Optic Nerve Sheath Meningioma in the First Decade of Life: Case Report and Review of the Literature

Background/Aim

Tumors of the optic nerve are mostly either optic nerve gliomas or optic nerve sheath meningiomas (ONSMs). While gliomas occur in children, most meningiomas are slow-growing tumors affecting middle-aged individuals with progressive visual loss, proptosis, disc edema and optociliary veins on fundus examination. ONSMs are extremely rare in children, with only 14 cases reported in children under the age of 10 years. The purpose of this study is to describe an additional case and review clinical, radiologic and histopathological findings that can help differentiate such tumors in children and allow an early and accurate diagnosis.

Case Report

An 8-year-old girl had a 2-year history of progressive proptosis and total visual loss on the right side. A computed tomography scan revealed a well-defined intraconal mass with perioptic calcification. Magnetic resonance imaging showed a tumor surrounding the optic nerve and extending intracranially, with enhancement following gadolinium injection. The patient was submitted to fronto-orbital craniotomy for complete tumor excision. Histological studies identified the neoplasm as ONSM.

Conclusions

Though unusual, ONSMs may occur in children and, in these cases, present a much more aggressive behavior than in adults. ONSM should therefore be differentiated from optic glioma in children because of its aggressive behavior and need for different treatment modalities.Key Words: Optic nerve neoplasms, Benign optic nerve sheath neoplasm, Meningioma     

Acute visual loss due to a calcified optic nerve glioma

Optic nerve gliomas are slow-growing tumours most commonly seen in children under 10 years of age. Rapidly progressive proptosis and rapid visual deterioration are uncommon but may occur owing to accumulation of mucoid material, necrosis or hemorrhage. We describe a patient with an optic nerve glioma who manifested sudden proptosis and blindness caused by hemorrhage within the optic nerve sheath. The visual acuity returned to 20/25 after surgical decompression of the nerve and high-dose steroid therapy. Histopathological examination was required to establish the diagnosis of optic nerve glioma with extensive calcification. Optic nerve decompression or short-term high-dose steroid therapy, or both, may be helpful in recovering visual function in selected patients with optic nerve gliomas who have acute visual loss.     

Optic nerve sheath meningiomas.

Meningiomas are benign neoplastic lesions arising from meningothelial cells of the meninges. Primary orbital meningiomas, originating in the optic nerve sheath, represent 1-2% of all meningiomas, and are the second most common optic nerve tumor after gliomas. They primarily affect middle-aged adults. Patients typically present with visual loss, frequently associated with optic atrophy and often with optociliary shunt vessels. The lesion is usually unilateral, but is bilateral in about 5% of cases. Meningiomas show characteristic indolent growth over years, progressing inexorably to blindness in the affected eye. Management should be conservative in most cases. In very rare situations, surgery has improved visual prognosis. In most patients, however, surgery offers no benefit, and should be reserved for those with blindness or severe proptosis, or when extension toward the optic canal is documented. Although preliminary results of radiotherapy are encouraging, very few patients have been treated using this modality, and the long-term advantage for vision remains unproven. Even when untreated, the prognosis for life is excellent, with an overall tumor-related mortality of 0%.     

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.     

Hemangioblastoma of the optic nerve

Optic nerve hemangioblastoma is a rare tumor that is usually unilateral and most commonly occurs in the context of von Hippel-Lindau disease. Differential diagnosis is based on clinical history and imaging. Magnetic resonance imaging with gadolinium enhancement is the most useful imaging modality as it can reveal flow voids and an absence of dural attachment, differentiating optic nerve hemangioblastoma from other more commonly encountered optic nerve tumors. Optic nerve hemangioblastoma are usually well-circumscribed vascular lesions composed of stromal cells and vascular endothelial cells. These lesions are diagnosed at a mean age of 37 years and can be asymptomatic, but over time, patients may develop reduction in vision, proptosis, and pain. Surgical excision is well described via orbital, transsphenoidal, or transcranial approaches. Given the risks associated with surgery, a stepwise conservative approach is advocated by most clinicians in the absence of severe symptoms. Although uncommon, this optic nerve tumor should be considered in young patients presenting with pain, proptosis, and optic nerve pallor, with or without a history of von Hippel-Lindau disease.     

Arachnoid cyst of the optic nerve: report of two cases and review of the literature

PURPOSE: To describe two patients with arachnoid cysts of the optic nerve mimicking optic nerve/orbital neoplasms. METHODS: The histories, ophthalmic examinations, and pathologic findings of two patients with arachnoid cysts of the optic nerves were reviewed and compared with previously reported cases. RESULTS: One patient had progressive proptosis and the other had decreased vision with visual field changes. Pathologic examination of optic nerve sheath biopsies showed redundant proliferations of meningothelial cells with associated thickened dura. CONCLUSIONS: Arachnoid cysts of the optic nerve are benign, slowly progressive conditions that may result in proptosis and visual field changes. The clinicopathologic features of arachnoid cyst should be differentiated from optic nerve sheath meningioma and other conditions.     

Neural orbital tumors

Primary tumors of the optic nerve (optic nerve sheath meningiomas and optic nerve astrocytomas) are important causes of visual morbidity. They may elude clinical detection and have the potential to be lethal. Their treatment remains controversial but often includes radiation. The optic nerve may be involved secondarily by peripheral nerve tumors in the orbit or by metastatic and infiltrative neoplasms. Optic neuropathy may also develop as a result of treatment of these tumors. Recent contributions to the literature concerning the diagnosis and management of neural orbital tumors are reviewed.     

Unsuspected recurrent pituitary adenoma presenting as an orbital mass

PURPOSE: Orbital invasion of pituitary tumors is rare and usually accompanied by optic nerve head pallor and visual loss. We describe a case of unilateral massive orbital invasion by a recurrent pituitary tumor with preserved visual acuity and normal optic nerve appearance. METHODS: Case report. RESULTS: Progressive proptosis developed 15 years after transphenoidal removal of a pituitary tumor. Based on the radiological appearance and the clinical history, the patient was suspected to have a sphenoid wing meningioma secondary to previous radiation treatment. A combined neurosurgical and orbital approach was used to remove the intraorbital mass, which extended from the cranial cavity through the superior orbital fissure and the optic canal. Histopathologic examination demonstrated a recurrent nonsecreting pituitary adenoma. CONCLUSIONS: Orbital extension of a recurrent pituitary adenoma should be considered in the differential diagnosis of progressive proptosis even in the absence of significant optic neuropathy.     

Meningiomas of the anterior visual system

Meningiomas, whether primary in the orbit, optic canal or intracranial area, typically cause slowly progressive loss of vision covering months to years. They usually occur in white women of middle age. Tumors in the orbit or optic canal almost always affect vision unilaterally; intracranial tumors, while usually causing unilateral visual loss initially, eventually cause bilateral loss of vision, often with blindness in one eye. Depending upon the size and location of the tumor, the ocular signs and symptoms of meningiomas may include visual field abnormalities, optic atrophy, edema of the ipsilateral optic disc, papilledema, diplopia, and proptosis. The diagnosis of meningiomas in all locations has been greatly facilitated by recent advances in computer assisted tomography. However, tumors confined to the optic canal or its foramina are small when vision is first lost and therefore are still difficult to detect. Hypocycloidal polytomography may be useful; other neuroradiologic studies rarely are. Treatment is surgical. Based on a review of more than 3000 meningiomas reported in the literature, the frequency and characteristics of the signs and symptoms of meningiomas in each location are discussed, as are the anatomy, pathology, natural history, and probable mechanisms.     

Intraosseous meningioma: a rare cause of chronic optic neuropathy and exophthalmos

BACKGROUND: The association of optic neuropathy, proptosis and chorioretinal folds is highly suggestive of an orbitopathy. Usual etiologies include an inflammatory orbitopathy, optic nerve sheath meningioma, sphenoid wing meningioma, exterioration of a sinus disorder, or an orbital tumor be it primary or secondary. HISTORY AND SIGNS: A 40-year-old man treated for systemic hypertension complained of decreased vision and floaters in his right eye. Initial examination revealed decreased visual acuity to 20/50 of the right eye with a slight dyschromatopsia, but a lack of afferent pupillary defect and normal visual fields. Fundus examination showed the presence of a slightly swollen right optic disc and chorioretinal folds. A diagnosis of presumed anterior ischemic optic neuropathy was made. Symptoms persisted and, five months later, right proptosis was noted. Magnetic resonance imaging revealed a diffuse thickening of the parieto-temporal bone and the greater wing of the sphenoid bone on the right side. Radiological differential diagnosis included fibrous dysplasia and metastasis. THERAPY AND OUTCOME: Bone biopsy revealed a grade I intraosseous meningioma. Conservative management was chosen because the lesion was too extensive to be resected and radiotherapy is usually not efficient on grade I meningiomas. CONCLUSIONS: Intraosseous meningiomas are benign tumors which are due to meningeal cells entrapment during vaginal delivery. It is a rare tumor of slow progression. Therapy usually consists of resection and cranioplasty and/or radiotherapy. In the present case, decompression of the optic canal remains feasible in case of further visual loss.     

Tumors of the optic nerve and chiasm

Optic nerve gliomas and meningiomas, which remain puzzling to clinicians, are reviewed with special attention to natural history, magnetic resonance findings, and appropriate management. New histopathological insights established the true neoplastic nature of optic nerve gliomas considered by most clinicians to be benign hamartomas. Unusual presenting manifestations and therapeutic alternatives of optic glioma have been also considered. Since the association of optic nerve gliomas and meningiomas with neurofibromatosis has long been recognized, special attention has been drawn to clinical, genetic, and ophthalmologic aspects of neurofibromatosis. New and unusual presenting manifestations, diagnostic and treatment opportunities concerning optic nerve involvement in metastatic disease, and lymphomatous proliferation are also described.     

Optic glioma of childhood: clinical, histopathological, and histochemical observations.

This report documents the long-term clinical and histopathological behaviour of eight intraorbital and 16 intracranial optic nerve gliomas and relates the therapeutic data to the prognosis for both visual acuity and survival. The mean age at onset of symptoms was 8.6 years and at the diagnosis 10.9 years. It is generally held that proptosis is mild in intraorbital glioma, but we encountered marked proptosis ranging from 7 to 12 mm in six of the eight intraorbital gliomas, which contained abundant Alcian-blue-positive mucoid material. Of the patients with intracranial optic nerve gliomas 37.5% survived for a mean of eight years after treatment with radiotherapy or surgery combined with radiotherapy. At follow-up ranging from five months to 11 years only one of the six patients with intracranial gliomas had full visual acuity. Our observations emphasise that, although optic nerve gliomas are benign hamartomas, the prognosis for visual acuity and survival is unfavourable in cases which are diagnosed and treated late. Histopathological and histochemical observations suggest that increase in the amount of mucoid material may contribute to rapid enlargement of intraorbital and intracranial optic gliomas.     

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.     

Orbital optic nerve gliomas in children with neurofibromatosis type 1

PURPOSE: To describe the clinical course and treatment of symptomatic orbital optic nerve gliomas in children with neurofibromatosis type-1 (NF-1). METHODS: A retrospective review of the records of patients with NF-1 and symptomatic orbital optic nerve gliomas seen in a large multidisciplinary NF-1 clinic of a tertiary care children's hospital. The main outcome measures included presenting symptoms and signs, ophthalmologic examination at diagnosis, the presence of progressive disease following diagnosis, type of therapy, and the reasons therapy was instituted. RESULTS: Twelve patients with symptomatic orbital optic nerve gliomas, all of which led to proptosis (eight girls, four boys), were identified. The mean age of diagnosis of NF-1 was 20 months; the mean age of diagnosis of the orbital optic nerve glioma was 26 months. At the time of diagnosis of the tumor, 10 of 12 patients (83%) had decreased visual acuity in the affected eye. Three patients underwent optic nerve resection; eight received chemotherapy, and one was observed without therapy. Of the eight children who received chemotherapy, progressive disease prior to treatment could be documented in only three; none of these eight children had a reproducible improvement in vision following chemotherapy. There was no demonstrable improvement in vision in any treated patient with NF-1-associated orbital optic nerve gliomas. CONCLUSIONS: Although not definitively proven, our data and previous studies suggest that NF-1-associated orbital optic nerve gliomas should not be treated unless there is clear evidence of either ophthalmologic or radiographic progression. Surgical excision of tumors which have led to proptotic eyes without functional vision should be reserved for cosmetic purposes or to treat complications of exposed globes.     

Meningeome im Bereich der vorderen Sehbahn

Meningiomas are found incidentally or because of characteristic but non-specific symptoms. In Germany approximately 1,000 new diseases in the area of the anterior visual pathways are expected per year. Around one quarter of all incidentally diagnosed meningiomas eventually need treatment. The typical patient is a female around the age of 50 years as more than 80?% of the patients are women. The typical presenting symptom is visual acuity loss followed by diplopia, visual field defects and proptosis are experienced in about 10?%. In primary optic nerve meningiomas the optic disc is swollen or atrophic, rarely normal and in meningiomas invading the optic nerve from another origin the optic disc is either normal or atrophic and rarely swollen.     

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.     

Clinical features of dysthyroid optic neuropathy: a European Group on Graves' Orbitopathy (EUGOGO) survey

BACKGROUND: This study was performed to determine clinical features of dysthyroid optic neuropathy (DON) across Europe. METHODS: Forty seven patients with DON presented to seven European centres during one year. Local protocols for thyroid status, ophthalmic examination and further investigation were used. Each eye was classified as having definite, equivocal, or no DON. RESULTS: Graves' hyperthyroidism occurred in the majority; 20% had received radioiodine. Of 94 eyes, 55 had definite and 17 equivocal DON. Median Clinical Activity Score was 4/7 but 25% scored 3 or less, indicating severe inflammation was not essential. Best corrected visual acuity was 6/9 (Snellen) or worse in 75% of DON eyes. Colour vision was reduced in 33 eyes, of which all but one had DON. Half of the DON eyes had normal optic disc appearance. In DON eyes proptosis was > 21 mm (significant) in 66% and visual fields abnormal in 71%. Orbital imaging showed apical muscle crowding in 88% of DON patients. Optic nerve stretch and fat prolapse were infrequently reported. CONCLUSION: Patients with DON may not have severe proptosis and orbital inflammation. Optic disc swelling, impaired colour vision and radiological evidence of apical optic nerve compression are the most useful clinical features in this series.     

Optic Nerve Involvement in Dysthyroidism

Although its prevalence in dysthyroidism is estimated at less than 5%, optic neuropathy should be recognized as a preventable cause of disabling visual loss. Obscured by more obvious external congestive signs, it is usually insidiously progressive and bilateral, but may be acute and unilateral. Neuro-ophthalmic findings are nonspecific (prechiasmal field defects, normal or swollen nerveheads), and the diagnosis should be suspected in the context of typical adnexal changes of dysthyroidism. Optic nerve involvement is probably secondary to apical orbital compression by congested muscles. Treatment with oral corticosteroids, irradiation, or orbital decompression has been beneficial, especially in the early phases.     

Optic nerve sheath meningiomas and advanced treatment options

PURPOSE OF REVIEW: Recent trends in the management of optic nerve sheath meningiomas have shifted towards fractionated conformal external beam radiotherapy. The purpose of this paper is to review the current literature on this subject and review the clinical characteristics and natural history of optic nerve sheath meningiomas. RECENT FINDINGS: Articles published within the last 2 years have reported the efficacy of using fractionated radiation therapy alone. A total of 62 patients in three studies have been reported; 40.3% retained stable visual acuity, 41.2% percent had improvement in their vision, and 18.4% percent had a decrease in visual acuity. Median follow-up time ranged from 22 to 51 months in these studies. SUMMARY: Optic nerve sheath meningiomas may be safely managed with fractionated external beam radiotherapy in select patients with possible improvement or stabilization of visual acuity. This represents an enormous improvement in a disease with typically poor visual prognosis.