Unusual glioma of the optic nerve.

A 3 1/2-year-old white girl presented with unilateral proptosis and an orbital tumor that was diagnosed histopathologically as an unusual form of glioma of the optic nerve. The optic foramen was not enlarged but the ultrasonogram indicated a definite retrobulbar mass.     

A case of amblyopia with contralateral optic nerve hypoplasia

We describe an unusual case of unilateral optic nerve hypoplasia (ONH) in a patient with contralateral anisometropic/strabismic amblyopia. A seven‐year‐old boy presented with visual acuities of 6/12 R and 6/18 L and eccentric fixation in the left eye. Cycloplegic retinoscopy was R +1.50/‐0.50 × 180 and L +5.25 DS. Funduscopy revealed optic nerve hypoplasia of the right eye. The patient fixated with his better‐seeing right eye, despite the optic nerve hypoplasia. His reduced vision may be attributed to optic nerve hypoplasia in the right eye and amblyopia in the left. Although optic nerve hypoplasia can occur with ipsilateral amblyopia, we believe this is the first reported case of unilateral optic nerve hypoplasia in the fellow eye of an amblyopic patient.     

Bilateral optic disk edema caused by sarcoidosis mimicking pseudotumor cerebri

PURPOSE: To present a case of retrobulbar optic nerve and chiasm sarcoidosis that mimicked pseudotumor cerebri. METHODS: A 34-year-old, thin, black woman presented with transient visual obscurations, normal visual acuity, bilateral optic disk edema, and enlarged blind spots. Clinical, medical, and radiologic evaluations were consistent with pseudotumor cerebri. The patient improved while taking acetazolamide, but 6 months later her symptoms worsened. Neuroimaging disclosed enhancement of the optic nerve and chiasm. RESULTS: Despite administration of intravenous corticosteroids, the patient's vision worsened. Bilateral optic nerve sheath fenestrations were performed, and pathology disclosed sarcoidosis. CONCLUSION: Sarcoidosis of the optic nerves and chiasm may mimic pseudotumor cerebri.     

Optic nerve cyst associated with optic disk pits

Purpose To report a case with two optic disk pits which were associated with an optic nerve cyst in the same eye.Methods Observational case report.Results A 47-year-old patient noted visual impairment in the right eye. On examination the best corrected visual acuity in the right eye was 20/80 and in the left eye was 20/20. Biomicroscopy revealed, in the right eye, a very pale optic disk with two optic disk pits without macular elevation. Magnetic resonance imaging (MRI) revealed a well circumscribed 6×6-mm² round cystic lesion within the right optic nerve sheath adjacent to the temporal aspect of the right optic nerve at its retrobulbar segment, which compressed and displaced the nerve.Conclusions In the case of an extremely pale optic disk with congenital pits and visual impairment without macular detachment, radiological examination is indicated in order to exclude the possibility of coexisting optic nerve anomalies.     

Optic nerve glioma in Japanese patients with neurofibromatosis 1. Case reports and literature review.

Case 1, a 6-year-old boy, had multiple café-au-lait spots, no light perception OS, Lisch nodules OU, pale optic disc OS, enlarged left optic nerve on computed tomographic scan and magnetic resonance imaging, and histopathologically verified pilocytic astrocytoma (glioma). The patient's mother had neurofibromatosis 1 (NF-1). Case 2, a 12-year-old boy, had multiple café-au-lait spots, decreased visual acuity OU, Lisch nodules OU, pale optic discs OU, enlarged optic nerves and chiasm on computed tomographic scan, and histopathologically verified pilocytic astrocytoma. We also examined 38 Japanese patients with NF-1 and found Lisch nodules in 95% and optic nerve glioma in 5%. After reviewing the recent literature, we found that the association of optic nerve glioma and NF-1 in the Japanese population was less than that reported in North America.     

Atypical anterior optic neuropathy caused by toxoplasmosis.

PURPOSE: To report atypical anterior optic neuropathy due to toxoplasmosis. METHODS: Interventional case report. A 33-year-old male presented with sudden painless loss of vision and floaters in the right eye. Examination demonstrated a best-corrected visual acuity of 20/200, optic nerve head edema, retinal hemorrhages, and vitreous opacities. RESULTS: Nine days later, a granuloma at the optic nerve head was apparent, and the patient was treated with pyrimethamine, sulfadiazine, folinic acid, and prednisone. Six weeks after initiating therapy, best-corrected visual acuity had improved to 20/25. CONCLUSION: Optic nerve involvement in toxoplasmosis is uncommon and, when it occurs, usually presents with a white inflammatory mass on the optic disk. The current case demonstrates the importance of including toxoplasmosis in the differential diagnosis of unilateral anterior optic neuropathy, even if a focal inflammatory mass is not apparent.     

Bilateral visual field defects with optic disc drusen and secondary open angle glaucoma with PEX--clinical correlation with the HRA

BACKGROUND: Arcuate visual field defects are a typical sign of glaucomatous damage. Elevated intraocular pressure in combination with pseudoexfoliation syndrome (PSX) manifests the diagnosis glaucoma. Beyond this state, in microdiscs with optic disc drusen, the exact classification of the visual field defects is crucial. CASE REPORT: A 57-year-old male with pseudoexfoliation glaucoma was referred because of progressive glaucomatous visual field defects. The visual acuity was right 20/40 and left 20/25. Maximum intraocular pressure was 36 mm Hg. A simple optic nerve atrophy was diagnosed superonasally. The optic disc size was OD 2.24 mm(2) and OS 1.89 mm(2) (HRT I). An Ultrasound B-mode scan demonstrated the diagnosis of optic disc drusen. Over a follow-up of 1 year, a growth tendency was observed, especially in the superonasal quadrant. The mulberry-shaped surface of the drusen was visualized with infrared reflection images (HRA II, 830 nm). Confocal scanning laser ophthalmoscopy (HRA II, excitation 488 nm, 500 nm notch filter) showed an increased intrapapapillary autofluorescence (> 50 % papillary area: OD 1.67 mm(2), OS 1.26 mm(2)). This technique could detect drusen in areas that looked normal in classical retinoscopy. CONCLUSION: The differential diagnosis of arcuate scotomas includes simple optic nerve atrophy and glaucomatous optic nerve atrophy. Optic disc drusen in glaucoma eyes can obscure the main cause of progressive visual field loss. Superficial optic disc drusen can be measured planimetrically over the years. An adequate reduction of intraocular pressure should be realized in these eyes.     

Spheno-orbital meningioma with optociliary veins.

A 40-year-old white woman had slowly progressive unilateral loss of visual acuity and increasing proptosis during an eight-year period. Ophthalmoscopy and fluorescein angiography revealed chronic disk edema and optociliary shunt vessels in the right eye. Polytomography showed an enlarged right optic canal. Cerebral arteriography demonstrated a dumbbell-shaped tumor blush in the right juxtasellar region and a diffuse tumor blush in the right orbit. Right frontal craniotomy and orbital exploration showed a cranio-orbital junction (spheno-orbital) meningioma that invaded the sclera and peripapillary choroid. Anomalous optociliary veins were demonstrated histologically at the optic disk.     

Atypical visual prognosis with an optic nerve glioma

A 13-year-old girl presented with a 1-year history of gradual decline of vision in her right eye. She was diagnosed by clinical examination and computed tomographic scan as having a right optic nerve glioma. While awaiting surgery, she experienced spontaneous improvement of her symptoms. Four years after the onset of symptoms, the Snellen acuity, color testing, and visual fields have returned to normal, while the computed tomographic appearance of the tumor is essentially unchanged. Possible mechanisms for such a fortuitous outcome are discussed.     

Optic disk vasculitis associated with chronic active Epstein-Barr virus infection

PURPOSE: To report two cases of optic disk vasculitis associated with chronic active Epstein-Barr virus infection (CAEBV). METHOD: We examined the eyes of two patients with CAEBV. RESULTS: In the first case, a 6-year-old boy, visual acuity was 20/20 in both eyes. Papillary and peripapillary exudates were observed in the right eye. Fluorescein angiography showed hyperfluorescence of the optic disk and a leakage from the peripapillary retinal vessels in the right eye. Two months later, the exudates increased and preretinal hemorrhages appeared in the right eye. Visual acuity decreased to 20/60. He was treated with systemic administration of corticosteroid, globulin and acyclovir. Visual acuity returned to 20/20, but peripapillary exudates remained in the right eye. In the second case, a 16-year-old girl, visual acuity was 20/20 in both eyes. The right eye showed optic disk swelling and dilated retinal veins. Fluorescein angiography showed hyperfluorescence of the optic disk but no leakage from the retinal vessels. Visual field examination revealed an enlarged blind spot in the right eye in both cases. These ocular manifestations are compatible with those of optic disk vasculitis, which shows swelling of the optic disk and dye leakage on and around the optic disk in fluorescein angiography, with an almost normal visual acuity and an enlargement of the blind spot. CONCLUSION: Persistent Epstein-Barr virus infection may cause optic disk vasculitis.     

Isolated optic nerve lymphoma diagnosed by optic nerve biopsy

PURPOSE: To report a case of isolated optic nerve lymphoma diagnosed by optic nerve biopsy. DESIGN: Case report. METHODS: A 66-year-old woman was referred to the Neuro-Ophthalmology Service because of a decrease in visual acuity and right optic disk edema. RESULTS: A magnetic resonance image of the brain showed only enhancement of the optic nerve. An examination that included ANA, c-ANCA, p-ANCA, Lyme titers, FTA-ABS, ACE level, chest x-ray, and lumbar puncture was negative. Because of rapid progression on clinical examination and serial imaging, an optic nerve biopsy was performed, which showed B-cell lymphoma. CONCLUSION: Optic nerve lymphoma can be confused with a variety of inflammatory and neoplastic infiltrations of the optic nerve on clinical and radiographic examinations. Optic nerve biopsy can be valuable in diagnosing isolated optic nerve lymphoma if other diagnostic tests are unrevealing, but the procedure carries considerable risk of loss of visual acuity and should be recommended judiciously.     

An optic nerve tumor in von Hippel-Lindau disease, masquerading as a retinal hemangioma

CLINICAL CASE: A 35-year-old man with a family history of von Hippel-Lindau disease was diagnosed to have two retinal hemangiomas in the right eye and another in the left eye. The hemangiomas were treated with cryotherapy and laser photocoagulation respectively. Despite apparent good resolution of the retinal lesions, progressive visual loss was observed. An MRI was then performed and showed bilateral tumoral lesions of the optic nerve compatible with a glioma, meningioma or hemangioblastoma. Currently the visual acuity in his right eye is hand movement, and is 0.6 in the left eye. DISCUSSION: The presence of the retinal hemangiomas delayed the diagnosis of an optic nerve tumor in this patient.     

Malignant transformation of an optic disk melanocytoma

PURPOSE: To report a case of malignant transformation of an optic disk melanocytoma with a second melanocytoma in the ciliary body. METHODS: Clinical data including visual acuity, visual fields, color fundus photographs, fluorescein angiogram, and ultrasonogram and histopathologic studies of this case were reviewed. RESULTS: The right eye of a 65-year-old white woman was diagnosed with melanocytoma of the optic nerve. Four years later, the tumor became significantly larger. The best-corrected visual acuity declined from 20/40 to counting fingers and the size of the tumor increased fourfold in 2 years. The right globe was enucleated. Histopathologic studies demonstrated moderately pigmented spindle-B malignant melanoma cells adjacent to and within a population of large, polyhedral, heavily pigmented melanocytoma cells that extended to the lamina cribrosa and optic nerve. There was also a deeply pigmented melanocytoma in the ciliary body. CONCLUSION: This is a rare case of malignant melanoma transformed from an optic disk melanocytoma. Periodic follow-up of the patient with optic disk melanocytoma is necessary.     

Familial exudative vitreoretinopathy.

At age 26 years a woman who had been blind in the left eye from birth had visual acuity of R.E.: 6/18 (20/60); L.E.: 6/120 (20/400), with 40 prism diopters of left exotropia. The left eye showed a decreased anteroposterior diameter of the globe and a complete retinal detachment behind a cataractous lens. The right eye had a posterior subcapsular lens opacity severe vitreous fibrosis, dragging of the optic disk, and intraretinal and subretinal exudation with a fibrovascular mass in the temporal retina. The patient's 3-year-old daughter had 45 to 50 prism diopters of exotropia, a pendular nystagmus with intraretinal and subretinal dragging of each disk, and early degeneration and band formation in the periphery of each fundus.     

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).     

A case of orbital myositis complicated with optic neuropathy--analysis of the pathological mechanism of optic neuropathy from magnetic resonance imaging findings

BACKGROUND: We observed a rare case of orbital myositis involving the optic nerve. Case: A 52-year-old woman complained of visual disturbance, lid swelling, ocular pain, and conjunctival injection in her right eye. Her corrected vision was 0.15 in the right eye and 1.2 in the left eye. Relative afferent pupillary defect(RAPD) and central scotoma in visual field test were noted in the right eye. Ocular movement of her right eye was moderately disturbed in all directions. Although laboratory data showed elevation of erythrocyte sedimentation rate, other hematological data such as thyroid function, autoimmune antibodies, and viral infection antibodies were normal. Magnetic resonance imaging(MRI) findings showed compression of the optic nerve at the orbital apex by marked thickening of the right lateral rectus muscle and superior rectus muscle, and inflammation directly invading the optic nerve. Based on the above findings, we diagnosed the case as orbital myositis complicated with optic neuropathy, and started corticosteroid therapy. Her right corrected vision improved dramatically, and the RAPD, central scotoma, and lid swelling disappeared shortly after administration. Enlargement of the extraocular muscles was still present one month after corticosteroid therapy. CONCLUSION: MRI findings suggested that optic neuropathy in this case was induced not only by mechanical compression by the enlarged extraocular muscles at the orbital apex but also by direct inflammatory infiltration from the extraocular muscles.     

Optic nerve sheath meningioma with intraocular invasion--a case report]

BACKGROUND: In case a sonographically high reflective intraocular tumor is detected, some different processes with calcium embedding could be considered. PATIENT: A 56 year-old female patient was seen in our department for the first time because of secondary glaucoma of the amaurotic right eye. The left eye has had no morphological changes and visual acuity was 5/5. CASE HISTORY: More than 20 years ago the patient had noticed decreased visual acuity of the right eye for the first time. 10 years ago in a first report from another eye hospital, a large white tumor in the area of the optic disc had been described. Sonographic size was 12 x 8 mm, 5.3 mm prominence. Visual acuity was perception of light with incorrect projection. A CT scan showed an inhomgeneous structure of calcification in the area of the right optic disc which continued along the optic nerve to intracranial structures. Enucleation was performed. Histopathological examination showed meningothelial meningeoma (grade I WHO) with its origin in the dural sheath of the optic nerve and intraocular growth. A postoperative MRI confirmed the intracranial spreading of the meningeoma along the dural sheath of the optical nerve. A meningeoma of the olfactorius region was found as a second independent tumor. CONCLUSION: The intraocular spread of an optical nerve sheath meningeoma is a rare case.     

Resolution of optic nerve edema and improved visual function after optic nerve sheath fenestration in a patient with osteopetrosis

PURPOSE: To describe the outcome of a patient with visual loss and optic nerve edema that resulted from osteopetrosis who underwent an optic nerve sheath fenestration (ONSF). DESIGN: Interventional case report. METHODS: A 33-year-old man with osteopetrosis had bilateral visual field loss and optic nerve edema. Computed tomography and magnetic resonance imaging demonstrated open optic canals. Although a lumbar puncture showed a normal opening pressure, there were other findings that were suggestive of increased intracranial pressure. The patient elected to undergo a unilateral optic nerve sheath fenestration. RESULTS: After ONSF, the patient experienced markedly improved visual acuity, visual field, and optic nerve appearance. CONCLUSION: Individuals with visual loss and optic disk edema that is associated with osteopetrosis may benefit from ONSF if the optic canals appear to be open and the optic nerve edema is thought to be related to increased intracranial pressure.     

High-dose intravenous immunoglobulins for treatment of optic neuritis in Guillain-Barré syndrome

BACKGROUND: Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating disease of the peripheral nervous system. Treatment strategies include systemic steroids, immune adsorption, plasmapheresis, and intravenous immunoglobulins. Optic neuritis as an affectation of the central nervous system does not belong to the normal spectrum of neurological symptoms in GBS, which is thought to be restricted to the peripheral nervous system. CASE REPORT: A 55-year-old female patient with unilateral optic neuritis secondary to GBS was referred to our department. Visual acuity was 0.04 in the affected left eye, L. E. and 1.25 in the right eye, R. E. Visual field testing revealed a large centrocecal scotoma. Ophthalmoscopy disclosed a slight oedema of the left optic disc. High-dose steroid treatment based on the diagnosis of optic neuritis secondary to GBS could not improve vision in the patient's left eye. Therefore, a repetitive treatment with high-dose intravenous immunoglobulins (IVIg) was initiated. The patient underwent three treatment cycles - 0.4 g per kg daily for 5 days - with intervals of two weeks between each cycle. Visual acuity and visual field improved gradually after the initiation of the immunoglobulin treatment. At the end of the last treatment course - 7 weeks after the begin of ocular symptoms - visual acuity had recovered to 0.8. A small residual paracentral scotoma resolved completely within the following weeks. Further follow-up examinations revealed a complete recovery of visual acuity to 1.0. Side effects of the immunoglobulin treatment were not observed throughout the treatment period. CONCLUSIONS: Based on the observation that the clinical improvement in our patient coincided with the initiation of the IVIg treatment after steroid treatment had failed, we feel justified in drawing attention to IVIg as a potential treatment option in patients with GBS and involvement of the optic nerve.     

Unilateral Papilledema in Pseudotumor Cerebri

Abstract

Purpose: To report a case of a 25-year-old girl with pseudotumor cerebri who presented with unilateral swollen optic disk. Methods: A 25-year-old obese patient admitted to our ophthalmic department complaining of headaches, tinnitus, and transient visual obscurations for the last three months. Upon ophthalmic examination, the left optic nerve was swollen with a few hemorrhages compared to the normal-appearing right optic nerve. Following lumbar puncture with opening pressure of 350?mmHg, a diagnosis of pseudotumor cerebri was made and treatment with acetazolamide was started. Results: Three months later there were no episodes of visual obscurations and headache improved. Conclusion: Although rare, unilateral swollen disk could be a sign of unilateral papilledema due to increased intracranial pressure.