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.     

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.     

Fractionated stereotactic radiotherapy in optic nerve sheath meningioma

CASE REPORT: A 56-year-old woman with a right optic nerve sheath meningioma and visual loss, was treated with fractionated stereotactic radiotherapy. This resulted in an almost complete recovery of her visual acuity. DISCUSSION: Currently there are many different alternatives available for the management of an optic nerve sheath meningioma: observation, surgery, conventional radiotherapy and fractionated stereotactic radiotherapy. The last of these treatments has been demonstrated to preserve or improve vision in 2 out of 3 treated patients, with fewer side effects, and has not been associated with recurrent disease or tumor progression.     

Is primary optic nerve sheath schwannoma a misnomer? Report of two cases and literature review

Aims: To report clinicopathological characteristics of two patients with optic nerve sheath schwannoma (ONSS) and review the literature.

Method: The first patient (22-year-old man) presented with left eye proptosis and decreased vision in 2012 whose orbital imaging showed a large cystic lesion around the optic nerve. The second patient (52-year-old man) presented with decreased vision in the left eye (without proptosis) in the 2006. His imaging showed a small orbital apex lesion between the medial rectus and optic nerve. Both lesions were histopathologically consistent with ONSS. ONSS has previously been reported in 12 patients.

Results: Orbital biopsy and subsequently external beam radiotherapy were performed for the first patient who showed a temporary improvement of vision. However, his proptosis progressed and vision decreased to light perception (LP) a few months after radiotherapy when the lesion was resected in April 2013. Second patient declined any procedure until his vision gradually decreased to LP in October 2007. It was then removed through a combined medial and lateral orbitotomy procedure. Both patients ended up with visual acuity of no LP and no recurrence in their last follow up visits in April 2018. No histopathological evidence was found to show that optic nerve sheath could be the origin for presenting and previously reported cases in the literature.

Conclusion: Presenting cases and literature review imply that ONSS is a misnomer and all the lesions are different types of orbital schwannomas with optic nerve sheath attachment.     

The Surgical Management and Outcomes for Spheno-orbital Meningiomas: A 7-year Review of Multi-disciplinary Practice

Aims/Purpose: Spheno-orbital meningiomas account for 9% of all adult intracranial meningiomas. Complete resection is extremely difficult with this condition. We report on our experience in the surgical management of spheno-orbital meningiomas.

Methods: A retrospective review was performed of all patients with spheno-orbital meningiomas who underwent joint neurosurgical and ophthalmic procedures between January 2000 and December 2007. Radiological findings, presenting clinical signs, indications for surgery, surgical approach, histopathological findings, surgical complications and post-operative results were recorded.

Results: Twelve patients were included in the study. Visual function deterioration was the main indication for surgery. Six patients underwent an optic canal decompression along with their initial tumour resection and all 12 patients underwent an extensive lateral orbital wall decompression. Post-operatively 6 patients had reduced proptosis. Two patients had an improvement in their visual acuity, 5 patients had a stable visual acuity and 5 patients had a progressive deterioration in visual acuity following surgery. Six patients maintained a stable visual field. Cranial nerve palsy was the commonest post-operative complication. Three patients required postoperative fractionated radiotherapy. Three patients required further surgery.

Conclusion: Spheno-orbital meningiomas are difficult tumours to manage. Surgical resection can reduce the degree of proptosis and stabilise visual function in patients with failing vision, although sustained improvement is difficult to achieve if the tumour behaves in an aggressive manner. The risk of post-operative visual loss is considerable, either due to surgery or tumour progression. Outcomes from surgical decompression may not necessarily be better than the natural history of these tumours.     

Optic nerve sheath meningiomas

PURPOSE: To study the natural history and growth of optic nerve sheath meningiomas and evaluate their management outcome. DESIGN: Clinicopathologic retrospective noncomparative case series. METHODS: A retrospective study of 88 patients who were treated between 1976 and 1999 at the University of British Columbia and the University of Amsterdam. Clinical reports, imaging studies, and histopathologic findings were reviewed. RESULTS: The mean age at onset of symptoms was 40.3 years, and most were seen in middle-aged females. Patients typically presented with visual loss, frequently associated with optic atrophy or papilledema and occasionally optociliary shunt vessels. On imaging, the optic nerve demonstrated segmental or diffuse thickening of the sheath or globular growth. Calcification was seen in 31% of cases and was associated with slower tumor growth. Tumors with posterior components in the orbit had more frequent intracranial involvement. Intracranial extension was more frequent and had a greater growth rate in younger patients. Irregular margins in the orbit implied local invasion. A presenting visual acuity better than 20/50 correlated with longer preservation of vision. Patients who underwent radiotherapy showed improvement in their visual acuity, and tumor growth was halted. Optic sheath decompression did not preserve vision. En bloc tumor excision was associated with no detectable recurrence in contrast to debulked tumors that recurred. CONCLUSIONS: Meningiomas show characteristic indolent growth. Management therefore should be conservative in most cases. Radiotherapy is indicated in patients with progressive visual deterioration. Surgery, when indicated, should be an en bloc excision.     

Optic Nerve Sheath Meningioma: Associations and Implications

The classical clinical presentation of optic nerve sheath meningiomas is painless, slowly progressive, unilateral optic neuropathy with a swollen and/or pale optic disc. It affects middle-aged women more frequently than men. Optic nerve sheath meningiomas occur frequently in patients with neurofibromatosis type 2. This association parallels the well known link between neurofibromatosis type 1 and optic gliomas. Due to advances in neuroimaging, management decisions have to be made in patients with excellent visual function. Fractionated conformal stereotactic radiotherapy holds the best potential to improve, prevent or at least delay severe visual loss in patients with optic nerve sheath meningiomas.     

Optic nerve sheath meningioma: diagnosis and new treatment options, a case study of monocular blindness during pregnancy

Optic nerve sheath meningiomas are challenging lesions to manage. We report here a case of primary optic nerve sheath meningioma in a pregnant woman with sudden unilateral vision loss. Then we review the current literature on the subject, prognosis factors, and report the results of fractionated radiotherapy and current therapeutic guidelines. Pregnancy may accelerate growth of meningiomas and this diagnosis must be considered in all cases of optic neuropathy in pregnant woman.     

关于视神经鞘脑膜瘤诊断和治疗的新观点

视神经鞘脑膜瘤是视神经鞘最常见的肿瘤,占原发性视神经瘤的三分之一。随着先进的神经影像技术的发展,其发生率提高了。神经影像技术对该病的早期诊断具有重要的意义。大多数临床所见的病例诊断甚是可疑,许多病例并未做活组织检查,只是通过神经影像学支持诊断。视神经鞘脑膜瘤治疗的目的是确保一定的视力,控制肿瘤的局部增长,减少死亡率。对视力轻微下降或无视力障碍以及视力保持稳定的患者进行随访观察,分次立体定向放射疗法对进行性和早期的病例可以维持视力。外科切除术和活组织检查的致盲率比较高,适用于向外生长到硬脑膜的肿瘤。     

Optic nerve sheath meningiomas. Clinical manifestations

A retrospective clinical study of optic nerve sheath meningiomas based on 22 patients showed that symptoms most commonly develop in women between the ages of 35 and 60 years. The most common presenting symptoms were decreased vision and transient visual obscurations. In the earliest stages, many patients presented with normal to mildly impaired acuity (despite subjectively decreased vision), optic disc edema and enlargement of the blind spot. Optic disc edema was frequently associated with refractile bodies indicative of chronic swelling. Optic disc edema preceded the development of optic atrophy. Another group of patients presented with a history of longstanding vision loss, visual acuity of 20/200 or worse and optic atrophy. Optociliary shunt vessels were late findings only seen in five patients. The most consistent visual field abnormality was peripheral constriction. Cecocentral scotomas were uncommon. Intracranial involvement was present in five patients. There were two patients with bilateral optic nerve sheath meningiomas without CT evidence of intracranial involvement. Computerized tomography was found to be indispensable in the diagnosis of optic nerve sheath meningiomas and the detection of intracranial involvement.     

Primary radiotherapy for optic nerve sheath meningioma

Radiotherapy has recently been recognized as the treatment of choice for most primary optic nerve sheath meningiomas (ONSM). Radiotherapy is incorporated into the treatment of non optic nerve sheath meningiomas for unresectable or subtotally resected tumors. Most primary ONSM are not surgically approachable without a high risk of visual deterioration. Radiotherapy has been found to prevent or delay tumor growth for the majority of patients and improve vision in some. We review the rational and current methods of the use of radiotherapy for these tumors.     

Primary radiotherapy for optic nerve sheath meningioma

Radiotherapy has recently been recognized as the treatment of choice for most primary optic nerve sheath meningiomas (ONSM). Radiotherapy is incorporated into the treatment of non optic nerve sheath meningiomas for unresectable or subtotally resected tumors. Most primary ONSM are not surgically approachable without a high risk of visual deterioration. Radiotherapy has been found to prevent or delay tumor growth for the majority of patients and improve vision in some. We review the rational and current methods of the use of radiotherapy for these tumors.     

经颅视神经减压开放术治疗外伤后迟发失明

目的 :报告 2 0例经颅视神经减压开放术治疗外伤后迟发失明。方法 :经冠状切口额部开颅硬膜下入路 ,视神经管上壁骨性减压 ,并剪开视神经鞘膜。评价手术后视力恢复的标准为 :失明、眼前手动、眼前数指、光感、能见视力表符号 5个级别。术后视力提高 1个级别以上者为有效 ,否则为无效。结果 :2 0例患者共 9例术后恢复了视力 ,有效率为 45 %。结论 :迟发性失明的患者应行急诊手术治疗 ,术后视力恢复与损伤后至手术时间、手术方法相关 ,也与视神经损伤程度密切相关     

Gaze-evoked amaurosis

Gaze-evoked amaurosis is a transient monocular loss of vision occurring in a particular direction of eccentric gaze. Six cases are reported with a mean follow-up of 5 years; three with optic nerve sheath meningiomas and three with orbital cavernous hemangiomas. Five of the six patients have had no visual deterioration during follow-up. Bilateral optic nerve sheath meningiomas led to visual deterioration in both eyes of one patient, but gaze-evoked amaurosis was present in only one eye. The symptom of gaze-evoked amaurosis, while alarming, is not predictive of permanent visual loss. Possible mechanisms for gaze-evoked amaurosis include inhibition of axonal impulses or transient optic nerve ischemia.     

A long-term visual outcome comparison in patients with optic nerve sheath meningioma managed with observation, surgery, radiotherapy, or surgery and radiotherapy

PURPOSE: Comparison of treatment outcome in patients with primary optic nerve sheath meningioma (ONSM). DESIGN: Retrospective, nonrandomized, comparative interventional case series. PARTICIPANTS: Sixty-four patients with at least 50 months of well-documented follow-up. INTERVENTION: Observation, surgery only, radiotherapy only, or surgery and radiotherapy. MAIN OUTCOME MEASURES: Visual acuity expressed as ratio, radiographic progression, and treatment complications. RESULTS: Sixty-four patients (55 women [85.9%] and nine men [14.1%]) were followed for a mean of 150.2 months (range, 51-516 months; standard deviation [SD], 74.7). The mean age at diagnosis was 47.1 years (range, 17-81 years; SD, 15.4). Fifty-eight patients had unilateral disease and six patients had bilateral disease. Of 59 patients with vision greater than no light perception at diagnosis, 13 patients were observed only, 12 had surgery only (four biopsies or partial resections, eight total resections), 18 received radiation alone, and 16 had surgery and radiation (14 biopsies or partial resections and radiation, two total resections and radiation). Irradiated patients received 4000 to 5500 cGy of conventional multiport or conformal external beam therapy, typically fractionated over 6 weeks. Visual acuity measurements at diagnosis among the four groups were not different (ratio, P = 0.186). Visual acuity at diagnosis was > or =0.5 in 56.3%, 0.4 to 0.050 in 12.5%, and <0.050 in 31.3%. Visual acuity measures at last follow-up were different among the four groups (ratio, P = 0.004). At last follow-up the acuity was > or =0.5 in 28.1%, 0.4 to 0.050 in 15.6%, and <0.050 in 56.3%. Visual acuity fell significantly for the observed only (ratio, P = 0.002), surgery only (ratio, P = 0.019), and surgery with radiation groups (ratio, P = 0.030). The radiation only group showed a decrease in visual acuity that was not significant (ratio, P = 0.301). Complication rate was 33.3% in radiation only cases, 66.7% in surgery only cases, and 62.5% in surgery with radiation cases. Twenty-one patients (32.8%) showed radiographic progression. Four patients who were observed, seven patients who had surgery alone, and eight patients who had surgery and radiation developed radiographic progression. Two patients who had radiation alone had radiographic progression before treatment. Only two patients treated with radiation only showed radiographic progression after radiotherapy, and both had at least one surgical procedure before the radiotherapy. CONCLUSIONS: Patients with ONSM receiving radiation alone demonstrated the best visual outcome during the follow-up period. We recommend that fractionated external beam radiation (5000-5500 cGy) be considered as initial treatment in adults in selected cases of ONSM when preservation of visual function is a reasonable therapeutic goal.     

Radiation retinopathy after fractionated stereotactic radiotherapy for optic nerve sheath meningioma

PURPOSE: To report a patient with radiation retinopathy after fractionated stereotactic radiotherapy for treatment of optic nerve sheath meningioma (ONSM). DESIGN: Interventional case report. METHODS: The clinical presentation, radiation treatment, and subsequent visual complications of a patient with ONSM are described. MAIN OUTCOME MEASURES: Development of radiation retinopathy and Snellen visual acuity. RESULTS: A 36-year-old man presented with a left optic neuropathy. Magnetic resonance imaging studies showed abnormalities consistent with ONSM. Because of progressive vision loss, the patient underwent fractionated stereotactic radiotherapy for the lesion. Vision initially improved after treatment, but 22 months later, retinal abnormalities consistent with radiation retinopathy were noted in the left eye. Visual acuity worsened over the next 24 months in association with the development of retinal hemorrhages, lipid, and retinal edema predominantly within the nasal portion of the posterior pole. Fluorescein angiography showed edema associated with microaneurysms, retinal telangiectasia, and capillary nonperfusion. Laser photocoagulation was performed on several occasions in areas of microaneurysms and retinal telangiectasia; however, the retinal edema and lipid remained, associated with persistent decreased vision. CONCLUSIONS: Radiation retinopathy and vision loss may occur after fractionated stereotactic radiotherapy for ONSM and should be discussed as a potential complication.     

Pseudotumor cerebri and optic nerve sheath decompression

OBJECTIVE: To determine the efficacy and safety of optic nerve sheath decompression in a large population of patients with pseudotumor cerebri with visual loss despite medical treatment and to suggest a treatment algorithm on the basis of these data. DESIGN: Retrospective, noncomparative, interventional case series. PARTICIPANTS: One hundred fifty-eight eyes in 86 patients with pseudotumor cerebri. INTERVENTION: Optic nerve sheath decompression. MAIN OUTCOME MEASURES: Visual acuity, visual fields, and surgical complications. RESULTS: After optic nerve sheath decompression for pseudotumor cerebri, visual acuity stabilized or improved in 148 of 158 (94%) eyes, and visual fields stabilized or improved in 71 of 81 (88%) eyes. Surgical complications, most of which were transient and benign, were seen in 39 of 86 patients. Only one eye in one patient had permanent severe visual loss secondary to an operative complication. CONCLUSIONS: In patients with pseudotumor cerebri with progressive visual loss despite maximum medical therapy, optic nerve sheath decompression is a safe and effective means of stabilizing visual acuity and the visual fields of those tested.     

Optic nerve sheath decompression for visual loss in intracranial hypertension: report from a tertiary care center in South India

Aim: Severe visual loss is the only serious complication of intracranial hypertension secondary to idiopathic intracranial hypertension (IIH) and some cases of cerebral venous thrombosis (CVT). Optic nerve sheath decompression (ONSD) has been shown to improve or stabilize visual function in patients with IIH, while its role in CVT is yet to be established. We report our experience with optic nerve sheath decompression for visual loss in IIH and CVT. Materials and Methods: In this prospective noncomparative, interventional study, 41 eyes of 21 patients with IIH and CVT and visual loss underwent ONSD. The main outcome measures included best-corrected visual acuity (BCVA), visual fields, pupillary light reflex, optic nerve sheath diameter on B-scan and resolution of papilledema which were evaluated preoperatively and at follow-up at four days, two weeks, one month, three months and final follow-up. In 7/41 eyes with absent light perception preoperatively, the functional outcome was analyzed separately. Results: Following ONSD BCVA and visual fields stabilized or improved in 32/34 (94%) eyes. Statistically significant improvement in BCVA, visual fields and pupillary light reflex occurred over the three month follow-up period. Surgical success was indicated by reduction in optic nerve diameter and papilledema resolution occurred in all patients. The outcome in the IIH and CVT groups was comparable. Four eyes with absent light perception showed marginal improvement in visual acuity. Four eyes had transient benign complications. Conclusion: Optic nerve sheath decompression is an effective and safe procedure to improve or stabilize vision in patients with visual loss caused by IIH and CVT.     

Visual Function Improvement After Optic Nerve Sheath Fenestration in Osteopetrosis Patients with Optic Canal Stenosis: A Report of Two Cases

The outcome of two patients with visual loss from osteopetrosis who underwent an optic nerve sheath fenestration (ONSF) is reported. A 20-year-old male and 26-year-old female with osteopetrosis had optic nerve edema. Computed tomography and magnetic resonance imaging demonstrated optic canals stenosis. Both patients underwent unilateral ONSF.?After ONSF, the patients experienced improvement in visual acuity and optic nerve appearance. Therefore, when papilledema is recognized in osteopetrosis patients, it may be reasonable to start with an ONSF even if the optic canal seems to be stenotic because of the lower morbidity that is associated with this procedure compared with other surgical options.     

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.