Anesthetic management of Craniosynostosis

1. CRANIOSYNOSTOSIS
Dr Ratnesh Shukla (Neuroanesthesiology and critical care)

2. CRANIOSYNOSTOSIS
• Definition – Characteristic skull deformities that occur as a result of premature fusion of
one or more cranial vault sutures
• Results in abnormalities in size & shape of the calvarium, cranial base, orbits and dental
occlusion and constitutes a diverse group of deformities.
• Craniosynostosis affects
• cosmetic appearance
• brain growth (developmental delay)
• ICP (increased- HCP)
• vision (impairment)
• Incedence-‐1 / 2000-‐2500 live birth
• Males - sagittal and metopic stenosis
• Females - coronal
2

3. HISTORY
• Sommerring (1700s) : bone growth in skull occurred primarily at suture lines and that when this growth site
was prematurely bridged with bone, an abnormal skull shape developed
• Otto (1830) : coined the term craniosynostosis
• Stahl and Hyrtl : premature closure of cranial vault sutures abnormal skull shape
• Virchow (1851): described how skull growth is restricted to a plane perpendicular to the affected,
prematurely fused suture and is enhanced in a plane parallel to it
• Gave classification of skull deformities
• Lannelogue (1890) : performed B/L strip craniectomies
• Paul Tessier : father of modern craniofacial Surgery
• First to attempt major surgical procedures on the craniofacial skeleton
3

4. 4

5. PATHOGENESIS
• Premature fusion of calvarial sutures
• 1 in 2500 live births
• Syndromic and non-syndromic.
• Phenotypic and non-phenotypic.
• Primary and secondary.
• Most common, sagittal, unilateral coronal, bilateral coronal,
metopic and lambdoid
• Sutures allow growth perpendicular to them
• Growth at suture lines related to brain growth
5
CRANIOSYNOSTOSIS
NORMAL SUTURE

6. NORMAL SKULL OF INFANT
6

7. 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
• The cranial base was proposed as the primary locus of the
abnormality in children with multi sutural craniosynostosis and
may not be the primary anomaly in bicoronal or sagittal
craniosynostosis
• 90% of adult size achieved by age of 1 year
• 96% by the age of 6 years
7

8. TIMING OF CLOSER OF SUTURES & FONTANELLES
Sutures & Fontanelles Timing of closer
Metopic Suture 9 months -‐ 2 year
Coronal , Sagittal and Lamdoid suture 40 years
Anteior fonanelles 18-‐24 months
Posterior fontanelles 3 -‐ 6 months
Mastoid fontanelles 1 year
Sphenoid fontaneles 2-‐3 months
8
Rodger E, Hall CM. Craniosynostosis. Clin Radiol 2002;57:94

9. BRAIN & CRANIUM
• Brain Growth
 At term nearly 40% of adult brain volume and increases to 80%
by 3 years of age and continues to grow until the age of 12 yrs
• The cranium
 At term -‐ 40% of adult size and by 7 years of age -‐ increases to
90%.
Principles and practice of pediatric neurosurgery. Thieme
Medical, 1999:219-‐42
9

10. ETIOLOGY
• Exact etiology is not known
• Sporadic in most instances
• Potential risk factors :
• White maternal race
• advanced maternal age
• male infant
• 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
10

11. ETIOLOGY..
• Familial
• Non-syndromic craniosynostosis,
• Transmitted as an Autosomal Dominant disorder
• Fibroblast growth factor and fibroblast growth factor receptor (FGFR) : regulate fetal
osteogenic growth and possibly influence fetal suture patency.
• Mutation in gene coding for FGFR1 -‐Pfeiffer’s syndrome
• FGFR2 : Apert’s syndrome and Crouzon’s 10/10/2012
• syndrome TWIST – Saethre chotzen syndrome
11

12. 12

13. CLASSIFICATION OF CRANIOSYNOSTOSIS
• Primary
• Simple (single suture) : Non syndromic: sagittal, coronal, metopic, lambdoid
• Compound ( ≥2 suture): Non syndromic: bicoronal
Syndromic: Crouzon’s disease, Apert’s syndrome,
Pfeiffer’s disease, Saethre-Chotzen syndrome
• Secondary
• Metabolic disorders: e.g., hyperthyroidism (bone mature faster )
• Malformations : e.g. holoprosencephaly, shunted hydrocephalus, microcephaly,
encephalocele.
• Exposure of fetus: e.g. valproic acid, phenytoin.
• Mucopolysaccharidosis : e.g., Hurler’s syndrome, Morquio’s syndrome.
13
Aviv RI, Rodger E, Hall CM. Craniosynostosis. Clin Radiol 2002;57:94

14. PRIMARY VS SECONDARY CRANIOSYNOSTOSIS 14
Primary Secondary
Primary defect of ossification: suture growth is altered Secondary to brain malformation
Head frequently asymmetric Head symmetric
Brain continues to grow in areas where sutures are
open
growth of brain is impaired
most individuals are normal neurologically neurologically abnormal usually
benefit from surgery No benefit of surgery

15. TYPE OF CRANIOSYNOSTOSIS CHARACTERISTIC HEAD SHAPE
Single‐Suture Synostosis
Sagittal
Coronal
Metopic
Lambdoid
Scaphocephaly (Dolichocephaly)
Anterior plagiocephaly,
Trigonocephaly
Posterior plagiocephaly
Double-Suture Synostosis
Bicoronal
Bilambdoid
Saggittal plus metopic
Anterior brachycephaly
Posterior brachycephaly
Scaphocephaly
Complex Multisuture Synostosis
Bicoronal, sagittal,metopic
Multisuture, pansynstosis
Turribrachycephaly
cloverleaf (Kleeblattschadel)
15

16. 16

17. TYPES OF CRANIOSYNOSTOSIS:
A. Sagittal
• Sagittal craniosynostosis
(Scaphocephaly, dolichocephaly )
• Most common: 40-55%,
• 1 in 5000 live birth
• Male : Female = 3:1
• Skull shape :
1. biparietal narrowing
2. ridging of the sagittal suture
3. bilateral bulging of the frontal or
occipital region or both
17

18. B. CORONAL CRANIOSYNOSTOSIS
• Second most common type (20 -‐25 %)
• 1/10,000 live births
• Female > male with ration of 2 : 1
• Two types :
i) Bilateral coronal cranosynostosis: Brachycephaly
ii) unilateral coronal cranosynostos: Anterior plegiocephaly
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19. B.(I) BILATERAL CORONAL CRANOSYNOSTOSIS:
BRACHYCEPHALY
19
1. Fused bilateral coronal suture
2. Recessed superior orbital rim
3. Prominent frontal bone
4. Flattening of occiput
5. Anteriorly displaced skull vertex
6. Shortened anterior cranial fossa
7. Harlequin deformity of greater wing of sphenoid
8. Protrusion of squamous portion of temporal bone

20. B. (II) UNILATERAL CORONAL SYNOSTOSIS
1. Prematurely fused one coronal suture,
2. Flattening of the ipsilateral frontal and parietal
bones,
3. Bulging of the contralateral frontal and parietal
bones
4. Bulging of the ipsilateral squamous portion of
the temporal bone,
5. Ipsilateral ear displaced anteriorly compared
with the contralateral ear.
6. Radiographic findings include the “harlequin”
orbit deformity ( elevation of supra orbital
margin )due to elevation of the greater and
lesser wings of the sphenoid
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21. C. METOPIC SYNOSTOSIS 21
• Also known as trigonocephaly (as seen from above-
triangular look)
• Incidence : 4-10 %
• M > F
• Features:
1. Ridging of fused suture
2. Temporal narrowing (bilateral lateral retrusion of
frontal bones)
3. Patent coronal sutures displaced anteriorly
4. Compensatory bulging of the parieto-occipital region,
contributing to the skull’s triangular appearance
5. Narrowed bizygomatic dimension
6. Posterior displacement of the superolateral orbital rim
7. The distance between the medial canthi of the eyes
is reduced (hypotelorism)

22. D. Lambdoid synostosis
• Represents 2-‐ 4% ,
• prevalence of 1 / 150,000 live births
• unilateral fusion: Posterior plegiocephaly
• bilateral fusion: Posterior brachycephaly
E. Oxycephaly
• Turricephaly ( high head syndrome)
• Fused coronal and sagittal suture results in a an
abnormally high conical head shape with bossing in the
region of the anterior fontanelle
• Seen in syndromic craniosynostosis
F. Cloverleaf skull deformity
• Pansynostosis/multiple suture synostosis
• Head shaped like a cloverleaf (Kleeblattschadel)
• Three bulges-‐two temporal and top pronounced
constrictions in both sylvian fissures
22

23. CRANIOSYNOSTOSIS SYNDROMES
1. Crouzon’s syndrome (craniofacial dysostosis)
• one in 60,000 live births (most common craniosynostosis $)
• 5 % of cases of craniosynostosis
• Autosomal dominant pattern, paternal age older than 35 years
• Mutation of gene coding FGFR2, FGFR3
Clinical features:
• Brachycephaly, proptosis, and midface (maxillary) hypoplasia
• significant hypertelorism, beaked nose
• Intracranial anomalies : Hydrocephalus (30%), Chiari 1 malformation &
hindbrain herniation (70 %)
• No visceral or extremity involvement
• Normal intellect
• Normal extremities
• 5 % have acanthosis nigricans
• 30 % have progressive hydrocephalus
Anesthetic concerns:
Airway management: May be a difficult intubation (uncommon),
airway obstruction with sedation or anesthesia, caution with premedication
Neurologic: Caution with premedication if elevated ICP,
Eyes require protection if ocular proptosis present
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24. 2. APERT’S SYNDROME (ACROCEPHALOSYNDACTYLY)
• Crouzon’s with Hand Involvement
• 1 in 65,000 to 88,000 live births,
• Mutation FGFR2
• Clinical features:
• HEENT: Turribrachycephaly, midface hypoplasia, orbital
hypertelorism, cleft palate in 30%, occasional choanal atresia
and tracheal stenosis, airway obstruction. OSA (40-85%)
• Cardiac: Congenital heart disease occurs in 10%; may include
VSD, pulmonary stenosis.
• Genitourinary: Hydronephrosis in 3%, cryptorchidism in 4.5%.
• Musculoskeletal: Syndactyly of the hands and feet, fusion of
digits 2 to 4, fusion of the cervical vertebrae can occur.
• Neurologic: Mental retardation (50%), may have elevated ICP.
• Dermatologic: Acne vulgaris is common
24

25. • Anesthesia issues:
• Preoperative laboratory tests: HCT, blood type, and screen, X-ray cervical spine.
• Airway management:
• Mask ventilation may be very difficult because of midface hypoplasia, choanal, atresia, and tracheal
stenosis.
• Intubation may be difficult because of facial anomalies and decreased neck mobility.
• FOB, LMA (Nishimoto K, et al. Masui. 2014;63:1125-1127.)
• Cardiac: Emphasis on balancing pulmonary and systemic blood flow, de-air IV lines, SBE
prophylaxis.
• Musculoskeletal: Cervical fusion may decrease neck extension; syndactyly may make vascular
access difficult.
• Neurologic: Caution with premedication if ICP elevated
• For OSA adno-tonsillectomy advised (Zandieh SO et, al., Plast Reconstr Surg. 2013;131:847-852)
25
2. APERT’S SYNDROME (ACROCEPHALOSYNDACTYLY)..

26. 3. PFEIFFER SYNDROME
• Incidence 1 in 100,000
• Mutation of gene coding for FGFR1, FGFR2
• 3 types-Typically clinical features, degree of airway obstruction,
and mortality increase with types 2 and 3
• Suture: Coronal and occasionally sagittal
• Clinical features
• HEENT: Tower skull, midface hypoplasia, orbital hypertelorism,
proptosis; choanal atresia-uncommon.
• Pulmonary: OSA
• Cardiac: May have cardiac defects.
• Musculoskeletal: mild syndactyly involving broad thumbs and great
toes, rarely ankylosis of elbow, fusion of cervical vertebrae reported
• Neurologic: Generally normal but mild developmental delay can
occur, may have increased ICP.
26

27. 3. PFEIFFER SYNDROME
• Anesthesia issues:
• Preoperative laboratory tests: HCT, blood type, and screen.
• Airway management: No reported cases of difficult intubation; airway obstruction may occur
intraoperatively or postoperatively.
• Cardiac: Emphasis on balancing pulmonary and systemic blood flow, de-air IV lines,
endocarditis prophylaxis.
• Musculoskeletal: Cervical fusion may decrease neck extension.
• Neurologic: Caution with premedication if ICP elevated, eyes require protection if ocular
proptosis present
27

28. 4. Saethre-Chotzen syndrome
• Suture involved: coronal & other
• Clinical features:
• HEENT: Brachycephaly, maxillary hypoplasia, orbital hypertelorism,
beaked nose, occasional cleft palate, low hairline
• Genitourinary: Renal anomalies and cryptorchidism
• Musculoskeletal: Short stature, mild syndactyly, cervical fusion possible
• Neurologic: Mild developmental delay, rare increased ICP
• Anesthesia issues:
• Preop lab tests: HCT, blood type, and screen
• Airway management: No reported cases of difficult ventilation or
intubation
• Musculoskeletal: Cervical fusion may decrease neck extension;
syndactyly may make vascular access difficult.
• Neurologic: Caution with premedication if elevated ICP
28

29. 5. Carpenter Syndromes
• Suture involved: coronal & other (lambdoid suture closes first coronal last)
• Autosomal recessive
• Clinical features
• HEENT: Tower skull, down-thrust eyes, orbital hypertelorism, low-set ears,
small mandible
• Cardiac: Cardiac defects- common (VSD, ASD)
• Genitourinary: Hypogonadism
• Musculoskeletal: Syndactyly of hands and feet
• Neurologic: Developmental delay common but variable, may have
increased ICP
• Other: obesity
• Anesthesia issues
• Preoperative laboratory tests: HCT, blood type, and screen
• Airway management: Small mandible may make intubation difficult;
obesity may make ventilation difficult
• Musculoskeletal: Syndactyly may make IV access difficult
• Neurologic: Caution with premedication if elevated ICP
29

30. 6. Muenke syndrome
• Suture involved: coronal
Clinical features
• HEENT: Wide-set eyes, hearing loss
• Neurologic: Developmental delay uncommon
Anesthesia issues
• Preoperative laboratory tests: HCT, blood type, and
screen.
• Neurologic: Caution with premedication if elevated
ICP.
30

31. CONSEQUENCE OF CRANIOSYNOSTOSIS
Intracranial hypertension
• 4-20%: single suture craniosynostosis
• 42-60 % : multiple suture/syndromic craniosynostosis
• Neurologic symptoms of elevated ICP (Headaches, vomiting, sleep disturbances,
feeding difficulties, behavioral changes, and diminished cognitive function)
• If ICP : 10 -15 mmHg were considered borderline & > 15 mmHg abnormal
• Causes-
• abnormal venous drainage
• respiratory obstruction
• Chiari malformations
31

32. CONSEQUENCE OF CRANIOSYNOSTOSIS
Hydrocephalus
• 4% to 18%
• Multiple suture craniosynostosis >> non-syndromic single suture craniosynostosis
• Communicating
• Causes
• Cerebral maldevelopment
• Brain atrophy
• Abnormal CSF circulation
• Venous outflow obstruction
• Hind brain herniation
• Aqueduct stenosis
32

33. CONSEQUENCE OF CRANIOSYNOSTOSIS
Ophthalmologic Effects
• Papilloedema, optic nerve atrophy, and even loss of vision may occur with
prolonged, untreated elevated ICP
• Visual function and eye motility are often impaired
• orbital dystopia secondary to unilateral coronal synostosis result in disturbances
of extra-‐ocular muscle movement (i.e., strabismus), upper eyelid ptosis, and poor
binocular vision
• Decreased orbital volume (in coronal craniosynostosis) causes proptosis, corneal
exposure, and increased risk of direct ocular trauma
33

34. SURGICAL OPTIONS
• Open Cranial Vault (Frontal Orbital Advancement)
• Calvarial vault reconstruction : plastic surgery (craniofacial) and neurosurgery
• “Bandeau” osteotomy is performed along lateral temporal bones and nasion to
mobilize the superior orbital rim
34

35. STRIP CRANIECTOMY WITH HELMET
• Endoscopic approach
• less invasive
• less blood loss
• shorter lengths of stay
• Age: 2-3 month
• Helmet therapy needed
for 6 months
35

36. STRIP CRANIECTOMY WITH HELMET..
36

37. SPRING-MEDIATED CRANIOPLASTY
• Synostotic suture removed surgically & a spring between the two bone
edges is placed
• Over time, the force exerted on the two bone edges corrects the defect
• A second operation- to remove the spring
• Less blood loss and lengths of stay
37

38. 38

39. MIDFACE ADVANCEMENT
• It is to correct midface hypoplasia
• Different types of advancements include:
• Le Fort I maxillary advancement (maxillary hypoplasia due to cleft lips
& palates)
• Le Fort III maxillary and upper face advancement
• Monoblock advancement (maxilla, upper face, orbits, and forehead)
• The Le Fort III and Monoblock osteotomies typically performed for
patients with midface hypoplasia due to syndromic craniosynostosis
(Apert, Pfeiffer, Crouzon)
39
Midface
mobilized
Rigid fixation
Distraction bar
anchored to
mobilized mid
face
(distracted @1-
2 mm/day)

40. INDICATIONS FOR SURGERY
• Correction of cosmetic abnormality
• Early treatment of intracranial hypertension
• Optimizing brain growth
• Severe proptosis and impending corneal damage
40

41. TIMING OF SURGERY
• Early operation(3-‐6 months)
• Better compliance of brain ,dura and scalp
• Calvarium is much more malleable, easier to shape and providing a better outcome
• Rapid brain growth reshape the bone
• Surgery within 3-6 months time frame to take advantage of the ability of the rapidly
expanding brain and skull to grow more normally and so that the skull can be remodeled
more readily
John A. Persing, et. al. Management Considerations in the Treatment of Craniosynostosis.
Plastic and Reconstructive Surgery; April 2008
41
YN Anantheswar, NK Venkataramana, Pediatric craniofacial surgery for craniosynostosis.
Pediatric neurosciences,2009 , 4 ; 86-‐99

42. ANAESTHESIA MANAGEMENT
42

43. DIAGNOSIS
(A) Detailed history:
• Birth history, sleeping position
• H/O OSA: daytime somnolence, enuresis, behavioural changes, snoring
40% to 80% syndromic infants
Zandieh et. al. Plast Reconstr Surg. 2013;131:847-852
• Cardiac anomaly: h/o fatigue or sweating with feeds, cyanosis, and
syncope, VSD most common, Syndromic infants more likely to have it
Hidestrand P et. Al. J Craniofac Surg. 2009;20:254-256
• Head tilt, torticollis (deformational plagiocephaly), delayed developmental
mile stones
• family history abnormal head shape or multiple systemic problems (e.g.
cardiac, genitourinary, musculoskeletal)
43

44. 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
• Look for associated anomalies (CVS, genitourinary, musculoskeletal)
• Fundus examination (syndromic craniosynostosis~ 50% papilledema)
Bannink N et al. J Craniofac Surg. 2008;19:121-127
• Check hands & feet
• Check airway if possible (infant)
• Identifying potential IV and arterial access sites
• Subacute bacterial endocarditis (SBE) prophylaxis - uncorrected or recently
corrected cyanotic heart disease receiving intraoral surgery
44

45. DIAGNOSIS: (C) RADIOLOGICAL EVALUATION
• Plain radiography- AP and lateral views of the skull- bony bridging across the suture, sclerosis,
straightening and narrowing of the suture and loss of suture clarity
• CT scan Head: more accurate, structural abnormalities (e.g., hydrocephalus, agenesis of the
corpus callosum). 3D CT scanning accurately delineate the craniofacial deformity and plan
surgical reconstruction
• USG: Non invasive, Accuracy depends on a reliable and experienced operator
• MRI: Complex craniosynostosis, improved definition of intracranial soft tissue structures
Hindbrain herniation, can identify sites of respiratory obstruction
• Radio isotope scanning: Diminished uptake → complete fusion
• ICP monitoring: Clinically occult – majority, Radiological signs inconclusive,
Features of ↑ ICT Mean pressure > 15 mm Hg
Raised base line value & Prolonged plateau wave
45

46. PERIOPERATIVE ANESTHETIC 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
47

47. MONITORING
• Standard ASA monitors
• ECG,
• BP,
• SPO2,
• TEMP
• Cardiac output monitoring
• Arterial line/ CVP
• Co-oximetry
• ABG/ ROTEM
• Precordial Doppler
• Urine output
48

48. SPECIFIC MONITORING 49
PRECORDIAL DOPPLER TEE
ROTEM
CARDIAC OUTPUT

49. INDUCTION, AIRWAY MANAGEMENT, &
ANAESTHETIC MAINTENANCE AIRWAY
• Airway management challenging (upper airway obstruction)
• Difficulty may be at ventilation, intubation, or both
• Maintain spontaneous ventilation till the airway is secured
• Alternative airway management techniques (e.g., LMA, FOB with difficult airway cart)
Fiadjoe JE et al. Paediatr Anaesth. 2015;25:801-806
• RAE tube for sphinx position/armored tubes (intra-op suctioning difficult)
• Induction: Inhaled commonly performed, if ICP high go for IV induction
• In the presence of increased ICP, avoid
• sedative premedication, ketamine, succinylcholine.
• hypercapnia, hypoxemia, and arterial hypotension.
• Some infants require tracheostomy in view significant upper-airway
obstruction Perkins JA. Cleft Palate Craniofac J. 1997;34:135-140
Sculerati N et. Al. Laryngoscope. 1998;108:1806-1812
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50. 51

51. POSITIONING
• Supine
• Prone
• Sphinx (modified prone)
• Spinal cord injury d/t hyperextended neck
 Proper padding pressure points
 Lifting head for 15 s 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
52

52. LINES
• Peripheral Venous Access: at two least 20/ 22G
cannula
• Blood Tx: potentially high potassium content as well
as citrate to mix and be diluted before it returns to the
heart
• Arterial Catheter:
• Real time IBP measurements
• Repeated blood sampling (ABG, electrolytes &
metabolic status)
• Electrolyte and metabolic correction
• Central Venous Catheter: for CVP, VAE, inotrope or
difficult IV access
53

53. 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
• Blood loss ~ one-half to one blood volume (Tunçbilek G, J Craniofac Surg. 2005;16:59-62)
• Nearly 90% to 100% of infants need blood transfusion (Stricker PA Paediatr Anaesth. 2010;20:150-159)
• Predictors of excess blood loss: (White N et. al. J Craniofac Surg. 2009;20:378-381)
• surgery time > 5 hours (Stricker PA, Paediatr Anaesth 2010; 20: 150–9)
• age < 18 months
• multi-suture craniosynostosis
• syndromic craniosynostosis
• Intraoperative assessment of BVL is difficult
• Postoperatively- collection in surgical drains
54

54. REDUCING BLOOD LOSS
Techniques include
• 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 (C/I in infants < 6month )
• Preoperative autologous donation
• Recombinant human erythropoietin (EPO) given s.c. weekly for 3–4 weeks prior to
surgery (Fearon JA Plast Reconstr Surg. 2002;109:2190-2196)
55

55. REDUCING BLOOD LOSS..
• Fibrinogen concentrate:
• Lyophilized, purified, pasteurized product
• Advantages:
 pathogen inactivation and elimination of infectious disease transmission risk,
 ability to be stored for prolonged periods before reconstitution and administration
 ability for precise dosing
• Disadvantage:
 High cost
• Effective in maintaining normal hemostatic function in infants undergoing craniofacial
surgery without the need for FFP transfusion
Haas T et al. Anesth Analg 2008;106:725–31
56

56. REDUCING BLOOD LOSS..
Recombinant activated factor VII
• Acidosis, hypothermia, thrombocytopenia, and hypofibrinogenemia all impair the efficacy
of recombinant factor VIIa and
• should be corrected to maximize efficacy.
• associated with significant thrombotic complications; therefore, so the use should be
reserved for life-threatening situations in which all other methods of achieving
hemostasis have failed.
Stricker PA et al. Successful treatment of intractable hemorrhage with recombinant factor VIIa
during cranial vault reconstruction in an infant. Paediatr Anaesth 2009;19:806–7
57

57. COMPLICATIONS OF TRANSFUSION
• Acute hemolytic reactions
• Transfusion related acute lung injury (TRALI)
• Infection
• Dilutional coagulopathy (1.2 times blood vol loss: FFP needed)
Hiippala ST et. al. Anesth Analg 1995; 81:360–5
• 1 unit of FFP:1 unit of PRBCs: near elimination of postoperative dilutional coagulopathy
Stricker PA et. al. Paediatr Anaesth 2011;21:54–61
• Refrigerated Fresh whole blood (less no of donor exposure, no need of FFP, platelets)
Kaufman RM. Transfusion 2005;45:1407–12
58

58. COMPLICATIONS OF TRANSFUSION..
• Electrolyte acid–base disturbance:
• Hyperkalemia (from high potassium content of stored PRBCs),
• Hypocalcemia (citrate toxicity)
• Rapid administration of PRBCs with prolonged storage, particularly in the setting of
hypovolemia, can result in hyperkalemic cardiac arrest
Brown KA, et. al. Can J Anaesth 1990;37: 747–54 & Bhananker Smet. Al. Anesth Analg 2007;105:344–50
• Wake up Safe group recommends: use PRBCs less than 7 days from collection
http://www.wakeupsafe.org/Hyperkalemia_ statement.pdf. WakeUpSafe. 2011
• Metabolic acidosis
• Hypothermia
59

59. ANTIFIBRINOLYTIC AGENTS
• Attractive strategy : simple to administer, inexpensive, and low incidence of adverse
events
• Tranexamic acid: reducing blood loss and perioperative transfusion in children
undergoing craniofacial surgery
dose 10 to 100 mg/ kg loading dose followed by an infusion of 5–10 mg /kg/ h for
the duration of the surgery
• Aminocaproic acid: comparable efficacy as tranexamic acid
• Aprotinin: also effective but no longer available
D’Errico CCet al. Efficacy of aprotinin in children undergoing
craniofacial surgery. J Neurosurg 2003;99:287–90
60

60. 61

61. 62

62. VAE IN CHILDREN 63

63. VAE AND CRANIOSYNOSTOSIS 64

64. INTRAOPERATIVE VAE
Symptoms
• Bronchoconstriction/wheezing
• Hypotension/circulatory collapse
• Hypoxaemia (V/Q mismatch)
• Dysrhythmias, Myocardial ischaemia
Signs
• Abrupt decrease/loss end-tidal CO2 •
• Turbulent flow detected on transoesophageal echo or Doppler ultrasound
Management
• Notify surgeon,
• call for help
• 100% oxygen
• Discontinue nitrous oxide/volatile
• Flood surgical wound with saline
• Position head below the heart
• Perform valsalva with manual ventilation
• Chest compressions (even if not in cardiac arrest, these may help break up bubbles)
• Treat cardiovascular compromise with usual inotropes, e.g. epinephrine
• Standard PALS protocol if in cardiac arrest
• Call for emergent transoesophageal echocardiography to confirm diagnosis
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65. FLUID DEBATE
• Lactated Ringer appears unsuitable for pediatric patients
• at risk for brain edema (e.g.: neurosurgery, head trauma, central nervous
system 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
Santi et al. Italian Journal of Pediatrics (2015) 41:47
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67. 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
• Adequate pain relief with opiates and Acetamenophen
• Blood product transfusion/ metabolic abnormalities
• Obstructive sleep apnea/delayed awakening
• Vision loss/increased exophthalmos/conjunctival chemosis
• Long term follow up for rehabilitation/psychosocial cognitive therapy
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The Journal of Craniofacial Surgery Volume 30, Number 3, May 2019

69. 70

70. LONG TERM FOLLOW-UP
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Speech Training
Feeding / Swallowing trainingGenetic counselling
Ophthalmological
reivews

71. THANK YOU
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