Original Article

Anaesthetic Challenges and Management of

Myelomeningocele Repair
Chand MB,* Agrawal J.**, Dr. P. Bista***
*Associate Professor, **Chief Consultant Anaesthesiologist, Department of Anaesthesia & ICU,
***Associate Professor, Department of Neurosurgery

ABSTRACT
Introduction: Failure of neural tube closure early in intrauterine development results in
spectrum of abnormalities ranging from spinabifidaocculta, a relativelybenigncondition to
meningomyeocele, an abnormality involving vertebral bodies, spinal cord and brainstem. These
babies also have associated congenital anomaliese.g.intestinalmalrotation, renal anomalies,
cardiac malformations and tracheoesophageal fistula.Anaesthesiafor meningomyelocele always
poses a challenge to anaesthesiologist as one has to deal with neonates and infants who
already have so many anatomical and physiological differences compare to the adults or normal
children.Meticulous anaesthetic management is crucial for early repair and to prevent sequele of
meningomyelocele.
Methods: To identify the anaesthetic challenges, perioperative and postoperative complications
during myelomeningocelerepair, a retrospective study has been carried out on all the children
operated during April 2006 to March 2011 at National Academy of Medical Sciences, Bir hospital,
Kathmandu. All together 37 babies were operated on during the study period of which 59.5%
were male and 40.5% were female.
Results: Overall complications were seen in 64.9% and the most common complications were
respiratory like laryngospasm, bronchospasm, hypoxemia and endobronchial intubation. Most of
the complications were manageable through early and precise diagnosis.
Conclusion: Children with Myelomeningocele are prone to have peri-operative conclusions
which can be managed by meticulous anaesthetic managenement.
Key Words: Meningomyelocele, MMC, hydrocephalus, general anaesthesia, complication.

INTRODUCTION

Myelomeningocele (MMC), one of the most common
congenital malformation of the central nervous
system, has been described as “the most complex
treatable congenital anomaly compatible with life”1.
Failure of neural tube formation and closure may
occur anywhere along the neural axis. MMC occurs
in 0.4-1 per 1000 live birth and incidence varies with
environmental and genetic factors1, 2, 3. In addition to
the neural tube defect, several associated anomalies
like hydrocephalus concerns the anaesthesiologist.
Most infants with hydrocephalus are usually associated
with Arnold- Chiari type II malformation (downward
displacement of the cerebellum into the brain stem
and cervical canal with medullary kinking). This may
Correspondence :
Dr. M. B. Chand, NAMS, Bir Hospital
Email Address: drmbchand@gmail.com
cause cervical cord compression, during extension
for intubation leading to brain stem compression.
Airway management may be difficult in patients with
significant hydrocephalus3. Most patients show a
diminished response to hypoxia, and may be more
susceptible to post-operative apnoeic episodes4.
The effect of positioning in neonates for induction of
general anaesthesia must also be considered. No direct
pressure should be applied to the exposed neural
placode.3 In addition, children with MMC have a higher
incidence of intestinal malformations, renal anomalies,
cardiac malformations and tracheoesophageal fistula.5
Surgical management of MMC is challenging, not
only for administration of anaesthesia but also for
providing perioperative care owing to paediatric age
group, comorbid conditions, and associated systemic
abnormalities.6

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 Anaesthetic Challenges and Management of Myelomeningocele Repair

METHODS Lactate was the fluid of choice in most patients, but

2/3 dextrose + 1/3 sodium chloride were also used
All the available medical records of children who especially in neonates. At the end of surgery, residual
underwent excision and repair of MMC over a period neuromuscular block was reversed with calculated
of five years (April 2006 to march 2011) were analysed. dose of Neostigmine and atropine and the trachea was
For each child, data were collected by detailed review extubated when the patient fully awake.
of records related to pre-anaesthetic evaluation, intra-
operative course and postoperative complications. RESULTS
Pre-operative evaluation included age, sex, weight,
height, Hb%, electrolytes, chest x-ray, site of lesion, Out of a total 37 children operated, majority 89.1%
CSF leak from the sac, neurological presentation, (n=33) were above 60 days of age. Only 10.9% (n=4)
associated systemic abnormalities, venous and airway were less than 60 days old. Remaining patients were
assessment. All the patient had their MRI and CT older than one year. (Table 1) More male child (n=22)
Scan of relevant part to see the extent of disease. were operated than female child (n=15) with male:
Intraoperative data included anaesthetic technique, female ratio of 3:2.
intraoperative monitoring (heart rate, saturation,
Table 1: Age And Sex Of The Children
ventilation, BP, Urine output, temperature), fluids
Total
infused, blood loss and transfusion. Intraoperative Age Male female Percentage
number
parameter such as heart rate and blood pressure
were recorded. Below and above 20% alteration from 0-60 days 3 1 4 10.81
baseline values of heart rate and BP were regarded >60 days-
9 8 17 45.95
as abnormal. Respiratory complications (hypoxemia, 1 year
hypercarbia, bronchospasm, laryngospasm), >1 year 10 6 16 43.24
endobronchial intubation, accidental extubations were 22 15
Total 37
noted. Miscellaneous complications like hypothermia, (59.45%) (40.55%)
dislodgement of intravenous catheter were reviewed.
The most common location of neural tube defect was
Post-operative complications like nausea, vomiting,
found to be lumbar spine 67. 6% (n=25) followed by
mechanical ventilation, surgical complications were
lumbo sacral 27.4% (n=10) cervical and dorsal 1 each
reviewed.
(Table 2). The mean weight of babies undergoing MMC
ANAESTHETIC TECHNIQUE repair was 7.25kg (range 2.5kg-12kg). Four children
presented with leaking meningocele and had to
A standard anaesthetic technique was followed for all undergo emergency repair.
children. Intravenous line was secured with or without
sedation. Those who needed sedation were given Table 2: Site of Myelomeningocele
midazolam 0.25-5mg/kg body weight. Intravenous Site No. (%)
induction was done with thiopental sodium (3-5 Lumber MMC 25 (67.6)
mg/kg). Vecuronium was used to facilitate tracheal Lumbosacral 10 (27.4)
intubation. The intubation was performed mostly in Cervical 1 (2.5)
supine position (32 cases) putting placode within the Dorsal 1 (2.5)
padding made in the shape of a doughnut. In patients Total 37
associated with large hydrocephalus pillow behind Among associated abnormalities ( table 3)
shoulders were kept during intubation. Intubations Hydrocephalus was found to be most common in
in lateral position (5 cases) were done in patients 25 children (67.56%) who underwent ventriculo-
with large MMC. Repair of MMC was done in prone peritoneal shunt followed by MMC repair in the
position whereas in patients with large hydrocephalus same setting. Chiari malformation II was found in 2
VP Shunt was inserted in supine position followed (5.40%) patients, all in association with hydrocephalus.
by MMC repair in prone position. Anaesthesia was Scoliosis seen in 12 cases (32.43%) diagnosed clinically
maintained with oxygen and Isoflurane with controlled by features of uneven shoulders, prominent ribs and
ventilation. Meperidine was used for analgesia. Ringer typical look of the hip where one hip was higher

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 Anaesthetic Challenges and Management of Myelomeningocele Repair

than the other. Electrolyte imbalance was present 3(8.1%). Post operative complications included wound
in 10(27.02%) as hypokalaemia, hyperkalaemia, infection 3(8.1%), hydrocephalus 4(10.81%), wound
hyponatraemia. Two of the children were also anaemic dehiscence 2 (5.4%), postoperative CSF leaking from
and malnutrition was present concurrently. repair 3(8.1%) were recorded. These postoperative
complications were recorded as those cases had to
Table 3. Associated Abnormalities undergo various revision operative procedures like,
No. (%) resuturing for wound dehiscence, debridement of
Central nervous system abnormalities wound infection, VP shunting for HCP, exploration of
• Hydrocephalus 25(67.56) leaking CSF (table 5).
• Chiari malformation 2(5.40)
Orthopaedic abnormalities Table 5. Post-operative complications
• Scoliosi 12(32.43) Complication No (%)
• Talipes Equinovarus 9(24) Hydrocephalus 4(10.81)
• Flat/ high arched foot and tropic 11(29.72) CSF leak 3(8.10)
ulcer Wound infection 2(5.40)
Co-existing medical conditions Wound dehiscence 2(5.40)
• Upper respiratory tract infections 11(29.72)
• Electrolyte imbalance 10(27.02) At the end of surgery 32 cases were extubated on the
operating table. 5 cases had delayed recovery and one
Table 4. Intra-Operative Complications of them needed postoperative ventilation.
Intra-operative No(%)
The ICU stay was 2 to 7 days whereas the hospital stay
Cardiovascular
was 4-24 days.
• Bradycardia 4 10.8
• Tachycardia 3 8.1 Among those 11 cases with post-operative
• Hypotension 3 8.1 complications 4 cases died due to shunt infection,
Respiratory wound infection, CSF leaking, aspiration and chest
• Bronchospasm 3 8.1 infection respectively.
• Hypoxemia 3 8.1
• Endobronchial 2 5.4 DISCUSSION
intubation
• Accidental 3 8.1 MMC is the most common malformation of the CNS
extubation 3 8.1 worldwide, with an incidence evaluated at 0.4-1 per
• Laryngospasm 1000 live births, and is one of the leading causes of
Total 24 infantile paralysis along with lifelong disabilities
including paraplegia, hydrocephalus, Chiari II
The duration of anesthesia ranged from 2hrs – 6hrs, an malformation, incontinence, sexual dysfunction,
average of 4 hrs. Comparatively less time was taken for skeletal deformities and mental impairment 7,8. In
MMC repair alone then for VP shunt followed by MMC developed countries incidence is decreasing thanks
repair. In few cases especially with hydrocephalus to antenatal screening procedures, as well as dietary
there were problems during intubation. Among the supplementation with folic acid to the women at risk
24 intraoperative complications respiratory was most prior to and during pregnancy 7,8 but there is no study
common as seen in 14 than cardiac complication (table about the incidence in our country .Moreover the
4). Among respiratory complications Bronchospasm, male: female ratio in the series is 3:2.The vast majority
hypoxemia, laryngospasm, accidental extubation were of patients with spina bifida exhibit some degree of
more common. Endobronchial intubation was found in 2 Chiari type II malformation (CM II). Approximately one
cases. Cardiac complications in the form of bradycardia, third of babies with MMC develop symptomatic Chiari
tachycardia and hypotension created problems during II malformation 9, 10 consisting of a small posterior fossa
the operative procedure. Intraoperative blood loss was and downward displacement of the cerebellar tonsils
20 ml- 130 ml, an average of 75 ml. Other uncommon below the foramen magnum with elongation and
complications were hypothermia 3(8.1%), facial compression of the brain stem and obliteration of the
edema 3(8.1%), dislodgement of intravenous catheter cisterna magna 2,3 .

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 Anaesthetic Challenges and Management of Myelomeningocele Repair
Despite aggressive surgical and medical management
15-30% of neonates with MMC die within the first
5 year of life, majority of deaths are attributable
to severe CM II 5. Developmental abnormalities of
brain such as corpus callosum agenesis, microgyria,
porencephalic cyst, and arachnoid cyst are commonly
associated apart from Chiari malformations 11.
The timing of surgery, usually in the first 48 hours after
birth, is important because an increased infection
rate is associated with delayed surgery 12. The MMC
is usually located in the lumbosacral area. In rare
instances, it may be located higher; it is not clear
whether the mechanism of the high spinal MMC is the
same as that of the low MMC. Cervical MMC seems
to have a better long-term neurological prognosis than
low spinal MMC 13, 14. In our study, 67% cases were
Lumbar MMC followed by lumbosacral 27%.
As mentioned earlier MMC is associated with multiple
abnormalities. One of the commonest finding is
hydrocephalus (85-90%). As largely debated in
the literature, many factors may contribute to its
occurrence, namely aqueduct stenosis, fourth ventricle
outlet obstruction, and obliteration of the posterior
fossa subarachnoid spaces or their obstruction at the
tentorial notch9,10. In our study as well the association
of MMC with Hydrocephalus was most common 67%
(25 cases). Hydrocephalus affects neurocognitive
outcome and result in morbidity and mortality caused
by shunt malfunction and infection11,15. Significant
Hydrocephalus may complicate the management
of airway, reduced response to hypoxia and hence
susceptible to post-operative apnoeic episodes16.
Similarly, Chiari II malformation is symptomatic in
20% of children especially in those aged 3 months
or less 9. The symptoms are manifested as brainstem
dysfunction and may present with inspiratory stridor
from vocal cord paralysis, apnoea from central
hypoventilation, swallowing dysfunction, bradycardia,
nystagmus, torticollis, hypotonia, upper extremity
weakness, and spasticity. Inspiratory stridor is believed
to be caused by stretching of vagus nerve due to
coning of hindbrain structures after development of
hydrocephalus 17. But this association of MMC with
Chiari malformation was just 5% (2 cases) in our study
partly owing to low number of neonatal cases and the
difficulty in diagnosis. Other common associations
were Scoliosis 32% (12cases),Flat/ high arched foot,
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upper respiratory tract infections (29%) and electrolyte
imbalance(27%) .
Preoperative evaluation especially of cardiac,
gastrointestinal, genitourinary system is very important
because embryologically they are formed concurrently
with malformed neurologic system 2. Literature
review suggests that 37% of MMC are associated with
congenital heart lesions however present study did
not show any of those.11. The short trachea is found
in 36% chest radiograph of MMC patients, few of
them get endobronchial intubation.17In our study
we could not diagnose short trachea preoperatively
but endobronchial intubation occurred in 2 patients
probably owing to short trachea..
Although local anaesthesia with mild sedation has
been utilized in the past, at present the operation
is performed only under general anaesthesia with
orotracheal intubation 19. Operative mortality is
practically absent while morbidity may be significant
20,21
. Thus, under proper technique and qualified
personnel the operative procedure is relatively safe.
Paediatric endotracheal tubes should be uncuffed until
at least six years of age. A correctly sized tube allows
adequate ventilation with a small audible leak of air
present when positive pressure is applied at 20 cmH2O
10
. The main problems encountered were particularly
difficult intubation owing to huge hydrocephalus, huge
MMC in cervical region and dorsal region, anatomical
and physiological variations in the paediatric airway.
The perioperative complications pose challenges
to the anaesthesiologist and more common are
respiratory complications followed by cardiovascular
complications. Respiratory complications are
hypoventilation, sleep apnoea, bronchospasm,
laryngospasm, prolonged breath holding as a result of
structural derangement of post medullary respiratory
control centre or in its afferent and efferent pathways.
Cardiovascular complications included bradycardia,
hypotension and tachycardia. Brainstem compression
and coning causes most of the cardiac complications
including cardiac arrest when Chiari malformation is
associated with MMC. 15 But in our study, bradycardia
was more common during laryngoscopy which might
be due to brainstem compression and during surgery
due to stimulation of lumbosacral parasympathetic
segment . Hypotension was found second most
 Anaesthetic Challenges and Management of Myelomeningocele Repair
common CVS complication and sometimes they failed
to respond to fluid and blood transfusion. The cause of
unexplained hypotension may be attributed to sudden
loss of CSF from sac leading to increased craniospinal
pressure gradient and hence, brain herniation.15 Since
hypotension occur in 50% of the cases 22, it may be
reasonable to provide intravenous fluid replacement
of the calculated deficit in an effort to decrease the
incidence of hypotension.
Other intraoperative complications included facial
oedema (8%) due to the prone positioning of the
patient during the procedure, dislodgement of
iv catheter, hypothermia. Hypothermia occurred
commonly owing to the age group of the patient
and the use of general anaesthetic agent as they
depress the thermoregulatory response in children.
Heat is lost from the core to the cooler peripheral
tissues, particularly in non-shivering thermogenetic
neonates23. This problem is compounded by cold
operating rooms, wound exposure or dry anaesthetic
gases. Prolonged hypothermia can lead to a profound
acidosis and impaired tissue perfusion, which have
been associated with delayed awakening from
anaesthesia, cardiac irritability, respiratory depression,
increased pulmonary vascular resistance and altered
drug responses. Hypothermia can be prevented by
several simple measures such as raising operating room
temperature to 28-30°C, wrapping the extremities
and head with cotton blankets, administering warm
infusion solutions and blood. Inhaled anaesthetic
gases should also be heated and humidified.
The process of recovery is crucial as well. The criteria
for extubation include intact cough and gag reflex, a
negative inspiratory force of 15-30 cmH2O, a force
vital capacity breath in excess of 10 ml/kg, an air leak
around the tube with inflation pressures less than
25 cmH2O as well as the maintenance of acceptable
oxygenation and ventilation on minimal ventilatory
support. Adequate reversal of neuromuscular blockade
is demonstrated by either a sustained arm lift or leg lift.
Extubation should be performed only when the child is
awake and breathing well.26 In our study 32 cases were
extubated on the operating table but 5 patients had
delayed recovery, one of them needed post operative
ventilation. The possible causes of delayed recovery
are hypothermia, inadequate reversal, prolonged
effect of the anaesthetic agent, electrolyte imbalances
and malnutrition.
Post operative complications occurred in 11 cases
(29.72%) mainly hydrocephalus, CSF leak, wound
infection, wound dehiscence. Four cases (10.81%) died
due to shunt infection, CSF leak, aspiration and chest
infection.
Limitations of the study: It is a retrospective study
carried out in small number of children without any
neonatal or paediatric backup, therefore, the study
may not reflect the true morbidity and mortality.
CONCLUSION
The children with MMC have several associated
CNS abnormalities which are very prone to have
perioperative complications. Most of the complications
are manageable through early and precise diagnosis,
meticulous preoperative preparations, vigilant
intraoperative monitoring and preparation for
anticipated complication.
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