Endoscopy

Management of Hydrocephalus
Associated with Occipital
Encephalocoele using Endoscopic
Third Ventriculostomy: Report of
Two Cases
Ranjith K. Moorthy, M.B.B.S., and Vedantam Rajshekhar, M.Ch.
Department of Neurological Sciences, Christian Medical College Hospital, Vellore, India

Moorthy RK, Rajshekhar V. Endoscopic third ventriculostomy
for hydrocephalus associated with occipital encephalocoele: re-
port of two cases. Surg Neurol 2002;57:351355.
BACKGROUND
Occipital encephalocoele is the most common cranial
dysraphism in the western hemisphere and is often com-
plicated by hydrocephalus. Management of hydrocepha-
lus and reducing the CSF pressure is crucial in preventing
dehiscence at the site of the encephalocoele repair.
METHODS
Two female patients had presented with occipital en-
cephalocoeles. The first patient (aged 42 days) had un-
dergone repair of the occipital encephalocoele and then
developed hydrocephalus with recurrence of the enceph-
alocoele. The second patient (aged 12 months) had hy-
drocephalus associated with an occipital encephalocoele
at initial presentation.
Both the patients underwent endoscopic third ventric-
ulostomy (ETV) through a right frontal burr hole. In the
first patient, ETV was performed after shunt dysfunction
at the age of 9 months. Because she presented with re-
currence of the encephalocoele 15 months later, a repeat
endoscopic third ventriculostomy was performed. She
required a ventriculoperitoneal shunt during the same
admission because of the early failure of the ventriculos-
tomy. In the second patient, it was performed before the
encephalocoele repair, both ETV and the repair being
conducted under the same anesthesia. ETV was per-
formed using a rigid scope and the perforation in the
third ventricular floor was enlarged using a No. 4 Fogarty
catheter.
RESULTS
The first patient had no recurrence of encephalocoele at
follow-up of 10 months but she presented with recur-
rence of the occipital encephalocoele after 15 months.
The second patient had no evidence of recurrence at
follow-up after 16 months. The lateral and third ventric-
Address reprint requests to: Dr Vedantam Rajshekhar, Department of
Neurological Sciences, Christian Medical College Hospital, Vellore 632004,
India.
Received January 10, 2001; accepted November 28, 2001.
2002 by Elsevier Science Inc.
655 Avenue of the Americas, New York, NY 10010
ular volumes had decreased in both the patients at initial
follow-up.
CONCLUSION
ETV can be an effective treatment option for
encephalocoele-associated hydrocephalus, even in chil-
dren under the age of 1 year. It may obviate the need for
placement of CSF shunts that have a risk of infection and
dysfunction. However, delayed failure of ETV may occur
as seen in our first patient, indicating the need for careful
and long-term follow-up. 2002 by Elsevier Science Inc.
KEY WORDS
Occipital encephalocoele, endoscopic third ventriculos-
tomy, shunt failure.
ccipital encephalocoele is the most common
O type of cranial neural tube fusion defect seen
in the west although in the orient, basal encepha-
locoeles are more common [1]. Studies have re-
vealed that hydrocephalus is often associated with
this condition [2,10,13]. CSF diversion procedures
in the form of ventriculoperitoneal shunts have
been reported to improve the morbidity related to
the condition [2]. Moreover, CSF diversion proce-
dures done before repair of the encephalocoele
would help to protect the repair. We report the use
of endoscopic third ventriculostomy (ETV) in the
management of hydrocephalus associated with oc-
cipital encephalocoele in two infants.
Case Description
CASE 1
A 42-day-old female child presented to us with a
history of a large swelling in the back of the head
since birth. The antenatal period was uneventful.
There were no other complaints. Physical examina-
tion revealed polydactyly in the right hand. There
0090-3019/02/$see front matter
PII S0090-3019(02)00696-1
352 Surg Neurol
2002;57:351355
CT scan (Patient 1) showing dilated lateral ventri-
1 cles with the ventricular end of the shunt tube in
situ.
was a 15 12 10 cm occipital swelling arising
from below the external occipital protuberance.
The swelling was soft, fluctuant, and translucent
and there was a transmitted impulse on coughing.
The posterior fontanelle was lax and pulsatile and
the head circumference was 32 cm. Computed to-
mography (CT) scan of the head showed a swelling
in the region of a bony defect in the occipital region
consisting of hypodense areas and neural elements.
The ventricular system was normal. Excision of the
encephalocoele sac with repair of the defect was
conducted. She had an uneventful postoperative
period.
Five months later she was brought in with com-
plaints of delayed motor development with associ-
ated increase in head size. Physical examination
revealed that the swelling in the occipital region
had recurred. The head circumference was 39 cm.
An ultrasonogram of the ventricular system showed
dilated third and lateral ventricles. A right ventricu-
loperitoneal shunt was placed.
Two months later (at 9 months of age) the occip-
ital swelling recurred and there was significant in-
crease in the head circumference. Physical exami-
nation revealed head circumference of 43.5 cm with
a lax and pulsatile posterior fontanelle. A translu-
cent, soft occipital swelling was palpable. CT scan
of the brain showed dilated lateral and third ven-
tricles with the shunt tube in place indicating shunt
dysfunction (Figure 1).
An ETV was performed to treat shunt dysfunction
and provide alternate CSF diversion. Intraopera-
tively, the CSF was under moderate pressure and
Moorthy and Rajshekhar
CT scan (Patient 1) 10 months after ETV showing
2 decompressed lateral ventricles. The subarachnoid
spaces over the convexity are well visualized.
the ventricle was tapped at a depth of 4 cm. The
right lateral ventricle was enlarged. The right fora-
men of Monro was large and the floor of the third
ventricle was thinned out and easily perforated
with a No. 4 Fogarty catheter. She had an uneventful
postoperative period. Follow-up after 10 months
revealed that the occipital swelling had decreased
and the head circumference was 44.5 cm. She was
still unable to walk. CT scan done at this time
showed that the ventricles were decompressed and
there was no soft tissue swelling over the occipital
region (Figure 2). The volume of the lateral ventri-
cles had decreased from 55 mL to 14 mL. She re-
mained asymptomatic for hydrocephalus for 15
months after the procedure. The swelling had since
recurred and there was regression in her mile-
stones with intermittent fever and vomiting. The
head circumference had increased to 47 cm. She
underwent a repeat endoscopic third ventriculos-
tomy. Intraoperatively, the opening in the third ven-
tricular floor was found to have closed up. The
encephalocoele sac regressed after the repeat ETV.
However, she developed CSF leak from the wound
on the seventh postoperative day. A left ventricu-
loperitoneal shunt was placed to ensure CSF diver-
sion at this stage and she was discharged after an
uneventful early postoperative period.
CASE 2
A 1-year-old female child was brought with com-
plaints of progressively increasing occipital swell-
ing since birth and progressively increasing head
size associated with delayed motor milestones. The
Endoscopic Third Ventriculostomy
CT scan (Patient 2) showing A) a large occipital
3 swelling protruding through the bony defect and B)
dilated third and lateral ventricles.
antenatal and perinatal period had been uneventful.
Physical examination revealed head circumference
of 50 cm. There was an 8 6 cm midline translucent
occipital swelling with an expansile cough impulse.
Central nervous system examination revealed left
lateral rectus paresis with no other focal neurologic
deficits.
CT scan of the head showed a large cystic mass in
the occipital region extracranially with a bony de-
fect overlying the region of the torcula. There was
dilatation of the third and the lateral ventricles
(Figure 3). The fourth ventricle was in normal posi-
tion and was normal in size. An ETV was done
followed by repair of the occipital encephalocoele.
Intraoperatively the right frontal horn was tapped
Surg Neurol 353
2002;57:351355
CT scan (Patient 2) 16 months after ETV showing
4 mild decrease in size of the ventricles.
at a depth of 3 cm and CSF was drained under
moderate to low pressure. The floor of the third
ventricle was thinned out and was perforated with
a No. 4 Fogarty catheter. There was a cystic mass in
the occipital region containing CSF with brain tissue
seen protruding through the bony defect. The sac
was excised and the walls were closed watertight.
The postoperative period was uneventful. A re-
peat CT scan at 16 months showed adequate de-
compression of the ventricles with a small residual
occipital swelling protruding through the midline
bony defect. The volume of the lateral ventricles
had decreased from 132.2 mL to 95.69 mL and the
volume of the third ventricle had decreased from
4.09 mL to 3.75 mL (Figure 4).
Discussion
HYDROCEPHALUS ASSOCIATED
WITH OCCIPITAL CEPHALOCOELES
Mealey et al. [10] have reviewed the prognostic
factors associated with occipital encephalocoeles.
Hydrocephalus, which was seen in 36% of their
patients, was an unfavorable factor with increased
morbidity and mortality. In their experience, a
shunt did not alter quality of survival. In another
series, hydrocephalus and other abnormalities of
ventricular development like holoprosencephaly
have been associated with occipital cephalocoeles
[13]. Shunts were the modality of treatment used
for CSF diversion in this series also. In another
report of occipital meningocoeles associated with
Dandy-Walker syndrome, CSF diversion via a shunt
354 Surg Neurol
2002;57:351355
procedure was found to collapse the encephalo-
coele [2].
Studies on the pathogenesis of occipital encepha-
locoeles have shown that herniation and displace-
ment of the mesencephalon and roof of the dien-
cephalon is the primary occurrence [9]. This would
lead to stretching of the midbrain and distortion of
the aqueduct thus resulting in hydrocephalus.
THIRD VENTRICULOSTOMY FOR
HYDROCEPHALUS ASSOCIATED
WITH NEURAL TUBE DEFECTS
Hydrocephalus associated with myelomeningo-
coele has been ascribed to aqueductal stenosis and
it is known to be exacerbated after closure of the
defect. While 15% of patients show clinical signs of
hydrocephalus at birth, 80% present in early in-
fancy and a ventriculoperitoneal shunt is the pre-
ferred mode of management [6]. McLone et al. [5]
have observed that the degree of ventricular en-
largement may not significantly affect development
in these children. However, ventriculitis secondary
to shunt infection has a definite negative impact on
the intelligence outcome in these children.
ETV is being used successfully to treat shunt
failures in children with lumbar meningomyeloco-
eles who developed hydrocephalus after closure of
the defect [7,8,11,14]. However, it has been ob-
served that the rate of failure of ETV is higher in
infancy [7]. In a series of 27 children under the age
of one year with aqueductal stenosis undergoing
ETV, it functioned only in 6 [3]. Cinelli et al. [4], in
their review of 213 children who underwent third
ventriculostomy (114 underwent ETV and the rest
had ventriculographic guided ventriculostomy) for
mechanical obstruction of the aqueduct, observed
that failure was detected soon after the procedure
in children less than 6 months of age. The age at
which the procedure was conducted did not affect
the long-term outcome in their patients. They have
concluded that age should not be a deterrent to
performing ETV. In their series, there were three
patients with encephalocoeles but it is not stated
whether they underwent third ventriculostomy
with endoscopic guidance.
The high rate of failure of ETV in neonates and
infants has been attributed to the suboptimal de-
velopment of the subarachnoid spaces as well as
the absorptive pathways over the brain (because of
intrauterine escape of CSF) in rapidly progressive
hydrocephalus. Malposition of the floor of the third
ventricle also makes ETV a more difficult and riskier
option in neonates [7]. While Natelson [11] has
reported success with percutaneous third ventric-
ulostomies in patients with meningocoele, the pa-
Moorthy and Rajshekhar
tency of the opening has not been proved as all his
patients had concomitant shunt placement.
In our first case, hydrocephalus manifested itself
later in the course of the illness after the occipital
encephalocoele had been repaired. Although a
shunt was placed, it failed to function and hence it
was decided to perform a third ventriculostomy. It
is possible that one of the causes of shunt dysfunc-
tion was that the mild elevation in CSF pressure was
inadequate to open the shunt valve. However, after
about 15 months, there was evidence of dysfunc-
tion of the ventriculostomy and a repeat ventricu-
lostomy was conducted. This also failed and an
alternate CSF diversion procedure was required.
Learning from the initial experience in our first
patient, in the second case, ETV was utilized to
divert the CSF before the repair of the encephalo-
coele. In this patient also the CSF was only under
moderate pressure and a shunt may not have been
sufficient to divert the CSF.
In conclusion, ETV is an useful option in patients
with hydrocephalus associated with occipital en-
cephalocoele and can be performed before the re-
pair of the encephalocoele. Although delayed fail-
ure of the ventriculostomy may occur, it can be
used at least as a temporary measure to delay
placement of the shunt, especially in cases of recent
shunt dysfunction or infection.
REFERENCES
1. Bhagwati SN, Mahapatra AK. Encephalocoeles and
anomalies of the scalp. In: Choux M, Di Rocco C,
Hockly AD, Walker ML, eds. Paediatric Neurosurgery.
London: Churchill Livingstone, 1999:101120.
2. Bindal AK, Storrs BB, McLone DG. Occipital meningo-
coeles in patients with Dandy Walker syndrome. Neu-
rosurgery 1991;29:844 7.
3. Buxton N, Macarthur D, Mallucci C, Punt J, Vloe-
berghs M. Neuroendoscopic third ventriculostomy in
patients less than one year old. Pediatr Neurosurg
1998;29:73 6 (abstr).
4. Cinelli G, Sainte-Rose C, Chumas P, et al. Failure of
third ventriculostomy in the treatment of aqueductal
stenosis in children. J Neurosurg 1999;90:448 54.
5. Dias MS, McLone DG. Hydrocephalus in the child with
dysraphism. Neurosurg Clin N Am 1993;4:71526.
6. Hahn YS. Open myelomeningocoele. Neurosurg Clin N
Am 1995;6:231 41.
7. Jones RFC, Brazer DH, Kwok BC, Stening WA. Neu-
roendoscopic third ventriculostomy. In: Cohen AR,
Haines SJ, eds. Minimally Invasive Techniques in Neu-
rosurgery, Concepts in Neurosurgery. Philadelphia:
Williams and Wilkins, 1995;7:33 49.
8. Jones RF, Kwok BC, Stening WA, Vonau M. Third
ventriculostomy for hydrocephalus associated with
spinal dysraphism: indications and contraindica-
tions. Eur J Paediatr Surg 1996;1:5 6 (abstr).
9. Leong ASY, Shaw CM. The pathology of occipital en-
Endoscopic Third Ventriculostomy Surg Neurol 355
2002;57:351355

cephalocoele and a discussion of pathogenesis. Pa-
thology 1970;11:22334.
10. Mealey J Jr, Dzenitis AJ, Hockey AA. The prognosis of
encephalocoeles. J Neurosurg 1970;32:204 218.
11. Natelson SE. Early third ventriculostomy in meningo-
myelocoele infantsshunt independence? Childs
Brain 1981;8:3215.
12. Patterson RH, Bergland RM. The selection of patients
for third ventriculostomy based on experience with
33 operations. J Neurosurg 1968;29:252 4.
13. Simpson DA, David DJ, White J. Cephalocoeles. Treat-
ment, outcome and antenatal diagnosis. Neurosur-
gery 1984;15:14 21.
14. Teo C, Jones R. Management of hydrocephalus by
endoscopic third ventriculostomy in patients with
myelomeningocoele. Pediatr Neurosurg 1996;25:57
63.
COMMENTARY
Moorthy et al present two cases of occipital en-
cephalocele in which hydrocephalus was managed
using a third ventriculostomy. In one of those pa-
tients, a third ventriculostomy appeared to be the
only treatment necessary; in the other, a shunt was
finally needed. The authors are right to point out
the potential dangers of placing a ventriculoperito-
neal shunt; however, endoscopic third ventriculos-
tomy has its own problems. It certainly can be a
successful treatment of hydrocephalus, but the
question is always What does successful treatment
mean?
In both of these cases, an endoscopic third ven-
triculostomy seems to have had a significant effect.
In the first case, however, one could say that the
child had been exposed to the potential risks of a
third ventriculostomy with no benefit. In the second
case, the childs ventricles were not normal and the
child was not neurologically normal, so it is a fair
question to ask whether that child would be better
with small ventricles or not. In all fairness, the only
answer one can give to that question is that we do
not know.
The place of endoscopic third ventriculostomy in
pediatric neurosurgery is certainly one that is open
to debate. I think only by honest reporting of long-
term outcomes will we be able to make any kind of
statement about its true worth.
John A. Grant, M.D.
Pediatric Neurosurgery
Childrens Memorial Hospital
Chicago, Illinois

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