This document outlines the learning objectives and clinical considerations regarding hydrocephalus, meningocele, and craniosynostosis in pediatric patients, including anatomy, neurophysiology, and management strategies. It details the pathophysiology, clinical presentation, and various treatment modalities, such as shunt placement. Additionally, it covers anesthetic management, pre-operative assessments, intraoperative concerns, and post-operative care specific to these conditions.

1. Prepared by:-
Gammachis Akkuma(Ass’t lecturer)
March 2023
3/4/2023 1

2. Outline
• Objectives
• Neurophysiology and development
• Hydrocephalous?
• Meningocele
• Craniosynstosis

3. 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

4. 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.

5. 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.

6. • 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.

7. 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

8. 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

10. • 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.

11. 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.

12. 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).

13. Ethology and classification
• Congenital :-
– Myelomeningocele
– Stenosis of the cerebral
aqueduct
– Arnold-Chiari
malformation
– Arachnoid cysts
– Vascular malformations
– Idiopathic
• Acquired :-
– Infection
– Intraventricular
hemorrhage
– Tumour
– Trauma
– Nutritional deficiencies

15. 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

16. 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)

18. 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

19. • 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).

20. 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.

21. Shunt Surgery

22. 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

23. 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

24. • 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

25. • 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

26. • 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

27. 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.

28. • 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

29. • 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

30. 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

31. 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

32. 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

33. • 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.

34. – 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?

35. 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

36. 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

37. 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

38. 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

39. Meningomyelocele

40. Thoracolubar 85%
Dorsal 10%
Cervical 5%

41. 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

42. 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

45. Pathophysiology
• Depending on severity, manifests as neurologic,
motor and sensory deficits
• Associated congenital anomalies may be
present
• Brainstem dysfunction
• Hydrocephalus and ↑ICP

46. 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

47. Clinical features
• Fluid filled swelling at back
• Paraparesis with muscle wasting
• Sensory symptoms
• Neurogenic bladder and bowel
• Convulsions/ tonic spasms
• Cranial nerve dysfunctions

48. Complications
• Rupture of sac during birth process
• CSF leak
• Raised ICP- vomiting, convulsions, altered
sensorium, irritability
• Infection - fever, unconsciousness, altered
sensorium (meningitis)

49. 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

50. 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)

51. 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

54. 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

55. 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

57. 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

60. • 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

61. 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

62. 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

63. • 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??.

65. 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

66. 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

68. 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

69. Anesthetic Considerations for
Craniosynostosis repair

70. 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

71. 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

72. •

73. 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

76. 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

77. • 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

78. 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

79. 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

80. Syndromes Associated with
Craniosynostosis
• Most show an autosomal dominant pattern of
inheritance
• Most commonly associated with
craniosynostosis:
 Apert
 Crouzon
 Pfeiffer
 Others: Saethre-Chotzen, Muekne

81. 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

82. 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

83. 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

84. Indications for Surgery
• Increased ICP
• Severe exophthalmos
• OSA
• Craniofacial deformity
• Prevention of neurologic sequelae
• Psychosocial concerns

85. 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

86. 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

87. 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

88. Surgical Techniques
• Removal of plates and
reconstruction
• Longer surgery
• Higher blood loss
• Specific procedures
include:
 Frontal advancement
 Posterior vault
expansion
Total Vault Reconstruction, fronto-orbital
remodeling

89. 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

90. 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

91. 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

92. 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

93. 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

94. MONITORING
• Standard ASA monitors:- ECG, BP, SPO2, TEMP
• Cardiac output monitoring
• Arterial line/ CVP
• Co-oximetry
• ABG/ ROTEM
• Precordial Doppler
• Urine output

95. 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.

96. • 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

98. 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

99. 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

100. 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)
Blood Management (continued)

101. 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

102. 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)

103. 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
Venous Air Embolism (continued)

104. 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

105. 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

106. 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

107. • 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