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Perioperative Management of Children With

Giant Encephalocele: A Clinical Report of 29
Cases
Article in Journal of neurosurgical anesthesiology February 2016
Impact Factor: 2.99 DOI: 10.1097/ANA.0000000000000282

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CLINICAL REPORT

Perioperative Management of Children With Giant

Encephalocele: A Clinical Report of 29 Cases
Charu Mahajan, MD, DM,* Girija P. Rath, MBBS, MD, DM,* Parmod K. Bithal, MD,* and
Ashok K. Mahapatra, MS, MChw

Background: Giant encephalocele, a rare entity, makes anesthesiologists wary of challenging anesthetic course. Apart from
inherent challenges of pediatric anesthesia, the anesthesiologist
has to deal with unusual positioning, dicult tracheal intubation, and associated anomalies during the perioperative
course.
Materials and Methods: Medical records of 29 children with
giant encephalocele, who underwent excision and repair, during
a period of 13 years, were retrospectively analyzed. Data pertaining to anesthetic management, perioperative complications,
and outcome at discharge were reviewed.
Results: The average age at admission was 164 days. Hydrocephalus and delayed milestones were present in 19 (65.5%) and
7 (24.1%) children, respectively. Diculty in tracheal intubation
was encountered, in 15 (51.7%) children. Tracheal intubation
was attempted with direct laryngoscopy, most often, in lateral
position (24 [82.8%]). Intraoperative hemodynamic and respiratory complications were observed in 9 (31.0%) and 5 (17.2%)
children, respectively. Intraoperative hypothermia was observed
in 4 (13.8%) children. The average stay in the intensive care unit
was 2.7 days and average hospital stay was 11.5 days. The
condition at discharge remained same as the preoperative period
in 24 children (82.7%), deteriorated in 2 (6.9%), and 3 children
(10.3%) died.
Conclusions: Management of children with giant encephalocele
requires the updated knowledge on possible diculties encountered during the perioperative period. They need specialized
anesthetic care for dealing with dicult tracheal intubation,
associated congenital anomalies, unusual positioning, electrolyte
abnormalities, hypothermia, and cardiorespiratory disturbances. For securing the airway, we suggest the practice of
direct laryngoscopy in lateral position after inhalational in-

Received for publication September 18, 2015; accepted December 30,

2015.
From the *Department of Neuroanaesthesiology & Critical Care,
Neurosciences Centre; and wNeurosurgery, All India Institute of
Medical Sciences (A.I.I.M.S.), New Delhi, India.
Presented at ASA Annual Meeting (Anesthesiology 2014) held at New
Orleans, LA.
The authors have no funding or conicts of interest to disclose.
Reprints: Girija P. Rath, MBBS, MD, DM, Department of Neuroanaesthesiology & Critical Care, Neurosciences Centre, All India
Institute of Medical Sciences (A.I.I.M.S.), New Delhi 110029, India
(e-mail: girijarath@yahoo.co.in).
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J Neurosurg Anesthesiol

Volume 00, Number 00, 2016

duction. Muscle relaxant should be administered only after

visualization of the glottis.
Key Words: giant encephalocele, anesthesia, airway management, complication
(J Neurosurg Anesthesiol 2016;00:000000)

ncephalocele is a type of cranial dysraphism in which

protrusion of cranial contents occur through a defect
in cranial vault leading to the formation of a sac. Defects
of embryogenesis, viral infections, hyperthermia, irradiation, hypervitaminosis, and use of salicylates in early
pregnancy have been implicated in its pathogenesis.1
Encephaloceles constitute 8% to 19% of all craniospinal
dysraphisms and about 15.6% of them are giant in size.2,3
The term Giant is often used to describe an encephalocele sac larger than the size of head (Fig. 1).4 Apart
from inherent challenges of pediatric anesthesia, the anesthesiologists may have to deal with unusual positioning
and dicult airway. Associated anomalies may occur in
approximately 60% of such children which may have a
bearing on the perioperative management.5 The literature
search on giant encephalocele revealed only isolated cases. Here, we describe the largest series of 29 such children
with an objective to share our clinical experience on
perioperative management.

MATERIALS AND METHODS

Medical records of children with giant encephalocele
who underwent excision and repair during a period of 13
years (2000 to 2012) were retrospectively analyzed. Data
pertaining to preanesthetic evaluation, intraoperative
course, and postoperative follow-up were collected. Preoperative data included age, sex, weight, signicant antenatal history, neurological presentation, cerebrospinal uid
(CSF) leak and associated systemic abnormalities. Record
of all available investigations like hemoglobin concentration, serum electrolytes, electrocardiogram, chest x-ray
and other radiologic ndings, was made. Intraoperative
data included anesthetic technique, uids infused, and
blood loss or transfusion. Cases with dicult airway (a
clinical situation in which a conventionally trained anesthesiologist experiences diculty with face mask ventilation, diculty with tracheal intubation, or both) were
noted. Intraoperative complications such as, hemodynamic
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Mahajan et al

J Neurosurg Anesthesiol

Volume 00, Number 00, 2016

RESULTS

FIGURE 1. Showing giant occipital encephalocele in an eight

month old child.

alterations (bradycardia and tachycardia, hypotension, and

hypertension; dened as 20% alteration in baseline values of
heart rate or systolic blood pressure, respectively), cardiac
arrest, respiratory events (bronchospasm, laryngospasm,
endobronchial intubation), hypothermia (< 351C), and hyperthermia (>381C) were also recorded. Postoperative parameters included duration of mechanical ventilation,
reintubation, complications, duration of intensive care unit/
hospital stay, and the condition at discharge (same as in
preoperative period [good], deteriorated, died). Data are
presented as mean (SD) or number (%) or median (range).

Anesthesia Technique
A standard anesthetic protocol was followed for all
the children. They were premedicated with oral atropine
20 to 40 mg/kg, 30 minutes before induction. Dicult
airway cart was kept ready. Sevourane was commonly
used for induction; and succinylcholine or rocuronium
was used to facilitate tracheal intubation. Trachea was
intubated with direct laryngoscopy most often in the right
lateral position. Insertion of ventriculoperitoneal (VP)
shunt and repair of encephalocele sac present on the
cranial vault was carried out in the supine position,
whereas for the repair of occipital encephalocele sac the
child was turned prone. Anesthesia was maintained with
either halothane or sevourane in a mixture of O2 and
N2O (1:2). Analgesia was provided with intermittent boluses of fentanyl. Rectal paracetamol suppository was
inserted just before surgical positioning for postoperative
analgesia. At the end of surgery, the residual neuromuscular block was reversed and trachea was extubated.

2 | www.jnsa.com

Medical records of 29 children who underwent excision and repair of giant encephalocele over the study
period were reviewed. Most of them were infants (Table 1) with the median age of admission being 4 months
(range, 2 d to 18 mo). The site of lesion was occipital in all
children except 2 in which the lesion was located in the
right fronto-parietal region. Two children were born
preterm by cesarean section, and the antenatal history in
rest of the children was unremarkable. The average size of
encephalocele sac was 2733 cm3 (range, 7350 to 960 cm3);
leakage of CSF was present in 4 children, but none of
them were associated with meningitis. Hydrocephalus
(HCP) was present in 19 (65.5%) children. The associated
cranial abnormalities were microcephaly (27.6%), corpuscallosal agenesis (17.2%), Arnold-Chiari malformation
(17.2%), and craniosynostosis (17.2%). Other associated
systemic anomalies encountered were syringomyelia, ectopic kidney, congenital acyanotic heart disease, and bilateral inguinal hernia. The milestones of development
were delayed in 7 (24.1%) children.
Inhalational induction was the technique of choice
in majority of these children (26 [89.7%] vs. 3 [10.3%] as
IV induction). Halothane was used for induction, before
2005, until sevourane was made available in our institute. The choice of muscle relaxant varied from succinylcholine or rocuronium for tracheal intubation to
atracurium or rocuronium for maintenance of anesthesia.
An initial attempt at direct laryngoscopy was made, most
often, in lateral position (24 [82.8%]). None of the children had diculty in mask ventilation whereas tracheal
intubation was dicult in 15 (51.7%) children (Table 2).
Three children, who could not be intubated in lateral
position, were subsequently placed supine with head held
beyond the edge of table. Encephalocele sac was decompressed with needle under sterile precaution to facilitate
proper positioning of the head and thus, easing tracheal
intubation in 3 patients. Anesthesia was maintained with
sevourane in a mixture of O2 and N2O (1:2). The average
blood loss and blood transfusion was 10.4 and 12.3 mL/
kg, respectively; average uid transfusion was 272.6 mL.
Two children suered intraoperative cardiac arrest and
were resuscitated successfully (Tables 2 and 3). HCP and
seizures were the common postoperative neurologic
complications (Table 4). Insertion of VP shunt followed
by excision-repair of encephalocele was done, in the same
sitting, in 9 children. Average duration of anesthesia and

TABLE 1. Demographic Variables

Variable

n (%)/mean SD

Age
0-30 d
> 30 d-1 y
>1y
Sex
Male
Female
Average weight (kg)

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7 (24.1%)
17 (58.6%)
5 (17.2%)
16 (55.2%)
13 (44.8%)
5.66 2.08

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Signicant Association

Anesthetic
Induction

Intraoperative Complications

4 mo

Microcephaly
Inhalational
Delayed milestones acyanotic CHD
bilateral inguinal hernia

8 mo

ACM III
Corpus callosal agenesis

Inhalational

3
4

1y
1 mo

HCP
HCP

Intravenous
Inhalational

5
6

3 mo HCP
13 mo Delayed milestones, HCP, corpus
callosal agenesis
Syringomyelia
5 mo HCP, ACM IV
Coronal synostosis microcephaly
cervical spina bida

Inhalational
Inhalational

9 mo

Inhalational

Dicult intubation: DL failed twice in lateral

position, child placed supine with head held
beyond the edge of table, successfully
intubated in next attempt by direct
laryngoscopy, CL III
Episodes of desaturation, hypercarbia,
tachycardia, hematuria, tall T waves
Hypotension

2d

Inhalational

Uneventful

Condition at
Discharge

10

11

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12

ACM III
Corpus callosum agenesis
Polymicrogyria
Craniosynostosis
Microcephaly
Delayed milestones
Corpus callosal agenesis, HCP

2.5 mo Anemia, HCP

10 d

Inhalational
(halothane)

Intravenous+SCh

HCP, syrinx from C3 level to lower Inhalational

dorsal
(halothane)
Sacral dermal sinus

1.5 mo Craniosynostosis
Microcephaly

Inhalational
(halothane)

Uneventful

Good

Pseudomeningocele, seizures, HCP,

respiratory distress, reintubation, VP
shunt

Good

Uneventful
VP shunt on POD2

Good
Good

HCP, pneumoventricle VP shunt POD3

Blood transfused

Good
Deteriorated (lower
limbs weakness)

Uneventful

Good

Fever

Good

Physiological jaundice, VP shunt on

POD17
Dicult intubation: failed 2 attempts of DL in Postoperatively ventilated in view of
right lateral position, atropine 0.1 mg given,
hypothermia
intubated in third attempt. CL III
Fever
Hypothermia33 C
Dicult intubation: failed 2 attempts of DL in Uneventful
left lateral position, child placed supine with
head held beyond the edge of table,
successfully intubated with DL in next
attempt. CL III
Hypercarbia
Mask ventilation possible
Electively ventilation in view of
Dicult intubation: failed 3 attempts of DL in
hypothermia
right lateral position, child placed supine with Wound infection, meningitis, fever, HCP,
head held beyond the edge of table,
brain abscess, sepsis, gangrene foot,
successfully intubated over bougie with DL in metabolic acidosis, hyperkalemia,
fth attempt. CL III
cardiac arrest (asystole), resuscitation
Bradycardia, hypothermia
unsuccessful

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Dicult intubation: inhalational induction, 2

attempts of DL failed, secured airway in third
attempt, CL III
Hypothermia
Dicult intubation: DL failed twice in lateral
position and once in supine with head held
beyond the edge of table, sac aspirated, child
placed supine and intubated by direct
laryngoscopy, CL II with external laryngeal
maneuver
Uneventful
Dicult intubation: DL failed twice in left
lateral position, childs head held beyond the
edge of table, intubated in third attempt, CL II
Hypotension
Tachycardia
Endobronchial

Good
Good

Good

Died

Management of Children With Giant Encephalocele

Postoperative Complications

Volume 00, Number 00, 2016

Age

No

J Neurosurg Anesthesiol

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TABLE 2. Details of the Children With Giant Encephalocele

No
13

Age

Signicant Association

Anesthetic
Induction

11 mo Preterm, delayed milestones, HCP Inhalational

(halothane)

14

18 d

15

4 mo

Corpus callosal agenesis, HCP

Ectopic kidney

Inhalational
(halothane)

Intraoperative Complications

Condition at
Discharge

Dicult intubation: failed 3 attempts of DL in HCP, CSF leak, wound infection and
Good
right lateral position, ventilated with LMA,
dehiscence, VP shunt on POD2, revision
successfully intubated over bougie with DL in on POD6
fth attempt. CL III. No episode of
desaturation
Uneventful
Received blood transfusion for anemia,
Died
hematuria, apneic episodes, cardiac
arrest
Uneventful
Focal seizures
Good

18 mo HCP, delayed milestones

18

1 mo

Intravenous

19

4 mo

HCP

Inhalational
(sevourane)

20

30 d

Inhalational
(sevourane)

21

5 mo

HCP

Inhalational
(sevourane)

22

Inhalational
(sevourane)

23

18 mo HCP, Chiari malformation with

HCP, C2-3 blocked vertebrae
Delayed milestones
19 d Microcephaly, HCP

Inhalational
(sevourane)

Multiple episodes of hypoxia (SpO2: 90%) and

bradycardia during airway management

24

2 mo

HCP, cervico-dorsal syrinx

25

2 mo

HCP, anemia

26

6 mo

Microcephaly delayed milestones

Anemia

Inhalational
(sevourane)
Inhalational
(sevourane)
Inhalational
(sevourane)

27

2 mo

ACM with HCP stridor,

aqueductal stenosis

Inhalational
(sevourane)

Uneventful

Good

Dicult intubation, intubated in third attempt

by DL in lateral position. CL III

Wound infection, fever, dehiscence,

Died
wound resuturing done, CSF leak,
meningitis, vomiting, hyponatremia,
seizures, microaspiration, reintubated,
HCP, pneumonitis, sepsis, cardiac arrest
Dicult intubation: failed 2 attempts of DL in In view of cardiac arrest, child ventilated Deteriorated
right lateral position, successfully intubated
in postoperative period
with DL in third attempt. CL III
Hematuria, HCP, pneumonia,
Intraoperative cardiac arrest (bradycardia
hypothermia, hypotension, seizures
followed by asystole) during decompression of
sac, resuscitated successfully within 3 min
Hypothermia
Dicult intubation: intubated at third attempt Stridor, inltrates in chest x-ray, seizures, Good
by direct laryngoscopy in lateral position, CL
reintubation
III
Dicult intubation: intubated in second attempt Uneventful
Good
by direct laryngoscopy in lateral position, CL
III
Dicult intubation: intubated after fourth
Fever
Good
attempt, with direct laryngoscopy in supine
position. CL III
Uneventful
Fever
Good

Uneventful

Postoperative elective ventilation in view

of intraoperative complications
Fever, wound dehiscence
VP shunt on POD8

Good
Good

Uneventful

Uneventful

Good

Dicult intubation: direct laryngoscopy

Elective ventilation in view of inadequate Good
attempted in lateral position, failed 3 times,
reversal
child turned supine and successfully intubated
in fourth attempt. CL III
Elective ventilation in view of
Good
Dicult intubation: induced with sevourane
induction in right lateral position, DL failed 4 intraoperative cardiac arrest
times, mask ventilation possible. SpO2 never

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17

Ectopics with halothane, changed to isourane

J Neurosurg Anesthesiol

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16

Microcephaly
Anemia
2 mo HCP, craniosynostosis

Inhalational
(halothane)
Inhalational
(halothane)
Inhalational
(halothane)

Postoperative Complications

Mahajan et al

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TABLE 2. (continued)

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Volume 00, Number 00, 2016

ACM indicates Arnold-Chiari malformation; CHD, congenital heart disease; CSF, cerebrospinal uid; DL, direct laryngoscopy; F, female; GCS, Glasgow-Coma score; Good, same as during preoperative period;
GOS, Glasgow outcome score; HCP, hydrocephalus; LMA, laryngeal mask airway; M, male; POD, postoperative day; VP, ventriculoperitoneal.

Good
Uneventful
Uneventful
Inhalational
(sevourane)
29

28

5 mo

Craniosynostosis
Microcephaly
Anemia
16 mo Anemia

Inhalational
(sevourane)

dropped below 90%. To facilitate intubation,

550 mL of uid aspirated from sac. Patient
developed sudden bradycardia followed by
asystole. Patient made supine, intubated and
resuscitation successfully. Surgery continued
Uneventful
Operative site hematoma

Good

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Management of Children With Giant Encephalocele

TABLE 3. Intraoperative Adverse Events

Events

N/%

Hemodynamic events
Cardiac arrest
Hypotension
Bradycardia
Tachycardia
Ectopics
Total
Respiratory events
Hypoxia
Hypercarbia
Endobronchial intubation
Total
Hypothermia

2
2
2
2
1
9 (31.0%)
2
2
1
5 (17.2%)
4 (13.8%)

surgery was 222.6 and 160.2 minutes, respectively. Postoperatively, trachea was not extubated in 6 children
(20.7%) owing to various intraoperative complications; 2
suered cardiac arrest, 2 had hypothermia, 1 child had
inadequate reversal, and another suered multiple episodes of bradycardia and hypoxia (Table 2). The average
duration of postoperative ventilation was 39.4 hours.
Postoperatively, 5 children (17.2%) required tracheal reintubation; 2 for respiratory distress and 3 for cardiac
arrest. Six children underwent insertion of VP shunt for
HCP. The intensive care unit stay was 2.7 days and
hospital stay was 11.5 days. The condition at discharge
remained similar as in preoperative period (good) in 24
children (82.7%), deteriorated in 2 (6.9%), and 3 (10.3%)
children died.

DISCUSSION
Most cases of encephalocele present with a healthy
skin and hence, surgery is carried out electively based on
the general condition of child. However, in case of giant
swellings, excision and repair should be planned as early
as possible to prevent its rupture, minimize further herniation, and facilitate the nursing care. HCP was present
in 65.5% of cases, in this series. Several factors are implicated for the occurrence of HCP namely aqueduct
stenosis, fourth ventricle outlet obstruction, and obliteration of the posterior fossa subarachnoid spaces or
their obstruction at the tentorial notch.6 A VP shunt may
be inserted before denitive surgery to prevent postoperative CSF leak. Often the children with giant enceTABLE 4. Postoperative Complications
Complications

n (%)

Hydrocephalus
Wound infection
Sepsis
Fever
Respiratory infection
Seizures
Anemia
Hemodynamic instability
Meningitis
Cardiac arrest

7
4
2
7
3
5
2
1
2
3

(24.1)
(13.8)
(6.9)
(24.1)
(10.3)
(17.2)
(6.9)
(3.4)
(6.9)
(10.3)

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Mahajan et al

J Neurosurg Anesthesiol

phalocele would have associated microcephaly due to the

herniation of the cranial contents into the sac. Other developmental abnormalities may be present such as corpus
callosal agenesis, Arnold-Chiari II malformation, spina
bida, cleft lip or palate, renal agenesis, pulmonary hypoplasia, patent ductus arteriosus, and septal defects.5
Microcephaly and delayed developmental milestones were
the most common abnormalities encountered, in this
series, which usually aect the long-term outcome in such
children. Available antenatal history was insignicant
except in 2 children who were born preterm. The important issue in preterm children is immature physiological development of all systems, making them
susceptible to cardiorespiratory problems, apneic episodes, hypoglycemia, hypothermia, inadequate metabolism of anesthetic drugs, and rapid blood loss due to small
blood volume. The perioperative course was uneventful in
these 2 children and condition at discharge was same as
during the preoperative period (good).
The main concern during induction of anesthesia in
children with giant encephalocele is to secure the airway.
The oxygen reserve is low and the children may desaturate
rapidly during induction; hence, prompt intervention is
essential. The pediatric airway is anatomically dierent and
more challenging than the adult one. In addition, children
with giant occipital encephalocele pose diculty during
intubation mainly because of suboptimal positioning due to
a large sac behind their neck rather than any structural
abnormality in the airway. Moreover, a large sac present at
the occiput may make the movement of neck grossly restricted. Hence, a dicult airway cart should be made
available. None of the children, in this series, had any
congenital anomaly involving upper airway.
In such cases, inhalational agent is preferred for
induction as it can be rapidly washed o during dicult
circumstances. For children with HCP, a concomitant
hyperventilation (along with inhalational induction) may
prevent further increase in intracranial pressure (ICP). In
such scenario, muscle relaxant is given at the discretion of
the attending anesthesiologist, once adequate mask ventilation is ascertained. The decision is based on absence of
apparent dysmorphic features and the ability to perform
mask ventilation. Although use of muscle relaxants is
controversial, Davys et al7 observed improvement of intubating conditions and decreased the frequency of adverse respiratory events (laryngospasm, bronchospasm,
and SpO2 < 90%) on addition of 0.3 mg/kg of rocuronium to 8% sevourane. It indicates, an omission of
muscle relaxant may lead to paradoxical increase in respiratory complications.
In most of these children in the series, a quick and
gentle check laryngoscopy was performed to assess the
airway after inhalational induction. Airway was secured
with a tracheal tube during the laryngoscopy, or muscle
relaxant was administered to do it depending on preference of the attending anesthesiologist. Succinylcholine
may be the best agent for use in such cases; but, in this
series, it was not preferred possibly due the perceived
complications of raised ICP, associated with it.

Fiberoptic bronchoscopy is considered as gold

standard for securing the airway but its use in pediatric
population requires a lot of experience and skill. Presence
of even small amount of blood and secretions may
completely obscure the view of airway. Videolaryngoscopes may overcome this particular drawback.
Optical stylets, pediatric beroptic bronchoscope, and
videolaryngoscopes were unavailable, at our Institute,
during the study period. Hence, the classic laryngeal mask
airway (LMA) and bougie were relied upon as a backup
support for securing the airways. LMA is an indispensible
gadget for securing airway if mask ventilation and tracheal intubation fail; proseal LMA provides a better
oropharyngeal seal as compared with classic LMA.
Necessary arrangement for performing surgical tracheostomy was made as a routine, lest it was required.
Thus, not every case of giant encephalocele had
dicult airway, and on other hand, a seemingly easy
airway may pose unanticipated diculty. On the basis of
our experience, we recommend performing a gentle check
laryngoscopy after anesthetic induction with sevourane.
If glottis is visualized, muscle relaxant can be administered before attempting tracheal intubation. Nonetheless, it is important to attain a good experience with all
available pediatric airway gadgets under normal circumstances for the optimal use in dicult airway scenario.
The children should be positioned carefully to prevent compression of sac, which may lead to raised ICP or
even, rupture. Recommended positions for intubation in
children with giant occipital encephalocele vary from lateral to supine.812 Although child is placed supine, the
head may be placed beyond the edge of table, with assistants supporting it. Alternatively, the sac can be supported
with foam cushion devices or silicone support.12,13 In this
series, majority of children were intubated by direct laryngoscopy in lateral position. Hence, we recommend laryngoscopy in right lateral position which may be
comfortable for the right-handed anesthesiologists.14 Singh
et al15 oered a similar suggestion to practice tracheal intubation in lateral position in patients with normal airway
so as to manage a complicated scenario. Generally, children in whom tracheal intubation is failed in lateral position, were placed supine with head beyond the edge of
table supported by an assistant. The drawback is requirement of an additional assistant which may not always be
possible. A big sac at occipital region may limit the neck
movement. CSF aspiration is one of the maneuvers to
decompress the sac, which allows more space for extension
of neck and actually improves the glottis view. In one of
the children in this series, neck extension was completely
absent owing to presence of a giant occipital encephalocele
and intubation failed several times. Slow drainage of CSF
was performed for shrinkage of the encephalocele sac;
later, the child was placed supine and the trachea was
successfully intubated. This procedure has been suggested
by other authors, as well.1618 Presence of Arnold-Chiari
malformation may cause brainstem compression during
excessive exion or extension of head which should be
taken into consideration during laryngoscopy.19

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J Neurosurg Anesthesiol

Volume 00, Number 00, 2016

Management of Children With Giant Encephalocele

The repair of giant occipital encephalocele is carried

out in prone position. The child should be carefully
positioned to avoid accidental dislodgement of cannulae,
catheter, and endotracheal tube. Maintenance of body
temperature may be dicult in these children due to lack
of central autonomic control. Hypothermia may also
occur after increased area of exposure to environmental
temperature owing to the large size of the sac. Despite
adequate precaution to maintain normal body temperature, 4 children in this study developed hypothermia
during intraoperative period. In 2 children, the temperature was reduced to <341C, at the end of surgery; hence,
mechanical ventilation was continued until the body
temperature was normalized.
CSF drainage from the giant encephalocele sac may
be associated with a number of complications. At times,
slow aspiration of the sac is practiced, just before skin
incision, by the neurosurgeons. This maneuver helps
providing a slack sac for better surgical dissection. A
higher rate of CSF drainage may lead to intraoperative
cardiac arrest; observed in one of these children. Moreover, sudden decompression of ventricles may cause
traction on brainstem nuclei leading to cardiac arrest.20,21
Two children, in this series, suered bradycardia followed
by asystole during CSF aspiration from the sac. Further
aspiration of CSF was stopped, and both the children
could be resuscitated. Hemodynamic instability and
electrolyte imbalance may occur after loss of large volume
of CSF. Dissection of large encephalocele sac, cutting
through rich vascular suboccipital bone, and damage to
contents of sac-like blood vessels, dural sinuses, or torcula, may lead to signicant blood loss and that needs
urgent replacement. Normally, the gliotic brain is excised
and defect is repaired. The neural tissue present in the sac
is attempted to be kept inside cranial cavity; it may lead to
raised ICP.22,23 During postoperative period, the raised
ICP in a newborn is unpredictable and may manifest with
episodes of sudden respiratory arrest.23
In this series, 6 (20.7%) children required elective
postoperative ventilation because of various reasons; hypothermia and cardiac arrest being the major ones. Five
children (17.2%) required tracheal reintubation (Table 2).
Two of them who required early tracheal reintubation
after respiratory distress were discharged from the hospital with good general condition. One child developed
HCP and became drowsy, whereas another child developed stridor, and thus, both required tracheal intubation,
subsequently. Another 3 children, who required intubation as a part of cardiopulmonary resuscitation had
unfavorable outcome; 2 of them developed sepsis and
multiorgan failure before experiencing cardiac arrest.
Both of these children developed wound infection, meningitis, HCP, sepsis followed by nonresuscitable cardiac
arrest. Sepsis occurred in them despite prophylactic antibiotic administration at induction of anesthesia and
strict aseptic precaution during the perioperative period.
Hence, the persistent CSF leak leading to postoperative
meningitis may not be ruled out. Meticulous closure of
dura mater during surgery is imperative to avoid such

complications. Another child who died of sudden cardiac

arrest had postoperative apneic episodes. Structural derangements of the cardiorespiratory center seem to be a
possible association, in this child.
The prognosis of children after repair of encephalocele depends on the location of the defect, amount of
brain tissue herniated into the sac, presence of HCP, and
other anomalies. Generally, children with posterior encephaloceles would have a poor prognosis as compared
with anterior types.24 Giant encephalocele invariably
contains brain tissue and hence, is associated with poor
outcome. In this series, the condition at discharge was
good, in 82.7% of children. A similar study observed
good mental function on long-term follow-up, in 66% of
children with giant encephalocele.23
It is a retrospective study and hence, the study is
being limited by the assessment of conditions of the
children only at the time of discharge from the hospital.
These children might have unpredictable mental development and hence, a long-term follow-up could have
provided insight to the overall outcome. Although contents of the sac are considered as the major determining
factor for long-term outcome in these children, precautions should be taken to avoid any further intraoperative insult.
To conclude, the management of children with giant
encephalocele requires updated knowledge on possible
diculties encountered during the perioperative period.
They need specialized anesthetic care for dealing with
dicult airways, associated congenital anomalies, unusual positioning, electrolyte abnormalities, hypothermia,
and cardiorespiratory disturbances. Before the airway is
secured, we suggest the practice of check laryngoscopy in
lateral position after inhalational induction. Muscle relaxants should be administered only after visualization of
the glottis. We also emphasize on the practice of tracheal
intubation in lateral position to overcome airway-related
crises.

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