerve to the optic chiasm are reviewed., Main Outcome Measure: Clinical course of retrobulbar optic nerve hemangioblastomas., Results: The mean age of VHL diagnosis was 24+/-14 years, and mean follow-up was 5+/-4 years. All had other CNS lesions and retinal hemangioblastomas. Approximately 50% (5/9) had a previous enucleation or had visual acuity loss (4/9), some due to other VHL ocular complications. Four patients underwent surgical resection of an intracranial hemangioblastoma. Growth patterns and pathology are similar to those of other hemangioblastomas in the CNS., Conclusions: Although these lesions are rare, patients with VHL who present with signs of optic neuropathy should be evaluated for anterior visual pathway hemangioblastomas impinging on the optic nerve from the orbit to the chiasm. On neuroimaging, the hemangioblastomas may demonstrate chiasmal or optic tract edema, associated cysts, and T(2) flow voids. Lesions may remain radiologically and clinically stable, evolve radiographically with no visual or neurological progression, or progress clinically and radiographically. Patients at risk for visual loss should be considered for surgical resection. Close coordination among neuroradiology, neurosurgery, and ophthalmology patient care teams is advised for optimal management of these patients.

The aim of this study was to evaluate longitudinally functional and neuro-radiologic findings in childhood optic gliomas (OG), by comparing flicker visual evoked potentials (F-VEPs) with brain magnetic resonance imaging (MRI) changes. Fourteen children (age range: 1-13 years) with OGs underwent serial F-VEP, MRI and neuro-ophthalmic examinations over a 38 month (median, range: 6-76) follow-up. F-VEPs were elicited by 8 Hz sine-wave flicker stimuli presented in a mini-Ganzfeld. Contrast-enhanced MRI examinations were performed. Results of both tests were blindly assessed by independent evaluators. F-VEPs were judged to be improved, stable or worsened if changes in the amplitude and/or phase angle of the response exceeded the limits of test-retest variability (+/-90th percentile) established for the same patients. MRI results were judged to show regression, stabilization or progression of OG based on its changes in size (+/-20%) or extension. Two to seven pairs of F-VEP/MRI examinations per patient (median: 4) were collected. Based on a total of 38 pairs of F-VEP/MRI examinations, both tests agreed in showing worsening (progression), stabilization and improvement (regression) in 5, 15 and 10 cases, respectively. In 3 cases, F-VEPs showed a worsening and MRI a stabilization, while in 5 cases F-VEPs showed an improvement and MRI a stabilization. Agreement between F-VEP and MRI changes was 78.9% (95% CI: +/- 37%, K statistics = 0.67, P < 0.001). The results indicate that longitudinal F-VEP changes can predict changes in MRI-assessed OG size and extension, providing a non-invasive functional assay, complementary to neuro-imaging, for OG follow-up.

Purpose: Restoration of visual function after neurosurgery for parachiasmal tumors is variable and unpredictable. The current study was conducted to determine whether in vivo retinal nerve fiber layer (RNFL) thickness measurements predict the visual recovery of such patients., Methods: Forty patients undergoing surgical resection of parachiasmal lesions were prospectively assessed before surgery with a neuro-ophthalmic examination, involving standard automated visual field (VF) testing and optical coherence tomography (OCT) measurements of RNFL thickness, which was the prespecified marker for axonal loss. Tests were repeated within 6 weeks after surgery., Results: Thinner preoperative RNFL thickness was associated with worse visual acuity (VA) and VF mean deviation (MD). Patients with normal preoperative RNFL had significant improvement in mean VA after surgery, from 20/40 to 20/25 (P = 0.028), whereas patients with thin RNFL did not improve (20/80 to 20/60, P = 0.177). Eyes with normal RNFL showed improvement in MD (-7.0 dB before surgery, -3.5 dB after surgery, P = 0.0007) unlike eyes with thin RNFLs, which had no significant improvement after surgery (-15.3 dB before and -13.3 dB after surgery, P = 0.191). RNFL thickness increased by 1% after surgery among all eyes (P = 0.04). Eyes with severe VF defects (MD

Objectives: Analysis of form and function relationships with tumour-mediated dislocations and deformations of the optic chiasm., Materials and Methods: Results of magnetic resonance tomography and two forms of quantitative, computerized perimetry were contrasted in 31 patients with pituitary adenomata impinging on the chiasm, prior to treatment., Results: Perimetric abnormalities ranged from none to modest. High-pass Resolution Perimetry (HRP) produced abnormal results in 10 cases and RareBit perimetry (RBP) in 15 cases. Overall, field defects and qualitative stages of deformation and dislocation of the chiasm were well correlated. Among quantitative indices, the best correlations were obtained by a measure of the cranio-caudal position of the chiasm., Conclusions: On average, an elevation of the chiasm by 6 mm will be associated with abnormal visual fields in 50% of the cases. An additional elevation of 5 mm will raise the incidence of field defects to 90%.

A 72-year-old previously healthy man developed rapidly progressive visual loss, and brain imaging showed features suggestive of a malignant glioma of the anterior visual pathway. Biopsy of one optic nerve yielded a diagnosis of lymphoma. There was no evidence of an extracranial non-Hodgkin lymphoma, so the conclusion was that this represented a primary central nervous system lymphoma (PCNSL). PCNSL isolated to the optic chiasm has been described only once in an immunocompetent patient. Our patient is unusual in that the lymphoma involved the optic nerve, chiasm, and tract in an immunocompetent patient.

A 30-year-old man who developed acute blurred vision in the right eye and right periocular pain on eye movement proved to have a bilateral optic neuropathy and imaging evidence suggestive of bilateral optic nerve sheath meningiomas. This is an unusual presentation for optic nerve meningioma but a reminder that this entity may mimic optic neuritis.

Unlabelled: Optic nerve sheath meningioma (ONSM) accounts for one-third of primary optic nerve tumors, and 2% of all meningiomas. ONSM must be distinguished from other meningiomas, in particular from cavernous meningiomas because of the different prognosis and treatment. The most frequent clinical sign is a progressive or sudden unilateral visual loss. Treatment of ONSM is still subject to discussion. This report covers a series of eight ONSM patients treated with fractionated stereotactic radiotherapy., Material and Methods: Between 2000 and 2006, we managed eight patients with ONSM. The average patient age was 47 years. There were five women and three men. The most frequent clinical signs were visual loss (100%), proptosis (35%), diplopia (25%). One patient was initially treated with surgery. All patient have been treated by fractionated stereotactic radiotherapy. 45 Gy in 25 fractions were delivered on the meningioma area at a rate of 5 fractions of 1.8 Gy per week. We used a Brainlab framework associated with a thermo-formed mask. A computed tomography then magnetic resonance imaging was obtained for each patient. The data was merged and planning took place on a Brainlab dosimetric console. The treatment was performed with a head-only Varion linear accelerator, with a Brainlab multi-blade collimator., Results: The average follow-up was 27 months. Each patient had a complete radiological and ophthalmologic exam every 3 months during the first year, then every 6 months thereafter. Tumor control rate was 100%. Vision was re-established in five patients and three patients had improvement, including one patient during treatment. 100% of proptosis and diplopias regressed. No side effect was reported., Conclusion: This is still a preliminary study, but the results suggest that fractionated stereotactic radiotherapy may emerge as a primary treatment for ONSM, delaying surgery, which has proven functionally disappointing.

An 82-year-old man presented with optic disc melanocytoma and glaucomatous optic neuropathy. The coexistence of these findings is particularly challenging, as there is no specific means for differentiating whether the optic nerve damage is glaucoma- or melanocytoma-related.

Background: Prechiasmal lesions of the visual pathway are produced by visual field defects that either affect only one eye or, if both eyes are affected, they are clearly different. Typically, the defects respect the vertical meridian. This report of a case shows that the classical nerve fibre bundle defect must be taken into consideration for differential diagnosis., Case Report: In a 67-year-old patient, a meningeoma of the sphenoid was diagnosed that had spread into the optic canal. Examination of the visual field showed an inferior altitudinal nerve fibre bundle defect., Conclusions: Apparently the retinal pattern of nerve fibres remains intact even when approaching the optic chiasm. Nerve fibre bundle defects are therefore sometimes found in prechiasmal lesions and should be considered for differential diagnosis (e. g., in the case of a suspected low tension glaucoma).

Purpose: To describe four patients with optic nerve sheath meningioma in whom visual improvement began to occur even before completion of a standard course of radiation therapy., Design: Retrospective case review., Methods: A review was conducted of 35 patients who received stereotactic three-dimensional conformal radiotherapy for optic nerve sheath meningioma from 1990 to 2005., Results: Four patients with radiographic confirmation of optic nerve sheath meningioma were identified who had neuro-ophthalmologic assessment, which included perimetry, either while radiation treatment was underway or immediately afterwards. All of the patients showed improvement in visual acuity and visual fields., Conclusion: A rapid response to radiation therapy may occur in some patients with optic nerve sheath meningioma. In such patients, it may be possible to customize the radiation dose by assessing of visual function during the course of therapy.

Objective: Many approaches have been recommended for the surgical treatment of anterior and middle cranial fossa lesions. The frontobasal approach and its many modifications have been proposed and developed for such situated lesions. An alternative approach is the frontolateral craniotomy through a supraciliary skin incision., Methods: This minimally invasive technique, a 2.5 x 3.0 cm craniotomy, just above the eyebrow through a supraciliary incision, is a simple but elegant modification of the traditional approach to the anterior cranial fossa., Results: A step-by-step description of the approach is offered in this report to facilitate a clear understanding of the lesions treatable with this minimally invasive technique., Conclusion: The supraciliary frontolateral keyhole craniotomy is a minimally invasive cosmetic approach that provides excellent exposure to a variety of intracranial lesions. This approach cannot be used for all intracranial pathologies, but is recommended for many anterior and middle cranial fossa lesions.

Objective: In 1904, Hermann Wilbrand reported that nasal ON fibers form a loop into the contralateral ON, subsequently referred to as Wilbrand's knee. He had further theorized that a lesion affecting Wilbrand's knee would develop a distinct visual field defect characterized by an ipsilateral central scotoma with a contralateral superotemporal visual field defect. We present clinical evidence that disputes the existence of Wilbrand's knee., Methods: A retrospective analysis of 3 patients whose ONs were divided at the ON-chiasm junction is presented. Two patients had an ONS meningioma and the other patient had sarcoidosis of the ON. Resection of the lesion and the ON up to the nerve-chiasm junction was done to prevent the disease from extending into the OC and the contralateral ON. The patients had detailed neuro-ophthalmologic evaluations preoperatively and postoperatively., Results: After the resection of the ON at the ON-chiasm junction in the 3 patients, junctional scotoma could not be detected by visual perimetry., Conclusion: No clinical perimetric evidence was found to support the existence of Wilbrand's knee in the anterior visual pathway.

Optic nerve sheath meningioma is an uncommon tumor of middle age. Patients most often present with progressive visual loss, mild proptosis, and, occasionally, ocular motility defect. With modern neuroimaging, these tumors are relatively easy to diagnose. However, the computed tomography scan or magnetic resonance imaging scan obtained must be a high-resolution study focused on the optic nerve. Clinical observation may be appropriate for patients with relatively preserved vision. However, when vision deteriorates, fractionated stereotactic radiotherapy is indicated and can often restore or preserve visual function.