Bilateral idiopathic inflammatory perioptic neuritis simulating optic nerve sheath meningioma on computed tomography

Idiopathic inflammatory perioptic neuritis is a rare inflammatory condition of the optic nerve sheaths. The differential diagnosis is important because the disease has radiologic findings resembling those of optic nerve sheath meningioma and management is quite different in each condition. A case of bilateral idiopathic inflammatory perioptic neuritis is reported in which the computed tomographic (CT) appearance simulated optic nerve sheath meningioma and there was progressive visual loss. Presented as a scientific poster at the XIth Congress of the European Society of Ophthalmology, Hungary, Budapest, June 1–5, 1997.     

Terson's syndrome

Intravitreal hemorrhage in association with acute intracerebral or subarachnoid hemorrhage is called Terson's syndrome, according to the author who first published this entity in 1900. Apart from vitreal hemorrhage, massive intracranial bleedings produce retinal, preretinal and subhyaloid hemorrhages, papilledema, hemorrhages in the optic nerve sheaths and optic nerve, as well as orbital hemorrhages and also bleeding in oculomotor nerves (third nerve, sixth nerve). The pathophysiology of the intraocular hemorrhages as well as of the hemorrhages in the optic nerve sheaths is extensively discussed by means of a careful examination of apathological specimen (globe and optic nerve) of a patient who died because of acute subarachnoid hemorrhage. The favorable results of 14 patients treated with pars plana vitrectomy for vitreous hemorrhage after subarachnoid or intracerebral or sub- resp. epidural bleeding are presented.     

Optic nerve sheath meningoceles. Clinical and radiographic features in 13 cases with a review of the literature

Thirteen patients with dilated intraorbital optic nerve sheaths with an expanded, patulous cerebrospinal fluid (CSF) space were studied with high-resolution computed tomography (CT) or magnetic resonance imaging (MRI). Eleven patients had bilateral findings. Headache or visual complaints, or both, were present in all patients. Signs of optic nerve dysfunction were present in eight patients. Three patients had visual acuity worse than 20/200. Cerebrospinal fluid pressure was mildly elevated in two patients. Three patients underwent a surgical procedure; visual acuity improved in one. The authors propose the term meningocele for this condition and suggest MRI with fat-suppression techniques and off-axis sagittal views as the radiographic procedure of choice.     

Complete evulsion of the optic nerve

Evulsion of the optic nerve is an uncommon traumatic event, probably because the intraorbital portion of the optic nerve is mobile and surrounded by sheaths. The present paper reports a case of complete evulsion of the optic nerve with retinal detachment and discusses the possible patho mechanisms involved. Fluorescein angiography showed no retinal circulation.     

Gradient-index (GRIN) endoscopic examinations from the inner structures of the optic nerve meninges

BACKGROUND: Due to the excellent image quality and the small outer diameter of the GRIN-(gradient index) endoscope tips we were able to examine the subdural and the subarachnoidal space of the optic nerve meninges by endoscopy. This examination was performed to obtain more information about the inner structure of the optic nerve meninges. MATERIAL AND METHODS: In this post-mortem study 7 optic nerves were examined from the chiasm to the globe by GRIN endoscopy (Volpi, Schlieren, Switzerland), with an outer diameter of 0.89 mm, integrated optic of 0.5 mm diameter and an integrated fluid channel of 0.2 mm diameter. RESULTS: In all cases the endoscopic examination of the optic nerve meninges was technically easy to perform. It was possible to study the inner surface of the nerve sheaths and the nerve sheath spaces in close-up. We found horizontal and vertical cords on the inner surface of the dura mater, which could tighten by movements of the optic nerve. CONCLUSIONS: With a gradient-index (GRIN) endoscope we obtained new information about the inner structure of the optic nerve meninges. New theories about the changes of the optic nerve meninges during movements of the optic nerve may evolve from this study. Further studies with this new method should be encouraged.     

Optic nerve diameters and perimetric thresholds in idiopathic intracranial hypertension.

AIMS/BACKGROUND--Idiopathic intracranial hypertension (IIH) is a central nervous disorder characterised by abnormally increased cerebrospinal fluid (CSF) pressure leading to optic nerve compression. An indirect estimate of increased CSF pressure can be obtained by the ultrasonographic determination of optic nerve sheaths diameters. Computerised static perimetry is regarded as the method of choice for monitoring the course of the optic neuropathy in IIH. The aims were to compare the echographic optic nerve diameters (ONDs) and the perimetric thresholds of patients with IIH with those of age-matched controls, and to examine the correlation between these two variables in individual patients with papilloedema. METHODS--Standardised A-scan echography of the mid orbital optic nerve transverse diameters and automated threshold perimetry (Humphrey 30-2) were performed in 20 patients with IIH with variable degree of papilloedema (according to the Frisén scheme) and no concomitant ocular diseases. Echographic and perimetric results were compared with those obtained from 20 age-matched controls. RESULTS--When compared with controls, patients with IIH showed a significant increase in mean ONDs and significantly reduced mean perimetric sensitivities. In individual patients with papilloedema, the transverse ONDs correlated negatively with Humphrey mean deviation values and positively with pattern standard deviation values. CONCLUSION--These results indicate that OND changes in IIH are associated with perimetric threshold losses, and suggest that IIH functional deficits may be related to the degree of distension of optic nerve sheaths as a result of an increased CSF pressure.     

Allergic Fungal Sinusitis Induced Visual Loss and Optic Neuropathy

Visual loss associated with allergic fungal sinusitis (AFS) is uncommon, with incidence estimates ranging from 1.46% to 3.7%. Uncertainty remains concerning the pathophysiology of visual loss in the setting of AFS. Proposed theories include direct or indirect compression of the optic nerve or orbital inflammatory changes causing an optic neuritis. A case of precipitous visual loss in an adult male with clinical and radiographic evidence of optic neuropathy is reported. After surgical and medical interventions this patient showed improvement in optic nerve function. Visual loss associated with AFS is a rare but potentially devastating condition.     

Optic atrophy following jejunoileal bypass

A 38-year-old woman presented with a bilateral, chronic, progressive optic neuropathy following jejunoileal bypass surgery for morbid obesity. Standardized A-scan orbital echography revealed grossly swollen optic nerve sheaths with a positive 30 degrees test, indicating increased subarachnoid fluid. Review of reported complications of jejunoileal bypass surgery revealed no similar case of what might be termed the "optic nerve ascites syndrome."     

Removal of horseradish peroxidase and fluorescein-labelled dextran from CSF spaces of rabbit optic nerve. A light and electron microscope study

Horseradish peroxidase (HRP) and fluorescein-labelled 150 000 mol. wt. dextran (FD) were introduced into the CSF spaces of rabbits by means of intraventricular infusions over approximately 3 hr. By light (LM) and electron (EM) microscopy HRP reaction product was found to have been taken up from the subarachnoid space of optic nerve into cells of the arachnoid around the optic nerve. HRP was also observed inter- and intracellularly in the optic nerve dura and in deep cervical lymph nodes. Fluorescence, associated with FD's was observed in the optic nerves most densely in the nerve sheaths particularly around blood vessels. Arachnoid cells of the optic nerves contained FD's in discrete locations.It is inferred that HRP and FD are carried by CSF currents into the subarachnoid spaces of optic nerves. Tracers are then taken up by the arachnoid and subsequently pass into the deep cervical lymph nodes.     

Traumatic evulsion of the globe

PURPOSE: To report the clinical and pathologic findings in 3 cases of traumatic evulsion of the globe, during which the optic nerve and its sheath were disrupted at different locations and to varying extents. METHODS: Case series (3 patients). RESULTS: We describe the clinical, gross, and microscopic pathologic findings in 3 globes that were traumatically evulsed from their orbits. The optic nerves and sheaths were disrupted at 2 different locations and in 2 distinct combinations. Two of these variations in discontinuity of the nerve and/or its sheath were unique. In 1 case, the eye and optic nerve sheath were evulsed without the nerve; in another case, the nerve and sheath were pulled from the posterior sclera at the lamina cribrosa. CONCLUSIONS: Traumatic evulsion of the globe may cause the optic nerve and its sheath to be disrupted at varying distances from the eye and may involve the optic nerve and its sheath together or separately. To the best of our knowledge, no cases have been reported in which orbital trauma caused the globe and optic nerve sheath to be removed together, leaving the nerve behind, or in which disruption of the optic nerve at the lamina cribrosa resulted in a complete posterior scleral defect. Three theories are proposed to explain possible mechanisms leading to optic nerve disruption during traumatic evulsion of the globe.     

Pneumatization of the intraorbital optic nerve after severe head trauma

Radiological evidence of pneumatization of the intraorbital optic nerve sheath following severe head trauma in an adult is reported. A young man was admitted to the emergency department following a high-speed motorcycle accident with unconsciousness, forehead laceration, and multiple fractures of the skull and extremities. On admission, the pupils were dilated and fixed. Computed tomography revealed right subdural hematoma with midline shift, brain stem hemorrhage, contusion of the left temporal lobe, multiple facial bone fractures, cerebral edema with intracerebral air, and meningeal pneumatization of the optic nerve sheaths bilaterally. This case demonstrates that after severe head trauma, air may extend in the optic nerve sheath, which could be a marker of severe optic nerve injury.     

Contralateral amaurosis after retrobulbar injection

Contralateral amaurosis after retrobulbar injection of local anesthetic agents occurred in two patients, a 64-year-old woman and a 57-year-old man. The amaurosis resulted from migration of the anesthetic agents to the optic chiasm and contralateral optic nerve via the subarachnoid space. In both cases visual acuity returned to preoperative levels within 90 minutes. A modified technique in which patients look straight ahead or slightly downward and outward during retrobulbar injection, as opposed to the traditional upward and inward positioning of the globe, avoids inadvertent piercing of the sheaths of the optic nerve and injection of substances into the subarachnoid space.     

Acute sarcoid optic neuropathy with spontaneous recovery

Sarcoid optic neuropathy without fundus lesions is unusual. We present a case of optic neuropathy with spontaneous remission in one eye that paralleled the clinical course of demyelinative acute optic neuritis. The fellow eye had disc swelling with normal visual acuity but with a large blind spot and distended optic nerve sheaths seen on ultrasonography. The diagnosis of sarcoidosis was made by characteristic chest roentgenographic findings and confirmed by skin biopsy.     

外伤性视神经病变经鼻内镜下视神经管减压及综合治疗

目的 回顾外伤性视神经病变经鼻内镜下视神经管减压合并药物治疗的结果,探讨手术适应证.设计回顾性病例系列.研究对象2006年～2010年北京同仁医院耳鼻咽喉头颈外科收治的外伤性视神经病变患者69例（69眼）.方法 患者术前均行视神经管CT检查,除外严重颅脑外伤合并症,行鼻内镜下经筛、蝶窦视神经管减压术,同时给予围手术期激素冲击、营养神经及扩张血管等综合药物治疗.主要指标手术后视力.结果 59例（85.5%）术前CT扫描显示视神经管骨折.67例（97.1%）行视神经管减压术,其中因鞘膜水肿或鞘膜下积血切开神经鞘2例 1例放弃手术 另1例视力自行恢复出院.随诊2～44个月,术后视力改善30例（44.8%）,其中从无光感到有光感18例,视力大于0.1者9例.结论 经鼻内镜视神经管减压联合药物治疗是目前治疗外伤性视神经病变的推荐方法之一,在除外手术禁忌的情况下,应积极手术并结合药物治疗,挽救视力.     

Type VI-A mucopolysaccharidosis (Maroteaux-Lamy disease). Clinico-pathologic case report

In this clinicopathological case report on a 27-year-old man suffering from mucopolysaccharidosis (MPS) type VI-A (Maroteaux-Lamy), histologic findings in both eyes, and histologic and ultrastructural findings in a keratoplasty button are presented for the first time in the German literature. Type VI-A is a very rare type of MPS, very benign as regards mental development. It results in a typical corneal opacification due to massive accumulation of mucopolysaccharides in all corneal layers. The opacification was treated successfully in one eye by penetrating keratoplasty. Thickening of the sclera and the optic nerve sheaths was demonstrated for the first time by computer-assisted tomography. Additionally, bilateral atrophy of the optic nerve, probably caused by elevated intracranial pressure, was found. The differential diagnostic differences between this and other types of MPS and the reason for the two cardinal symptoms (corneal opacification and optic atrophy) are discussed in extenso, and a review of the literature is given.     

Echographische Differenzialdiagnose von Optikusverbreiterungen

INTRODUCTION: Standardized echography is a well-established examination technique in ophthalmology. Among other things one of the more important uses is the evaluation of the optic nerve and its abnormalities. One aspect is the use of the stretch-test (originally called the "30 degrees-test") for the differential diagnosis of optic nerve widening. Such widening can be caused by solid thickening of the optic nerve and/or its sheaths or by solid infiltration of the subarachnoidal space. More frequent is a widening of the optic nerve as a consequence of sheath distension by increased subarachnoidal fluid. PATIENTS AND METHODS: We present various cases of widened optic nerve patterns and explain the technique and diagnostic value of standardized echography, especially of the stretch-test. RESULTS: In widened optic nerve patterns due to fluid around the optic nerve parenchyma, a decreased optic nerve thickness will be found when performing the stretch-test (positive test result), whereas in solid lesions of the optic nerve no change of optic nerve thickness will be seen (negative test result). CONCLUSION: The stretch-test provides a reliable tool for the differential diagnosis of widened optic nerves.     

米诺环素对急性视神经炎视神经轴突及Caspase-3表达的影响

目的:探讨米诺环素对急性视神经炎的影响,并与甲基强的松龙比较。方法:雌性Wistar大鼠22只随机分为正常组、EAE组、米诺环素组、甲基强的松龙组(MP组)。观察视神经病理改变,免疫组织化学法检测视网膜神经节细胞(retinal ganglion cells,RGCs)中Caspase-3蛋白表达。结果:EAE组视神经光镜下表现为神经纤维空泡样变性,轴突不规则肿胀,大量炎性细胞浸润。EAE组、米诺环素组、MP组与正常组视神经轴突占横切面积比例相比,差异均有显著统计学意义(P<0.01),米诺环素组、MP组与EAE组间的差异均有统计学意义(P<0.05)。正常大鼠视网膜几乎未见Caspase-3蛋白表达,EAE组、MP组与米诺环素组间的差异均有统计学意义(P<0.05),MP组与EAE组间的差异有统计学意义(P<0.05)。结论:甲基强的松龙可减轻脱髓鞘性视神经炎轴突损伤,但不能减少RGCs中Caspase-3的表达。米诺环素可下调Caspase-3在视网膜中表达,提示米诺环素可通过抑制RGCs中Caspase-3活性,介导对脱髓鞘性视神经炎RGCs的保护作用。     

Macroglial alterations after isolated optic nerve sheath fenestration in rabbit.

PURPOSE: To study the modifications undergone by the macroglial cells after meningeal breach of the optic nerve in the rabbit, without optic neuropathy. METHODS: The optic nerve sheath fenestration technique carried out in humans was adapted to rabbit without axonal injury in the optic nerve. The effects of meningeal fenestration on glial cells were examined by immunocytochemical procedures (day 15) using the antibodies against two astrocyte markers: glial fibrillary acidic protein (GFAP) and vimentin. Proliferation of glial cells was evaluated with single 5-bromodeoxyuridine (BrdU) labeling or double GFAP and BrdU labelings. Qualitative data on glial cells were evaluated with the electron microscope. RESULTS: Optic nerve sheath fenestration on healthy adult rabbits resulted in a decrease of volume of the subarachnoid space located at the level of the meningeal scar, with a significant increase of the optic nerve area. The meninges presented a fibrous scar. In the optic nerve parenchyma, astrocytes appeared hypertrophic in the vicinity of the fenestration. The whole nerve contains numerous BrdU-labeled mitotic cells, a number of which double-labeled for both BrdU and GFAP belong to the astrocyte line. There was no loss of optic nerve axons. CONCLUSIONS: The inflammation produced by the surgical breach of the peri-optic meningeal sheaths induces a significant reactivity, including proliferation of astrocytes in the optic nerve. Reactive astrocytes may interact positively with axons and may modify the extracellular environment in the optic nerve.     

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.