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Научно-практический журнал «Нейрохирургия и неврология детского возраста» (Pediatric Neurosurgery and Neurology), 2011, 2(28), 30-52
The Use of Endoscopic Technique in Surgical Interventions for Isolated Fourth Ventricle
Petraki VL, Simernitsky B.P., Asadov R.N.
Medical Center for Children with Craniofacial Malformations and Congenital Nervous Diseases,
Moscow, Russia
Abstract
Thirty six children aged from 20 days to 7 years were operated on the isolated Fourth ventricle
using endoscopic techniques during 2001 - 2010 period. The majority of them (66%) were infants.
Two types of surgery were performed. The first type dealt with cases of complete obliteration of
aqueductus cerebri. The paraaqueductal outflow of CSF was formed by fistulae between cisterna
ambiens and the Third or lateral ventricle, and between cisterna ambiens and the Fourth ventricle.
When oral parts of the Fourth ventricle protrude into tentorial incisure, and roof of the Fourth
ventricle become a thin “membrane” adherent to a similar thin wall of a lateral or the Third
ventricle, a fistula was made between a lateral and the Fourth ventricle and between the Third and
the Fourth ventricles. The operation was supplemented by perforation of the floor of the Third
ventricle and foramen Magendie plasty to improve CSF outflow to subarachnoidal space. The
second type of surgery was aimed at restoration of physiological CSF-circulation with plasty and
stenting of aqueductus, foramen Magendie and craniovertebral junction combined with endoscopic
Third-ventriculocisternostomy (ETV).
CSF outflow from the Fourth ventricle was restored after surgery in 35 children (94% cases).
Stable compensation of hydrocephalus without any other manipulations was achieved in 5 cases. In
31 cases it required additional shunting.
CONCLUSIONS: In children (and especially in infants) endoscopic restoration of CSF
circulation is a method of choice in treatment of the isolated Fourth ventricle. It is achieved by
elimination of occlusion between cerebral ventricles (aqueductoplasty, interventriculostomy) and
simultaneous restoration of CSF flow into subarachnoid space (plasty of foramina Magendie and
Luschka, craniovertebral junction, Third-ventriculocisternostomy, and Third-Fourth
ventriculocisternostomy). Use of endoscopic technique in implantation of panventriculoperitoneal
shunt allows to perform surgery without stereotactic and navigation devices. Forced dilatation of
slit lateral and the Third ventricles after shunting gives opportunity for aqueductoplasty and
aqueductal stenting in the isolated Fourth ventricle.
Key words: isolated Fourth ventricle, endoscopic aqueductoplasty, aqueductal stent,
interventriculostomy, panventriculoperitonal shunt, Third-Fourth-ventriculocisternostomy, foramen
Luschka plasty, foramen Magendie plasty, cisternoventriculoperitoneal shunt,
ventriculosubarachnoidal stent.
The isolated (or entrapped) Fourth ventricle (IFV) is a form of multilevel obstructive
hydrocephalus. It has the most severe clinical course and difficult to treat. The isolated Fourth
ventricle is characterized by combination of closure of foramina Luschka and Magendie and
acqueductal occlusion. These occlusions result from acute or chronic inflammation of arachnoidea
and ependima accompanied by adhesion and periventricular edema in physiological diminutions of
CSF pathways [ 2 ]. They also may be caused by mechanical factors such as intraventricular
hemorrhage (IVH), functional acqueductal occlusion, repeated revisions of CSF shunts and cerebral
malformations. Occlusion hydrocephalus is complicated by IFV syndrome in 5-43% cases [ 2, 5, 9,
20, 29, 34 ].
There are many different surgical techniques for treatment of TFV. Most frequently performed
are suboccipital craniotomy with dissection of cerebellar vermis or adhesions blocking foramen
Magendie, endoscopic aqueductoplasty or aqueductal stenting [ 1, 4, 9, 10, 11, 13, 16, 17, 21, 26,
31, 32, 33, 35 ], intreventriculostomia [ 7, 10, 27, 32 ], isolated or combined shunting of lateral
ventricles and the Fourth ventricle [ 12, 14, 18, 19, 30 ] and combination of the above mentioned
interventions [ 4, 16, 18 ]. The risk of postoperative complications substantially increases in
newborns and infants.
The aim of our paper is to demonstrate possibility of radical intervention in different types
of IFV in newborns and infants using neuroendoscopy.
Material and methods
Thirty six children (age from 20 days to 7 years) with IFV were operated on with
neuroendoscopic technique during 10-year period (from 2001 to 2010). Twenty seven children
(75%) were preterm babies born at 27-37 week of gestation. In 11 cases endoscopic interventions
were primary and in 14 cases they were performed after earlier implanted VP-shunts. In 11 cases
endoscopy procedures and shunting were done simultaneously. In 12 children IFV was formed after
intraventricular hemorrhages. In 14 cases TFV was caused by infection of CSF spaces after IVH (5
cases) or neonatal sepsis (9 cases). Table 1 presents distribution of cases by age and etiology.
Twenty four operated on children were infants (66 %).
Table 1
Etiology
Age
Total<1 mo. 1-3 mo. 3 - 6 mo. 6-12 mo. >1
yr.
IVH III-IV grade 3 4 4 1 - 12
Meningitis and ventriculitis 3 1 3 2 5 14
Functional aqueductal
occlusion
- 1 1 7 9
Dandy-Walker syndrome - - 1 - - 1
Total 6 6 8 4 12 36
Variants of endoscopic interventions
Neuroendoscopic interventions were performed only by supratentorial approach using rigid
endoscopes “Richard Wolf” (external diameter 3,5 mm) and “Karl Storz” (external diameter 3 mm).
We considered two types of surgical intervention related to structural changes in CSF pathways:
either creation of paraaqueductal anastomosis (type I) or restoration of aqueductal patency (type II)
- aqueductoplasty (see Table 2).
Table 2
Distribution of children according to age and types of surgical interventions
Types of surgery
Age
Total<1 mo. 1-3 mo. 3 - 6 mo. 6 - 12 mo. > 1 yr.
Paraaqueductal
anastomosis (Type I)
- 2 4 1 1 8
Aqueductoplasty (Type II) 6 4 4 3 11 28
Total 6 6 8 4 12 36
Surgery of paraaqueductal anastomosis (type I). This type of surgery was aimed at
circumvention of totally long length occluded aqueduct by creation of direct CSF passage from the
Third or a lateral ventricle into the Fourth ventricle. When oral parts of the Fourth ventricle
protrude into tentorial incisure, and roof of the Fourth ventricle becomes a thin “membrane”
adherent to a similar thin wall of a lateral or the Third ventricle, it is possible to dissect or puncture
these “membranes” via empty cisterna ambiens and form a fistula between ventricles. In 3 children
a fistula was made between a lateral and the Fourth ventricle (Latero-Fourth-interventriculostomy –
see Fig.1) and in 2 cases it was performed between the Third and the Fourth ventricles (Third-
Fourth-interventriculostomy – see Fig.2). At the same time we restored CSF drainage into
interpeduncular cistern (Third-ventricolocisternostomy) and cisterna magna (foramen Magendie
plasty).
A B C
Fig.1. Endoscopic Lateral-Fourth-interventriculostomy.
A. A sketch of surgery.
B. Endoscopic view of supratentorial protrusion of upper wall of the Fourth ventricle as seen
from a lateral ventricle cavity.
C. Endoscopic view of an artificial fistula between a lateral and the Fourth ventricle
(indicated by arrow).
A B
Fig.2. Endoscopic Third-Fourth interventriculostomy.
A. MRI before surgery. Planned trajectory of the endoscope is indicated by arrow;
B. MRI after formation of artificial fistula between the Third and the Fourth ventricles. The
Fourth ventricle is shrunken.
In 3 cases surgical technique had some peculiarities when there was cisterna ambiens’ cavity
between ventricles (i.e. between the Third and the Fourth ventricles or a lateral and the Fourth
ventricle).
Surgical technique of Endoscopic Third-Fourth-ventriculocisternostomy. The endoscope is
introduced into cavity of the Third ventricle via frontal horn of a lateral ventricle (1 case). Posterior
wall of the Third ventricle is perforated at the level of subpineal inversion (posterior Third-
ventriculocisternostomy) and the endoscope reaches cisterna ambiens. Arachnoid bands inside the
cistern are dissected. Dorsal surface of lamina quadrigemina is an anatomical landmark of cisterna
ambiens. Roof of the Fourth ventricle is seen distally. It is presented as a thin and dome-shaped
deformed upper velum cerebelli. Velum is pointwise coagulated and perforated along midline. Then
the newly formed fistula to the Fourth ventricle is enlarged to the size of the endoscope diameter
(proximal Fourth-ventriculocisternostomy). Fluctuation of stoma margins and turbulent CSF flow
indicates fistula functioning.
At the same time we perform foramen Magendie plasty. The endoscope is inserted into the
dilated Fourth ventricle along midline and a surgeon inspects the ventricle cavity. The endoscope is
aimed at projection of foramen Magendie. Its obstructing membrane is perforated, and diameter of
resulting stoma is enlarged up to 5-7 mm (distal Fourth-ventriculocisternostomy or foramen
Magendie plasty). Then the endoscope is introduced in cisterna magna which is being inspected.
Finally it is feasible to perform standard Third-ventriculostomy which gives additional
possibilities for outflow of ventricular CSF into subarachnoid space. Fig. 3 provides an outline of
main stages of surgical intervention, and Fig.4 presents MRI of its result.
Occlusion
Fig.3. Main stages of endoscopic Third-Fourth ventriculocisternostomy.
Arrow 1 indicates posterior Third-ventriculocisternostomy (via recessus subpinealis to
cisterna ambiens). Arrow 2 indicates proximal Fourth-ventriculocisternostomy (from cisterna
ambiens via upper velum cerebelli in the Fourth ventricle cavity).
A B
Fig.4. Endoscopic Third-Fourth-ventriculocisternostomy.
A. MRI before surgery. Main planned stages of surgery are indicated by arrows: posterior
Third-ventriculocisternostomy (dotted arrow), proximal Fourth-ventriculocisternostomy (white
arrow), and plasty of foramen Magendie (black arrow);
B. MRI after surgery: perforation of the floor of the Third ventricle (white arrow) and
restoration of foramen Magendie (black arrow).
Surgical technique of Endoscopic Lateral-Fourth-ventriculocisternostomy. The
endoscope is introduced into anterior horn of a lateral ventricle by frontal approach and oriented
towards midline (2 cases). Medial wall of posterior horn of a lateral ventricle is perforated in
projection of cisterna ambiens with subsequent revision of this cistern. After main anatomical
landmarks (lamina quadrigemina, roof of the Fourth ventricle) are determined we perforate upper
cerebellar velum and formed a fistula to the Fourth ventricle. The fistula is enlarged to the size of
the endoscope diameter.
At the same time we perform foramen Magendie plasty thus connecting Fourth ventricle to
cisterna magna and restored CSF drainage into interpeduncular cistern (Third-
ventricolocisternostomy).
It should be mentioned that in our experience vital functions remained stable during surgery.
Surgery for restoration of aqueductal patency (type II). It was aimed at consecutive
restoration of pathways of CSF circulation by eliminating occlusion of aqueductus, foramen
Magendie and cranio-vertebral junction. Endoscopic aqueductoplasty was performed in cases of
membrane occlusion or aqueductal stenosis. A membrane which occluded aqueductus was
perforated and a newly created stoma was dilated until aqueductus was fully patent. In case of
stenosis aqueductus was widened first by bougienage and pendular movements of the endoscope’s
working instrument (1 mm diameter electrode), and then in similar fashion by the endoscope’s
corpus. By the end of the procedure aqueductal lumen could usually be dilated up to 4 – 4,5 mm,
and the endoscope might be inserted into the Fourth ventricle with minimal diversion from midline
(Fig.5).
A B
C D
E F
Fig.5. Endoscopic aqueductoplasty.
A. MRI before surgery. Planned trajectory of the endoscope is indicated by arrow;
B-F – endoscopic images: B – membrane aqueductal occlusion; C – the endoscope’s
working instrument (an electrode) in projection of membrane is indicated by arrow; D –view after
membrane perforation; E –dilatation of entrance hole and aqueductus; F – restored aqueductus after
its bouginage.
In 25 children aqueductoplasty was combined with endoscopic Third-ventriculocisternostomy
(ETV) to provide CSF leakage into subarachnoid space (Fig.6). It should be noted that in ETV in
case of the entrapped Fourth ventricle basal cisterns might be difficult to explore due to their
compression by displaced brainstem. That is why exploration of basal cisterns was performed in
final stage of surgery after the Fourth ventricle had been drained and cisterns decompressed.
A B
Fig.6. Combination of aqueductoplasty and Third-ventriculocisternostomy.
A. MRI before surgery. Planned trajectories of the endoscope are indicated by arrows:
aqueductoplasty is shown by black arrow, and Third-ventriculocisternostomy – by white arrow;
B. MRI after surgery. Perforation of the Third ventricle floor is indicated by white arrow and
restored aqueductus – by black arrow.
In 15 children we performed plasty of foramen Magendie in order to restore CSF leakage from
the Fourth ventricle into cisterna magna after aqueductoplasty (Fig.7).
A B
C D
E F
Fig.7. Endoscopic plasty of foramen Magendie.
A. Fourth ventricle and hemosiderosis near foramen Magendie (arrow);
B. Decollement of foramen Magendie (two arrows);
C. Revision of cisterna magna (arrow);
D. Brainstem vessels (arrow);
E. Revision of craniovertebral junction and decollement (arrow);
F. Free outlet from Fourth ventricle (arrow) at final stage of surgery.
In one case foramina Magendie and Luschka were obstructed after septic ventriculitis in
combination with numerous adhesions in aqueductal lumen and the Fourth ventricle. Restoration of
CSF outflow was achieved in this case by combination of aqueductoplasty and decollement (Fig.8).
A B
Fig.8. Combination of aqueductoplasty and decollement in the Fourth ventricle.
A. MRI before surgery. Multiple adhesions of aqueductus and the Fourth ventricle (arrow);
B. MRI after surgery. Aqueductus is patent (arrow) and volume of the Fourth ventricle is
reduced.
In another case it was impossible to open foramen Magendie which was obliterated by
adhesions to brainstem. But CSF outflow from the Fourth ventricle was restored after opening of
membrane adhesion which obstructed left foramen Luschka – foramen Luschka plasty (Fig.9).
A B
C D
Fig.9. Endoscopic plasty of foramen Luschka.
A and B: MRI before surgery;
C and D: MRI after surgery. Arrows indicate perforation of left foramen Luschka.
For prevention of aqueductal reocclusion an autonomous stent was inserted in 15 cases. It was a
silicon catheter with perforated walls in projection of the Fourth, the Third and a lateral ventricles.
Its length was prior calculated using MRI, CT or neurosonography. It amounted to a distance from
dura mater at the point of immersion to a lower third of the Fourth ventricle. The stent was
introduced into a lateral ventricle parallel to the endoscope which navigated the stent through the
Third ventricle and aqueductus into the Fourth ventricle. Proximal end of the catheter was fixed by
angular clips to burr hole margin or to dura mater in case of big open fontanelle (Fig.10, Fig.11).
A B
C D
Fig.10. Aqueductal stenting.
A. MRI before surgery: adhesive occlusion of aqueductus (arrow) and the entrapped Fourth
ventricle;
B. MRI after surgery. Longitudinal position of stent (arrow) inside the Fourth ventricle
along brainstem;
C. CT after surgery. Stent position (arrow) in ventricular system;
D. Relation of stent (arrows) to cranial structures (3D CT-reconstruction).
A B
C D
Fig.11 Aqueductal stenting. Endoscopic views.
A. Aqueductus as seen from the Third ventricle;
B. Stent (arrow) is navigated through aqueductus;
C. Stent position (arrow) inside the Fourth ventricle;
D. Stent position (arrow) in a lateral ventricle.
Aqueductal stent was used as ventricular catheter of a VP shunt in 7 cases when the Fourth
ventricle foramina were closed. After the catheter was navigated through aqueduct by the above
mentioned technique its distal end was connected with contour valve (Delta, “Medtronic”) and
peritoneal catheter. Thus combination of aqueductoplasty and aqueductal stenting with CSF
shunting enabled simultaneous draining of all cerebral ventricles by a singular shunting system –
panventriculoperitonal shunt (PVP-shunt) in the entrapped Fourth ventricle. Fig.12 presents the
results of such surgery.
A B
C D
Fig.12. Panventriculoperitoneal shunt (PVP- shunt).
A. MRI before surgery. The isolated Fourth ventricle (arrow);
B. MRI after surgery. Position of ventricular catheter (arrow) in ventricular system and
contraction of the Fourth ventricle;
C. CT after surgery. Midline position of ventricular catheter (arrow) in the Fourth
ventricle;
D. 3D CT reconstruction of PVP-shunt. Ventricular catheter is indicated by arrow.
PVP-shunting might be modified. In one case we inserted ventricular catheter into the Fourth
ventricle directly from a lateral ventricle after Lateral-Fourth-ventriculocisternostomy and in
another case – after Lateral-Fourth-interventriculostomy.
Similarly we inserted autonomous catheters between a lateral and the Fourth ventricle. Thus we
achieved sufficient internal decompression of the Fourth ventricle which enabled us to avoid
modification of previously implanted standard shunt systems or from implantation of an additional
VP-shunt from the Fourth ventricle (Fig.13).
A B
Fig.13. Endoscopic lateral Fourth-ventriculocisternostomy and implantation of
autonomous catheter (MRI after surgery).
A. Fistula between a lateral and the Fourth ventricle (arrow);
B. Catheter between a lateral and the Fourth ventricle (arrow).
It should be noted that both in paraaqueductal catheter implantations and in aqueductal stenting
catheters and stents are placed longitudinally to brain stem.
Within type II surgery there was ventriculosubarachnoid stenting in 9 patients - that is insertion
of one stent throughout foramen Monroe, aqueductus, foramen Magendie and craniovertebral
junction. Prevalence of adhesive process both in diminutions of ventricular system and in basal
subarachnoid space determines an increased risk of reocclusion after endoscopic intervention which
provokes adhesive process. Ventriculosubarachnoid stenting was aimed at preventing reocclusion at
different levels, and at preventing functional stenosis and obliteration of CSF pathways due to
contraction of ventricular system after shunting. In this procedure endoscopic aqueductoplasty,
plasty of foramen Magendie followed by revision of craniovertebral junction and dissection of
adhesions were the first stage of surgery. Then the stent was introduced into aqueductus and
navigated through Fourth ventricle. Its distal end was placed in cisterna magna (1 case) or
subarachnoid space of cervical part of spinal cord (8 cases) at C2-C7 levels (Fig.14 and Fig.15).
A B
C D
Fig.14. Ventriculosubarachnoidal stenting.
A. MRI before surgery. Status of ventricular system;
B. MRI after surgery. Shrinkage of cerebral ventricles;
C-D. CT (C) and 3D CT-reconstruction (D) after surgery. Distal end of the stent is
located at C3 level (arrows).
A B
C D
E F
G H
Fig.15. Ventriculosubarachnoidal stenting. Endoscopic views.
A. Decollement (arrow) of foramen Magendie;
B. Cavity of cisterna magna (arrow);
C. Margin of foramen magnum (arrow);
D. Adhesions (arrow) in craniovertebral junction;
E. Revision and decollement (arrow) in craniovertebral junction;
F. End of the catheter (arrow) at foramen magnum margin;
G. Navigation of the catheter to dorsal spinal space with working instrument (arrow) of
the endoscope;
H. Stent position (arrow) in craniovertebral junction at the end of surgery.
There were no significant reactions from structures at the floor of the Fourth ventricle during
ventriculosubarachnoidal stenting and after surgery. Postoperative MRI confirmed correct position
of stent in the Fourth ventricle and in spinal canal as well as absence of compression of brain stem
and cervical part of spinal cord (Fig.16).
A B
C D
Fig.16. Stent position in spinal canal after endoscopic ventriculosubarachnoidal stenting.
A. MRI after surgery. The stent is located along brainstem (white arrow);
B. Enlarged MRI after surgery which demonstrates craniovertebral junction and the
stent;
C. Relation of the stent (white arrow) to spinal cord (black arrow) in spinal canal on
MRI;
D. Position of distal end of the stent (yellow arrow) in spinal canal on craniospinal CT.
When calculating length of the catheter in spinal subarachnoid space we assumed that during
child’s growth a distal end of the catheter should stay in spinal canal outside the zone of adhesions.
We believe that the catheter should be plunged into spinal subarachnoid space as low as possible
but not lower than C6-C7 level (3 cases). In a newborn the length of cervical part of the stent from
foramen magnum to C7 level is 3, 5 cm.
In 2 children ventriculosubarachnoid stent was used as part of a shunting system. This enabled
to drain CSF into peritoneal cavity from ventricles and cerebral cisterns simultaneously – cisterno-
ventriculo-peritoneal shunt (CVP-shunt). Such shunt is shown at Fig.17.
A B
Fig.17. Cisternoventriculoperitoneal shunt on CT.
A. Position of ventricular catheter of a shunt (arrow) in craniocervical space;
B. Distal end of a ventricular catheter is located at C7 level (arrow).
Characteristics of endoscopic intervention at combination of the entrapped Fourth ventricle
and the slit Third and lateral ventricles.
In 4 children routine VP-shunting of tetraventricular occlusion hydrocephalus resulted into
contraction of lateral and the Third ventricles to a slit-like shape and functional aqueductal
occlusion with formation of IFV. The size of supratentorial portion of ventricular system prevented
the use of endoscopic technique for decompression of the Fourth ventricle. In 3 cases lateral and the
Third ventricles were dilated by transformation of peritoneal shunt catheter into external drainage
with gradual reduction of CSF outflow. Within 1-3 weeks lateral and the Third ventricles became
wide enough for endoscopic plasty and aqueductal stenting with simultaneous ETV as described
above (Fig.18).
I. A B
II. C D
III. E F
Fig.18. Ventricular system after VP-shunting on MRI.
A. Slit lateral ventricles (arrows);
B. The isolated Fourth ventricle (arrow);
C. Dilatation of lateral ventricles ( arrows) 16 days after transformation of a VP-shunt
and controlled intraventricular hypertension;
D. The Fourth ventricle maintain the same size (arrow);
E. Normal lateral ventricles (arrows) after aqueductal stenting with autonomous stent
and restoration of functioning of VP-shunt;
F. Autonomous stent of aqueductus (black arrow), the normal Fourth ventricle (white
arrow).
There was forced dilatation of ventricles during surgery in one case with lateral ventricles 15
mm wide and the Third ventricle – 5 mm wide. After the endoscope was inserted into a lateral
ventricle the latter was moderately dilated due to constant intraventricular infusion of normal saline
under increased pressure (400 mm H2O). This was sufficient for endoscopic procedures. Narrowed
foramen Monroe (functional stenosis after shunting) was dilated by bougienage movements of the
electrode and endoscope corpus. While infusion was continuing we passed the endoscope into the
Third ventricle and performed aqueductoplasty and aqueductal stenting combined with ITV
(Fig.19).
A B
C D
E F
Fig.19. Dilatation of ventricles by infusion technique on MRI.
A. Slit lateral ventricles (arrows) after VP-shunting;
B. Functional occlusion of foramina Monroe (arrow);
C. The isolated Fourth ventricle (arrow);
D. Intraoperative dilatation of lateral ventricles (arrows) by infusion technique;
E. Patent foramen Monroe (arrow) after its endoscopic plasty;
F. Autonomous stent of aqueductus (black arrow) and diminution of the Fourth
ventricle (white arrow).
Inspection of ventricular system at final stage of the surgery did not reveal macroscopic signs of
brain damage along trajectory of our intervention. This case demonstrates that intraventricular
infusion of normal saline under controlled pressure is an effective and sufficiently safe method of
ventricular dilatation during surgery. Its use allows excluding manipulations upon shunting system
and reducing preoperative period and total length of hospitalization.
In 3 cases aqueductal autonomous stent had to be removed after 1-2,5 months after surgery
(infection in CSF space -1, stent displacement-1, pleocitosis -1). Follow-up exam showed patent
and functioning aqueductus in all cases (Fig.20).
A
1 2 3
B
1 2 3
Fig.20. Restoration of aqueductal patency after its temporal stenting.
A. Postinflammatory occlusion of the Fourth ventricle’s foramina;
B. Posthemorrhagic occlusions of the Fourth ventricle’s foramina.
1. The isolated Fourth ventricle (arrows);
2. Aqueductal stenting (arrows);
3. Restoration of aqueductal patency after stent removal (arrows).
Results
CSF outflow from the Fourth ventricle was restored after surgery in 35 children (94% cases). It
was accompanied by reduction of neurological signs of compression of posterior fossa structures. In
one case there was slow progression of the Fourth ventricle enlargement due to reocclusion of
stoma between a lateral and the Fourth ventricle which required second surgery: suboccipital
craniotomy, foremen Magendie revision with removal of adhesions and implantation drainage
between the Fourth ventricle and cisterna magna. In 5 cases there was stable compensation of
hydrocephalus after endoscopic interventions due to restoration of physiological CSF resorption
which allowed avoiding implantation of a VP-shunt at final stage of treatment. Treatment results are
presented in Table 3.
Table 3
Results of surgical correction of the entrapped Fourth ventricle
Age Type of surgery OutcomeI II Hydrocephalus
compensation (without shunt)
Hydrocephalus stabilization(with VP-shunt)
0-1 months
- 6 1 5
1-3 months
2 4 4 2
3-6 months
4 4 - 8
6-12 months
1 3 - 4
1-6 years 1 11 - 12Total 8 28 5 31
Complications occurred in 5 cases (14, 4%): ventriculitis (1 case), stent displacement (1 case),
and transitory (2-7days) oculomotor impairments (3 cases). It should be noted that vital functions
were normal during surgery in most cases (33 patients). There was no perioperative mortality. Four
children died in remote postoperative period from causes unrelated to our intervention.
Discussion
Our study is dedicated to possibilities of radical treatment of the isolated Fourth ventricle which
is one of the most difficult types of multilevel occlusion hydrocephalus. Most cases (66%) were
newborns and infants.
Traditional methods of normalization of CSF circulation in case of the entrapped Fourth
ventricle are mostly aimed at elimination of just one level of occlusion or creation of alternative
outflow of CSF outside CSF space (e.g. VP-shunting). However routine VP-shunting of lateral
ventricles in case of the isolated Fourth ventricle is not effective unless the latter is previously
connected to supratentorial parts of ventricular system. Combined shunting of lateral and the Fourth
ventricles with two separate shunting systems or Y-shaped connection of two ventricular catheters
of one shunt is feasible. Although such interventions are less traumatic than direct suboccipital
craniotomy they are often (up to 50%) coupled with different complications which result to shunt
dysfunction and its repeated revisions [ 32 ], as well as with brainstem injury during implantation of
ventricular catheter in the Fourth ventricle [ 6, 8, 15, 30, 32 ]. Shrinkage of the Fourth ventricle
after surgery causes a risk of contact of ventricular catheter with brainstem because the former is
located inside the ventricle cavity at acute or even right angle towards brainstem.
Restoration of intracerebral CSF circulation by direct removal of occlusion is an alternative to
combined VP-shunting of a lateral and the Fourth ventricles. It includes the above mentioned
suboccipital craniotomy and interventriculostomy, endoscopic plasty and stenting of aqueductus
and foramen Magendie, as well as combination of these techniques.
Indications for endoscopic interventriculostomias between the Fourth and lateral or the Third
ventricles are limited. They are performed when aqueductoplasty is impossible and ventricles are
significantly enlarged after chronic hydrocephalus. Walls of ventricles are coming in close contact
and become thin which enables to perform fistula between the Fourth and the Third or lateral
ventricles by dissection or puncture of a separating “membrane” [ 10, 27, 32 ].
Aqueductoplasty as well as interventriculostomy are more effective than the Fourth ventricle
shunting and less traumatic than suboccipital craniotomy [ 10, 32 ]. However there are possibilities
of acqueductal reocclusion (20%) and development of oculomotor disorders [ 27, 32 ].
Occlusion hydrocephalus is prevalent in infants. CSF spaces are usually blocked at aqueductus,
the Fourth ventricle outlets, craniovertebral junction and basal cisterns due to adhesions [ 28 ].
Endoscopic interventions might irritate the existing adhesive process both in ventricular stenoses
and in basal subarachnoid space which increase a high risk of reocclusion. To prevent this
complication we combined endoscopic aqueductoplasty and stenting, and supplemented
interventriculostomias by implantation of the catheter between ventricles. It is important to note that
during postoperative reduction of the Fourth ventricle the stent remains located parallel to
brainstem. It eliminates the risk of irritation of brainstem structures. Fixation of stent or catheter
position in ventricular system and prevention of their migration is an important condition of
successful surgery. It is achieved by fixation of the stent or the catheter to cranial bone or dura
mater as well as to the shunt pump. In the last case the stent functions as a ventricular catheter of a
panventricular shunt and allows draining all cerebral ventricles evenly. Interestingly, in case of
panventricular shunting a ventricle shrinks similar to separate implantation of aqueductal stent and
standard VP-shunting. It should be noted that endoscopic stenting did not require stereotactic or
navigation equipment [ 34 ]. In all cases of stenting we managed to restore CSF outflow from the
Fourth ventricle.
The result was positive even in case of temporal stenting with subsequent stent removal. In 3
cases aqueductal stenting during 1 - 2,5 months was sufficient for a stable restoration of aqueductal
patency.
Aqueductal stenting was always combined with ITV in cases when we could not open
outlet foramina of the isolated Fourth ventricle. This enabled to divert CSF into subarachnoid
space and thus fully restore CSF circulation.
According to our experience and published literature aqueductoplasty is possible only in case of
membrane occlusion or “short” aqueductal stenosis [ 21, 25, 27 ]. In case of extensive aqueductal
obliteration the problem of isolated Fourth ventricle might be solved by interventriculostomy or
communication between the Fourth ventricle and the Third or lateral ventricles in circumvention of
aqueduct via cisterna ambiens. This could be done with or without implantation of catheters. In one
case we performed paraaqueductal Third-Fourth ventriculocisternostomia: in circumvention of
aqueduct we perforated posterior wall of the Third ventricle and connected it with cisterna ambiens,
then we perforated of upper velum cerebelli and penetrated into the Fourth ventricle. Combination
of two procedures provided communication between the Third and the Fourth ventricles and
cisterna ambiens. Occlusion of caudal parts of the Fourth ventricle was removed by resection of a
membrane in foramen Magendie and outing to cisterna magna.
Similarly in 2 cases we performed communication between the Fourth and lateral ventricles and
cisterna ambiens with simultaneous implantation of autonomous (1) or ventriclar (1) shunt catheter
in order to prevent obliteration of created stomae. Thus we provided internal decompression of the
Fourth ventricle without its extracranial shunting.
In direct surgery of the Fourth ventricle with suboccipital craniotomy the occlusion is removed
by dissection of adhesions which obliterate foramen Magendie or by dissection of vermis cerebelli
[ 7, 35 ]. Some authors recommend completing this intervention by implantation of the catheter
between the Fourth ventricle and spinal subarachnoid space [ 7 ]. This technique allows placing the
catheter along midline of the Fourth ventricle which reduces a risk of brainstem damage. The
surgery restores physiological CSF circulation. It does not require use of valve devices and presents
a justified alternative to a “classical” ventriculoperitoneal shunting of the Fourth ventricle. However
this surgery is traumatic which is a serious obstacle to its use in newborns (especially in premature
neonates) and infants. Our technique of endoscopic perforating ventriculosubaracnoidal stenting of
CSF system is much more conservative. In our opinion, there are two basic advantages of our
method of stenting of CSF system. First, it restores CSF circulation inside ventricular system and
simultaneously allows CSF outflow into subarachnoid space. Second, traumatic suboccipital
craniotomy is unnecessary which makes possible to use our method in newborns and infants.
The efficacy of the proposed method of perforating ventriculosubaracnoidal stenting is not yet
proven. However it should be noted that we achieved compensation of hydrocephalus without
additional VP-shunting in 2 children out of 6 who underwent ventriculosubarachnoidal stenting.
There was reduction of cavities of the Third and lateral ventricles to slit ventricles,
diminution of foramen Magendie, aqueductal occlusion and formation of the isolated Fourth
ventricle in 4 children after VP-shunting for tetraventricular obstructive hydrocephalus.
Similar changes in ventricular system after shunting procedures are reported by other authors
[ 2, 3, 22, 23, 24, 32 ].
In such cases we used a method of controlled forced enlargement of lateral and the Third
ventricles in order to eliminate occlusions. This enables us to use endoscopic technique for restoring
communication between all compartments of ventricular system and achieve normal functioning of
a VP-shunt. There were two options for ventricles’ enlargement. First, a peritoneal catheter of a
previously implanted and functioning VP-shunt was removed from peritoneal cavity and connected
to a system of external drainage [ 3, 32 ]. This created resistance to CSF within the system which
contributed to gradual collection of CSF inside ventricles and their enlargement. After occlusion
was eliminated the surgery ended by restoration of VP-shunt.
Second option was used when the width of lateral ventricles was sufficient for insertion of the
endoscope. There was single-step forced dilatation of ventricles by filling them with normal saline
under endoscopic control. This option allowed reducing time for preparation for surgery and
preserve previously implanted VP-shunt.
In general we can assert that our technique of endoscopic plasty and stenting of obstructed CSF
pathways at different levels (from foramen Monroe to craniovertebral junction) and catheterization
of the artificial Fourth ventricle fistulae provides long-lasting effect of our minimally-invasive
intervention.
There are several advantages of surgical tactics. First, there is one supratentorial approach via
fontanelle or a burr-hole. Second, it can be combined with other endoscopic procedures. Third,
endoscopic control allows performing surgery without navigation and stereotactic equipment.
Fourth, the stent (catheter) is placed at midline of the Fourth ventricle along brainstem and
maintains such location after reduction of the ventricle volume in remote postoperation period
which allows avoiding injury of brainstem structures by the catheter. Fifth, in case there is a need
for extracranial CSF drainage we can use the stent (catheter) as a ventricular catheter of VP-shunt or
as an external drainage thus providing CSF outflow both from ventricles and subarachnoid space.
Conclusions:
1. In children (and especially in infants) endoscopic intervention is a method of choice in treatment
of the isolated Fourth ventricle.
2. Restoration of physiological CSF circulation is a priority in the isolated Fourth ventricle. It is
achieved by elimination of occlusion between cerebral ventricles (aqueductoplasty,
interventriculostomy) and simultaneous restoration of CSF flow into subarachnoid space (plasty of
foramina Magendie and Luschka, craniovertebral junction, Third- ventriculocisternostomy, and
Third-Fourth ventriculocisternostomy).
3. The technique of forced aqueductal enlargement does not induce irreversible neurological deficit.
This technique is essential for success of our surgical approaches.
4. Endoscopic aqueductal stenting and catheterization of artificial fistulae of the Fourth ventricle
prevents reocclusions and provides optimal position of a stent or a catheter in relation to brainstem
in the Fourth ventricle cavity.
5. Craniovertebral junction and foramina Luschka are accessible targets in endoscopic surgery for
the entrapped Fourth ventricle in children of all ages.
6. The use of endoscopic technique in implantation of panventriculoperitoneal shunt which equally
drains all cerebral ventricles allows to perform surgery without stereotactic and navigation devices.
7. In case of impaired CSF resorption ventriculosubarachnoidal stenting which provides
communication between all CSF spaces is concluded by implantation of a system for extracranial
drainage. Unlike routine VP-shunting for occlusion hydrocephalus this allows to evacuate CSF both
from ventricular system and subarachnoid space.
8. Formation of paraaqueductal pathway from the Fourth ventricle to cisterna ambiens by
endoscopic approach via the Third or a lateral ventricle is one of options for draining of the Fourth
ventricle into subarachnoidal space in case of intractable aqueductal occlusion.
9. Forced dilatation of the slit lateral and Third ventricles after shunting for occlusion
hydrocephalus gives opportunity for aqueductoplasty and aqueductal stenting in the isolated Fourth
ventricle.
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Research and Practice Center for Children with Craniofacial Malforvations and Congenital Nervous System Diseases.
(119620, Moscow, Aviatorov str., 38)
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