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

Histology and Molecular Genetics

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Surgery in Primary Optic Nerve

6

K E Y P O I N t S

6.1

Introduction

68

Various surgical procedures, depending on the ex-

6.2

treatment Goals

68

tent of the tumor, have been practiced in patients

6.3

A subset of Data on surgery

with primary optic nerve sheath meningioma.

6.3.1

in Primary onsM

68

Extirpation, sometimes including removal of the

Demographics

68

optic nerve from chiasm to globe, have occasion-

6.3.2

Results 69

ally been suggested. Some have proposed micro-

6.3.3

Complications

69

6.4

General Indications

surgical resection, but patients risk high prob-

ability of postoperative blindness due to almost

for surgical Intervention 70

certain inadvertent dissection of the pial vascula-

6.4.1

Diagnostic Biopsy

70

6.4.1.1

Case (from Turbin

ture. PROOFSDespite cases with isolated surgical benefit,

et al. 2006)

70

the long-term experience of surgical manipulation

6.4.2

Decompression of Compartment

D

Eof ONSM is limited. Several articles addressing

Syndrome

72

t intra-cranial meningioma undergoing gross total

6.4.3

Role of Resection

74

C

resection have indicated that residual tumor re-

6.4.4

Resection as Extirpative

E

mains in situ and may even be spread during sur-

Surgery

74

R

gery. Therefore, surgical resection alone will not

6.4.5

Resection of Secondary

R

result in cure of “focal” tumors, and partial nerve

ONSM

74

CO

sheath surgery could result in orbital recurrence.

6.5

Canalicular Involvement

75

Although transient postoperative improvement

6.6

Conclusion

75

N

is well documented after relief of congestion, it

References

u75

is typically of brief duration. Extirpative surgery

is rarely required and obviously associated with

complete and irreversible loss of function as well

as potential for significant other morbidity and

mortality. Therefore, radiation therapy most likely

still represents the best treatment option for long-

term preservation of vision in ONSM patients with

progressive visual loss.

R. E. Turbin, MD, FACS

Assistant Professor, Neuro-ophthalmology and Orbital Sur-

gery, University

of Medicine

and

Dentistry, New

Jersey

Surgery in Primary Optic Nerve Sheath Meningioma

69

table 6.1. Surgical description of 33 patients undergoing surgical biopsy, subtotal or total resectiona

operation

number

Description

Needle biopsy (or ONSF†)

8

Four required additional surgical intervention (one ONSF†, two orbito-

tomy, one craniotomy)

Orbitotomy (subtotal or total)

9

Two required additional craniotomy, one required embolization and

craniotomy‡

Craniotomy (subtotal or total)

16

Four required additional surgical intervention three craniotomy, one

ONSF†

optic nerve sheath meningioma managed with observation, surgery, or surgery and radiotherapy. Ophthalmology 109:890–899

NLP at diagnosis, one had a partial resection, and four

OnlyPROOFS2 of 25 (8%) patients not treated with radia-

opsy or partial resection, 8 total resection), 18 received

surgery followed by radiation, showed at least two lines

radiation alone, 16 had surgery and radiation (14 bi-

of improvement (including improvement from light

opsy or partial resection and radiation, 2 patients total

perception to finger counting) that remained stable

resection and radiation). Of the five patients who were

through the last follow-up.

had total resection. Of the eight patients who under-

D

tion ever improved at least one line of Snellen acuity.

went needle biopsy or optic nerve sheath fenestration,

EOne patient (previously reported in 1978) with a rare

four had additional surgical procedures. Of the ninetfocal “globular” nerve sheath meningioma, had the tu-

patients undergoing subtotal or total resection by orbiC-

mor completely resected, but it recurred in the orbit,

totomy, three required further surgical procedures.E Of

although vision was preserved at 20/25 as late as 1997

the 16 patients who underwent subtotal or Rtotal exci-

(Mark et al. 1978). Another patient developed severe

sion by craniotomy (which may have includedR orbito-

worsening of vision during pregnancy, which sponta-

CO

neously improved post-partum. The tumor ultimately

tomy as well), 4 required additional surgical procedures

(Table 6.1).

N

extended intracranially, and was excised in its entirety,

u

leaving NLP vision.

In atypical cases, biopsy remains an important option

and is the commonest indication for which I operate

6.4

on these lesions. Although many lesions that mimic

General Indications

optic nerve sheath meningioma will respond to radio-

for Surgical Intervention

therapy, some inflammatory lesions require ongoing,

Diagnosis of primary ONSM is typically based on clini-

lesion specific immunosuppressive therapy. In addition,

malignant lesions exist that require systemic staging or

cal and radiographic criteria without histologic con-

other modes of systemic and local control (Fig. 6.1a,b).

firmation. Similarly, vision preserving treatment para-

Biopsy may be performed in a limited fashion via nee-

digms are primarily based on various radiotherepeutic

dle aspiration or via transorbital approaches. These

options, often without histologic confirmation. How-

techniques have been well described and are beyond the

ever, on occasion atypical clinical or radiographic char-

scope of this chapter. The biopsy itself may give tem-

acteristics lead to sufficient clinical uncertainty to re-

porary improvement in visual function that is probably

quire histologic confirmation of ONSM rather than to

short lived via decompressive effects (Kennerdell et

rely on an empiric course of corticosteroids to rule out

al. 1988; Cristante 1994; Turbin et al. 2006; Dutton

other inflammatory lesions. Therefore biopsy remains a

1992). Recurrence or spread into periorbital tissues may

reasonable option in selected cases. In other cases, le-

occur (Wright et al. 1989), and the biopsy should usu-

sions which are focal, exophytic and predominantly

ally be followed by radiotherapy after lesion confirma-

extradural may anatomically lend themselves to near

tion (Turbin et al. 2006; Saeed et al. 2003).

total resection. Surgical extirpation also remains an op-

6.4.1.1PROOFS

tion if intracranial spread threatens contralateral visual

pathways and ipsilateral recovery of visual function is

hopeless, or severe proptosis and the ocular surface

Case (from turbin et al. 2006)

abnormalities it causes are disfiguring or otherwise un-

D

E

manageable. Surgery may remain an option in selectedtA 32-year-old woman developed loss of visual acuity

patients with a constrictive compartment syndromeCof

over 5 months to 20/300 in the left eye (OS). She had

the intraorbital optic nerve causing congestiveEoptic

1 mm of left proptosis, mild resistance to retropul-

nerve edema with macular edema. Finally, surgeryR

re-

sion, and a 4+ RAPD OS. The funduscopic examina-

mains a viable option in patients with secondaryR

men-

tion revealed disc edema of the left optic nerve with

ingioma of nearby intracranial structures that affect the

telangiectasia. A high-resolution MRI showed diffuse

optic nerve. Each of these clinical indications are subse-

enhancement of the intra-orbital left optic nerve sheath

quently discussed in more detail.

CO

complex. The optic nerve substance, rather than the

N

u

Surgery in Primary Optic Nerve Sheath Meningioma

71

Surgery in Primary Optic Nerve Sheath Meningioma

73

a

b

Fig. 6.5. a Preoperative fundus photograph showing severe

assumed to be related to perioptic congestion from the con-

congestive disc edema and macular exudation approximately

PROOFS

strictive orbital meningioma. b Significant resolution of optic

2 years after receiving radiotherapy. Visual acuity dropped to

nerve and macular edema with return of visual acuity to 20/25.

20/200, and was accompanied by progressive intensity of an

Adapted from Figure 1 of Turbin RE, Wladis EJ, Frohman LP,

ipsilateral afferent pupillary defect. Typical vascular changes

et al. Role for surgery as adjuvant therapy in optic nerve sheath

of radiation retinopathy were lacking, and the findings were

meningioma. Ophthal Plast Reconstr Surg 2006;22(4):278–82

D

E

t

Fig. 6.7. Fronto-temporal craniotomy with unroofing of the

6.4.5

Resection of Secondary ONSM

left orbit allows for exposure of the intraorbital optic nerve

complex. The scalloped, cut edge of the orbital roof is marked

Discussion of this topic is important to contrast pri-

by white arrowheads, and is located at the top of the photo-

graph. The levator superioris is retracted laterally with a trac-

mary and secondary ONSM. The clinical similarities

tion suture. The superior rectus is marked by a small white ar-

and differences of primary and secondary ONSM are

row, and the frontal lobe is retracted underneath the malleable

important to consider because differences between

retractor at the bottom of the image (long white arrow). Cour-

these two entities emphasize fundamental differences in

tesy of Paul Langer, MD

treatment strategies. These two entities may be difficult

to clinically differentiate, present with similar manifes-

tations, and are frequently discussed together by au-

thors. DifferencesPROOFSin lesion architecture and especially

anatomic location lend support to a greater surgical role

6.4.3

D

Ein patients with secondary ONSM. The same is not typi-

Role of Resection

tcally true of primary ONSM, nor secondary ONSM that

C

invades the intraorbital nerve sheath or its pial vascula-

Predominantly extradural, focal nerve sheath lesionsE or

ture. Delineation of the surgical goals therefore remain

ectopic orbital tumors are very rare but may beRresected,

remains an important concept in selecting surgical can-

leaving a rim of meningeal tissue that doesRnot disrupt

didates.

pial blood supply. In fact, the most commonly cited case

In experienced hands, partial tumor resection is

reported by Mark et al. (1978) developed local recur-

possible with good visual outcome in patients with in-

CO

tracranial meningioma that secondarily affect the optic

rence (without visual loss), and was re-reported in a

N

nerve; this disease remains a surgical entity (Schick

later publication (Turbin et al. 2002). Other authors

u

et al. 2004). Schick and colleagues developed a clas-

report total excision with improvement of vision and no

tumor recurrence (Saeed et al. 2003).

sification based on tumor location which emphasizes

In cases of primary ONSM, patients must be care-

location of secondary ONSM that may be amenable to

fully selected, consented for complete visual loss, and

partial resection. These authors provide impressive evi-

approached via transorbital or transcranial extradural

dence that their group could decompress the intracra-

orbitotomy to allow microsurgical techniques using the

nial optic canal from a pterional intradural (54 patients)

operating microscope.

or extradural approach (10 patients) with relative safety

and relatively low incidence (21.7%) of iatrogenic visual

6.4.4

loss. The authors reported a transient nonvisual mor-

bidity of 12.3% and permanent morbidity of 2.7%, with

Resection as Extirpative Surgery

5 cases of worsening vision in each of 2 groups of 29

patients with good vision (Snellen >0.5) and 17 with fair