Despite an initial improvement with acetazolamide, his vision subsequently worsened. With an elevated lumbar puncture opening pressure and imaging showing right optic nerve sheath enhancement, the differential diagnosis included ONSM, perineuritis and idiopathic intracranial hypertension (IIH) The primary differential considerations are optic neuritis or optic nerve vasculitis (most commonly related to infection, radiation, or autoimmune disorders). Optic neuritis is much more common and is the correct diagnosis in this case. Optic nerve tram-track sign
Optic nerve meningiomas are benign tumors arising from the arachnoid cap cells of the optic nerve sheath and represent ~20% of all orbital meningiomas, the majority of which are direct extensions from intracranial meningiomas.. These tumors typically appear as masses within the optic nerve, isointense to grey matter on both T1 and T2 weighted imaging, demonstrating vivid enhancement which. The classic clinical signs of optic neuropathy are visual field defect, dyschromatopsia, and abnormal papillary response. There are ancillary investigations that can support the diagnosis of optic neuropathy. Visual field testing by either manual kinetic or automated static perimetry is critical in the diagnosis The diagnosis of OPN was made in patients who had an acute optic neuropathy and/or disc edema plus either radiographic demonstration of enhancement of the optic nerve sheath or histopathologic evidence of perineural inflammation
Tram-track enhancement occurs when neoplastic or inflammatory lesions involve the optic nerve sheath and spare the optic nerve. 4 The differential diagnosis for this imaging sign is broad, as many neoplastic and inflammatory conditions can involve the optic nerve sheath Differential diagnosis for macular star includes hypertensive retinopathy, papilledema, anterior ischemic optic neuropathy, diabetic papillopathy, posterior vitreous traction, disc and juxtapapillary tumors, and toxic etiologies, including bis-chloroethyl,nitrosourea and procarbazine Differential Diagnosis Optic Nerve Glioma, Metastatic disease, Leukemic infiltration, Neurosarcoidosis, Tuberculosis, Gummatous syphilis, Optic Perineuritis, Myelin Oligodendrocytic Glycoprotein (MOG Enlargement of the optic nerves is uncommon. Swelling of the optic nerves has a surprisingly broad differential
sheath,15,16,19 or to vascular occlusion due to vasculitis.16 For OPN associated with other etiologies,11-13,20 optic nerve histology was lacking. Diagnosis was made either on clinical grounds or radiologic appearance of the optic nerve, considered together with other systemic features. Epidemiology For OPN associated with syphilis and other. Optic nerve sheath inflammation is accompanied by eye pain as well as varying levels of optic nerve dysfunction (decreased vision, decreased color vision, visual field changes, and relative afferent pupillary defect). Less frequently, orbital signs (proptosis, decreased motility etc.) may be present
Mitochondrial DNA testing revealed a mutation at nucleotide position 3460 thereby confirming a diagnosis of LHON. been described. 2,3 The increased optic nerve signal may not occur until several months after the onset of visual loss. 2-4 Optic nerve sheath distention has there are no other reported cases of optic nerve enhancement among. Optic nerve sheath meningioma (ONSM) is a term applied to primary and secondary meningiomas of the optic nerve. According to a recent meta-analysis and subse- enhancement of the meninges surrounding a hypodense Radiographic Differential Diagnosis The MRI study demonstrated tram track enhancement of the left optic nerve sheath. This finding shifted the differential diagnosis from neuroretinitis to optic perineuritis or optic nerve sheath meningioma. The subsequent clinical and radiological improvement with steroids was diagnostic of optic perineuritis rather than optic nerve sheath.
Radiographically, there is optic nerve sheath enhancement and, occasionally, orbital fat streaking. Biopsies have identified dural sheath lymphocytic infiltrate, perineural fibrous tissue, granulomas, or evidence of small-vessel vasculitis. Retroperitoneal fibrosis (RPF) is defined by abdominal organ fibrosis In women with bilateral enophthalmos, metastatic scirrhous breast cancer should be considered in the differential diagnosis. Neoplasms that arise from the optic nerve or its sheath include glioma and meningioma. At imaging, gliomas often cause fusiform expansion of the optic nerve, in which the nerve itself cannot be delineated from the lesion Differential diagnosis Venous varix, schwannoma, optic nerve sheath meningioma, and lymphoma
A 46-year-old woman was admitted to our hospital because of high fever and lumbago. CT revealed increase in density of fat tissue around the aorta, suggesting retroperitoneal panniculitis. The following day, she reported of leg pain, pain in the mouth, painful urination and right eye pain that was exacerbated by eye movement. We observed erythema nodosum, aphthous stomatitis and genital. It cannot, however, be used as a substitute for clinical diagnosis. An optic nerve sheath meningioma can look exactly like optic neuritis on MRI and should be suspected if the contrast enhancement.
Two patients with abnormal optic nerve/sheath complex enhancement. A: Patient 1 had a right optic neuropathy. The study shows enhancement of the optic sheath (arrow) and surrounding retrobulbar soft tissues (arrowhead). This was steroid-responsive disease and believed to be due to pseudotumor Introduction . Leptomeningeal carcinomatosis occurs in about 5% of cancer patients. Ocular involvement is a common clinical manifestation and often the presenting clinical feature. Materials and Methods . We report the case of a 52-year old lady with optic neuritis as isolated manifestation of neoplastic meningitis and a review of ocular involvement in neoplastic meningitis Optic nerve meningioma is seen most often in middle-aged adults. Patients usually note vision loss. Because the tumor originates in the arachnoid villi of the meningeal sheath of the optic nerve (a primary orbital tumor), visual loss caused by compression of the nerve is common before the tumor is large enough to cause much proptosis. The onset. A previously healthy 27-year-old Malay male presented with acute onset of painless, severe blurring of vision in his right eye. It was associated with headache and vomiting for the past week. Relative afferent pupillary defect was present in the right eye, with reduced optic nerve function. Patient also had bilateral generalised optic disc swelling, splinter haemorrhages, and tortuous vessels
Figure 1. Optic nerve sheath diseases (cases 1 and 3). (A) and (B) demonstrate imaging findings of case 1, showing intense contrast enhancement of the optic nerve extending through the optic canal almost reaching the chiasm (B) due to neurosarcoidosis. There is an asymmetrical thickening of the optic nerve compared wit Differential considerations for ON include optic nerve glioma (marked enlargement of the nerve) and optic nerve sheath meningioma (calcification and thickened enhancement of the nerve sheath but a normal nerve) References. Khanna S, Sharma A, Huecker J, Gordon M, Naismith RT, Van Stavern GP.. Enhancement of the optic nerve on MRI may involve the nerve itself or the surrounding meninges. Optic nerve enhancement has been reported in a variety of disorders including optic neuritis ( 2,3 ), carcinomatous meningitis ( 3,4 ), idiopathic orbital inflammation ( 5 ), sarcoidosis ( 6 ), Wegener granulomatosis ( 7 ), and radiation-induced.
Introduction. Optic perineuritis (OPN) is a rare form of orbital inflammatory disease targeting the optic nerve sheath ().OPN usually presents with minor visual impairment, optic disc edema, and visual field abnormalities that include arcuate defects and peripheral island defects ().It is very important to identify etiology and clinical characteristics of patients with OPN, and it will affect. We report findings of optic nerve enhancement, optic nerve sheath enhancement, and the first description in the English-language ophthalmic literature, to our knowledge, of chiasmal enhancement in.
Optic nerve sheath meningioma (ONSM) is a term applied to primary and secondary meningioma of the optic nerve. ONSM occurs more commonly in middle aged women. Primary ONSMs account for approximately one third of primary optic nerve tumors and 5% to 10% of orbital tumors [ 1, 2 ]. Primary ONSM represents a neoplasia of meningothelial cap cells. Patients with NF-1 rarely present with intraorbital optic nerve sheath dural ectasia, which should be included in the differential diagnosis of optic nerve glioma. We report the case of a 2½-year-old boy with NF-1 presenting with glioma of the optic pathway with involvement of the chiasm and optic nerve, orbital alveolar rhabdomyosarcoma (RMS.
Editor-In-Chief: C. Michael Gibson, M.S., M.D. Associate Editor(s)-in-Chief: Simrat Sarai, M.D. Overview. Optic nerve glioma must be differentiated from other diseases that cause optic nerve enlargement such as optic nerve sheath meningioma, orbital pseudotumor, optic neuritis, orbital lymphomas, metastasis, fibrous dysplasia, paranasal mucocele, rhabdomyosarcoma, neurofibromatosis, perioptic. Figure 18.1 Hematoxylin and eosin (H&E) staining. A meningothelial meningioma (right) with extension in subdural and subarachnoid spaces of the optic nerve (left). Epidemiology Meningiomas typically arise in adults in their fourth to sixth decades of life and are rare in children. They are more common in African Americans and in females, with a 2
Optic nerve sheath meningioma in a 62-year-old patient who presented with blurry vision of the right eye, axial T1+C fat-suppressed shows an avidly enhancing tumor along both sides of the right optic nerve, the intraoptic and intracanalicular portions of the optic nerve are involved (arrows), as well as the prechiasmatic portion of the. The complete differential diagnosis for DON is broad. Optic nerve sheath enhancement 'optic perineuritis' Orbital inflammatory syndrome, sarcoid, inflammatory bowel disease, Lyme disease. Pseudopapilledema is also part of the differential diagnosis for childhood optic disc swelling. Pseudopapilledema describes optic nerves that mimic the appearance of swollen optic nerves. This can include normal variants or congenital anomalies of optic nerves including optic nerve tilting, myelinated nerve fibers Optic Nerve Lesions The list of causes of optic nerve enlargement is long, but the differential is usually between optic nerve glioma and optic sheath meningioma. Optic gliomas These tumors occur in children, most often in association with neurofibromatosis (NF 1) . They are slow-growing, nonaggressive, and follow a relatively benign course Perineural enhancement was seen in four cases, optic atrophy in one. Three who had had unenhanced scans showed optic nerve enlargement. Nine patients had optic chiasmal involvement. Sarcoidosis should be considered in the differential diagnosis of optic nerve or nerve sheath enhancement on MR. Orbital sarcoidosis has MR characteristics very.
Histopathology of the resected optic nerve sheath was normal. Nine months after the procedure, the left eye was blind with significant optic nerve atrophy. The authors recommend prompt diagnosis and drainage of arachnoid cysts to preserve vision. Naqvi et al. reported 2 cases of post optic nerve sheath fenestration optic nerve cysts. The. Hemangioblastomas are World Health Organization (WHO) grade I tumors of uncertain histologic origin. These central nervous system tumors are most often found in the posterior fossa, brainstem, and spinal cord. There are fewer than 20 reported cases of optic nerve hemangioblastomas in the literature. We present a patient with visual decline found to have a mass arising from within the posterior. Optic nerve sheath meningiomas (ONSMs) account for 2 % of orbital lesions and are the second most common optic nerve tumors, after optic nerve gliomas [1-4].Clinically, optic nerve tumors closely mimic optic neuritis with painless unilateral vision impairment [2, 5].Diagnosis remains difficult because histologic confirmation carries a high risk of visual loss
Schwannoma is a benign tumor of the nerve sheath that arises from Schwann cells of the peripheral nervous system. It is important to make this distinction, as the optic nerve is part of the central nervous system, and thus schwannomas are lesions that do not arise from and are distinct from the optic nerve Enhancement of the optic nerve sheath is often poorly-defined, which, in addition to a history of pain, may serve as an important feature in distinguishing perineuritis from meningioma (Figure 8B). This finding, along with clinical history of acute, painful presentation, help distinguish perineuritic pseudotumor from en plaque optic nerve. Meningiomas of the optic chiasm are also extremely rare, and typically arise from the expansion of optic nerve sheath meningiomas . Astrocytomas are the most common tumors of the optic nerve and chiasm, and may be divided into two groups according to the age of the patient: A pediatric group and an adult group Optic neuropathy is damage to the optic nerve from any cause. Damage and death of these nerve cells lead to characteristic features of optic neuropathy. The main symptom is loss of vision (visual acuity and visual field damages), with colors appearing subtly washed out in the affected eye. The diagnosis is made on clinical examination. The history often points to the possible etiology of the.
The presence of optic nerve sheath meningioma was assumed. Discussion Optic nerve sheath meningioma (ONSM) is a term applied to primary and secondary meningioma of the optic nerve. ONSM occurs more commonly in middle aged women. Primary ONSMs account for approximately one third of primary optic nerve tumors and 5% to 10% of orbital tumors [1,2] MRI reveals a left-sided intra-conal lesion adjacent to the optic nerve. On T2-weighted images (Figs. 1a and 2a) the lesion appears iso-intense (arrow) displacing the optic nerve (arrow head).After administration of Gadolinium the lesion shows homogenous enhancement of contrast (Figs. 1b and 2c, arrow).The optic nerve does not seem to be invaded by the mass lesion Diagnosis: Presumed bilateral optic nerve glioma in NF-1 patient. Key points. General: - Most common cause of optic nerve enlargement. - Much more common than optic nerve sheath meningioma (up to 4 times more common) - 3% of all orbital tumors. - Treatment may include radiation, chemotherapy, or surgery if needed. - Strong association with NF-1 There was no optic nerve or optic nerve sheath enhancement on MRI in any of the patients. The opening pressure was normal in the two patients in which it was measured. The primary site of pathology appeared to be inflammation of the optic disc, as demonstrated by isolated enhancement of the optic disc on MRI in three of the patients
Methods: We retrospectively analysed a consecutive case series including all patients with an MRI study of brain and orbit and the clinical diagnosis of either ON or AION. We examined the scans for restricted diffusion of the optic nerve, optic sheath diameter, enhancement and location of enhancement of the optic nerve and distribution of the. Meningioma, also known as meningeal tumor, is typically a slow-growing tumor that forms from the meninges, the membranous layers surrounding the brain and spinal cord. Symptoms depend on the location and occur as a result of the tumor pressing on nearby tissue. Many cases never produce symptoms. Occasionally seizures, dementia, trouble talking, vision problems, one sided weakness, or loss of.
evolution, the tumor is considered an optic nerve sheath meningioma (ONSM). DIscUssION A differential imaging diagnosis should be made with: optic nerve glioma (enhancement of the optic nerve, more frequent in children), orbital schwan-noma (heterogeneous enhancement with cystic components), orbital pseudo tumor (rapid-onse Thickened optic nerve showing enhancement after gadolinium administration on T1-weighted fat-suppressed imaging. Differential Diagnosis sheath, anterior to the sternocleidomastoid muscle, and posterior to the sub
To analyze trends in the clinical presentation and diagnosis of optic nerve sheath meningiomas and to evaluate the effectiveness and side‐effect profile of three‐dimensional conformal radiotherapy versus other treatment modalities, a retrospective chart review wa 1.3 Optic nerve hypertrophy . Differential diagnosis of optic nerve hypertrophy is broad and includes both congenital and acquired etiologies, such as neurofibromatosis 1 (NF1), histiocytic or granulomatous infiltration, leukemia, ON sheath meningioma, medulloepithelioma, orbital pseudotumor, optic neuritis as well as Krabbe disease [5] There were only 2 pediatric cases, both classified as B cell lymphoma. Diagnosis was made by optic nerve biopsy in 12 cases and at autopsy in 4 more. Notably, 1 biopsy report noted that the optic nerve sheath was not infiltrated, 8 demonstrating that biopsy of the nerve itself is required to confidently rule out lymphomatous infiltration
track pattern of enhancement along theleft opticnerve sheath. T2-weighted coronal image (Fig. 3D) shows that the optic chiasm is normal. Differential considerations at this time would include both inflammatory and neoplastic processes. Dr Lincoff: The differential diagnosis of unilateral optic nerve thickenin Benign peripheral nerve sheath tumor with classic identifiable features including the presence of a neuronal component comprising transformed Schwann cells and a nonneoplastic fibrous component that includes fibroblasts Optic glioma Sphenoid dysplasia or thinning of long bone cortex with or without pseudoarthrosis Differential diagnosis asm, with homogeneous contrast enhancement (Fig. 2a, b). The differential diagnosis included an optic nerve sheath meningioma, an optic glioma, a lymphoma, and/or an in-flammatory mass. Further investigation with positron emis-sion tomography (PET)-CT scan demonstrated mediastinal and bilateral hilar lymph nodes, aside from a hypermeta A T1-weighted postcontrast magnetic resonance image (MRI) of the brain and orbits showed both multifocal areas of nodular leptomeningeal enhancement and abnormal enhancement of the posterior left optic nerve sheath; this abnormality also involved the left orbital apex and anterior clinoid process