Management of optic nerve gliomas.

Seventeen patients thought to have orbital optic nerve gliomas when first seen have been reviewed after up to 12 years. Enlargement of the optic canal was present in 15 of the 16 patients examined, but this finding was unreliable as an indicator of the posterior extent of the tumour. Nine patients had a stable course with little change over a period of up to 8 years; there was optic atrophy in all and neurofibromatosis was relatively common (7/9). Eight patients showed progressive enlargement of the tumour; 6 had swollen discs, and the incidence of neurofibromatosis was relatively low (3/8). The optic nerve was excised in 7 of the latter group. Biopsies of the optic nerve taken from the region of maximal enlargement were difficult to interpret and unhelpful in planning management. Radical surgery should be reserved for the minority of patients in whom there is progressively enlarging tumour without evidence of chiasmal involvement.     

Unilateral adult malignant optic nerve glioma

Introduction Adult malignant optic nerve gliomas are rare and rapidly fatal visual pathway tumours. They represent a clinical entity different from the more common childhood benign optic nerve gliomas, which are frequently associated with neurofibromatosis I.Case report A 61-year-old woman presented with rapidly progressing right vision loss, lower altitudinal visual field defect and papilloedema. MRI showed intraorbital and intracranial swelling of the right optic nerve. Resection of the intracranial part of the right optic nerve up to the chiasm revealed anaplastic astrocytoma grade III. Within 1 year, the patient died of leptomeningeal metastasis despite radiotherapy. Clinical and MRI evaluation of the left eye and optic nerve were normal at all times.Discussion Unilateral adult malignant glioma of the optic nerve is exceptional. The final diagnosis was only confirmed by optic nerve biopsy. In the literature, only one patient has been reported with a unilateral tumour manifestation; he was lost to follow-up 3 months later. All other cases were bilateral. To date, 44 case reports of adult malignant optic nerve glioma have been published, either malignant astrocytoma or glioblastoma. These tumours can mimic optic neuritis in their initial presentation. The diagnosis is seldom made before craniotomy. On MRI images, malignant glioma cannot be distinguished from optic nerve enlargement due to other causes. Although radiotherapy appears to prolong life expectancy, all presently available treatment options (radiation, surgery, radio-chemotherapy) are of limited value. Most patients go blind and die within 1 or 2 years.This paper was presented in part at the 101st Meeting of the German Ophthalmological Society (DOG).     

Primary optic nerve sheath meningioma.

Fifty patients with optic nerve sheath meningiomas have been reviewed with a follow-up of up to 15 years. The median age at onset of their symptoms was 40.0 years. The majority were middle aged females with a slowly progressive lesion. More aggressive lesions were encountered in a younger, predominantly male group of patients with frequent intracranial involvement. Our experience indicates that a more aggressive surgical approach to these lesions is needed to prevent this sequence of events. Meningiomas in older individuals often do not need treatment, though radiotherapy can be beneficial.     

Congenital anomalies of the optic nerve

Congenital optic nerve head anomalies are a group of structural malformations of the optic nerve head and surrounding tissues, which may cause congenital visual impairment and blindness. Each entity in this group of optic nerve anomalies has individually become more prevalent as our ability to differentiate between them has improved due to better characterization of cases. Access to better medical technology (e.g., neuroimaging and genetic analysis advances in recent years) has helped to expand our knowledge of these abnormalities. However, visual impairment may not be the only problem in these patients, some of these entities will be related to ophthalmologic, neurologic and systemic features that will help the physician to identify and predict possible outcomes in these patients, which sometimes may be life-threatening. Herein we present helpful hints, associations and management (when plausible) for them.     

High-dose steroid therapy of traumatic optic neuropathy may fail to protect the optic nerve permanently

Background: The optimal treatment of traumatic optic neuropathy (TON) is still unresolved due to the poor understanding of the pathology involved and the relatively small number of cases in the published clinical series. It is currently held that the results of conservative treatment with high-dose corticosteroids are similar to those obtained with surgical decompression. Purpose: To assess the late results of conservative treatment for traumatic optic neuropathy. Patients and methods: 15 patients (3 women and 12 men, age 14–64 years) who sustained a direct injury to the optic nerve as a consequence of closed head trauma. All were treated conservatively with high-dose dexamethasone therapy. Ten patients presented for follow-up examination 3–11 years after the injury, on average 5.3±2.4 years (SD). A full ophthalmologic examination and color-coded Doppler (CCD) study of the orbital vessels was performed in all subjects. Results: Full blindness of the affected eye, persisting since the injury, was noted in six patients. Their visual acuity did not improve in spite of vigorous treatment and their eyes remained without sight at the follow-up examination. The finding of an absence of flow in central retinal arteries at follow-up suggests structural damage to the nerve. Moreover, in five of these patients, distinct features of eyeball atrophy had evolved between the time of injury and the follow-up examination. Four patients, who on admission presented only with the ability to perceive light, responded to 2–3 weeks of steroid therapy with improvement of visual acuity to 3/50, 5/50, 5/10, and 5/7. After 4–6 years, however, the visual acuity of all of these patients had deteriorated, resulting in nearly total blindness in one case, light perception in two, and 1/50 in the fourth. Optic nerve atrophy was diagnosed in all of the affected eyes. Conclusion: Conservative treatment of TON, even if giving transient improvement in visual function, may not be reliable in providing permanent relief from the sequelae of traumatic optic neuropathy.     

Optic nerve glioma: an update

Optic nerve glioma is the most common optic nerve tumour. However, it has an unpredictable natural history. The treatment of optic nerve gliomas has changed considerably over the past few years. Chemotherapy and radiation therapy can now stabilize and in some cases improve the vision of patients with optic nerve gliomas. The treatment of optic nerve glioma requires a multi-disciplinary approach where all treatment options may have to be implemented in a highly individualized manner. The aim of this review article is to present current diagnostic and treatment protocols for optic nerve glioma.     

Optic nerve blindness due to paranasal sinus disease.

Patients with total blindness caused by paranasal sinus disease have rarely been reported. We retrospectively studied the prognosis of patients who had optic nerve blindness due to paranasal sinus disease. During the past 10 years, we identified 17 patients with optic nerve disease and posterior paranasal sinus disease. Seven of the 17 patients with optic nerve disease and paranasal sinus disease had no light perception. Five of these 7 patients with paranasal sinus disease had a final visual acuity of 20/200 or better. Two patients showed dramatic visual improvement after endonasal surgery. Because immediate surgery to correct paranasal sinus disease is required in affected patients, computed tomography should be done at the patient's first visit.     

Optic nerve sheath meningiomas and advanced treatment options

PURPOSE OF REVIEW: Recent trends in the management of optic nerve sheath meningiomas have shifted towards fractionated conformal external beam radiotherapy. The purpose of this paper is to review the current literature on this subject and review the clinical characteristics and natural history of optic nerve sheath meningiomas. RECENT FINDINGS: Articles published within the last 2 years have reported the efficacy of using fractionated radiation therapy alone. A total of 62 patients in three studies have been reported; 40.3% retained stable visual acuity, 41.2% percent had improvement in their vision, and 18.4% percent had a decrease in visual acuity. Median follow-up time ranged from 22 to 51 months in these studies. SUMMARY: Optic nerve sheath meningiomas may be safely managed with fractionated external beam radiotherapy in select patients with possible improvement or stabilization of visual acuity. This represents an enormous improvement in a disease with typically poor visual prognosis.     

Optic nerve involvement as the initial manifestation of sarcoidosis

Sarcoidosis is a multisystem granulomatous disease of unknown cause. It may have several diverse manifestations that may be progressive yet develop slowly. Ocular disease may occur with inactive systemic disease. We describe two patients in whom optic nerve involvement was the first manifestation of sarcoidosis and clinically mimicked an optic nerve tumour. Sarcoidosis involving the optic nerve should be considered when an optic nerve tumour is suspected.     

Primary optic nerve meningioma.

Twenty-seven patients with a meningioma arising from within the optic nerve sheath have been seen during the past 12 years. Twenty-one were women, the majority aged 39--64 years. The men were younger, all except 1 being between 20 and 41 years when first seen. There were no patients younger than 20 years. Twenty-two patients had noticed a deterioration in the vision as their initial symptom. The optic nerve head was abnormal in all patients; 14 were swollen and 13 atrophic and flat. Neurofibromatosis was not associated with this condition. Treatment was essentially surgical.     

Optic nerve compression by normal carotid artery in patients with normal tension glaucoma

AIM: To determine whether compression of the optic nerve by the intracranial carotid artery (ICA) can be a causative factor of normal tension glaucoma (NTG). METHODS: The medical records of 103 eyes of 54 Japanese patients with NTG and 104 eyes of 52 age matched control patients were reviewed. The neuroradiological findings of magnetic resonance images (MRI) were evaluated to determine the relation between the optic nerve and ICA. The clinical characteristics and general medical conditions, such as diabetes and systemic hypertension, were also compared between the two groups. RESULTS: The prevalence of optic nerve compression by the ICA in patients with NTG was 49.5%, which was significantly higher than that in control group with 34.6% (p = 0.035). Bilateral compression of the optic nerve was detected in 22 patients with NTG (40.7%), and this was also significantly higher (p = 0.029) than that in the control group (11 patients, 21.2%). In the NTG group, eyes with cup/disc ratio (C/D ratio) > or =0.7 showed a higher percentage of compression (52.6%) compared with eyes with C/D ratio of <0.7 (12.5%; p = 0. 042). The presence of diabetes and hypertension did not affect the incidence of optic nerve compression by ICA significantly. CONCLUSIONS: The significantly higher percentage of NTG patients who had optic nerve compression by the ICA suggests that compression of the optic nerve by ICA may be a possible causative factor or a risk factor for optic nerve damage in some patients with NTG.     

Late manifestations of the optic nerve damage after closed head trauma

PURPOSE: A follow-up assessment of the visual system in patients who had undergone close head injury and in whom simple atrophy of the optic nerve occurred several months after the trauma. PATIENTS: Among patients treated in Department of Ophthalmology in Bia?ystok in the years 1984-1995 there were 2 women and 3 men, aged 19-61 years, who suffered from advancing simple atrophy of the optic nerve and who had undergone severe closed head trauma 3-5 months earlier. In one case the time interval between trauma and the visual sequelae amounted to 11 years. METHODS: The patients underwent a follow-up examination at 3 to 11 years after their initial treatment in the Department of Ophthalmology. Besides a conventional ophthalmologic examination, a static perimetry was performed as well as ultrasonography in projection B and Colour-Coded Doppler sonography (TCCD) of the orbital vessels. RESULTS: When compared to the findings at discharge, in 2 patients visual acuity improved to 5/12, in the remaining 3 no improvement was noted. In all patients the optic discs were white, while other structures of the globe appeared within normal limits. Blood flow in the central retinal artery, ophthalmic artery and in the long posterior cilliary arteries remained undisturbed, as assessed with TCCD. CONCLUSIONS: 1. Lack of visual disturbances immediately after head injury does not preclude their development in the protracted period after the trauma. 2. The reason of the late development of visual sequelae after head trauma is not clear. Normal flow, found in the major vessels of the globe, might indirectly suggest disturbances of microcirculation within the optic nerve with a consequent optic atrophy.     

Topographical analysis of the optic nerve in migraine patients.

PURPOSE: Vascular dysfunction appears related to the development of migraines and has been associated with pressure-independent glaucoma. The purpose of this study is to investigate possible topographical differences in the optic nerve between migraine sufferers and normal age-matched control subjects. The identification of optic disc topographical differences between migraine and non-migraine sufferers may help clinicians determine if the presence of migraine influences the development and progression of glaucoma. METHODS: Sixty consecutively seen patient-volunteers (30 subjects in migraine group: mean age 37.7 years, range from 19 to 66 years, 28 females and 2 males; 30 subjects in the control group: mean age 37.6 years, range from 19 to 61 years, 29 females and 1 male) were categorized as either migraine patients or control group subjects. All migraine subjects had been medically diagnosed with a migraine syndrome and were being treated with prophylactic medication. Optic nerve head topographical analysis was performed using a confocal scanning laser ophthalmoscope. The data was evaluated using generalized estimating equations, a priori and post hoc power analysis. RESULTS: No significant differences were found in any of the optic nerve parameters examined between the two groups. CONCLUSIONS: This study indicates that the optic nerve parameters as measured by the Heidelberg retinal tomograph II do not differ between migraine sufferers and age-matched control subjects.     

Aniridia and optic nerve hypoplasia

BACKGROUND: This study evaluates, in patients with aniridia, the prevalence of optic nerve hypoplasia and its association with foveal hypoplasia. METHODS: The medical records of 56 patients with aniridia (31 female, 25 male, mean age 33 years, range 2-74 years) were retrospectively evaluated for optic nerve and foveal hypoplasia. The difference in prevalence of foveal hypoplasia in patients with and without optic nerve hypoplasia was compared using Fisher's exact test. RESULTS: Six of 56 patients, 10.7% (95% CI: 4.8-21.5%), had optic nerve hypoplasia; hypoplasia was found in both eyes of five binocular patients and in one monocular patient. The prevalence of foveal hypoplasia was higher in aniridia patients with optic nerve hypoplasia than in those without (50.0 vs 6.0%); this difference did not achieve statistical significance (P=0.10). CONCLUSIONS: Clinically apparent optic nerve hypoplasia is found in roughly 10% of patients with aniridia and may occur independently or in association with foveal hypoplasia.     

Optic nerve sheath decompression for progressive central retinal vein occlusion

OBJECTIVE: To review our results with optic nerve sheath decompression (ONSD) for progressive central retinal vein occlusions (CRVO). METHODS: Patients selected all had evidence of progressively worsening CRVO, a component of optic nerve swelling, and most were already monocular from prior disease in the contralateral eye. ONSD was performed using a nasal approach under retrobulbar anesthesia. RESULTS: Eight eyes from 8 patients with a mean follow-up of 12 months were analyzed. Six patients improved, 2 worsened. Mean preoperative visual acuity was 20/160, and mean final postoperative visual acuity was 20/70. No complications occurred. Nonischemic CRVOs, patients < 65 years old, and those undergoing ONSD within 3 months of presentation seemed to do better. CONCLUSION: ONSD may improve vision or stabilize visual loss in patients with progressive CRVO.     

Clinical presentation of retinoblastoma in Eastern Nepal

BACKGROUND: The purpose of the present study was to determine modes of presentation of retinoblastoma and the association between proptosis with orbital extension and histopathologically detectable optic nerve infiltration by the tumour. METHODS: Clinical records and histopathological reports of patients with retinoblastoma were studied retrospectively. The work was undertaken at a tertiary care hospital in eastern Nepal during the period from September 1995 to August 2002. RESULTS: A total of 43 patients with retinoblastoma were studied. Among them unilateral involvement was found in 39 patients (90.70%) and bilateral involvement in four patients (9.30%). The mean age of presentation was 3.04 +/- 1.80 years (95% confidence interval = 2.49-3.59). The commonest mode of presentation was proptosis with orbital extension in 19 eyes (40.42%) followed by leucokoria in 14 eyes (29.78%). Histopathological reports of 42 eyes (one patient died shortly after admission and did not have an autopsy) confirmed the diagnosis of retinoblastoma and revealed that the optic nerve cut section had been infiltrated in 18, of which 16 had the clinical finding of proptosis with orbital extension. This was statistically significant (relative risk = 21.33, P < 0.001). CONCLUSION: The commonest mode of presentation of retinoblastoma in Nepal is proptosis. Presence of proptosis with orbital extension is associated with optic nerve cut end infiltration by the tumour. The diagnosis or presentation of retinoblastoma in Nepal is delayed and there is tremendous scope for improvement in its management.     

Age-related changes in the human optic nerve

BACKGROUND: Recent morphologic research has demonstrated the presence of nerve fibres of different diameters in the human optic nerve. The purpose of this study was to investigate age-related changes in fibres of the human optic nerve. METHODS: We studied the left optic nerve of 50 male cadaveric donors, 16 aged 18 to 22 years (mean 20 [standard deviation 1.2] years) and 34 aged 68 to 76 years (mean 72 [standard deviation 1.6] years). The samples were carefully harvested during autopsy from the intracranial portion of the optic nerve. Each nerve was cut into four 4-mm segments. After morphologic, histochemical and immunohistochemical staining, the optic nerve fibres were counted and measured. Each segment was evaluated under light microscopy for microanatomic details, glial cells and glial fibrillary acidic protein (GFAP) staining. The protein content was determined under biochemical analysis. We performed morphometric analysis by examining the optic nerve images quantitatively. RESULTS: Compared with the younger group, in the older group there was an increase in mean diameter of the optic nerve (p < 0.001), due to an increase in the optic nerve:meningeal membrane ratio. There was also an increase in mean optic nerve area (p < 0.001) and in mean number of astrocytes and the related GFAP-immunoreactive area (p < 0.001). The mean number of nerve fibres of large diameter (greater than 4 pm) was decreased (p < 0.001). There was no difference in mean protein content of the fibres between the two groups. INTERPRETATION: The human optic nerve is sensitive to the aging process and may be considered as a model for studies on neuronal aging.     

Autoantibodies in patients with glaucoma: a comparison of IgG serum antibodies against retinal,optic nerve,and optic nerve head antigens

Purpose The aim of this study was to analyze and compare the entire IgG autoantibody patterns against different ocular antigens (retina, optic nerve, and optic nerve head) in sera of glaucoma patients and healthy subjects.Methods Sixty-six patients were included in this study: healthy volunteers without any ocular disorders (CO, n=30), patients with primary open-angle glaucoma (POAG, n=19), and patients with normal-tension glaucoma (NTG, n=17). The sera were tested for antibodies against retinal, optic nerve, and optic nerve head tissues. Immunodetection was performed using 4-chloro-1-naphthol staining. The autoantibody patterns were digitized and subsequently analyzed by multivariate statistical techniques.Results All patients showed a complex repertoire of IgG antibodies against retinal, optic nerve, and optic nerve head antigens. The analysis of discriminance revealed a statistically significant differences between the patterns of all three groups. Our multivariate approach could quantify the differences in immunoreactivities of patient sera against the three antigens. The POAG group had the most significant difference against retinal antigens (P=0.0021) compared with the other antigens. In the NTG group the highest reactivity appeared against optic nerve head (P=0.00053) and optic nerve (P=0.0025).Conclusions All groups showed different and complex antibody patterns against the three ocular tissues. These autoantibodies are highly specific for each patient group. The analysis of these patterns could provide further information about possible autoimmune mechanisms in the pathogenesis of glaucoma.     

Serum methanol levels in subjects with or without optic nerve head disease

We evaluated serum methanol levels in subjects with or without optic nerve head disease. Serum methanol levels were determined using gas chromatography in 71 patients with optic nerve head disease and in 127 subjects without optic nerve head disease. Their ages ranged from 17 to 89 years. Serum methanol levels in 127 subjects without optic nerve head disease ranged from 0.12 to 3.86 microg/ml (mean +/- standard deviation, 1.72 +/- 0.86 microg/ml). In the subjects without optic nerve head disease, the differences in the levels between those with cataract versus retinal detachment, men versus women, and between each age-group (50-80 years) were not significant. The methanol levels in patients with optic neuritis (n = 2), Wolfram syndrome (n = 1), Leber hereditary optic neuropathy at the late stage (n = 2), retinitis pigmentosa (n = 23), and primary open-angle glaucoma (n = 16) were less than 3.86 microg/ml. Methanol levels in 1 patient with Leber hereditary optic neuropathy at the acute stage was 5.28 microg/ml. Of 10 patients with primary angle-closure glaucoma, 1 had a slightly elevated level and 9 had levels less than 3.86 microg/ml. Of 17 patients with normal tension glaucoma, 5 had methanol levels higher than 3.86 microg/ml, and 12 patients had levels less than 3.86 microg/ml. The present study shows that serum methanol levels in subjects without optic nerve head disease ranged from 0.12 to 3.86 microg/ml and were much lower than the levels that produce acute ocular symptoms of methanol intoxication. It is possible that high serum methanol levels may play a part in the acute stage of Leber hereditary optic neuropathy and normal tension glaucoma in certain patients. It is unlikely that increased serum methanol levels participate in primary angle-closure glaucoma.