Keywords
neuroendoscopy - arachnoid cyst - hydrocephalus - infants
Introduction
Intracranial cysts are one of the most common conditions that come to a neurosurgeon's
attention. They can be congenital or acquired.[1]
[2] The most common intracranial cyst encountered is an arachnoid cyst.
Most arachnoid cysts remain asymptomatic throughout the life and are incidental findings
on computed tomography (CT)/magnetic resonance imaging (MRI) performed for other situations
such as trauma.[3] Only symptomatic and large intracranial cysts require treatment. In the present
era of minimally invasive neurosurgery, an endoscopic procedure for an intracranial
arachnoid cyst has made the treatment easier and safer.
Surgery for these cysts has to be tailored according their location. Data about the
outcome of intracranial cysts in adults have been well reported, but the comparable
data in children < 1 year of age are still sparse.[4] In the present study, we evaluated the surgical approaches, indications, and outcome
of endoscopic procedures performed at our institution for arachnoid cysts in different
locations in infants and analyzed similar data reported in the literature.
Methods and Materials
A prospective study was performed in all pediatric patients admitted with arachnoid
cysts in our institution from 2005 to 2013. Malignant iatrogenic postoperative cystic
lesions and other cysts such as a Rathke cyst were excluded from the study. Detailed
history and a thorough physical examination were performed in all cases. Data included
age, sex, clinical symptoms, site, and location of cysts based on CT/MRI findings,
surgical approach, and postoperative status. All patients had both preoperative and
postoperative ophthalmologic checkups and psychomotor developmental assessments. Postoperative
CT/MRI brain scans were performed in all patients after 3 to 6 months or as and when
required. All patients had a minimum of a 6-month follow-up.
Study Population
The demographic data included 13 patients (M:F ratio 5:8) < 1 year of age operated
on during this period. Details of each case are described in [Table 1]. The mean age was 5 months (range: 1–12 months). Three cases diagnosed antenatally
were asymptomatic. Hydrocephalus was present in seven cases; four patients presented
with seizures. Other symptomatology included infantile spasms (in one patient) and
macrocephaly with involuntary head movements (in one patient). The follow-up period
ranged from 8 months to 6 years.
Table 1
Clinical characteristics of 13 patients with intracranial cysts
Case no.
|
Age/Sex
|
Diagnosed
|
Location of cyst
|
Presentation
|
HPE
|
Complications
|
Surgery
|
Follow-up
|
1
|
7 mo/F
|
Postnatal
|
Rt occipital (IV)
|
Infantile spasm
|
AC
|
None
|
Cystoventriculostomy
|
4.5 y
|
2
|
8 mo/F
|
Postnatal
|
Rt CP angle and juxta fourth ventricular
|
Macrocephaly; hydrocephalus
|
AC
|
Subdural hygroma
|
Transaqueductal cystoventriculostomy and ETV and R
|
6 y
|
3
|
8 mo/M
|
Postnatal
|
Posterior fossa extending into fourth ventricle
|
Macrocephaly; hydrocephalus
|
AC
|
None
|
Transaqueductal cystoventriculostomy and ETV and R
|
5 y
|
4
|
1 mo/M
|
Antenatal
|
Lt CP angle
|
Asymptomatic
|
AC
|
None
|
Cystocisternostomy
|
4 y
|
5
|
12 mo/F
|
Postnatal
|
Posterior fossa
|
Macrocephaly; hydrocephalus
|
AC
|
Pseudo meningocele
|
Cystocisternostomy and cystoventriculostomy and CP shunt
|
4 y
|
6
|
2 mo/F
|
Postnatal
|
Posterior fossa
|
Macrocephaly; hydrocephalus
|
AC
|
Pseudo meningocele and CSF leak
|
Third ventriculostomy and R and CV shunt
|
2 y
|
7
|
10 mo/F
|
Postnatal
|
Suprasellar
|
Involuntary head movements; hydrocephalus
|
AC
|
None
|
Transventricular transforaminal cystoventriculostomy and R
|
2.5 y
|
8
|
2 mo/M
|
Antenatal
|
Lt parietal
|
Asymptomatic
|
AC
|
None
|
Cystoventriculostomy and R
|
3 y
|
9
|
7 mo/F
|
Postnatal
|
Bilateral temporoparietal
|
Seizures; hydrocephalus
|
AC
|
Subdural hygroma
|
Cystoventriculostomy and R
|
2 y
|
10
|
2 mo/F
|
Postnatal
|
Rt parietal
|
Seizures
|
AC
|
Subdural hygroma
|
Cystoventriculostomy and R
|
2 y
|
11
|
12 mo/F
|
Postnatal
|
Lt frontal
|
Seizures
|
AC
|
None
|
Third ventriculostomy and cystoventriculostomy and R
|
18 mo
|
12
|
20 d/M
|
Antenatal
|
Rt CP angle juxta fourth ventricular
|
Asymptomatic hydrocephalus
|
AC
|
None
|
Transaqueductal cystoventriculostomy and ETV and cisternostomy and R
|
10 mo
|
13
|
3 mo/M
|
Postnatal
|
Interhemispheric (Rt)
|
Seizures
|
AC
|
None
|
Cystoventriculostomy
|
8 mo
|
Abbreviations: AC, arachnoid cyst; CP angle, cerebellopontine angle; CP shunt, cystoperitoneal
shunt; CSF, cerebrospinal fluid; CV, cystoventricular; ETV, endoscopic third ventriculostomy;
F, female; HPE, histopathologic examination; Lt, left; M, male; R, reservoir placement;
RC, Rathke cyst; Rt, right.
Location of Cysts
Posterior fossa and cerebellopontine (CP) angle cysts were seen in three patients
each. Four patients had cerebral convexity cysts, one had an interhemispheric cyst,
one had a supraseller cyst, and the one with infantile spasms had a cyst in the periventricular
region.
Treatment and Surgical Procedure
Treatment and Surgical Procedure
All patients underwent endoscopic procedures depending on the location of the cyst.
Cysts present around the ventricles underwent cystoventriculostomy; cystocisternostomy
was planned for cysts around the cisterns. Third ventriculostomy was an added procedure
in patients with a fourth ventricular outlet obstruction, and the procedure was performed
via a precoronal burr hole. All procedures were performed under general anesthesia.
Proper positioning of the patient for surgery was planned and determined depending
on the cyst location. Preoperative antibiotics were administered 30 minutes before
the time of induction. The surgical field was prepared and draped under all aseptic
precautions. A curvilinear skin incision was placed and a skin flap was reflected
with stay sutures. A burr hole was performed to allow insertion of the neuroendoscope.
Both rigid rod and flexible endoscopes (Karl Storz, Germany) were used. A cruciate
incision was placed over the dura after adequate coagulation. An endoscopic sheath
with trocar was introduced through the burr hole and the lateral ventricle was tapped.
The endoscope was negotiated into the cyst through the ventricular cavity or directly
into the cyst in some cases. Cyst wall was visualized all around. Continuous irrigation
was done with Ringer lactate and gentamycin at 37°C by connecting an irrigating channel
to the endoscope. The flow was adjusted to maintain an adequate intracystic pressure
and to prevent collapse of the cyst wall during the procedure.
After careful examination of the cyst wall, an avascular zone was selected for fenestration.
A piece of cyst wall was excised and sent for histopathologic study in all the cases.
Stoma was created using a blunt endoscopic bipolar tip or Fogarty catheter. Multiple
communications were created between the cyst cavity and cerebrospinal fluid (CSF)
pathways. These stomas were dilated as wide as possible with a 3F and 4F Fogarty catheter.
CSF cavity and subarachnoid cisterns were examined for any bleeding or adhesions that
were also breached. Bleeding from the cyst wall was controlled with continuous Ringer
lactate irrigation, by maintaining continuous pressure on the cyst wall with the Fogarty
balloon catheter and bipolar coagulation. Third ventriculostomy and aqueductoplasty
were performed as and when required. Finally, a reservoir was placed in the burr hole
for controlled tapping and to prevent early CSF leak/failure and then anchored to
the subcutaneous tissue. No major intraoperative or immediate postoperative complications
were encountered. No patient required craniotomy for any untoward complications. All
patients were extubated on the table.
Follow-up and Outcome
All patients had a postoperative CT scan of the brain after 3 to 6 months that showed
a significant reduction in the cyst dimensions. One patient had a CSF leak through
the surgical wound that was treated conservatively with regular CSF tapping from the
reservoir and oral acetazolamide. Three patients had subdural hygroma on follow-up
scans but showed clinical improvement with complete resolution of the hygromas over
time. One patient with a posterior fossa cyst presented with pseudomeningocele at
the reservoir site and required a ventriculoperitoneal shunt for CSF diversion. Overall,
all the patients showed symptomatic improvement.
Case 1: Endoscopic Cystoventriculostomy for Intracranial Cysts with Infantile Spasms
Case 1: Endoscopic Cystoventriculostomy for Intracranial Cysts with Infantile Spasms
A 7-month-old girl presented with recurrent episodes of sudden forward flexion movements
since age 3 months. Her electroencephalogram (EEG) was suggestive of hypsarrhythmia,
and CT and MRI of the brain ([Fig. 1]) showed a large right occipital juxtaventricular cyst. She was started on intravenous
adrenocorticotrophic hormone injections at age 5 months. Initially, the patient responded
to treatment, but soon her symptoms recurred. Finally, her symptoms and hypsarrhythmia
decreased following endoscopic cystoventriculostomy. Her postoperative CT brain scan
([Fig. 2]) correlated with the clinical findings and showed a significant reduction in cyst
dimensions.
Fig. 1 Case 1: Preoperative magnetic resonance imaging showing a large right periventricular
arachnoid cyst presenting with infantile spasm.
Fig. 2 Case 1: Postoperative computed tomography showing a well-decompressed cyst.
Cases 4 and 5: Endoscopic Cystocisternostomy for Posterior Fossa and Cerebellopontine
Angle Cysts
Cases 4 and 5: Endoscopic Cystocisternostomy for Posterior Fossa and Cerebellopontine
Angle Cysts
One patient was diagnosed with a posterior fossa cyst and the other with a CP angle
cyst. Endoscopic cystocisternostomy (cyst opened into the CP angle cistern) was performed
through an occipital retromastoid burr hole for a 1-month-old asymptomatic boy (case
4) diagnosed antenatally with a large left CP angle cyst ([Fig. 3]). A postoperative CT brain scan showed a considerable reduction in the cyst dimensions
([Fig. 4]). The patient with the posterior fossa cyst (case 5) was a 12-month-old girl with
macrocephaly who also underwent cystocisternostomy and cystoventriculostomy through
an occipital burr hole. The cyst was communicated with the fourth ventricle, and then
a third ventriculostomy was performed using the classical approach. Later, she presented
with a pseudomeningocele at the operative site that required a cystoperitoneal shunt.
Fig. 3 Case 4: Preoperative magnetic resonance imaging of a left-sided cerebellopontine
angle cyst.
Fig. 4 Case 4: Postoperative computed tomography brain showing reduced cyst size following
cystocisternostomy.
Case 6: Endoscopic Third Ventriculostomy with Cystoventricular Shunt for Large Posterior
Fossa Cysts
Case 6: Endoscopic Third Ventriculostomy with Cystoventricular Shunt for Large Posterior
Fossa Cysts
A 2-month-old girl presented with progressive enlargement of head size since 3 weeks
with downward deviation of the eyeballs since 2 weeks. Her MRI showed a large posterior
fossa cyst with compression of the cerebellar hemispheres (more on the right side)
with gross dilatation of the lateral and third ventricles. Endoscopic third ventriculostomy
with reservoir placement followed by a cystoventricular shunt was performed because
the location of the cyst was not adjacent to any CSF pathway (fourth ventricle). A
postoperative CT brain scan showed mild reduction in cyst size and mass effect over
surrounding structures. The patient developed a CSF leak with a pseudomeningocele
in the immediate postoperative period that was managed conservatively.
Case 7: Transventricular Transforaminal Cystoventriculostomy for Suprasellar Cysts
Cases 8 to 11: Endoscopic Cystoventriculostomy/Cystocisternostomy with Reservoir Placement
for Supratentorial Cysts
Cases 2, 3, and 12: Posterior Fossa Cyst Decompressed through the Transaqueductal
Route
Case 13: Interhemispheric Arachnoid Cyst
Case 13: Interhemispheric Arachnoid Cyst
A 3-month-old boy presented with seizures/tonic posturing. MRI revealed a large interhemispheric
arachnoid cyst predominantly extending to the right side and corpus callosum agenesis
with displacement and compression of the lateral ventricles more laterally ([Fig. 18]). The EEG was suggestive of right-side hemispheric discharges. The child underwent
an endoscopic cystoventriculostomy via a classical precoronal burr hole. Postoperatively
the child did well, and a postoperative scan revealed a decompressed cyst and opened
up ventricles and sulcal spaces ([Fig. 19]).
Fig. 18 Case 13: Magnetic resonance images showing large interhemispheric arachnoid cyst
displacing and compressing the lateral ventricles (right to left).
Fig. 19 Case 13: Postoperative computed tomography showing decompressed cysts and opened
up lateral ventricles.
Results
The follow-up period ranged from 8 months to 6 years. All patients were assessed using
clinical characteristics, neuropsychological assessment, and follow-up imaging. The
patient with infantile spasms showed a decreased frequency of spasms, and the postoperative
anticonvulsants required was reduced from four to only one. The EEG also showed decreased
episodes of hypsarrhythmia. All patients were relieved symptomatically. Only one patient
with a posterior fossa cyst required a CSF diversion procedure because she presented
with a pseudomeningocele at the occipital burr hole site 6 months after the primary
surgery. Postoperative imaging showed a significant decrease in cyst size in nine
patients and a slight decrease in the cyst size in the remaining five. Only one patient
had a CSF leak from the surgical wound that was treated conservatively with oral acetazolamide
followed by repeated CSF tapping from the reservoir.
Discussion
Intracranial arachnoid cysts are developmental abnormalities of the arachnoid membrane
that get duplicated or split. The other mechanism of arachnoid cyst formation is an
incomplete separation of the endomeninx, which is the perimedullary mesh. This occurs
during the early embryonic period.[2]
[5]
[6] Arachnoid cysts are also associated with other central nervous system anomalies
such as partial or complete agenesis of lobes. These cysts can be diagnosed antenatally
with ultrasonography (USG).[7] In our current series, four cases were diagnosed incidentally in the antenatal period
with routine USG. Although asymptomatic, the CSF dynamics can result in an increase
in the size of some cysts or rupture leading to elevated intracranial pressure (ICP).
The probable mechanisms include active secretions from the cyst wall, a valvelike
mechanism that causes inflow of CSF from CSF-filled cavities because of arterial pulsations
and obstructed outflow. Others include aberrant fluid dynamics and osmotic changes
along the cyst wall.[2]
[5]
[8] This leads to a persistent and continual mass effect that can hinder the normal
development and function of the surrounding brain.[9]
[10]
[11]
[12] The management options include either clinical monitoring with serial MR imaging
and timely intervention or elective definitive surgery for a large asymptomatic cyst.
Young patients are prone to cyst enlargement and hence need surgical interventions
as early as possible.[13]
The symptomatology depends on the location of the cyst and age of the patient. Supratentorial
cysts can be both intra- or extra-axial and usually present with macrocephaly or elevated
ICP. Suprasellar cysts usually present with typical bobble-head doll syndrome and
endocrine or visual abnormalities. Hydrocephalus is a common manifestation in posterior
fossa cysts and periventricular cysts either due to compression of the fourth ventricle
or aqueduct causing fourth ventricular outflow obstruction. In neonates and younger
children, presenting symptoms are usually due to elevated ICP. In our case series,
common presenting symptoms were macrocephaly, hydrocephalus, and seizures. Other symptoms
encountered were poor gaze fixation, infantile spasms, and involuntary forward flexion
movements of the head.
Varied surgical options have been described for intracranial cysts, but the optimal
option is still debated. These include simple burr hole and cyst aspiration, cyst
shunting, ventricular shunting, microsurgical cyst excision, endoscopic fenestrations
of cysts, and marsupialization of the cyst wall. Age is not a limiting factor for
these surgical options.[14] Sikorski et al concluded that endoscopic fenestration is the preferred initial surgical
management of symptomatic intracranial cysts in infants ≤ 1 year of age.[4] Pros and cons of different surgical options are described in [Table 2].
Table 2
Advantages and disadvantages of the different surgical techniques
Procedure
|
Advantages
|
Disadvantages
|
Cyst shunting
|
Safe procedure; useful in distorted anatomy of ventricles
|
• Shunt infection, dependency, and failure[11]
• Need for revisions
|
Ventricular shunting
|
Decreases raised ICP
|
• All disadvantages of cyst shunting
• Cyst may persist and continue to grow and cause mass effect
|
Microsurgical cyst excision
|
Whole cyst can be visualized; multiple fenestrations; less blood loss
|
• Relatively more time consuming and a more invasive technique[14]
[20]
• More brain handling and likely increased tissue damage in deep-seated cysts compared
with endoscopic fenestration
|
Endoscopic fenestrations
|
Less time consuming; easy and safe; comparatively less blood loss
|
• Requires trained personnel and equipment
• Reformation of membrane
|
Marsupialization
|
Less recurrence
|
• Same as microsurgical cyst excision
|
Abbreviation: ICP, intracranial pressure.
The major goals of the surgery for intracranial cysts are decompression of the cyst
and preventing further compression of neural structures. Endoscopic decompression
of the cyst is minimally invasive, less time consuming, safe, and easy with a decreased
incidence of postoperative complications. Other endoscopic procedures include endoscopic
cystocisternostomy, cystoventriculostomy, or partial cyst wall excision.
Most arachnoid cysts do not resolve completely as seen in our case series. In this
series, postoperative imaging showed a significant decrease in cyst size in seven
patients and only a slight decrease in cyst size with a resolution of symptoms in
others. The reason for noncollapsibility of these cysts is that they are developmental
cysts and associated with agenesis of lobes.[2]
[5]
[8]
In this current series, endoscopic fenestration helped successfully in decreasing
the frequency of seizures. Koch et al reported 37 cases with seizures and found that
23 (62%) were seizure free postoperatively, nine patients (24%) had a reduction of
seizures, and five children (14%) remained unchanged.[15]
All types of cysts can be treated with endoscopic fenestrations.[15]
[16] In our case series, suprasellar, supratentorial, infratentorial, paraventricular,
posterior fossa, and CP angle cysts were successfully treated with endoscopic cystoventriculostomy
or cystocisternostomy. Hopf and Perneczky used different endoscopic techniques in
36 patients with intracranial cysts and concluded that intraventricular and suprasellar
cysts are better managed with endoscopic neurosurgery.[17] Endoscopic fenestration is a minimally invasive technique that is a safe and effective
alternative treatment for suprasellar arachnoid cysts.[14]
[18]
Posterior fossa cyst is another challenge for the neurosurgeon. These cysts as in
cases of fourth ventricular outlet obstruction can cause obstructive hydrocephalus
and also decrease the prepontine space, thus making third ventriculostomy difficult.[19] Posterior fossa cysts bulging into the fourth ventricle could easily be accessed
through the transaqueductal approach using a flexible neuroendoscope. This procedure
of transaqueductal cyst decompression with a flexible endoscope was not described
in the literature. This procedure thus avoids the more aggressive treatment options
like shunting the cyst or a major craniotomy and cyst excision. Reservoir placement
after the endoscopic procedure is an additional technique for postoperative CSF access
for controlled tapping and as a preventive measure for early CSF leak, which is major
concern for any neurosurgeon.
Conclusion
No generally accepted solution exists for the treatment of intracranial cystic lesions
including arachnoid cysts. There has always been controversy regarding the best surgical
option. For some cysts, a permanent solution like endoscopic fenestration is possible.
Endoscopic neurosurgery in experienced hands is an easy, safe, and less invasive surgical
technique. The surgical approach has to be tailored according to the location of the
cyst. A flexible endoscope is a useful adjunct in selected cases where a rigid scope
cannot be manoeuvred. Outcome with neuroendoscopic procedure in infants is as effective
as in older children. The endoscopic procedure does not cause the complete collapse
of the cyst, but it definitely alters the fluid dynamics resulting in shrinkage of
the cyst and decreasing mass effect. It prevents major perioperative and postoperative
complications and avoids diversionary procedures in most cases.