CASE REPORT

Full Recovery of Severe Vertigo in Patient with Bilateral Cerebellopontine

Angle Arachnoid Cyst Following Microsurgical Treatment

Aji Setia Utama1, Asra Al Fauzi2

1
Resident of Department of Neurosurgery, Dr. Soetomo Hospital; Airlangga University Surabaya, Indonesia
2
Staff of Department of Neurosurgery, Dr. Soetomo Hospital; Airlangga University Surabaya, Indonesia

Abstract

Arachnoid cysts are benign, rare. These benign cysts, which contain cerebrospinal fluid, develop in the intra-
arachnoid space. Even the pathogenesis of those cysts is unknown; they are thought to be congenital. Symptoms are
produced by the mass effect of the cyst on surrounding structures. The presenting symptoms are frequently
nonspecific. The management of arachnoid cysts of the cerebellopontine angle is controversial. Asymptomatic
arachnoid cysts do not require treatment, and such patients should be monitored clinically and
radiologically. But serious symptoms need to be performed an operation. We report a 19-year-old man with
an bilateral arachnoid cyst of cerebellopontine angle that led to severe vertigo. One months after Microsurgical
surgery, the patient is symptom free
Key words: Arachnoid Cysts; Bilateral Cerebellopontine Angle; Severe Vertigo; Microsurgical Treatment

Introduction The most common site of occurrence is the

Arachnoid cysts are benign, rare, intra arachnoid
lesion filled with fluid similar to or exactly like
cerebrospinal fluid. They are thought to be
congenital or develop secondary to infection,
trauma, splitting abnormalities of the arachnoid
membrane, alteration of the cerebrospinal fluid
(CSF) flow, and/or change in the CSF pressure.1
Arachnoid cysts can occur all along the
craniospinal axis, but a large majority are located
intracranially.2
sylvian fissure followed by the cerebellopontine
angle (CPA) and supracollicular area. However,
arachnoid cysts of the CPA are often diagnosed
incidentally as they usually remain
asymptomatic.4
In this article, we describe a 19-year-old man with
a bilateral arachnoid cyst of the cerebellopontine
angle that led to progressive vertigo, and we
discuss the treatment in this case.

Lesions of the cerebellopontine angle account for Case Report

6% to 10% of all intracranial lesions. Acoustic
neurinomas and meningiomas represent
approximately 85% to 90% of all tumors of the
cerebellopontine angle, and the remaining lesions
of this location are primary cholesteatomas and
facial nerve schwannomas. Arachnoid cysts of
the cerebellopontine angle are rare.3 Bilateral
CPA arachnoid case are very rare and after
reviewing the available English literature we
could find only one case report.1
A 19-year-old man with a progressive vertigo
approximately 1 years-duration was admitted to
our hospital. There was no significant
neurological deficit. He come with full
awareness. He feel at the time of vertigo relapse,
others like moving around. There was no
disturbance of balance, no hearing loss, no history
of drug consumption, no previous illness history.

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The results of the neurological examination were
completely normal. Preoperative magnetic
resonance imaging showed a hyperintense on T2-
weighted imaging, in the bilateral CPA,
resembling an arachnoid cyst.
A under moderate pressure. The wall of the cyst was
B majority are located intracranially.2 Lesions of the
C is in the temporal fossa followed by the frontal
FIGURE 1. Preoperative magnetic resonance imaging. The axial T2-
Page 2 of 5
weighted (A) and coronal T2-weighted (B) and sagittal T2-weighted
(C) images show a hyperintense on T2 weighting, resembling an
arachnoid cyst in bilateral CPA.
Using a standard rectosigmoid approach, with
microneurosurgical technique, the cyst was
opened and typical cerebrospinal fluid flowed
excised. The postoperative was uneventful and
vertigo disappeared immediately. Histologically,
the cyst wall was a typical arachnoidal
membrane. One months after Microsurgical
surgery, the patient is symptom free.
Discussion
Arachnoid cysts are benign, rare, intra arachnoid
lesion filled with fluid similar to or exactly like
cerebrospinal fluid.1 Arachnoid cysts can occur
all along the craniospinal axis, but a large
cerebellopontine angle account for 6% to 10% of all
intracranial lesions. Arachnoid cysts of the
cerebellopontine angle are rare.3
Arachnoid cysts are benign developmental cysts
that occur throughout the cerebrospinal axis in
relation to the arachnoid membrane and
subarachnoid space. The cysts generally contain
clear, colourless fluid resembling normal spinal
fluid. They expand progressively, causing
pressure on contiguous brain and the skull.6
The most common location of the arachnoid cyst
convexity, posterior fossa, CPA and other
locations within the neurocranium.5 There were
significantly more males than females. This
difference was attributed solely to the
preponderance of cysts in the temporal fossa in
males, with ratio male/female 2:1. For the other
main locations (frontal convexity, posterior fossa,
and other locations) there was no significant
difference in sex distribution. However, for the
CPA sublocation there was a significant female
preponderance, with ratio male/female 1:2.5 For
arachnoid cysts in the temporal fossa, both male
and female patients demonstrated a significantly
increased number of left-sided cysts. For cysts
overlying the frontal convexity, no difference in
sidedness could be detected for either sex. As
noted above, there was a female predominance
for cysts in the CPA, with a significant tendency
for right-sided localization.5
They are thought to be congenital or develop
secondary to infection, trauma, splitting
abnormalities of the arachnoid membrane,
alteration of the cerebrospinal fluid (CSF) flow,
and/or change in the CSF pressure.1 Arachnoid
cysts can occur all along the craniospinal axis, but
a large majority are located intracranially.2
The structure features of the arachnoid cyst wall
that distinguish it from the normal arachnoid
membrane are as follows: (1) splinting of the
arachnoid membrane at the margin of the cyst, (2)
a very thick layer of collagen in the cyst wall, (3)
the absence of traversing trabecular processes
within the cyst, and (4) the presence of
hyperplastic arachnoid cells in the cyst wall,
which presumably participate in collagen
synthesis.6 Arachnoid cysts represent a
congenital anomaly of the developing
subarachnoid cistern in early intrauterine life. It is
postulate that, during the process of the complex
folding of the primitive neural tube and the
formation of normal subarachnoid cisterns, an
anomalous splitting of the arachnoid membrane
occurs.6
Usually, arachnoid cysts are asymptomatic.
Symptoms from an arachnoid cyst are caused by
an increase in the osmotic gradient of the liquid
content of the cyst; the creation of a valve
mechanism between the arachnoid cyst and the
subarachnoid space, which leads to an increase in
the size of the cyst; or the secretion of liquid from
the cyst wall, which enlarges the cyst. The onset
of the symptoms and signs are usually due to
cortical irritation, compression of the cerebral
parenchyma, or the obstruction of CSF
circulation.7 Symptoms are produced by the mass
effect of the cyst on surrounding structures. When
an arachnoid cyst is located in the
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cerebellopontine angle, the patient’s presenting
symptoms are frequently neurotologic, such as
sensorineural hearing loss, vertigo, or tinnitus.
Those symptoms are due to the dysfunction of the
eighth cranial nerve7 Sometimes, nonspecific
symptoms (headache, ataxia, gait disturbances,
dizziness, generalized unsteadiness) develop.
Compression of cranial nerves VII or V can cause
facial palsy, hemifacial spasm and/or neuralgic
pain.8,9,10 Arachnoid cyst in the cerebellopontine
angle manifesting as spastic hemiplegia11,
neuralgia20, contralateral trigeminal neuralgia12,
inverted burns’ nystagmus13, diplopia and
headaches7, congenital peripheral facial palsy14,
gliosis of the eighth cranial nerve15, auditory
neuropathy16, hearing loss17,18, tinnitus18,
hemifacial spasm19, Our patient exhibited only
severe vertigo.
The differential diagnosis of CPA arachnoid cysts
includes epidermoid and neurenteric cyst, cystic
acoustic schwannomas. MRI is helpful in
differentiating arachnoid cysts from other cystic
lesions. On MRI, arachnoid cysts appear as
smooth-surfaced lesions that exhibit a signal
characteristic of CSF in all MR sequences (DWI
and FLAIR).1
The management of arachnoid cysts of the
cerebellopontine angle remains controversial.
Asymptomatic arachnoid cysts do not require
treatment, and such patients should be monitored
clinically and radiologically with serial MRIs. If
the patient demonstrates no significant
compromise in local neural or vascular structures,
no severe symptoms, and no suspected or proven
rapid cyst growth, a watch-and-wait policy
should be implemented. The risks of surgery are
few, but complications (meningitis, hemiparesis,
oculomotor palsy, subdural hematoma, grand mal
epilepsy, and death) have been reported.1
The surgical treatment of arachnoid cysts of the
cerebellopontine angle consists of total resection
and surgical drainage. To reduce the likelihood of
complications, surgical drainage via the
retrolabyrinthine or retrosigmoid exposure is the
recommended therapy. Some authors recommend
the placement of a cystoperitoneal shunt in
patients with hydrocephalus. In recent years,
endoscopic cyst decompression, has been shown
to be safe and effective.1,21
Outcome
Using a standard rectosigmoid approach, with
microneurosurgical technique, the cyst was
opened and typical cerebrospinal fluid flowed
under moderate pressure. The wall of the cyst was
excised. The postoperative was uneventful and
vertigo disappeared immediately. Histologically,
the cyst wall was a typical arachnoidal
membrane. One months after Microsurgical
surgery, the patient is symptom free from severe
vertigo.
Conclusion
Bilateral arachnoid cysts of CPA is very rare
clinical entity with poorly defined etiology,
pathogenesis and can cause various symptoms in
this case with only severe vertigo or may be
asymptomatic. It becomes even more challenging
when more uncertainties exist regarding rare
bilateral arachnoid cysts operations.
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