Prepared by:-

Gammachis Akkuma ( lecturer)

March 2023

03/06/2023 1
 Outline
• Objectives
• Neurophysiology and development
• Hydrocephalous?
• Meningocele
• Craniosynstosis
 Learning Objectives:
• At the end of this lesson student will be able
– Identifies clinical features of Hydrocephalus and ICP
– Identifies anesthesia consideration for VP or atrial
shunt placement/hydrocephalus
– Define mengiolcele, craniosynostosis and describe
the underlying pathogenesis and pathophysiology
– Describe the pre-, intra- and postoperative anesthetic
considerations for infants and toddlers requiring
craniofacial surgery and Meningocele
 Neurophysiology and development

• The skull is a closed box with brain tissue,

blood and CSF as its contents.
• An increase in volume of one of these
components with increase in ICP will result a
compensatory reduction of other components
(Monro–Kellie doctrine).
• Anatomical differences between children and
adults affect CNS physiology, especially ICP.
CBF affects the CBV and the intracranial volume,
thereby affecting the ICP, is higher in children
compared to adults (100 vs 50 ml/100g/min).
• CBF is coupled tightly to metabolic demand, increase
proportionally after birth.
• CBF peaked between 2-4 years and settled at 7-8years
• In children the CMRO2 is higher at than the adults (5.2
vs 3.5 ml/100 g/min) and hence, less tolerant to
hypoxia.
• The autoregulation range of blood pressure in normal
newborn is narrow between 20 and 60 mmHg.
• The neonate at risk for cerebral ischemia and IVH for
extreme BP
Brain tissue, the largest component of the cranial
contents, may be pathologically enlarged lead to
a gradual increase in ICP.
CSF is continuously produced by the choroid
plexus, and after circulating through the
ventricles is absorbed at the arachnoid villi?.
• In children the rate of production of CSF is 0.2-
0.4 ml/min and any given time around 70 ml is
present in the head.
– By Choroid Plexus of ventricles, 0.25% of total
volume replaced every minute
– Extra CSF formation [25%] by glucose oxidation
 CSF
• Mechanical cushion to brain
• Source of nutrition to brain
• Excretion of metabolic waste products
• Intracerebral transport medium
• Control of chemical environment
• Autoregulation of intracranial pressure
• Circulation= circulates from lateral ventricle Foramen
of Monro 3rd ventricle  Aqueduct of Sylvius  4th
ventricle & then to Sub arachnoid space through
foramen of Lushka & Magendie.
• Reabsorption
– By arachnoid villi & arachnoid granulations at dura
walls superior sagittal sinus & venous lacunae.
– 85-90% of CSF reabsorbed in cranium.
 What is hydrocephalous?
• Hydrocephalus is the medical term for a condition
that is commonly called “water? on the brain.” 
– Abnormal accumulation of CSF due to disturbance in
formation, flow or absorption; which may lead to raised
ICP.
– This build-up of CSF causes the brain to swell, and for
pressure to increase inside the skull, resulting in nerve
damage.
 Epidimology
• The incidence of hydrocephalus is between
0.5-0.8 per 100 live births in the United States.
(Sivagnanam M, Jha Nk, February 2012].)
• The burden of infant hydrocephalus in East
Africa is significant, with more than 6000 new
cases estimated per year (Warf BC;. 2010).
 Ethology and classification

• Congenital :- • Acquired :-
– Myelomeningocele – Infection
– Stenosis of the cerebral – Intraventricular
aqueduct hemorrhage
– Arnold-Chiari – Tumour
malformation – Trauma
– Arachnoid cysts – Nutritional deficiencies
– Vascular malformations
– Idiopathic
 Clinical presentation
• It depend on underlying pathology, site of obstruction,
speed of onset, its duration and the patient’s age.
• Acute onset cases usually present with symptoms of
raised ICP and the ventricles may not be markedly
enlarged
• In chronic forms of hydrocephalus, the ventricles are
usually enlarged.
• symptom in infant include poor feeding, irritability,
reduced activity, and vomiting
 Examination in infants may reveal the
following findings
• Head enlargement
• Dysjunction of sutures
• Dilated scalp veins
• Tense fontanelle
• Setting-sun sign?
• Increased limb tone (spasticity preferentially
affects the lower limbs)
 Treatment modality
– Relieve hydrocephalus
– Treat complications.
• Surgical correction involves draining fluid from
the ventricular system to one of three places:
peritoneum, atrium, or the pleural cavity.
• As the child grows, the shunts often require
replacement or revision VP ,most common
pediatric neurosurgical procedures
• VP shunt is used most commonly. The lateral
ventricle is the usual proximal location.
• A ventriculoatrial (VA) shunt also is called a
"vascular shunt.“
– It shunts the cerebral ventricles through the
jugular vein and SVC into the right cardiac atrium.
– It is used when the patient has abdominal
abnormalities (eg, peritonitis, morbid obesity, or
after extensive abdominal surgery).
 How a shunt works
All shunts perform two functions.
• They allow CSF to flow in only one direction, to
where it is meant to drain.
• They all have valves, which regulate the amount
of pressure inside the skull.
– When the pressure inside the skull becomes too great
the valve opens, lowering the pressure by allowing
excess CSF to drain out.
Shunt Surgery
 PREOPERATIVE ASSESSMENT

• History-
A. History common to all surgery  GA
– Birth history (premature, birth trauma, forcep delivery)
– Immunization history
– H/O allergy
– H/O problem with previous anesthesia / surgery
– Fluid status/Last oral intake specially in emergency.
– Underlying cause of hydrocephalus
– Associated congenital anomalies
B. History specific to hydrocephalus
– Drug history- anticonvulsant, diuretics acetazolamide
– Associated cong. anomalies (Cardiac, Resp.)
– H/o fever (meningitis)
– H/o recurrent resp. infection due to sensorium &
aspiration.
– H/o nausea, vomiting, headache features ICP
– H/o Convulsion
• Examination (general): -
– Pt may have poor GCS.
– Depleted volume status i.e. dehydration &
electrolyte imbalance due to prolonged vomiting and
poor oral feeding.
– Search for the presence of any other congenital
• Systemic
– Assessment of Difficult Airway(big head, edentulous)
– CVS for cong., Cardiac anomaly,
– Resp. system - anomalies like Brovhopulm. dysplasia,
– kyphoscoliosis, recurrent RTI due to aspiration.
– Assessment of neurological status
• Level of consciousness/irritability, lethargy,
• altered sensorium and cranial nerve palsies.
• Features of  ICP (papilledema), Cushing’s triad
• Features of Brain Stem involvement.
• failure to feed, bulging fontanel and cranial enlargement
• CBC- leukocytosis & ESR in presence of infection
• Coagulation profile
• serum electrolytes :- dehydration and electrolyte
imbalances.
• renal and hepatic function test
• CXR, Abdominal U/S
• Special investigations
– X-ray skull (silver bitten appearance)
– CT scan/MRI brain for etiology
– ECG/ECHO-to evaluate/rule out congenital heart disease
 Anesthesia management
• Goals of Anesthesia
– Maintenance of CPP
– Control of hemodynamics
– Smooth temperature
– Prevent gastric aspiration
• NPO for 6 hours for solids and 2 hrs for clear fluids.
• Premedication
– Usually not required (lethargic, drowsy)
– Any resultant respiratory depression may cause
hypercapnia and therefore increase ICP.
• The method of induction depends upon
– The level of anxiety of the child.
– The child's ability to cooperate and communicate
(because of age or developmental delay)
– The presence or absence of a full stomach
– Intravenous access
• IV induction preferred in high ICP
• Ensure venous cannula placement
– Thiopentone (4-7mg/kg)- most commonly used
– Propofol [2-4mg/kg] - can be used
– Except for ketamine?
– Fentanyl (1.5-2 mcg/kg) iv
• children without IV access or with difficult IV
access, inhalational induction sevoflurane
preferred
 Muscle relaxants :

• Muscle Relaxation –sux?

• NDMR is preferred
– Rocuronium 0.6-1.2 mg/kg iv
• Atracurium - as the surgical procedure is of short
duration
– Can cause histamine release cerebral vasodilatation  CBF
incre ICP
– - Metabolite laudanosine has convulsive effect, but dose used
is too small
• Vecuronium-can cause bradycardia due to its PSN action-
better avoided
 Maintenance of anesthesia...

• O2 +air+volatile agent
– -Isoflurane preferred, Sevoflurane can be used
– All volatile anesthetics cause an increase in CBF, and thence the
ICP
• N2O avoided:
– Significantly increase CBF  ICP.
– strong emetic effect- it may confuse pt evaluation postop
– chance of air embolism in diploic vein
• Spontaneous ventilation not recommended?
– Risk of air embolism during craniotomy
– Ventilatory depression can cause hypercarbia- raised ICP
 Intraoperative concerns

• ECG, NIBP, Pulse oximetry, Capnography

• Chance of difficult intubation due to large
head/intubation in lateral position
• To support head a dough nut may be placed under
the head or an assistant may support the head.
• Positioning and immobility
• Tube should be firmly secured
• Reinforced tube to be used if available
• Avoid hypercarbia, hypoxia, hyperglycemia
• Hemodynamic stability- usually dehydrated
– Maintain normovolemia
– H/d changes and arrhythmias during rapid drainage
• Specific issues:-
– hypothermia
– Surgical stimulation during tunnelling- short acting
opioid/ increasing depth of anaesthesia
– Scalp infiltration with LA + adrenaline analgesia &
less bleeding.
– Care of ET Tube - as there is chance of accidental
extubation during head manipulation, burr hole &
tunneling.
– Head is turned away from the surgeon with a pad
below operative site.
– Extreme rotation of head-impair venous return
– During shunt placement in abdomen - chance of
visceral injury
– On cannulating ventricle BP may drop abruptly as
Brain Step pressure is suddenly released Patchy
hmrge
– Prophylactic antiemetic 30-60min before extubation?
 REVERSAL & EXTUBATION

• Neuromuscular blockade is reversed.

• Extubation done once the pt is awake
– avoid hypercarbia
– minimize risk of aspiration
• Mask oxygenation following extubation & suction
• Extubate only if child is awake, return of protective
reflexes and normothermic
• Patients that can be extubated will need supplemental
oxygen and apnea monitoring for 24-hours
 POST OPERATIVE MANAGEMENT
• Post-operative care and Monitoring
– Vital parameters to be monitored (pulse, NIBP, Temp.,
Respiration), urine output.
– Positioning -Supine with head up 15 degree.
– Analgesic -PCM, opioids better avoided
– Maintenance of proper hydration- by 4-2-1
– Neurological status : GCS
– Observe for signs of Increased ICP(indicate obstruction of shunt).
• Assess pupil size
• Abdominal distention
• Respiratory distress or episodes of apnea
 POSTOP COMPLICATIONS
 Immediate postoperative :-
• Respiratory distress
– Pneumothorax
• Massive accumulation of CSF in abdomen
– Increased intra abdominal pressure
– Regurgitation & aspiration
• Deterioration of LOC + neuro deficit due to
– Intraventricular hemorrhage
– Over drainage of CSF-SDH (opposite)
• Seizure
 Postop Complications.....
• Delayed complication:-
– Shunt malfunction- due to obstruction,
disconnection, breakage, migration
– Infection
– Hardware erosion through skin
– Conduit for extra neural metastasis of certain
tumor
– CSF bag formation at the site where shunt is
suture into subcutaneous tunnel
Meningomyelocele
Thoracolubar 85%
Dorsal 10%
Cervical 5%
 Etiology

• Genetic
– Females are more affected
– More common in Caucasians and blacks
– Parenteral consanguinity increases incidence
• Environmental- folic acid deficiency
• Teratogenic drugs- valpraote, carbamazapine
• Higher prevalence in lower socio economic
groups
 Pathophysiology
• Meningocele (20%) - sac containing meninges bulges
through the defect.
– Overlying skin intact.
– Neurological manifestations usually absent
• Meningomyelocele (80%) - contains neural elements
along with meninges.
– Majority of defects occur in lumbosacral area
– Neurological deficits distal to defect are most severe.
• Spina bifida occulta - absence of bony spinous
process. No clinical significance
 Pathophysiology

• Depending on severity, manifests as neurologic,

motor and sensory deficits
• Associated congenital anomalies may be
present
• Brainstem dysfunction
• Hydrocephalus and ICP
 Associated anomalies
• Club feet- commonest
• Arnold Chiari malformation (80%-90%)
• Hydrocephalus (80%)
• Neurogenic bladder(90%)
• Musculo skeletal defects
• Urogenital anomalies
• Facial clefts
• Umbilical hernia
• Congenital heart diseases - rare
 Clinical features

• Fluid filled swelling at back

• Paraparesis with muscle wasting
• Sensory symptoms
• Neurogenic bladder and bowel
• Convulsions/ tonic spasms
• Cranial nerve dysfunctions
 Complications
• Rupture of sac during birth process
• CSF leak
• Raised ICP- vomiting, convulsions, altered
sensorium, irritability
• Infection - fever, unconsciousness, altered
sensorium (meningitis)
 Diagnosis
• Prenatal
• Acetyl cholinesterase and alpha fetoprotein in
maternal serum and amniotic fluid in 2nd
trimester)
• Prenatal ultrasound picks up the defect in
100% of the cases
• Many are now scheduled electively before
birth for repair
 Management
• Management of neural tube defects requires
team approach with cooperation of
pediatrician, neurologist, neurosurgeon and
anesthesiologist
• Surgery is mainstay of treatment and includes
repair of the defect and a VP shunt ( if
associated with hydrocephalus)
 Management...
• Meningomyelocele is repaired within first day
or two of life. Why it is an emergency?
– Rupture of sac
– Spinal cord vulnerable to infection
– Sepsis commonest cause of death
– Surgery < 24 hours for open defects which
minimizes bacterial contamination and further
neurological damage
– Closed lesions should be operated within 48hrs
 Pre Anesthetic Evaluation
• Neurological impairment including
– Cranial nerve function and ability to protect airway
– Neurological deficits
– signs of ICP
• Volume status and IV access
• Any drug therapy
– Steroids
– Mannitol, diuretics
– Anti - convulsants
 Pre Anaesthetic Evaluation
• Investigations
– Hb, Blood Grouping and Cross Matching
– X Ray chest ( if required)
• Pre ор preparation
• Pre ор advice
– Explain NPO to parents
– Adequate hydration to be maintained
– Usually no pre medication is required
 Induction of anaesthesia
• Intubation is a challenge
– In most cases, Supine position- with sac resting in a
donut shaped cushioned ring and weight of child
supported on pelvis and portions of spine not involved
with the defect
– For large defects, Lateral position- with head held in
midline by an assistant
• Assistant must be available to ensure that no
physical trauma to neuroplaque occurs
• Induction may proceed by mask inhalation or by
IV administration of thiopental, propofol,
glycopyrrolate, and a muscle relaxant.
• Either a NDMR or sux can be used Safely
• Difficult airway-awake intubation with atropine
premedication (20mcg/kg, minimum 0.1 mg)
and preoxygenation
• Intubation with reinforced tracheal tube is
recommended
 Maintenance of anesthesia
• Patient positioning - prone position
• Abdomen should be free - rolls under chest and
pelvis
• Excessive rotation/flexion of the neck avoided
brainstem compression, rise in ICP
• Extremities relaxed and padded
• Eyes protected with ointment and thick pads
 Maintenance ....
• Anesthetic agents
– 60% N20 in O2 with Isoflurane/Sevoflurane
– Analgesia maintained with IV Fentanyl. Avoid high
dose opioids- risk of post operative apnea
• Muscle relaxants
– NDMR (Atracurium, Vecuronium)
– Used with caution when nerve stimulation is used to
identify neural structures
• Ventilation
– Maintain normocapnia
– Prevent barotrauma
– Avoid prolonged exposure of high FiO2
• Fluid management
– IV fluids - RL is used, IV dextrose solution5% if patient
is hypoglycemic
– High third space losses
– Blood loss is not excessive a bout 30 ml, or 10% of
blood volume??.
 Latex allergy
• Myelodysplasia patients are at high risk of
developing allergic reactions to latex.
• Due to repeated exposure to latex products
during surgery, or repeated bladder catherization
• To prevent early sensitization, myelomeningocele
patients should be treated as1st if they are latex
allergic
• Manifests as hypotension and wheezing with or
without rash
 Latex allergy
• Anaphylaxis should always be anticipated
• Treatment - removal of source of latex and
administration of IV fluids and vasopressors
• Post operatively, respiratory status should be
carefully assessed.
• Prone to develop allergic reactions to other
substances later in life
 Emergence and postop care
• Elimination of anesthetic agents
• Reversal of neuromuscular blockade
• Assessment of airway patency
– Neonates at risk for apnea and extubated fully awake
• Oxygen therapy
• Nursing in prone/ lateral position
• Pain relief by IV fentanyl, PCM suppositories
• Watch for signs of brain stem dysfunction and ICP
Anesthetic Considerations for
Craniosynostosis repair
 Craniosynostosis: Definition
• Premature fusion of one or more cranial sutures,
resulting in abnormal skull development and
head shape and constitutes a diverse group of
deformities
• Growth of skull perpendicular to the affected
suture is restricted
• Compensation in growth occurs parallel to the
affected suture so brain growth can continue
 Classification

• Overall incidence: ~1 : 2,000 live births

• Simple (non-syndromic: sagittal, coronal,

metopic, lambdoid) – single suture
• Compound/complex: Non-syndromic with
multiple sutures or syndromic
•
 Anatomic Variations
• Trigonocephaly: fusion of the metopic suture that runs
from the top of the head toward the nose (5-15% of
cases)
• Scaphocephaly: The sagittal suture that runs from to
back on top of the skull (40-55% of cases)
• Plagiocephaly: a fusion of either the right or left coronal
suture (20-25% of cases)
• Brachycephaly: fusion of both coronal sutures
 Pathogenesis
• Exact etiology is unknown
• Intrauterine constraint of fetal head

• Metabolic and hematological disorders (rickets,

hypothyroidism, polycythemia vera, thalassemia)

• Teratogens (nicotine, retinoic acid, valproic acid, SSRI)

• Genetic defects
• Sporadic in most instances
• Potential risk factors :
– White maternal race
– advanced maternal age
– male infant vs female
– maternal smoking
– maternal residence at high altitude
– use of amine containing drugs (e.g. nitrofurantoin,
chlordiazepoxide)
– certain paternal occupations (e.g. agriculture and
forestry, mechanics)
– fertility treatments
 Clinical Features and
Complications
• Elevated intracranial pressure (ICP)
• Disturbances in intellectual and neurologic
development
• Brain growth (developmental delay)
• vision (impairment)
• Midface hypoplasia: associated with OSA, airway
complications, exorbitism
• cosmetic appearance
 INFANT SKULL
• Flexible- to get through the birth canal
• Expansile- to accommodate rapidly growing
brain
• The calvarial sutures serve 2 important functions
– (a) maintenance of head malleability &
– (b) growth sites
• 90% of adult size achieved by age of 1 year
• 96% by the age of 6 years
 Syndromes Associated with Craniosynostosis

• Most show an autosomal dominant pattern of

inheritance
• Most commonly associated with
craniosynostosis:
 Apert
 Crouzon
 Pfeiffer
 Others: Saethre-Chotzen, Muekne
 Apert Syndrome:
Anesthetic Implications
• Airway
 Midface hypoplasia
 Choanal stenosis/atresia
 Tracheal stenosis
 High arched palate
 C5-6 fusion
• Cardiovascular
 10% have congenital heart
disease
 Pulmonary stenosis
 ASD/VSD
 Tetralogy of Fallot
• Genitourinary
 10% have renal abnormalities
• Neuromuscular
 Possible cognitive delay
 Craniosynostosis
 Corpus callosum agenesis
 May have increased ICP
• Pulmonary
 40-50% have OSA
• Syndactyly
 Difficult IV access
 Crouzon Syndrome:
Anesthetic Implications
– Relative prognathism
 Possible difficult mask ventilation
– Possible difficult intubation secondary to high-
arched palate, retrognathism and other
dysmorphic features
– Over sedation may result in upper airway
obstruction
– Ocular proptosis requires careful attention to eyes
and adequate protection
 Pfeiffer Syndrome:
Anesthetic Implications
• All major sutures and skull base involved
 Increased ICP more common, as are Chiari malformations
and hydrocephalus
• OSA
- Monitor for airway obstruction
• Difficult airway management rare
• Proptosis requires careful attention to eyes
 Indications for Surgery
• Increased ICP
• Severe exophthalmos
• OSA
• Craniofacial deformity
• Prevention of neurologic sequelae
• Psychosocial concerns
 Timing of Surgery
• Emergency surgery required in cases of
raised ICP
• Benefits of early surgery (4-6 months of
age) include softer, more malleable bone
and continued growth of cranial vault
• Disadvantages include risks of anesthesia in a
younger patient, potential for increased
relative blood loss
 Surgery generally performed between 6-12 months of age
 Surgical Techniques

Strip Craniectomy

• Cranial vault split into multiple segments

• Allows skull to grow with brain
• Requires protective helmet post-op
• Used for children less than 6 months old
• May be done endoscopically
 Surgical Techniques
Spring Assisted Cranioplasty

• Used in infants 4-6 months

• Midline osteotomy along sagittal suture
 Springs placed to increase dimensions
• Advantages
 Lower transfusion requirement
 Shorter surgery, faster recovery
 Surgical Techniques
Total Vault Reconstruction, fronto-orbital
remodeling
• Removal of plates and
reconstruction
• Longer surgery
• Higher blood loss
• Specific procedures
include:
 Frontal advancement
 Posterior vault expansion
 Preanesthesia assassment
(A) Detailed history:-Birth history, sleeping position
• H/O OSA: daytime somnolence, behavioral
changes, snoring
• 40% to 80% syndromic infants (Zandieh et. al.
2013)
• Cardiac anomaly: h/o sweating with feeds,
cyanosis, and syncope,
• Family history abnormal head shape or multiple
systemic problems
 DIAGNOSIS: (B) CLINICAL EXAMINATION

• Head Circumference (micro/macrocephaly)

• Head shape (from above, side)
• Palpate suture lines & fontanelles (Look for ridging)
• Ear and facial symmetry, neck, spine, digits, and
toes, hands & feet
• Look for associated anomalies
• Subacute bacterial endocarditis (SBE) prophylaxis?
• Check airway if possible (infant)
• Identifying potential IV and arterial access sites
DIAGNOSIS: (C) RADIOLOGICAL EVALUATION

• Plain radiography-AP and lateral views of the skull-

bony bridging across the suture, straightening and
narrowing of the suture
• CT scan Head: more accurate, structural abnormalities.
• USG: Non invasive, Accuracy depends on a reliable and
experienced operator
• MRI: Complex craniosynostosis, improved definition of
intracranial soft tissue structures
• ICP monitoring
 PERIOPERATIVE CONSIDRATION.
• Most patients with non-syndromic craniosynostosis
are otherwise healthy
– Preoperative testing for these, should generally include a
CBC and BG and cross
– Coagulation profile routinely performed at some centers
• Syndromic craniosynostosis may be associated with
midface hypoplasia or retrusion resulting in OSA and
other perioperative airway considerations
• Use preoperative sedatives with caution in the
setting of severe obstructive disease
 PERIOPERATIVE CONCERNS...

• Syndromic concerns- associated systemic anomalies

– Difficult airway, OSA, Bronchospasm, kinking of ET
– Difficult IV access
– Positioning
– Ophthalmologic
– Blood loss
– VAE
– Hypothermia
– Long duration of surgery- facial, tongue edema, pushing out ET
– Metabolic and electrolyte disturbances
 MONITORING
• Standard ASA monitors:- ECG, BP, SPO2, TEMP
• Cardiac output monitoring
• Arterial line/ CVP
• Co-oximetry
• ABG/ ROTEM
• Precordial Doppler
• Urine output
 INDUCTION, AIRWAY MANAGEMENT, &
ANAESTHETIC MAINTENANCE
• Airway management challenging FVFI
• Alternative airway management techniques (e.g., LMA,
FOB with DA cart, tracheostomy?)
• RAE tube for sphinx position/armored tubes
• Induction: Inhaled commonly performed, if ICP high go
for IV induction propofol, fentanyl..
• In the presence of increased ICP, avoid
– sedative premedication, ketamine, succinylcholine,
hypercapnia, hypoxemia, and arterial hypotension.
• POSITIONING :-Supine, Prone, Sphinx (modified prone)
• Spinal cord injury d/t hyperextended neck
• Proper padding pressure points
• Lifting head for 15sec every 15 mins (recommended)
• Secure ET / avoid kinking/check airway pressure
• Check eye position/ tarsorrhaphy
– Generally surgeons want eye to be accessible
– Apply eye ointment periodically
– Proptosis may need tarsorrhaphy
• Forced air warming, blanket
 BLOOD LOSS MANAGEMENT
• Prone to massive blood loss due to:
– duration of the procedure,
– large surface of exposed skin, bone, and dural surfaces, and
– rare complication of injury of large vessels like sagittal sinus
• Nearly 90% to 100% of infants need blood transfusion
• Predictors of excess blood loss:
– surgery time > 5 hours
– age < 18 months
– multi-suture craniosynostosis and syndromic craniosynostosis
• Intraoperative assessment of BVL is difficult
 REDUCING BLOOD LOSS
• Surgical techniques that minimize blood loss include using
– infiltration of subcutaneous epinephrine
– needle tip cautery
– scalp clips for the scalp flap
– bone wax for the osteotomy edges
– Fibrin sealants or fibrin glue
• Cell saver (reduces blood unit transfusion by 1 unit)
• Preoperative acute normo-volemic hemodilution >6month
• Preoperative autologous donation
• Recombinant human erythropoietin (EPO) given S.C.
weekly for 3-4 weeks prior to surgery
Blood Management (continued)
Transfusion related concerns:
• Allergic/febrile/hypersenitivity reactions
• Metabolic/electrolyte derangements (hypocalcemia,
hyperkalemia in the setting of massive transfusion)
• Transfusion-associated Lung Injury (TRALI)
• Transfusion-associated circulatory overload (TACO)
 FLUID DEBATE

• Lactated Ringer appears unsuitable for pediatric

patients at risk for brain edema (e.g.: neurosurgery,
head trauma, CNS infection), overt or latent Cl-
deficiency (e.g.: vomiting, salt losing renal tubular
disorders, electrolyte disturbances due to diuretics)
• In these two conditions, saline still remains the
solution of choice
• Balanced crystalloids also have higher chloride
than reqd
 Venous Air Embolism (VAE)
Causes:
 Surgical field above heart with open sinusoids
 CVP decreased due to hypovolemia (bleeding) – air
entrained due to pressure gradient formed between
the surgical site and the right atrium
Diagnosis:
 A precordial doppler may assist in early detection
Hemodynamically significant VAEs rare (resulting from
RV outflow obstruction), and may be heralded by
hypoxemia, hypotension, decreased or absent end-tidal
CO2, cardiac arrest in extreme cases)
 Venous Air Embolism (continued)
Prevention:
 Surgeon should apply bone wax to open edges of bone
Limit head down, but remember that even when supine, children
may be at risk due to their larger heads
Treatment
 Notify surgeon to flood surgical field
 Lower surgical field relative to heart
 Increase central venous pressure with crystalloid, colloids, blood
products as indicated
 IV epinephrine may be necessary in the setting of hypotension
 Aspirate air from central venous catheter if placed
 POSTOPERATIVE CARE
• Extubation: Most patients are extubated at the completion of
surgery in OT/ ICU
• Conditions for delay extubation : difficult airway cart ready
when extubating
– prolonged procedure
– marked fluid shifts
– large-volume transfusions
– effects of prolonged prone positioning and
– patient factors such as: preoperative obstructive sleep apnoea or
airway concerns
– Mechanical ventilation/ tracheostomy
– Obstructive sleep apnea/delayed awakening
 Postoperative Considerations

• Analgesia best achieved with combination of

acetaminophen and IV opioids (PRN or continuous
infusion, such as nurse-controlled analgesia [NCA])
• Local infiltration by the surgeon may be utilized to
supplement
• Adequate pain relief with opiates and PCM
– Monitor for airway obstruction, electrolyte disturbances
(hyponatremia), and anemia/coagulation abnormalities
from blood loss
 References
• Smith’s Anaesthesia for Infants and Children, 7th Edn.
Philadelphia: Mosby; 2006.
 Miller’s Anesthesia 9th edition volume 2
 Morgan & Mikhail’s Clinical Anesthesiology 6th Edition
• Durham EL, Howie RN, Cray JJ. Gene/environment interactions in
craniosynostosis: a brief review. Orthod Craniofac Res. 2017
• Hughes K, Thomas K, Johnson D et al. Anesthesia for surgery
related to craniosynostosis. Part 2. Pediatr Anesth 2013: 22-7
• Pearson A, Matava CT. Anaesthetic management for
craniosynostosis repair in children. BJA Educ 2016;16
• Stricker PA, Goobie SM, Cladis FP et al. Perioperative
Outcomes and Management in Pediatric Complex
Cranial Vault Reconstruction: 2017;(126):276-287
• Thomas K, Hughes C, Johnson D et al. Anesthesia for
surgery related to craniosynostosis. Part 1. Pediatr
Anesth 2012(11): 1033-41
• White N, Bayliss S, Moore D. Systematic review of
interventions for minimising perioperative blood
transfusion for surgery in craniosynostosis. J
Craniofacial Surg, 25 (2015), pp. 126-136
 GALATOOMAA!!
Thank you for your time!