Idiopathic inflammatory perioptic neuritis simulating optic nerve sheath meningioma

Between 1976 and 1982, we examined 13 patients with clinical and radiographic findings consistent with optic nerve sheath meningioma. All patients had visual loss, evidence of optic nerve dysfunction, centrocecal visual field defects, and echographic and computed tomographic evidence of optic nerve or sheath enlargement. All underwent surgery for biopsy or excision of their presumed tumors. However, adequate histologic sampling of all lesions failed to demonstrate meningioma in four patients; two of these four showed inflammatory infiltration of the dural sheath, whereas the other two showed only edematous or dense fibrous tissue. There was no evidence of other systemic disease in any of these cases. We conclude that optic nerve or sheath enlargement, probably induced by an idiopathic inflammatory perioptic neuritis, may simulate a sheath meningioma. Proper diagnosis requires biopsy confirmation.     

Disk edema and cranial MRI optic nerve enhancement: how long is too long?

A 43-year-old woman presented with painful visual loss and optic disk edema in the right eye (OD) diagnosed as optic neuritis. Initial non-gadolinium-enhanced fat suppressed cranial magnetic resonance imaging (MRI) was normal. Three months later, the disk edema persisted and a gadolinium-enhanced MRI scan of the brain and orbits with fat suppression showed enhancement of the optic nerve OD, most consistent with an optic nerve sheath meningioma. The diagnostic difference between optic neuritis and optic nerve sheath meningioma is discussed.     

Bilateral idiopathic inflammation of the optic nerve sheaths. Light and electron microscopic findings

Idiopathic perioptic neuritis is a term used to describe noninfectious inflammatory disorders of the optic nerve sheaths, the causes of which are unknown. In the following report, a 68-year-old woman with bilateral visual loss was found to have chronic inflammation with necrobiotic granulomas of her optic nerve sheaths. The patient, who had no systemic condition known to be associated with necrobiotic granuloma, lost vision from infarction of the optic nerve parenchyma and from compression due to thickened meninges. Although there are similarities between the inflammatory reaction in this case to the necrobiotic dermatoses, the pathogenesis of this condition remains obscure.     

Aneurysm mimicking intracranial growth of optic nerve sheath meningioma

A patient with a 30-year history of blindness in the right eye developed progressive temporal visual loss in the left eye. Examination showed right optic atrophy with optociliary shunts and left band atrophy. These clinical findings suggested that the visual deficit was caused by a right optic nerve sheath meningioma that had grown intracranially to involve the chiasm. Magnetic resonance imaging and surgical exploration revealed a perioptic meningioma extending from the orbit through the optic canal and over the tuberculum sellae. The tumor did not impinge on the optic chiasm or the left optic nerve. The chiasm was compressed by a thrombosed giant right internal carotid artery aneurysm.     

Idiopathic Inflammatory Neuroretinitis Simulating Optic Nerve Sheath Dural Ectasia

Abstract

We report a case of a 32-year-old female patient who presented with decreased vision on both eyes and headache. In fundus examination, both eyes had elevation of the optic disc and star shaped hard exudates in the macula. Magnetic resonance imaging was completely normal except the saccular dilatation of bilateral optic nerve sheath. The patient was treated with oral steroids following high-dose intravenous methylprednisolone. She displayed good anatomical and functional results during the follow-up. This case raises the possibility that optic nerve sheath enlargement, probably induced by an idiopathic inflammatory optic neuritis, may simulate dural ectasia of the optic nerve sheath.     

Atypical features prompting neuroimaging in acute optic neuropathy in adults

BACKGROUND: Acute optic neuropathy due to an intracranial lesion may masquerade as optic neuritis or nonarteritic anterior ischemic optic neuropathy (NAION). We reviewed the records of patients who presented with acute unilateral optic neuropathy that was initially diagnosed as optic neuritis or NAION but who ultimately proved to have an underlying structural lesion. METHODS: Retrospective observational case series. We reviewed the records of patients with the initial diagnosis of optic neuritis or NAION in whom the diagnosis was changed to an intracranial etiology at four tertiary care neuro-ophthalmology centres between 1995 and 1998. RESULTS: Eight cases were identified in which atypical features prompted further investigation, including neuroimaging, leading to the diagnosis of an intracranial etiology for the optic neuropathy. Five patients were discovered to have neoplasms (a tuberculum sellae meningioma in two cases, an optic nerve sheath meningioma in two cases and a metastatic lesion in one case), and three patients had intracranial sarcoidosis. Atypical features for optic neuritis included a progressive course, absence of pain, optic atrophy at presentation, lack of significant visual improvement and age over 40 years. For NAION, the atypical features included progressive course, optic atrophy on presentation, absence of vasculopathic risk factors and preceding transient visual loss. INTERPRETATION: Clinicians should be aware that patients with intracranial lesions may present with acute optic neuropathy mimicking optic neuritis or NAION and that certain atypical features should warrant consideration for neuroimaging.     

Visusabfall bei seltenem Sehnerventumor

A 49-year-old male patient presented with acute symptoms of reduced vision and relative afferent pupillary defects. Static perimetry testing revealed severe visual field defects and the diagnosis of neuritis of the optic nerve was suspected. A magnetic resonance imaging (MRI) scan was performed and showed an intraocular right-sided semicircular mass around the optic nerve which was partially calcified in computer tomography (CT). The diagnosis of an optic nerve sheath meningioma was made. The visual acuity and visual field defects improved under therapy with prednisolone. Therapeutic options were discussed in an interdisciplinary board but no intervention is planned so far and only regular follow-up controls have been arranged.     

Orbital color Doppler imaging of optic nerve tumors

Objective: To report changes in retinal arterial and venous blood flow pattern in two patients with tumors involving the entire optic nerve. Methods: Retrospective review of one patient with clinical and neuroimaging characteristics typical of bilateral optic nerve gliomas and one patient with a probable meningioma of the left optic nerve sheath. Results: The optic nerve glioma patient had reduced peak systolic velocity of central retinal arteries bilaterally, while the patient with an optic nerve sheath meningioma had relatively low central retinal artery flow velocity and intermittent blood flow in the central retinal vein on the affected side. Conclusions: Reduced retinal arterial flowvelocities in the setting of optic nerve gliomas may correlate with thepresence of optic nerve disease. Phasic blood flow in the central retinalvein with optic nerve sheath meningioma may be the reason that somepatients with this tumor develop retinal choroidal venous anastomoses.     

视神经炎的疾病分类学研究进展

视神经炎指发生于视神经的一切炎性病变,是神经眼科最常见的视神经疾病之一,也是中青年人最易罹患的视神经疾病.目前,国内外对视神经炎的分类方法主要有两种,一种按发病部位分类,可分为球后视神经炎、视乳头炎、视神经周围炎和视神经视网膜炎;另一种根据病因学分类,可分为中枢神经系统炎性脱髓鞘性疾病相关视神经炎、感染性疾病相关性视神经炎及全身系统性自身免疫性疾病相关性视神经炎.目前,我国的视神经炎病因学特点尚有待进一步开展大样本的流行病学研究.     

Evaluation of the Optic Nerve Complex in the Orbit Using Coronal Fast Magnetic Resonance Imaging

Recently available coronal fast magnetic resonance imaging (MRI) has very high spatial resolution with good contrast between the optic nerves and cerebrospinal fluid (CSF). The aim of this study was to evaluate the diagnostic value of coronal fast imaging in optic nerve diseases. Thirty-five patients with various Neuro-ophthalmic conditions including 9 with optic neuritis, 6 with optic atrophy, 5 with glaucoma, 4 with segmental optic nerve hypoplasia and 11 with other optic neuropathies including orbital apex syndrome were evaluated with the three-dimensional fast imaging employing steady-state acquisition (FIESTA) sequence in addition to standard MRI protocols. The optic nerve complexes were evaluated on coronal images of the orbits. Detailed demonstration of the optic nerve complex—the optic nerve, the perineural CSF space and dural sheath—could be readily obtained with FIESTA sequence. The acute phase of both optic neuritis and perineuritis showed enlargement of the perineural CSF space; the optic nerve was swollen in optic neuritis but not in perineuritis. Cases of optic atrophy and glaucoma showed perineural CSF space enlargement with normal optic sheath circumference and a thinner optic nerve, while optic nerve hypoplasia showed a smaller dural sheath circumference without perineural CSF space enlargement. In the cases of orbital apex syndrome optic nerve compression by the extraocular muscles was clearly shown. Coronal FIESTA imaging of the orbit is capable of delineating detailed structural changes in the optic nerve complex and is of diagnostic value for the differentiation of optic nerve diseases.     

Optic nerve sheath meningioma: visual improvement during radiation treatment

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.     

Arachnoid hyperplasia in optic nerve glioma: confusion with orbital meningioma.

A case is reported of an optic nerve glioma with a marked degree of arachnoid hyperplasia which was initially diagnosed as an optic nerve meningioma. Hyperplasia of the arachnoid was also the underlying cause for expansion of the optic canal. The relationship between arachnoid hyperplasia in optic nerve glioma and meningioma of the optic nerve sheath in childhood is discussed.     

眶尖部及视神经肿瘤的早期诊断

目的探讨眶尖部及视神经肿瘤在眼球突出前的临床表现、影像学检查特征及临床诊断要点。方法回顾性分析22例无明显眼球突出单侧眼眶肿瘤患者的病历资料,包括主要症状、首发表现、临床诊治过程及影像学检查结果。结果22例肿瘤患者中,海绵状血管瘤6例,神经鞘瘤、视神经鞘脑膜瘤、鼻窦及鼻咽腔恶性肿瘤眶内蔓延各4例,后组筛窦黏液囊肿2例,神经纤维瘤和视神经胶质瘤各1例。以视力下降为首发症状者17例,视力下降合并复视者3例。全部患者均在CT或MRI检查后确诊。肿瘤位于眶尖部或起源于视神经。结论眶尖部及视神经肿瘤的早期症状是视功能障碍,无眼球突出表现。视力呈缓慢进行性下降,按视神经炎治疗效果差。CT或MRI检查对早期诊断起重要作用。     

Role for surgery as adjuvant therapy in optic nerve sheath meningioma

PURPOSE: To describe a role for optic nerve decompression as adjuvant surgical therapy in the management of optic nerve sheath meningioma in patients with severe, progressive visual loss and optic disc edema before or after radiation therapy. METHODS: Interventional case report. RESULTS: Two patients with unilateral optic nerve sheath meningioma had progressive visual loss (20/200 and no light perception) and disc edema. The first had previously undergone fractionated stereotactic radiotherapy and the second subsequently was treated with fractionated stereotactic radiotherapy after decompression surgery. After excision of a dural window and biopsy of the tumor from the nerve sheath, visual acuity improved to 20/25 and 20/200, respectively, both coinciding with resolution of disc edema. CONCLUSIONS: In patients with optic nerve sheath meningioma with severe disc edema and rapid vision loss, surgery may serve an important but restricted, adjuvant role to radiation therapy under special conditions.     

Caught by a masquerade: sclerosing orbital inflammation.

Idiopathic sclerosing inflammation of the orbit is a distinct form of orbital inflammatory disease characterized by slow and relentless involvement of orbital structures. It is this insidious and relentless course that makes distinction from neoplastic lesions clinically difficult. We report the case of a patient with a several-week history of headache and decreased vision that was originally thought to represent an optic nerve sheath meningioma, based on clinical and radiographic evaluation. Subsequent histopathology from an optic nerve biopsy, however, was more consistent with optic nerve glioma. Eventually, pathologic examination of whole sections through the optic nerve was required to establish and confirm the actual diagnosis of sclerosing orbital inflammation.     

Primary Optic Nerve Sheath Meningioma in Children

Primary optic nerve sheath meningioma represents a proliferation of meningothelial cap cells of the arachnoid villi within the optic nerve sheath. Patients younger than 20 years of age make up less than 5% of all cases of pediatric primary optic nerve meningiomas. Histopathologically, the most common subtypes in children are transitional (54%) and meningotheliomatous (38%). This tumor has been called aggressive in the pediatric population, with surgical excision recommended. However, the tumor may spread intraorbitally, intracranially, or intraocularly after subtotal surgical resection. Recent studies examined the use of fractionated, stereotactic radiation in children with this tumor; however, follow-up is limited. Neurofibromatosis type 2 is concomitantly diagnosed in 28% of patients with pediatric primary optic nerve sheath meningioma. There have been no known deaths attributed primarily to this tumor.     

Treatment of optic nerve sheath meningioma with three-dimensional conformal radiation

PURPOSE: To report a patient recovering vision after three-dimensional conformal radiotherapy for optic nerve sheath meningioma. METHODS: Radiotherapy was delivered by a three-dimensional conformal technique in 28-180-cGy fractions. RESULTS: Visual acuity improved from 20/200 to 20/30, and the visual field defect resolved. CONCLUSION: Visual loss from optic nerve sheath meningioma can be reversed by three-dimensional conformal radiotherapy.     

Meningioma of the optic nerve sheath. Diagnostic problems despite use of modern imaging procedures]

In spite of modern optical examination techniques such as sonography, nuclear magnetic resonance tomography (NMR) and x-ray computer tomography up to now the meningioma of the optic nerve sheath still poses a problem in respect of differential diagnosis. This problem is illustrated by two case reports. In both patients a meningioma of the optic nerve sheath could be diagnosed only after various attempts using all the imaging techniques mentioned above. NMR in combination with Gadolinium injection gave most important information.     

Involution of retinochoroidal shunt vessel after radiotherapy for optic nerve sheath meningioma

PURPOSE: To illustrate the development and involution of retinochoroidal shunt vessel of the optic disc in a patient with primary optic nerve sheath meningioma. CASE REPORT: A 38-year-old woman presented with gradual onset of blurred vision in her right eye. Examination revealed a right relative afferent pupillary defect and mild edema of the right optic disc. Computed tomography (CT) showed changes consistent with a primary optic nerve sheath meningioma affecting the orbital portion of the right optic nerve. Patient was observed without treatment and subsequently developed a retinochoroidal shunt vessel on her right optic disc. With progression of the meningioma towards the optic chiasm, treatment with stereotactic radiosurgery was done. Following treatment, the optic disc gradually became pale and the retinochoroidal shunt vessel decreased in caliber with complete involution three years after radiotherapy.     

Neoplasm of mixed mesenchymal and neuroepithelial origin of the optic nerve.

A case is reported of a 19-year-old male having right proptosis for 4 years because of a mixed meningioma and astrocytoma of the ipsilateral optic nerve. The sheath of this nerve is analogous to the leptomeninges, and neuroglial cells constitute the stroma of the nerve. Both meningioma and astrocytoma therefore can arise primarily in the optic nerve, and they may be combined in the same mass, forming a neoplasm of mixed mesenchymal and neuroepithelial origin.