Ординатура / Офтальмология / Английские материалы / Primary Optic Nerve Sheath Meningioma_Jeremic, Pitz_2008

Clinical Evaluation of Primary Optic Nerve Sheath Meningioma

33

more common causes of an optic neuropathy, a pale, or

3.6

a swollen optic nerve in appropriate age groups. Each

A Suggested Approach to Clinical Evaluation

of these potential mimics therefore requires appropri-

Unfortunately, there is no “magic” bullet to identify the

ate historical support, demonstration of typical clini-

cal course, or confirmation of absence of compressive

subtle ONSM, although a high quality MRI with orbital

intracranial lesion on clinical or radiographic grounds.

fat suppression and gadolinium contrast nears the tar-

Appropriate history, review of symptoms, and iden-

get. However, ONSM is a rare tumor and purchasing a

tification of systemic disease known to be risk factors

neuroimaging study for all cases of optic neuropathy is

to these more common entities is therefore time con-

neither cost efficient, safe, nor an effective practice of

suming but imperative. The pale or chronically swollen

medicine. Few cases present with the classic triad, and

optic nerve associated with worsening visual function

other forms of optic atrophy may be indistinguishable

or subacute progressive visual loss must be identified,

on clinical grounds in more subtle cases. Yet the clini-

and requires further evaluation as it may harbor the

cian is charged with appropriate identification and diag-

unexpected compressive, inflammatory, or infiltrative

nosis of these lesions prior to the occurrence of irrevers-

lesion.

ible visual loss. The physician, therefore, must develop a

protocol to effectively sort out high risk presentations of

3.6.2

optic neuropathy from more indolent forms. Again, be-

cause ONSM is a very uncommon entity, defining these

Physical Examination

strategies will serve the physician well and may even be

The physical examination in patients identified to man-

more relevant to identification of many other progres-

sive optic neuropathies. I strongly believe that the cli-

ifest an optic neuropathy should be painstakingly em-

nician is not always required to identify and diagnose

piric, quantifying current level of function to provide

ONSM at first clinical presentation given the similarity

benchmarks for future identification of potential visual

in presentation in most cases to other more “mundane”

loss. APROOFScareful, complete ophthalmologic examina-

and more common forms of optic neuropathy. The cli-

tion is in order, as in all cases of optic neuropathy, and

nician is charged with identifying and ultimately diag-

D

Eshould quantify best potential corrected visual acuity,

nosing these cases early as patients begin to manifesttkinetic or static visual field testing, color vision testing,

continued and progressive visual dysfunction. It isCthe

pupil testing, tonometry, as well as all components of a

follow-up, therefore, that is key to differentiatingEthese

slit lamp biomicroscopic and fundoscopic examination,

clinical entities in cases that are not “obvious.”R

including optic nerve head appearance and presence or

R

absence of nerve fiber layer loss. Ocular motility test-

3.6.1

CO

ing, prism measurement of ocular misalignment, ex-

ophthalmometry, detection of resistance to ocular ret-

History

ropulsion and assessment of cranial nerve testing are all

N

germane. There is simply no substitute for an accurate,

u

quantitative, complete examination. Precise assessment

With few exceptions, most cases of sudden, severe, or

painful visual loss that result in urgent patient visits are

of visual acuity is especially important. The notation

not related to primary ONSM. This generalization may

“finger counting” is imprecise, and a more quantitative

not apply during pregnancy, in which more fulminant

angular assessment such as 20/800, 20/1000, etc. pro-

or even painful visual loss simulating optic neuritis oc-

vides more information for future evaluation. Similarly,

curs, although remains exceedingly uncommon. ONSM

the measure of the depth of relative afferent pupil with

cases are typically identified after numerous clinical vis-

neutral density filters may provide a baseline for future

its, previous non-diagnostic imaging studies, or failed

comparison, and is a cost effective, rapid, and efficient

surgical interventions. I have seen a number of patients

alternative to pupillography which is currently primar-

who failed to improve after cataract extraction (with

ily available only as a research technique.

misidentification of underlying optic neuropathy), or

who developed progressive visual loss after optic nerve

3.6.3

pallor was incorrectly attributed to the assumption of

past “unobserved” disc swelling. On the other hand,

Ancillary testing

optic neuritis, non-arteritic ischemic optic neuropa-

Ophthalmology and radiology are technologically

thy, arteritic ischemic optic neuropathy, inflammatory

or autoimmune optic neuropathy are each very much

driven fields, and continuous advancements in imag-

Clinical Evaluation of Primary Optic Nerve Sheath Meningioma

35

tributed to other causes after months of observation are

Créange A, Zeller J, Rostaing-Rigattieri S et al. (1999) Neuro-

suspect. On the other hand, patients who have not un-

logical complications of neurofibromatosis type 1 in adult-

dergone biopsy and have lesions presumed to be ONSM

hood. Brain 122:473–481

based on clinical course and radiographic appearance

Cristante L (1994) Surgical treatment of meningiomas of the

may harbor other lesions (see Sect. 3.2.2).

orbit and optic canal: a retrospective study with particular

Occasionally, the physician is faced with significant

attention to the visual outcome. Acta Neurochir (Wien)

126(1):27–32

clinical uncertainty based on atypical appearance, clini-

Cunliffe IA, Moffat DA, Hardy DG, Moore AT (1992) Bilateral

cal or radiographic progression. If

additional testing

optic nerve sheath meningiomas in a patient with neurofi-

fails to identify concurrent telltale disease the clinician

bromatosis type 2. Br J Ophthalmol 76:310–312

may be forced by clinical uncertainty to utilize a brief

Cushing H, Eisenhardt L (1938) Meningiomas: their classifi-

corticosteroid trial or resort to a conservative, limited

cation, regional behavior, life history and surgical end re-

biopsy. Indications for surgery are addressed in a sub-

sults. Charles C. Thomas, Springfield, Ill, pp 250–282

sequent chapter.

Dutton JJ (1992) Optic nerve sheath meningiomas. Surv Oph-

thalmol 37(3):167–183

Egan RA, Lessell S (2002) A contribution to the natural history

References

of optic nerve sheath meningiomas. Arch Ophthalmol

120(11):1505–1508

Alper MG (1981) Management of primary optic nerve menin-

Elschnig A (1898) Ueber opticociliare Gefasse. Klin Mbl Au-

genheilk 36:93–96

giomas. J Clin Neuro-ophthalmol 1:101–117

Evans DG, Moran A, King A et al. (2005) Incidence of ves-

Alper MG, Sherman JL (1989) Gadolinium enhanced mag-

tibular schwannoma and neurofibromatosis 2 in the North

netic resonance imaging in the diagnosis of anterior vi-

West of England over a 10-year period: higher incidence

sual pathway meningiomas. Trans Am Ophthalmol Soc

than previously thought. Otolol Neurotol 26(1):93–97

87:384–419

Frasier BS, Green RL (2002) Ultrasound of the eye and orbit,

Als E (1969) Intraorbital

meningiomas

encasing

the optic

2nd edn. Mosby, St. Louis, p 424

nerve. Acta Ophthalmol 47:900–903

Frisen L,PROOFSHoyt WF, Tengroth BM (1973) Optociliary veins, disc

Amoli FA, Mehrabani PM, Tari AS (2007) Aggressive orbital

pallor and visual loss: a triad of signs indicating spheno-

optic nerve meningioma with benign microscopic fea-

D

tures: a case report. Orbit 26(4):271–274

E orbital meningioma. Acta Ophthalmol 51:241–249

Andrews DW, Faroozan R, Yang BP (2002) Fractionated stet-

Frohman LP, Guirgis M, Turbin RE, Bielory L (2003) Sarcoido-

reotactic radiotherapy for the treatment of optic nerveC

sis of the anterior visual pathway: 24 new cases. J Neuro-

sheath meningiomas: preliminary observations ofE33 op-

Ophthalmol 23(3):190–197

tic nerves in 30 patients with historical comparison to

Hartmann E (1933) Enlargissement du canal optique visible à

R

la radiographie chez les malades atteints de neurofibroma-

observation with or without prior surgery. Neurosurgery

tose avec tumeur du nerf optique. J Belge Neurol Psychiatr

51(4):890–902

R

33:763–772

Becker G, Jeremic B, Pitz S et al. (2002) Stereotactic fraction-

ated radiotherapy in patients with opticCOnerve sheath men-

Hirst LW, Miller NR, Allen GS (1979) Sphenoidal pneumosi-

ingioma. Int J Radiat Oncol BiolNPhys 54(5):1422–1429

nus dilatans with bilateral optic nerve meningiomas. Case

Bosch MM, Wichmann WW, Boltshauser E, Landau K (2006)

report. J Neurosurg 51(3):402–407

u

Imes RK, Schatz H, Hoyt WF et al. (1985) Evolution of op-

Optic nerve sheath meningiomas in patients with neurofi-

tociliary veins in optic nerve sheath meningioma. Arch

bromatosis type 2. Arch Ophthalmol 124(3):379–385

Ophthalmol 103(1):59–60

Castel A, Boschi A, Renard L et al. (2000) Optic nerve sheath

Ito M, Ishizawa A, Miyaoka M et al. (1988) Intraorbital men-

meningiomas: clinical

features, functional

prognosis

ingiomas: surgical management and role of radiation ther-

and controversial treatment. Bull Soc Belge Ophtalmol

275:73–78

apy. Surg Neurol 29:448–453

Jackson A, Patankara T, Laitt L (2003) Intracanalicular optic

Cibis GW, Whittaker CK, Wood WE (1985) Intraocular exten-

nerve meningioma: a serious diagnostic pitfall. Am J Neu-

sion of optic nerve meningioma in a case of neurofibroma-

roradiol 24:1167–1170

tosis. Arch Ophthalmol 103:404–406

Jakobiec FA, Depot MJ, Kennerdell JS et al. (1984) Combined

Clark WC, Theofilos CS, Fleming JC (1989) Primary optic

clinical and computed tomographic diagnosis of orbital

nerve sheath meningiomas. Report of nine cases. J Neu-

glioma and meningioma. Ophthalmology 1:137–155

rosurg 70:37–40

Karp LA, Zimmerman LE, Borit A et al. (1974) Primary in-

Cooling RJ, Wright JE (1979) Arachnoid hyperplasia in optic

traorbital meningioma. Arch Ophthalmol 91:24–28

nerve glioma: confusion with orbital meningioma. Br J

Kennerdell JS, Maroon JC (1975) Intracanalicular menin-

Ophthalmol 63(9):596–599

gioma with chronic optic disc edema. Ann Ophthalmol

Coston T (1936) Primary tumor of the optic nerve. With a re-

7(4):507–512

port of a case. Arch Ophthalmol 15:696–702

36

R. E. Turbin and J. S. Kennerdell

Kondziolka D, Mathieu D, Lunsford LD et al. (2008) Radio-

Saeed P, Rootman J, Nugent RA et al. (2003) Optic nerve sheath

surgery as definitive management of intracranial menin-

meningiomas. Ophthalmology 110(10):2019–2030

giomas. Neurosurgery 62(1):53–58

Salzmann M (1893) Zur Anatomie der angeborenen Sichel

Laitt RD, Kumar B, Leatherbarrow B et al. (1996) Cystic optic

nach innen-unten. Albrecht von Grefes Arch Ophtal

nerve meningioma presenting with acute proptosis. Eye

39:131–150

10:744–746

Schick U, Hassler W (2005) Surgical management of tuber-

Larson JJ, van Loveren HR, Balko MG, Tew JM Jr (1995)

culum sellae meningiomas: involvement of the optic ca-

Evidence of meningioma infiltration into cranial nerves:

nal and visual outcome. J Neurol Neurosurg Psychiatry

clinical implications for cavernous sinus meningiomas. J

76(7):977–983

Neurosurg 83(4):596–599

Schittkowski M, Hingst V, Stropahl G et al. (1999) Optic nerve

Lindblom B, Norman D, Hoyt WF (1992a) Perioptic cyst dis-

sheath meningioma with intraocular invasion–a case re-

tal to optic nerve meningioma: MR demonstration. Am J

port. Klin Monatsbl Augenheilkd 214(4):251–254

Neuroradiol 13(6):1622–1624

Sen C, Hague K (1997) Meningiomas involving the cavernous

Lindblom B, Truwit CL, Hoyt WF (1992b) Optic nerve sheath

sinus: histological factors affecting the degree of resection.

meningioma. Definition of intraorbital, intracanalicular,

J Neurosurg 87(4):535–543

and intracranial components with magnetic resonance

Shapland CD, Greenfield JG (1935) Case of neurofibromato-

imaging. Ophthalmology 99(4):560–566

sis with meningeal tumor involving left optic nerve. Trans

Mathiesen T, Kihlström L (2006) Visual outcome of tubercu-

Ophthalmol Soc U K 55:257–279

lum sellae meningiomas after extradural optic nerve deShuanshoti S (1973) Meningioma of the optic nerve. Br J Oph-

compression. Neurosurgery 59(3):570–576

thalmol 57(4):265–269

Maxwell M, Galanopoulos T, Neville-Golden J et al. (1993)

Spencer W (1972) Primary neoplasms of the optic nerve and

Expression of androgen and progesterone receptors in pri-

its sheaths: clinical features and current concepts of the

mary human meningiomas. J Neurosurg 78:456–462

pathogenetic mechanisms. Trans AM Ophthamol Soc

McNab AA, Wright JE (1989) Cysts of the optic nerve. Three

70:490–528

cases associated with meningioma. Eye 3:355–359

Susac JO, Smith JL, Walsh FB (1977) The impossible menin-

Mehra KS, Khanna SS, Dube B (1979) Primary menin-

gioma. Arch Neurol 34:36–38

D

gioma of the intraorbital optic nerve. Ann Ophthalmol

SwensonPROOFSSA, Forbes GS, Younge BR, Campbell RJ (1982) Ra-

11(5):758–760

E

diologic evaluation of tumors of the optic nerve. Am J

Miller NR (2006) New concepts in the diagnosis and manage-

Neuroradiol 3:319–326

t

Tan FL (1989) CT scan and clinical diagnosis of parasellar le-

ment of optic nerve sheath meningioma. J Neuroophthal-

mol 26(3):200–208

C

sions – analysis of 91 cases. Zhonghua Zhong Liu Za Zhi

E

Miller NR, Golnik KC, Zeidman SM et al. (1996) Pneumosi-

11(4):291–293

R

Trobe JD, Glaser JS, Post JD, Page LK (1978) Bilateral optic ca-

nus dilatans: a sign of intracranial meningioma. Surg Neu-

rol 46(5):471–474

CO

nal meningiomas: a case report. Neurosurgery 3(1):68–74

Newell FW, Beamon TC (1958) Ocular signs ofRmeningiomas.

Trobe JD, Glaser JS, Cassady JC (1980) Optic atrophy. Differ-

Am J Ophthalmol 45:30–40

ential diagnosis by fundus observation alone. Arch Oph-

N

thalmol 98(6):1040–1045

Ortiz O, Schochet SS, Kotzan JM et al. (1996) Radiologic-

pathologic correlation: meningioma of the optic nerve

Turbin RE, Pokorny K (2004) Diagnosis and treatment of

u

orbital optic nerve sheath meningioma. Cancer Control

sheath. Am J Neuroradiol 17(5):901–906

Parker H (1922) A case of von Recklinghausen’s disease with

11(5):334–341

involvement of the peripheral nerves, optic nerve and spiTurbin RE, Thompson CR, Kennerdell JS, Cockerham KP, Ku-

nal cord. J Nerv Ment Dis 56:441–452

persmith MJ (2002) A long-term visual outcome compari-

Peele KA, Kennerdell JS, Maroon JC et al. (1996) The role of

son in patients with optic nerve sheath meningioma man-

postoperative irradiation in the management of sphenoid

aged with observation, surgery, radiotherapy, or surgery

wing meningiomas. A preliminary report. Ophthalmology

and radiotherapy. Ophthalmology 109(5):890–899

103(11):1761–1766

Turbin RE, Wladis EJ, Frohman LP et al. (2006) Role for sur-

Probst C, Gessaga E, Leuenberger AE (1985) Primary menin-

gery as adjuvant therapy in optic nerve sheath menin-

gioma of the optic nerve sheaths: case report. Ophthalmo-

gioma. Ophthal Plast Reconstr Surg 22(4):278–282

logica 190(2):83–90

van Tilborg AA, Al Allak B, Velthuize SC et al. (2005) Chro-

Ringel F, Cedzich C, Schramm J (2007) Microsurgical tech-

mosomal instability in meningiomas. J Neuropathol Exp

nique and results of a series of 63 spheno-orbital menin-

Neurol 64(4):312–322

giomas. Neurosurgery 60(4 Suppl 2):214–221

Walsh F (1970) Meningiomas primary within the orbit and op-

Rootman J (2003) Diseases of the orbit. A multidisciplinary

tic canal. In: Smith JL (ed) Neuro-ophthalmology Sympo-

approach, 2nd edn. Lipincott Williams & Wilkins, Phila-

sium of the University of Miami and the Bascom Palmer

delphia, pp 232–240

Eye Institute. Mosby, St Louis, Mo, pp 240–266

Rosca TI, Carstocea BD, Vlãdescu TG et al. (2006) Cystic

Wan WL, Geller JL, Feldon SE et al. (1990) Visual loss caused

optic nerve sheath meningioma. J Neuroophthalmol

by rapidly progressive intracranial meningiomas during

26(2):121–122

pregnancy. Ophthalmology 97:18–21

Clinical Evaluation of Primary Optic Nerve Sheath Meningioma

37

D

PROOFS

E

t

C

E

R

R

CO

N

u

Imaging Diagnosis of the Optic

4

K E Y P O I N t S

4.1

Introduction

39

Optic nerve sheath meningiomas classically are

4.2

Clinical Presentation 40

seen in middle-aged or elderly females and pres-

4.3

Imaging Features

40

ent as slowly progressing axial proptosis and

4.5.1

Optic Nerve Glioma

49

imagingPROOFSis an essential diagnostic tool and mag-

4.3.1

Computed Tomography

gradual loss of vision. In addition to the appro-

Imaging 40

priate clinical presentation, the diagnosis of optic

4.3.2

Magnetic Resonance Imaging

42

nerve sheath tumors falls heavily on the imaging

4.4

special situations

46

findings, especially given the complexity of their

4.5

Differential Diagnosis

location and the considerable morbidities that

of optic nerve enlargement

46

may be associated with biopsy. Cross-sectional

D

4.6

Miscellaneous

51

netic resonance (MR) imaging in particular, with

Ethe increasing clinical availability of 3-Tesla mag-

References 53

t nets, remains the modality of choice for the imag-

C

ing diagnosis of optic nerve pathology. Computed

E

tomography (CT), however, is not without its ad-

R

vantages and can often reveal characteristic find-

R

ings to help make the diagnosis. Thus, knowledge

CO

of the MR and CT imaging characteristics of optic

N

nerve sheath meningiomas, in conjunction with a

suitable clinical context, is essential to accurate di-

u

agnosis and differentiation from other similar ap-

Imaging Diagnosis of the Optic Nerve Sheath Meningioma

41

42

M. F. Mafee and J. H. Naheedy

and at times may be seen in normal subjects. The CT

appearance of pneumosinus dilatans in optic nerve

sheath meningioma or periforaminal meningioma may

be seen as an expanded (“blistering”) posterior ethmoid

air cell or a pneumatized anterior clinoid.

Less conventional imaging methods, such as CT

cisternography, may also lead to diagnosis. Fox et al.

(1979) reported a very unusual case of tiny meningioma,

detected by intrathecal metrizamide cisternography.

The tumor was subsequently removed with a return

to normal vision of the previously blind eye. However,

with the advent of MRI, even small optic nerve sheath

meningiomas can be readily visualized without need for

intrathecal contrast injection to perform CT cisternog-

raphy.

4.3.2

Fig. 4.3. Optic nerve sheath meningioma. Axial CT shows

Magnetic Resonance Imaging

diffuse calcifications along the optic nerve sheath (arrows). The

Despite its decreased sensitivity for detecting calcifi-

meningioma itself is seen as a fusiform mass surrounding the

optic nerve

cation (relative to CT), magnetic resonance (MR) im-

aging remains the modality of choice for the imaging

diagnosis of optic nerve sheath meningioma, spheno-

without focal or diffuse enlargement of the optic nerve)

orbital PROOFSmeningioma and other optic nerve pathologic

conditions. On MR imaging, meningiomas can be seen

may be present. While calcification in meningiomas

D

Eas a localized (Fig. 4.5), diffuse or fusiform enlarge-

is not uncommon, in very rare instances, optic nervetment of the optic nerve sheath complex (Fig. 4.6) and

gliomas may also demonstrate calcification. However,C

may be eccentric (Fig. 4.7). The tumor retains an isoin-

Greenberg (1998) reported that idiopathic opticEnerve

tense appearance to the optic nerve and brain tissue

sheath calcifications can indeed occur.

R

on most MRI imaging pulse sequences (Fig. 4.4). The

Post-contrast images typically show Rhomogenous

T1-weighted (T1W) and T2-weighted (T2W) MR im-

and well-defined contrast enhancement. The classic CT

ages usually show no significant differences in signal

finding of a “Tram-Track” sign refers to the central lu-

intensity of meningiomas compared with that of the

CO

normal optic nerve or brain tissue. Compared with the

cency of the optic nerve pancaked between an enlarged

N

brain, however, meningiomas may also be hypointense

and enhanced optic nerve-sheath complex. However,

u

on T1W and proton-weighted (PW) MR images and

while this was originally described in optic nerve sheath

meningiomas, it is not specific for this entity as it may

hyperintense or even hypointense (depending on calci-

also be seen in CT scans of pseudotumors, lymphoma,

fication or pact fibroblastic histologic feature) on T2W

sarcoidosis, and leptomeningeal carcinomatosis. Men-

MR images.

ingiomas surround the optic nerve, and thus the caliber

Gadolinium-based contrast enhanced fat-suppres-

of the nerve itself is attenuated within the surrounding

sion T1W pulse sequences have made a significant con-

tumor. This is in contrast to the optic nerve gliomas,

tribution to the orbital imaging and are widely considered

where the nerve itself appears expanded. This feature is

the gold-standard examination for evaluation of

best appreciated in coronal sections, particularly on MR

disorders of the optic nerve (Mouton et al. 2007). T1W

images (Fig. 4.4).

MR images obtained following IV injection of the gad-

Sphenoid pneumosinus dilatans is an additional

olinium-based contrast material demonstrate moderate

CT sign that may be associated with intracanalicular

to marked contrast enhancement of meningiomas

optic nerve sheath meningiomas as well as spheno-

(Figs. 4.4–4.7). Post contrast enhanced fat suppression

orbital meningiomas (Hirst et al. 1982). The involved

T1W MR images are most valuable for defining and

expanded sinus may be the ethmoid, sphenoid, or

enhancing optic nerve pathology (Fig. 4.8).

frontal-sinuses. However, this is relatively nonspecific

Imaging Diagnosis of the Optic Nerve Sheath Meningioma

43

a

b

c

d

e

f