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Shikher Shrestha
History..
Cushing – reduced morbidity and mortality by intracapsular removal; however with high
recurrence rate
History
pyramid anteriorly
tent superiorly
cerebellum dorsally
CP angle Anatomy
Contains:
CN V, VI, VII and VIII
SCA and AICA
variable number of draining veins
flocculus of the cerebellum
choroid plexus that protrudes through the foramen of Luschka
VII Nerve exits from the lateral part
of the pontomedullary sulcus;
1-2 mm
anterior to the entry zone of VIII nerve.
Facial
Vestibular (superior and inferior) vii sv
Cochlear and
Nervus intermedius Ant
(accompanied by labyrinthine artery and
occasionally by branches of AICA or coch iv
a loop of the AICA itself)
Histopathology of Vestibular Schwannoma
Things to consider:
site of origin
location
Cystic
Epidermoid cysts, arachnoid cysts, Neurenteric cyst
Containing Fat
Dermoid cyst, Lipoma
Intrinsic brain tumor with a significant exophytic extension into the CP angle may be
difficult to differentiate from an extra axial lesion based on imaging
Bone window thin slice CT: bony changes of the pyramid and IAC – essential in surgical
planning
Gadolinium contrast:
intense and homogenous enhancement with exception of cystic portion
T1-weighted images:
isointense to slightly hypointense to brain parenchyma
T2-weighted images:
higher intensity than VS and with homogenous contrast enhancement
Criteria for radiological differential between VS and CPA
meningioma
Meningiomas are
IAC not widened and tumor very rarely extends into the IAC (secondary invasion of
the IAC might be observed in 10-20%)
Criteria for radiological differential between VS and CPA
meningioma
Tail of enhancement along the dura (the dural ‘tail’ sign) – 60-70% meningiomas
Hypodense on CT
Signal intensity related to cyst content. Eg. If cholesterol predominates, the cysts are
hyperintense on T1 weighted images and hypointense on T2
MRI with and without contrast; FIESTA MRI if available (Fast Imaging Employing Steady
State Acquisition): uses CSF as the contrast agent and NOT gadolinium
Audiometric evaluation
aka BAER
poor wave morphology correlates with lower chance of preserving hearing (even
with good hearing).
Pure Tone Audiogram (PTA)
PTA assesses the functionality of hearing (to help in treatment decision making) +
baseline for future comparison
The single numerical score is an average of the thresholds for frequencies across the audio
spectrum
Pure Tone Audiogram (PTA)
Xs denote the left ear (AS) and Os denote the right ear (AD)
DDx: age related and noise related hearing loss (usually symmetrical)
Open set word recognition score (WRS) is a more sensitive measure of communication
ability than PTA.
Gardener and Robertson modified hearing classification
(modification of Silverstein and Norrell system)
Class A & B are considered “useful”, Class C is “aidable”, & Class D is “non functional”
Definition of serviceable hearing
If WRS is good (> or = 70) but PTA is poor, a hearing aid may provide significant benefit.
Definition of serviceable hearing
1. AAO-HNS class A or B
4. In a patient with good hearing in the contralateral ear, a speech discrimination score
(SDS) of <70% in the affected ear is not considered good hearing; whereas if the
contralateral ear is totally deaf, a SDS of > or = 50% can be useful.
Addendum to CT scan imaging…
Small lesions may be visualized by introducing 3-4 ml of subarachnoid air via lumbar
puncture
Scanning is done with the affected side up (to trap air in region of IAC)
Although many VSs enlarge the ostium of the IAC (called trumpeting) (normal diameter
of the IAC is 5-8 mm), 3-5% of VSs do not enlarge the IAC on CT
Advantage over MRI: bony anatomy delineation and thus, useful for planning
translabyrinthine approach.
Vestibular Schwannomas
Originates from the transition zone between central and peripheral myelin that usually is
located in the medial part of the IAC – Obersteiner Reidlich zone
2 different forms:
SPORADIC NF 2
Presents at 5th or 6th decade Usually younger at presentation
Predisposition to multiple tumors like
meningiomas, ependymomas,
neurofibromas
Autosomal dominant inheritance
Mutation in chromosome 22q12: affects
gene encoding schwannomin/merlin:
implicated in cellular remodeling and
growth regulation
Tumors just displaces VIIIth nerve without NF2 tumors form grape like clusters that
infiltrating may infiltrate the nerve fibers
VS progression – Four Stage Concept of Growth
Hydrocephalic
stage with
Compresses the obstruction of
brainstem and IV th ventricle
the cerebellum and its outlets
Occupy the CP
angle cistern,
Gradually fills the displacing VII
IAC first and VIII cranial
nerves and AICA
Intrameatal:
vestibulocochlear dysfunction: hearing loss, tinnitus or vestibular dysfunction
Hearing loss is insidious and high frequency sensorineural type
Cisternal stage:
progressive hearing loss
sense of dysequilibrium gradually replaces vertigo
Later stage:
trigeminal symptoms, headache, ataxia and obstructive hydrocephalus
Symptomatolgy and signs
Sign %
Nystagmus 26
contralateral long tract signs
Papilledema 10
Babinski sign 5
Classification of VS based on size or degree of extension into
CP angle
According to size:
Intrameatal
up to 1 cm
1 to 2.5 cm
from 2.5 to 4 cm
larger than 4 cm
INI (International Neuroscience Institute) or Samii
Classification of Vestibular Schwannoma extension:
Expectant management
Radiation therapy
Surgery
Chemotherapy
Expectant management
a. little or no growth: applies to most (83%) VSs confined within the IAC and 30%
extending into CPA
b. Slow growth ~2 mm/yr
c. Rapid growth > or = 10 mm/yr
d. A few actually shrink
Intracanalicular or CPA tumors < or = 20 mm diameter that are non cystic and non NF2:
observation with serial imaging and hearing tests
Presence of cysts: cystic tumors may display sudden and dramatic growth; high recurrence
Q6mo for 2 yrs; if stable then annually until year 5 after diagnosis
b. Stereotactic radiation
Translabyrinthine (and its several variations): sacrifices hearing, may be slightly better for
sparing VII
Middle fossa approach (extradural subtemporal): only for small lateral VSs
Sacrifice hearing
Restricted access to the CP angle
Difficult dissection and hemostasis close to the brainstem
Poor visualization and access to the caudal cranial nerves
RETROSIGMOID APPROACH: Fedor Krause, 1903
Equipment:
microscope
ultrasonic aspirator
image guided navigation system
complications: CSF leak with possible meningitis, loss of hearing in ipsilateral ear (if
not already lost), paralysis of facial muscles on the side of surgery with possible need
for surgical procedures to help correct (correction is often far from perfect), post-op
balance difficulties/vertigo, brainstem injury with stroke.
Technique Summary..
Incision is shaped like the pinna of the ear, 3 finger breadths behind the external auditory
canal
The craniotomy has to be lateral enough to expose part of the sigmoid and part of the
transverse sinuses.
To prevent CSF leak, seal all bone edges with bone wax
Dural opening Y shaped making base to sinuses
Exposure is enhanced by opening the CP angle cistern and the cisterna magna under the
microscope and draining CSF (20-40 ml of CSF can also be drained via a lumbar
subarachnoid catheter)
The petrosal vein is often sacrificed at the beginning of the procedure to allow the
cerebellum to relax and fall back and to avoid tearing off the transverse sinus. Be careful
not to coagulate the SCA that often runs with the petrosal vein.
Using the facial nerve stimulator, the posterior aspect of the tumor is inspected to make
sure the facial nerve has not been pushed posteriorly
The thin layer of arachnoid that covers most tumors is identified. Vessels within the
arachnoid may contribute to cochlear function and may be preserved by keeping them
with the arachnoid.
The plane between tumor and cerebellum may be followed to the brainstem, and
occasionally to the VII nerve (this plane is harder to follow once bleeding from tumor
debulking occurs)
Posterolateral tumor capsule is opened, and internal decompression is performed. The
tumor is collapsed inward and the capsule is kept intact and is rolled laterally off of VII
and is eventually removed.
The most difficult area to separate VII from tumor is just proximal to the entrance to the
porus acousticus.
Large tumors: capsule adherent to brainstem portion must be left; recurrence rate: 10-
20% may involve V superiorly and IX, X, XI inferiorly; these cranial nerves are spared
by protecting with cottonoids
After extracanalicular portion of tumor is removed, the dura over the IAC incised
Meningiomas located lateral to trigeminal nerve regardless of the site of dural attachment
are termed CP angle meningiomas
Arise from groups of arachnoid cells located in high concentration around the IAC, lower
border of superior petrosal sinus, lateral border of inferior petrosal sinus, around cranial n.
foramina and in the region of the geniculate ganglion.
Classified as premeatal and retromeatal in relation to the IAC
The more medially located the tumor, the greater is the surgical challenge and the poorer is
the outcome
Tinnitus 10-12%
Hydrocephalus 20-31%
Three percent of the cases of trigeminal neuralgia are due to tumor compression at the
root entry zone
Premeatal tumors are diagnosed earlier and consequently are smaller. Their clinical
presentation is with trigeminal signs and facial and cochlear nerve signs
Principles:
adequate exposure
interruption of the blood supply along the dural attachment
internal decompression
cautious dissection of the tumor capsule from the brainstem and cranial nerves at the
arachnoid plane
With the retrosigmoid approach hearing is preserved in 82-90.8% of the patients
Compared to VS, CP angle meningiomas have higher tendency toward recurrence, with a
recurrence rate between 0-9.5%
Surgery may still be required in case of treatment failure or secondary trigeminal neuralgia
after irradiation
Epidermoids
Might expand through the tentorial incisura into the middle cranial fossa, grow toward the
contralateral CP angle, or extend toward the foramen magnum
Usually present between the third and fifth decades of life with long standing history of
tinnitus and hearing loss.
Cause relatively more frequently trigeminal neuralgia or hemifacial spasm when compared
to VS and meningiomas; lead to facial nerve signs much earlier than VS.
Treatment of choice is surgical and preferred approach is retrosigmoid
Small remnants of capsule firmly attached to important structures should not be removed
for risk of additional neurological deficit
Subtotal resected epidermoid have late recurrence up to 20-30 years and thus mandates
close follow up on a long term basis.
Thank you!!!