Seminars in Ophthalmology, 2014; 29(1): 52–55 ! Informa Healthcare USA, Inc. ISSN: 0882-0538 print / 1744-5205 online DOI: 10.3109/08820538.2013.810279
Optic Nerve Sheath Meningioma: A Case Report with 15-Year Follow-Up Zeljka Vukovic Arar1, Zoran Vatavuk2, Blazenka Miskic3, Zeljka Janjetovic1, Sandra Sekelj1, and Marijana Knezevic Pravecek3 1
Department of Ophthalmology, General Hospital ‘‘Dr Josip Bencevic’’, Slavonski Brod, Croatia, 2Clinic for Ophthalmology, University Hospital ‘‘Sisters of Mercy’’, Zagreb, Croatia, and 3Department of Internal Medicine, General Hospital ‘‘Dr Josip Bencevic’’, Slavonski Brod, Croatia
ABSTRACT Meningiomas are benign neoplastic lesions of arachnoidal cells of the meninges. These tumors may arise wherever meninges exists, such as in the nasal cavity, paranasal sinuses, middle ear, and mediastinum. Optic nerve sheath meningiomas (ONSMs) are usually unilateral and occur predominantly in middle-aged females, although they may be present at any age. We present a case of a 55-year-old female with ONSM diagnosed when she was 40 years old. Diagnosis and follow-up was based on the clinical picture, CT orbit scan, and magnetic resonance imaging. Keywords: Arachnoid, diagnosis, magnetic resonance, optic nerve neoplasms, treatment outcome
Meningiomas are important in ophthalmology, neuroophthalmology, neurosurgery, and head and neck surgery because they occur relatively frequently, they can seriously damage or even destroy vision, and they are difficult to remove surgically.1,2 What causes meningiomas is not fully understood, though they are more likely to occur in people exposed to radiation of the head.3,4 Patients with neurofibromatosis type 2 (NF-2) have a 50% chance of developing one or more meningiomas.1,5 Intraorbital meningiomas are usually meningoendotheliomatous or transitional type grade I, according to the WHO classification.1,2 The primary tumor location is usually in the orbit, after which it can spread to secondary locations in the orbit of the cranium.1 Optic nerve sheath meningiomas (ONSMs) are primary and secondary meningiomas arising from meningoendothelial cells of the optic nerve.6,8 ONSMs occur more frequently in middle-aged women.6–10
Meningiomas account for 4% of all orbital tumors,1,2,7 including 30% of all primary tumors of the orbit and 70% of all secondary tumors. Onethird of all meningiomas occur in the orbit; of these, 60% occur in the optic nerve. Primary meningiomas give rise to secondary tumors in 70% of cases.1,2,8
Meningiomas occur more frequently in women,2 suggesting a hormonal connection. Indeed, several studies have detected receptors for estrogen and progesterone in meningioma cells. The presence of these receptors is associated with tumor progression during pregnancy and lactation and with formation of secondary tumors in the orbit of the cranium.1,2
Received 1 January 2013; revised 24 March 2013; accepted 26 May 2013; published online 24 July 2013 Correspondence: Zeljka Vukovic Arar MD, Department of Ophthalmology, General Hospital ‘‘Dr Josip Bencevic’’, A. Stampara 42, 35 000 Slavonski Brod, Croatia. E-mail: [email protected]
Optic Nerve Sheath Meningioma
METHODS: CASE REPORT Results A female, 55 years old, was treated in the Department of Ophthalmology General Hospital ‘‘Dr. Josip Bencevic,’’ Slavonski Brod (Croatia), on several occasions since 1997. Initially, she presented with blurred vision in the right eye, changes in the visual field, and frontal headache. Best corrected visual acuity (BCVA) was 0.2 in the right eye (Snellen optotype at 6 m distance) and 0.8 in the left eye. Fundoscopic examination showed mild edema of the optic disc in the right eye, while the left optic disc was normal. Computed tomography (CT) of the brain showed no clear signs of expansion. Findings of a lumbar puncture were normal. The patient was treated for retrobulbar neuritis and administered systemic and local steroid therapy. After normalization of the visual field and development of a visual recovery plan, the patient was discharged from hospital. At discharge, BCVA was 1.0 in both eyes, fundoscopic examination showed normal color of the optic disc, and intraocular pressure (IOP) was 17 mmHg in both eyes. Six months later, the patient returned to our department with the same clinical presentation. BCVA was 1.0 in both eyes, fundoscopic examination showed mild edema of the optic disc in the right eye, and IOP was normal. A standard visual examination showed periferal scotoma in the right eye. A consulting neurologist recommended magnetic resonance imaging (MRI) of the brain to determine whether demyelination was occurring.MRI findings were normal and demyelination was excluded. However, visual evoked potential (VEP) findings showed conduction disturbances in both visual pathways. Systemic and local steroid therapy was administered as on the first admission, and the patient showed clinical and subjective improvement. For the next three years, the patient underwent regular checks every six months. Clinical findings were normal and the patient did not report any problems. However, three years after the first admission, the patient returned to our department with the same clinical presentation as the first time. BCVA was 0.5 in the right eye and 1.0 in the left eye. Fundoscopic examination showed mild temporal pallor in the right optic disc. Visual field of Goldmann perimetry showed that I1 excluded the blind spot for I2. Extraocular eye movement was abnormal. We initiated steroid therapy for suspected recurring retrobulbar neuritis, but we carried out additional tests to gain a more complete picture of the patient’s clinical situation and assist in differential diagnosis. Computed tomography (CT) of the right orbit revealed a C-shaped formation of !
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FIGURE 1. CT of the right orbit revealed a C-shaped formation located between the lower flat muscle and optic nerve. 24 18 mm (300 300 DPI).
unknown etiology measuring 13.2 8.2 mm and located between the lower flat muscle and optic nerve (Figure 1). Differential diagnosis suggested hemangioma, but we could not exclude meningioma, neurinoma, or ocular changes due to Graves disease. Analysis of cross-sections of the orbit area revealed a pathological substrate in the right orbit below the right retrobulbar optic nerve. This substrate measured 7 mm wide and 15 mm long; it was intense and separated from the optic nerve. Ultrasound of the right orbit revealed a circular formation around the optic nerve; this homogeneous structure seemed to be associated with the sheath of the optic disc but without propagation into the orbit and without compression of the optic nerve. The patient was diagnosed with magnetic resonance. Given the location, growth, and symptomatology of the ONSM, the ophthalmologist recommended regular monitoring of the patient. At the last check-up, the patient reported being in good condition, with no pain or visual disturbances. BCVA was þ0.75 D sphere = 1.0 in the right eye, and þ0.50 D sphere = 1.0 in the left eye. Slit lamp analysis revealed mild proptosis of the right orbital bulb. Fundus examination showed a normal optic nerve in both eyes. Intraocular pressure (IOP) was normal, as was color vision, which did not show any afferent pupillary defect. Visual field examination was normal. CT of the orbit at the last check-up revealed a hyperdense formation approximately 13.2 8.4 mm in the projection of the right orbit between the retrobulbar optic nerve and lower flat muscle. This formation could not be separated from the surrounding structures, and its etiology remains uncertain. Differential diagnosis suggests meningioma, neurinoma, or hemangioma as the most likely possibilities.
54 Z. Vukovic et al.
CONCLUSION Primary ONSMs account for 30% of primary optic nerve tumors, and 5–10% of orbital tumors.1,8 Lesions may be unilateral, bilateral (6%) or mulitifocal. They usually involve the optic nerve in the orbit but may extend into the intracranial space.11,12 En plaque meningiomas are a rare type of meningioma that infiltrate surrounding tissue, grow in a sheet-like manner, and occasionally invade bone.10 Proptosis and decreased visual acuity are more common in primary than secondary meningiomas.6,8 Blurred vision reveals depression of the visual field, and dim vision is caused by compression of the optic nerve by the great mass of the apex. The reduction in visual acuity leads to reduced sensitivity to light or projected light, and in some cases to complete insensitivity to light. Nevertheless, most patients have reasonably good visual acuity because their central vision is preserved.6 Signs and symptoms of visual dysfunction are decreased visual acuity, visual field defects (general constriction, visual field depression or scotomas), color vision impairment, and the presence of afferent pupillary defect.13–15 Fundoscopic examination reveals asymmetric disc color or pallor and characteristic changes in optociliary shunt vasculature in about 20% of cases.5,6,9 Extraocular eye movement may be normal or abnormal. Main lesions included in the differential diagnosis are optic glioma, orbital pseudotumor, and lymphoma.8 Standard radiographs are usually not diagnostic, unless end-stage enlargement of the optic canal or hyperostosis is present. CT with contrast is an excellent imaging technique for detecting and evaluating ONSMs. Meningiomas surround the optic nerve. Radiographic intensity of the nerve is attenuated within the surrounding mass, giving a bull’s-eye appearance on coronal magnetic resonance imaging (MRI) images and a tram-track appearance on axial images. Other helpful MRI techniques include enhanced fat-suppression on T1-weighted images. Intraorbital extension of intracranial meningioma can be detected easily by contrast-enhanced CT or MRI.1,8,16 In fact, MRI eliminates bone artifacts, allowing study of intracanalicular and intracranial parts of the optic nerve as well as chiasmal extension or expansion of intracranial meningiomas on axial and coronal sections.16,17 Angiography to examine expansion of very large meningiomas plays an important role in preoperative assessment. Tumor invasion of the cavernous sinus indicates that the meningioma has crossed the internal carotid artery. Ultrasound of the orbit can reveal neurogenic tumors of the smooth surface; while the front wall of the tumor is clearly visible, the back wall
is nearly invisible due to strong attenuation within the tumor mass.
Treatment of ONSM Treatment of ONSM may involve surgery,18–23 surgery and radiotherapy, radiotherapy only,24–27 or simply follow-up observation.1,10,16 Surgical resection of orbital meningiomas usually involves partial resection supero-lateral orbitotomy and modified orbitozygomatic craniotomy.19–21 Primary radiotherapy for patients with ONSM is associated with long-term improvement of visual acuity.28–30 In fact, treatment with radiation alone or following surgical removal is associated with a better chance of visual improvement. Patients with meningiomas are good candidates for radiotherapy because the tumors are extra-axial and are easily visualized by CT or MRI. The choice of treatment should take into account several factors: tumor location and size, patient age, histologic subtype (most meningiomas are meningoendotheliomas), and visual acuity. In all cases, the purpose of the therapy is to preserve vision. The diagnosis in our patient was made by clinical findings, CT orbit scan and orbit MRI. Clinical findings, visual field examination, and ultrasound indicated no disease progression. Neuroradiological checks should normally be carried out once a year.
ACKNOWLEDGEMENTS The authors woud like to thank all their colleagues for their help and support in preparing this paper.
DECLARATION OF INTEREST The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
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