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


20 13


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 !

2014 Informa Healthcare USA, Inc.

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.

REFERENCES 1. Jain D, Ebrahimi KB, Miller NR, Eberhart CG. Intraorbital meningiomas: a pathologic review using current World Health Organization criteria. Arch Pathol Lab Med 2010; 134(5):766–770. 2. Mitchell VG. Optic nerve sheath meningioma treatment and menagement. Available from: http://emedicine. [Last accessed 30 December 2012]. 3. Harrison MJ, Wolfe DE, Lau TS, et al. Radiation-induced meningiomas: experience at the Mount Sinai Hospital and review of the literature. J Neurosurg 1991;75(4):564–574. 4. Mack EE, Wilson CB. Meningiomas induced by high-dose cranial irradiation. J Neurosurg 1993;79(1):28–31. Seminars in Ophthalmology

Optic Nerve Sheath Meningioma 5. Saeed P, Rootman J, Nugent RA, et al. Optic nerve sheath meningiomas. Ophthalmology 2003;110(10):2019–2030. 6. Badr MA, Elkhamary SM, Al Sabbagh S, Al Turjoman A. Bilateral optic nerve sheath meningioma with intracanalicular and intracranial component in a 25-year-old Saudi patient. Middle East Afr J Ophthalmol 2008;15(3):138–141. 7. Malloy KA, Chigbu DI. Anterior temporal chordoid meningioma causing compressive optic neuropathy. Optom Vis Sci 2011;88(5):645–651. 8. Deftereos SP, Karagiannakis GK, Spanoudaki A, et al. Optic nerve sheath meningioma: a case report. Cases J 2008; 1:423. 9. Wilhelm H, Do¨rr S, Paulsen F, et al. Early symptoms and findings in optic nerve meningiomas. Klin Monbl Augenheilkd 2009;226(11):869–874. 10. Eddleman CS, Liu JK. Optic nerve sheath meningioma: current diagnosis and treatment. Neurosurg Focus 2007; 23(5):E4. 11. Radhakrishnan S, Lee MS. Optic Nerve sheath meningiomas. Curr Treat Options Neurol 2005;7(1):51–55. 12. Basu K, Majumdar K, Chatterjee U, et al. En plaque meningioma with angioinvasion. Indian J Pathol Microbiol 2010;53(2):319–321. 13. Tsaousis KT, Balatsoukas D. Fusiform appearance of optic nerve sheath meningioma: correlating clinical and imaging findings. Oman J Ophthalmol 2012;5(1):55–57. 14. Jaggi GP, Mironov A, Huber AR, Killer HE. Optic nerve compartment syndrome in a patient with optic nerve sheath meningioma. Eur J Ophthalmol 2007; 17(3):454–458. 15. Mourits MP, van der Sprenkel JW. Orbital meningioma, the Utrecht experience. Orbit 2001;20(1):25–33. 16. Turbin RE, Pokorny K. Diagnosis and treatment of orbital optic nerve sheath meningioma. Cancer Control 2004; 11(5):334–341. 17. Mafee MF, Goodwin J, Dorodi S. Optic nerve sheath meningiomas: role of MR imaging. Radiol Clin North Am 1999;37(1):37–58. 18. Oya S, Sade B, Lee JH. Sphenoorbital meningioma: surgical technique and outcome. J Neurosurg 2011; 114(5):1241–1249.


2014 Informa Healthcare USA, Inc.


19. Ulivieri S, Oliveri G, Motolese I, et al. Supero-lateral orbitotomy for resection of spheno-orbital meningioma: a case report. G Chir 2011;32(3):118–119. 20. Subramanyam S, Wojno TH, Grossniklaus HE. Malignant (anaplastic) transformation of orbital clear cell meningioma. Ophthal Plast Reconstr Surg 2012;28(1):e3–e4. 21. Cohen-Gadol AA. Intraorbital meningioma: resection through modified orbitozygomatic craniotomy. J Neurosurg 2012;32(Supll):E1. 22. Boulos PT, Dumont AS, Mandell JW, Jane Sr JA. Meningiomas of the orbit: contemporary considerations. Neurosurg Focus 2001;10(5):E5. 23. Schick U, Jung C, Hassler WE. Primary optic nerve sheath meningiomas: a follow-up study. Cent Eur Neurosurg 2010; 71(3):126–133. 24. Lesser RL, Knisely JP, Wang SL, et al. Long-term response to fractionated radiotherapy of presumed optic nerve sheath meningioma. Br J Ophthalmol 2010;94(5):559–563. 25. Romanelli P, Bianchi L, Muacevic A, Beltramo G. Staged image guided robotic radiosurgery for optic nerve sheath meningiomas. Comput Aided Surg 2011;16(6):257–266. 26. Adeberg S, Welzel T, Rieken S, et al. Prior surgical intervention and tumor size impact clinical outcome after precision radiotherapy for the treatment of optic nerve sheath meningiomas (ONSM). Radiat Oncol 2011;6: 117. 27. Abouaf L, Girard N, Lefort T, et al. Standard-fractionated radiotherapy for optic nerve sheath meningioma: visual outcome is predicted by mean eye dose. Int J Radiat Oncol Biol Phys 2012;82(3):1268–1277. 28. Saeed P, Blank L, Selva D, et al. Primary radiotherapy in progressive optic nerve sheath meningiomas: a long-term follow-up study. Br J Ophthalmol 2010;94(5):564–568. 29. Smee RI, Schneider M, Williams JR. Optic nerve sheath meningiomas: non-surgical treatment. Clin Oncol (R Coll Radiol) 2009;21(1):8–13. 30. Paulsen F, Doerr S, Wilhelm H, et al. Fractionated stereotactic radiotherapy in patients with optic nerve sheath meningioma. Int J Radiat Oncol Biol Phys 2012;82(2): 773–778.

Copyright of Seminars in Ophthalmology is the property of Taylor & Francis Ltd and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.

Optic nerve sheath meningioma: a case report with 15-year follow-up.

Meningiomas are benign neoplastic lesions of arachnoidal cells of the meninges. These tumors may arise wherever meninges exists, such as in the nasal ...
300KB Sizes 0 Downloads 0 Views