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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 ular volumes had decreased in both the patients at initial
for hydrocephalus associated with occipital encephalocoele: re- follow-up.
port of two cases. Surg Neurol 2002;57:351355.
BACKGROUND ETV can be an effective treatment option for
Occipital encephalocoele is the most common cranial encephalocoele-associated hydrocephalus, even in chil-
dysraphism in the western hemisphere and is often com- dren under the age of 1 year. It may obviate the need for
plicated by hydrocephalus. Management of hydrocepha- placement of CSF shunts that have a risk of infection and
lus and reducing the CSF pressure is crucial in preventing dysfunction. However, delayed failure of ETV may occur
dehiscence at the site of the encephalocoele repair. as seen in our first patient, indicating the need for careful
and long-term follow-up. 2002 by Elsevier Science Inc.
Two female patients had presented with occipital en- KEY WORDS
cephalocoeles. The first patient (aged 42 days) had un- Occipital encephalocoele, endoscopic third ventriculos-
dergone repair of the occipital encephalocoele and then tomy, shunt failure.
developed hydrocephalus with recurrence of the enceph-
alocoele. The second patient (aged 12 months) had hy-
drocephalus associated with an occipital encephalocoele ccipital encephalocoele is the most common
at initial presentation.
Both the patients underwent endoscopic third ventric-
O type of cranial neural tube fusion defect seen
in the west although in the orient, basal encepha-
ulostomy (ETV) through a right frontal burr hole. In the
first patient, ETV was performed after shunt dysfunction
locoeles are more common [1]. Studies have re-
at the age of 9 months. Because she presented with re- vealed that hydrocephalus is often associated with
currence of the encephalocoele 15 months later, a repeat this condition [2,10,13]. CSF diversion procedures
endoscopic third ventriculostomy was performed. She in the form of ventriculoperitoneal shunts have
required a ventriculoperitoneal shunt during the same been reported to improve the morbidity related to
admission because of the early failure of the ventriculos-
tomy. In the second patient, it was performed before the
the condition [2]. Moreover, CSF diversion proce-
encephalocoele repair, both ETV and the repair being dures done before repair of the encephalocoele
conducted under the same anesthesia. ETV was per- would help to protect the repair. We report the use
formed using a rigid scope and the perforation in the of endoscopic third ventriculostomy (ETV) in the
third ventricular floor was enlarged using a No. 4 Fogarty management of hydrocephalus associated with oc-
cipital encephalocoele in two infants.
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. Case Description
The second patient had no evidence of recurrence at
follow-up after 16 months. The lateral and third ventric- CASE 1
A 42-day-old female child presented to us with a
history of a large swelling in the back of the head
Address reprint requests to: Dr Vedantam Rajshekhar, Department of since birth. The antenatal period was uneventful.
Neurological Sciences, Christian Medical College Hospital, Vellore 632004,
There were no other complaints. Physical examina-
Received January 10, 2001; accepted November 28, 2001. tion revealed polydactyly in the right hand. There
2002 by Elsevier Science Inc. 0090-3019/02/$see front matter
655 Avenue of the Americas, New York, NY 10010 PII S0090-3019(02)00696-1
352 Surg Neurol Moorthy and Rajshekhar

CT scan (Patient 1) showing dilated lateral ventri- CT scan (Patient 1) 10 months after ETV showing
1 cles with the ventricular end of the shunt tube in
2 decompressed lateral ventricles. The subarachnoid
situ. spaces over the convexity are well visualized.

was a 15 12 10 cm occipital swelling arising the ventricle was tapped at a depth of 4 cm. The
from below the external occipital protuberance. right lateral ventricle was enlarged. The right fora-
The swelling was soft, fluctuant, and translucent men of Monro was large and the floor of the third
and there was a transmitted impulse on coughing. ventricle was thinned out and easily perforated
The posterior fontanelle was lax and pulsatile and with a No. 4 Fogarty catheter. She had an uneventful
the head circumference was 32 cm. Computed to- postoperative period. Follow-up after 10 months
mography (CT) scan of the head showed a swelling revealed that the occipital swelling had decreased
in the region of a bony defect in the occipital region and the head circumference was 44.5 cm. She was
consisting of hypodense areas and neural elements. still unable to walk. CT scan done at this time
The ventricular system was normal. Excision of the showed that the ventricles were decompressed and
encephalocoele sac with repair of the defect was there was no soft tissue swelling over the occipital
conducted. She had an uneventful postoperative region (Figure 2). The volume of the lateral ventri-
period. cles had decreased from 55 mL to 14 mL. She re-
Five months later she was brought in with com- mained asymptomatic for hydrocephalus for 15
plaints of delayed motor development with associ- months after the procedure. The swelling had since
ated increase in head size. Physical examination recurred and there was regression in her mile-
revealed that the swelling in the occipital region stones with intermittent fever and vomiting. The
had recurred. The head circumference was 39 cm. head circumference had increased to 47 cm. She
An ultrasonogram of the ventricular system showed underwent a repeat endoscopic third ventriculos-
dilated third and lateral ventricles. A right ventricu- tomy. Intraoperatively, the opening in the third ven-
loperitoneal shunt was placed. tricular floor was found to have closed up. The
Two months later (at 9 months of age) the occip- encephalocoele sac regressed after the repeat ETV.
ital swelling recurred and there was significant in- However, she developed CSF leak from the wound
crease in the head circumference. Physical exami- on the seventh postoperative day. A left ventricu-
nation revealed head circumference of 43.5 cm with loperitoneal shunt was placed to ensure CSF diver-
a lax and pulsatile posterior fontanelle. A translu- sion at this stage and she was discharged after an
cent, soft occipital swelling was palpable. CT scan uneventful early postoperative period.
of the brain showed dilated lateral and third ven-
tricles with the shunt tube in place indicating shunt CASE 2
dysfunction (Figure 1). A 1-year-old female child was brought with com-
An ETV was performed to treat shunt dysfunction plaints of progressively increasing occipital swell-
and provide alternate CSF diversion. Intraopera- ing since birth and progressively increasing head
tively, the CSF was under moderate pressure and size associated with delayed motor milestones. The
Endoscopic Third Ventriculostomy Surg Neurol 353

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
CT scan (Patient 2) showing A) a large occipital 4.09 mL to 3.75 mL (Figure 4).
3 swelling protruding through the bony defect and B)
dilated third and lateral ventricles.
antenatal and perinatal period had been uneventful. HYDROCEPHALUS ASSOCIATED
Physical examination revealed head circumference WITH OCCIPITAL CEPHALOCOELES
of 50 cm. There was an 8 6 cm midline translucent Mealey et al. [10] have reviewed the prognostic
occipital swelling with an expansile cough impulse. factors associated with occipital encephalocoeles.
Central nervous system examination revealed left Hydrocephalus, which was seen in 36% of their
lateral rectus paresis with no other focal neurologic patients, was an unfavorable factor with increased
deficits. morbidity and mortality. In their experience, a
CT scan of the head showed a large cystic mass in shunt did not alter quality of survival. In another
the occipital region extracranially with a bony de- series, hydrocephalus and other abnormalities of
fect overlying the region of the torcula. There was ventricular development like holoprosencephaly
dilatation of the third and the lateral ventricles have been associated with occipital cephalocoeles
(Figure 3). The fourth ventricle was in normal posi- [13]. Shunts were the modality of treatment used
tion and was normal in size. An ETV was done for CSF diversion in this series also. In another
followed by repair of the occipital encephalocoele. report of occipital meningocoeles associated with
Intraoperatively the right frontal horn was tapped Dandy-Walker syndrome, CSF diversion via a shunt
354 Surg Neurol Moorthy and Rajshekhar

procedure was found to collapse the encephalo- tency of the opening has not been proved as all his
coele [2]. patients had concomitant shunt placement.
Studies on the pathogenesis of occipital encepha- In our first case, hydrocephalus manifested itself
locoeles have shown that herniation and displace- later in the course of the illness after the occipital
ment of the mesencephalon and roof of the dien- encephalocoele had been repaired. Although a
cephalon is the primary occurrence [9]. This would shunt was placed, it failed to function and hence it
lead to stretching of the midbrain and distortion of was decided to perform a third ventriculostomy. It
the aqueduct thus resulting in hydrocephalus. 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-
Hydrocephalus associated with myelomeningo- lostomy was conducted. This also failed and an
coele has been ascribed to aqueductal stenosis and alternate CSF diversion procedure was required.
it is known to be exacerbated after closure of the Learning from the initial experience in our first
defect. While 15% of patients show clinical signs of patient, in the second case, ETV was utilized to
hydrocephalus at birth, 80% present in early in- divert the CSF before the repair of the encephalo-
fancy and a ventriculoperitoneal shunt is the pre- coele. In this patient also the CSF was only under
ferred mode of management [6]. McLone et al. [5] moderate pressure and a shunt may not have been
have observed that the degree of ventricular en- sufficient to divert the CSF.
largement may not significantly affect development
In conclusion, ETV is an useful option in patients
in these children. However, ventriculitis secondary
with hydrocephalus associated with occipital en-
to shunt infection has a definite negative impact on
cephalocoele and can be performed before the re-
the intelligence outcome in these children.
pair of the encephalocoele. Although delayed fail-
ETV is being used successfully to treat shunt
ure of the ventriculostomy may occur, it can be
failures in children with lumbar meningomyeloco-
used at least as a temporary measure to delay
eles who developed hydrocephalus after closure of
placement of the shunt, especially in cases of recent
the defect [7,8,11,14]. However, it has been ob-
shunt dysfunction or infection.
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 REFERENCES
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7. Jones RFC, Brazer DH, Kwok BC, Stening WA. Neu-
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Endoscopic Third Ventriculostomy Surg Neurol 355

cephalocoele and a discussion of pathogenesis. Pa- tomy has its own problems. It certainly can be a
thology 1970;11:22334. successful treatment of hydrocephalus, but the
10. Mealey J Jr, Dzenitis AJ, Hockey AA. The prognosis of
encephalocoeles. J Neurosurg 1970;32:204 218. question is always What does successful treatment
11. Natelson SE. Early third ventriculostomy in meningo- mean?
myelocoele infantsshunt independence? Childs In both of these cases, an endoscopic third ven-
Brain 1981;8:3215. triculostomy seems to have had a significant effect.
12. Patterson RH, Bergland RM. The selection of patients
In the first case, however, one could say that the
for third ventriculostomy based on experience with
33 operations. J Neurosurg 1968;29:252 4. child had been exposed to the potential risks of a
13. Simpson DA, David DJ, White J. Cephalocoeles. Treat- third ventriculostomy with no benefit. In the second
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endoscopic third ventriculostomy in patients with
question to ask whether that child would be better
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63. 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
Moorthy et al present two cases of occipital en-
to debate. I think only by honest reporting of long-
cephalocele in which hydrocephalus was managed
term outcomes will we be able to make any kind of
using a third ventriculostomy. In one of those pa-
statement about its true worth.
tients, a third ventriculostomy appeared to be the
only treatment necessary; in the other, a shunt was John A. Grant, M.D.
finally needed. The authors are right to point out Pediatric Neurosurgery
the potential dangers of placing a ventriculoperito- Childrens Memorial Hospital
neal shunt; however, endoscopic third ventriculos- Chicago, Illinois

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Nickel and Dimed: On (Not) Getting by in America