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ANESTHETIC CONSIDERATIONS
1. Considerations for the Pediatric (cooperation, physiology, pharmacokinetics)
2. Potential Difficult Intubation/Ventilation
a. Surgical airway vs intubation attempt
b. Awake vs asleep
c. Spontaneous ventilation vs paralyzed
3. Potential Aspiration Risk & Co-existing Obstructive Sleep Apnea
4. Potential for Unstable or Immobile Cervical Spine
5. Coexisting multiple congenital anomalies particularly CHD
ANESTHETIC GOALS
1. Establish
a. Aspiration Risk
b. Cervical Spine Stability
c. Presence of OSA & Associated Anomalies
2. Minimize anesthetic related post operative respiratory depression
HISTORY
Previous anesthetics and experience with AW management (but kids grow!)
Airway
Details of congenital syndrome
Evidence of acute on chronic AW obstruction (infection, foreign body)
Noisy breathing/ stridor
Snoring
Symptoms of OSA
Complete perinatal Hx
Complications of Pregnancy
History of acute/ chronic airway obstruction
Aspiration symptoms/ reflux
Symptoms of cervical spine involvement
Occipital/ neck/ upper extremity pain or paresthesias
Limited range of motion
Coexisting syndrome abnormalities e.g. cardiac (cyanosis, murmur)
PHYSICAL
Signs of distress including agitation, retractions, cyanosis, anxiety, weak or absent cry, or
stridor
Anatomical AW abnormalities including:
Oral cavity (macroglossia, microstomia [small mouth], distortion [hemangioma,
glottic webs])
Anterior mandibular space (micrognathia, anterior larynx)
Maxilla (hypoplasia, nasal obstruction/mouth breathing)
TMJ (restriction to opening [fixed vs. secondary], reduced translocation [inability to
jut jaw forward])
Vertebral column (restricted movement, instability)
Resp: pattern and rate, wheeze (aspiration?), stridor, positional dyspnea
CVS: pulses, cap refill, murmur, pulm HTN (OSA)
Signs of associated congenital pathology or previous surgery
INVESTIGATIONS
C-spine XR, PA & lateral (mobility, instability)
Echo (if congenital heart disease)
OPTIMIZATION
EMLA
ENT consult/ backup
Caution with Premedication with co-existing OSA
Aspiration prophylaxis
Antisialagogue
SBE Prophylaxis if CHD
ANESTHETIC OPTIONS
Maintenance of spontaneous ventilation is paramount in those patients with anticipated
difficult ventilation/ intubation – avoid muscle relaxants
Awake instrumentation of the AW is virtually impossible in the pediatric patient
Recognition of conflicts of difficult A/W plus: pediatric patient, full stomach, raised ICP,
CHD with shunt & pulmonary hypertension, malignant hyperthermia
ANESTHETIC SETUP
Experienced help
Difficult Airway Cart
LMAs oral and nasal A/W’s available to facilitate PPV
Selection of preferred blades (curved may be better with macroglossia)
Multiple ETT sizes
Surgeon/ Tracheostomy/ Percutaneous cricothyroidotomy equipment
ANESTHESIA MANAGEMENT
Inhalational – Sevo but recognized relatively quick offset
TIVA – Propofol/remi infusion +/- bolus
Consider preop lidocaine nebulization to facilitate FOB intubation under sedation, or to
facilitate placement of oral airway under light plane of anesthesia
BACKGROUND
Pediatric vs Adult Airway - 5 Major Anatomical Differences
Large Tongue
In proportion to rest of oral cavity, and so more easily obstructs airway
Position of Larynx:
Infant: (C3-4); adult (C4-5); premature baby C3
Epiglottis narrower/stiffer and angled away from the axis of trachea
Vocal Cords have lower attachment anteriorly
Whereas in adults they rest perpendicular to trachea; this difference can
sometimes lead to difficulty with intubation of infant
Subglottic area (cricoid cartilage) is narrowest part of airway
Whereas in an adult is the rima glottides
SPECIFIC SYNDROMES
Cleft lip & palate:
Most common congenital A/W abnormality (lip 1/1000 live
births, palate 1/2500)
Cardiac abnormalities in 20%
Possibly chronic aspiration
Associated with many other craniofacial disorders (over
150), commonly Pierre Robin syndrome (also Mohr, Shpritzen, 4P)
Technical specifics:
Isolated cleft lip usually doesn’t present AW difficulties
Obstruction by tongue is usually overcome with oral AW
Left-sided cleft palate may interfere with normal positioning of laryngoscope
blade
Craniofacial dysostosis:
Craniosynostosis (premature closure of cranial sutures)
Midface hypoplasia (normal-sized mandible with prognathia) +/-
hydrocephalus, hypertelorism, proptosis, cervical fusion, tracheal
rings
Associated with Apert, Crouzon (towering skull, beaked nose) and
Pfeiffer’s syndromes
May have obstructive apnea severe enough to require tracheostomy
May have inc ICP
May require massive transfusion if multiple sutures (large bore IV, blood products)
Technical specifics:
Mask ventilation may be difficult, as well as obstructed PPV due to high,
arched palate resulting in nasal obstruction & choanal stenosis
Intubation is not usually a problem unless there are associated C-spine
abnormalities which limit mobility, or the presence of tracheal rings (may
require smaller ETTs)
Klippel–Feil syndrome:
Cervical spine abnormalities including fusion, A-O instability,
spinal canal stenosis, and scoliosis
Associated with neurological, cardiovascular, genitourinary and
auditory abnormalities
Technical specifics:
BMV usually not difficult
Fiberoptic intubation may be necessary due to limited
mobility
Careful positioning due to possible c-spine instability
Beckwith-Wiedemann syndrome:
Gigantism highlighted by prominent macroglossia
Characterized by exophthalmos, hyperplasia of kidneys and
pancreas (leading to hyperinsulinemic hypoglycemia)
Severe obstruction may ultimately lead to cor pulmonale
Technical specifics:
BMV may be impossible
Direct laryngoscopy is usually possible with adequate displacement of
tongue (may require assistant)
Often present for glossectomy requiring nasal intubation
Vascular malformations:
Hemangiomas, AVM and cystic hygromas (venous lymphatic
malformations) can all complicate AW management. AVMs
may be associated with high-output cardiac failure and
consumptive thrombocytopenia (Kasabach-Merritt syndrome)
Technical specifics:
Anatomic distortion by vascular masses may make
BMV and direct visualization difficult
Trauma to lesion may lead to bleeding complications
May require prolonged AW protection, including possible tracheostomy,
during extended treatment (chemotherapy, sclerotherapy, radiation etc.)
REFERENCES
1. Nargozian, The Airway in Patients with Craniofacial Abnormalities, 2004 Pediatric Anesthesia
2. Infosino, Pediatric Upper Airway and Congenital Anomalies, 2002 Anes Clin of NA
3. Hall, The Difficult Pediatric Airway – Recognition, Evaluation and Management, 2001 CJA
4. Faust’s Anesthesiology Review, Ch 163.
Syndrome Airway Defect Multisystem Associations Anesthetic Significance
Cleft Lip±Palate Isolated or associated swallowing, speech, BMV-difficult because airway obstruction
(CL ± CP) with syndrome URTI & middle ear by tongue falling into cleft: reversible with
infections oral airway
pulmonary aspiration- DL usually OK in isolated CP - but
feed upright with cleft laryngoscope blade may “hang-up” on
nipple cleft
20% associateded with Cleft Palate surgery: oral RAE secured
CHD midline with gag (beware ETT
Associated with Pierre displacement)
Robin, Treacher Collins, High risk of airway obstruction postop
Klippel Fiel, Down (edema, blood, residual anesth drugs);
Syndrome tongue stitch often placed to help relieve
obstruction
Leave intubated if in doubt
Alice says Oxford blade specifically blade
made for clefty’s
Mucopolysaccharidoses- genetic storage disease leading to accumulation of mucopolysaccharides throughout the body; 8
clinical types have been described of varying degrees of severity
Hurler Macroglossia; narrowing widespread deposition of potential difficult BMV & DL
(TypeI) –more laryngeal inlet & mucopolysaccharides Postop subglottic edema
severe form Tracheobronchial tree → CAD, valvular disease, Potential for lower airway obstruction
progressive AW CMO & arrhythmia, because tracheobronchial tree
obstruction; sudden death abnormalities
Joint stiffness with ↓ OSA common Periop resp failure
neck mobility Restrictive Lung Disease Unstable C-spine
Odontoid hypoplasia and & recurrent pulm Severe multisystem disease esp cardiac
Atlantoaxial subluxation infections
Worsen with age Bone Marrow Transplant
M. Barker wants to has been effective
emphasize awareness treatments; may actually
that Hurler’s gets worse reverse the airway
with age abnormalities
death before age 10
Beckwith- Macroglossia-regresses genetic “overgrowth Chronic AWO and usual sequelae of pulm
Wiedemann with age, may require syndrome” HTN & cor pulmonale
(~common) partial glossectomy characteristized by BMV Difficult (oral AW or LMA)
exomphalos, DL usu OK
macroglossia, and May need to stay intubated post
gigantism glossectomy b/c of sig swelling
associateded with viscero- Avoid hypoglycemia
megaly (↑ liver, pancreas,
kidney,spleen),
abdominal wall defects
(omphalocele) & neonatal
hypoglycemia
CHD alice adds
Mandibular Micrognathia Sequelae of May have severe airway obstruction
hypoplasia Cleft Palate chronic AWO - especially when supine
Pierre Robin Glossoptosis pulm HTN, cor most severe as infant, decreases as mandible
(tongue posteriorly pulmonale grows
displaced) Pulm aspiration treat AWO with oral/nasal airways & prone
position but if very severe may require
glossolabiopexy (tongue sutured to lower lip
to keep anterior)
BMV Difficult b/c AWO – inhalational
induction in lateral position or use LMA
DL Difficult; may get easier as mandible
grows
Mandibulofacial Malar & mandibular Sequelae of BMV Difficult b/c severe AWO
dysostosis hypoplasia ±TMJ chronic AWO - DL V. Difficult – sedated FOI or LMA ± FOI
Treacher Collins small mouth, CP & pulm HTN & cor AW Sx (eg palatoplasty) ↑ risk postop AW
velopharyngeal (VP) pulmonale obstrⁿ
incompetence Pulm aspiration Trach often indicated for intra- & post-op
AW may worsen with ±CHD AW mgmt & resp care
age with ↑basilar nETT contraindicated post repair VP
kyphosis of cranial base insufficiency – disrupt flap
Hemifacial microsomia varying degrees of Normal IQ BMV Difficult - poor mask fit; AWO
Goldenhar’s (oculo- mandibular hypoplasia Hydrocephalus DL highly variable (?degree of mandibular
auriculo-vertebral macrostomia eye/ear abN’s on deformity, mouth opening-previous Sx’s on
syndrome) fused/hemivertebrae → affected side jaw/TMJ, neck mobility)
limited neck mobility ±CHD C-spine immobility
may present bilaterally Potential ↑ ICP
(confuse with Pierre-
robin)
Craniofacial Midface hypoplasia, Craniosynostosis Chronic AWO±OSA; may need long term
dysostosis narrow, hi-arch palate ± ±hydrocephalus Trach
Apert Syndrome CP, choanal stenosis ± CHD BMV Difficult- poor mask fit; ~ obstructed
Crouzon Syndrome Mandible usu Normal ± hydronephrosis, nares so need patent oral AW during inductⁿ;
(no assoc multisyst ±↓C-spine mobility PCKD use LMA
effects) abN tracheal rings → ± esophageal DL usu OK unless C-sp prob’s, prepare
smaller ETTs atresia smaller ETTs, nasal ETT not contraindicated
(craniosynostosis Prone to post intubation croup
& ↑ ICP only for Assoc’ed cardiac, renal, ↑ ICP
Crouzon)
Freeman-Sheldon Microstomia Rare genetic myopathic BMV OK
/Whistling Face/ short webbed neck with dysplasia of facial, limb ± Difficult DL
cranio-carpo-tarsal ltd mobility respiratory muscles Assoc with malignant
dysplasia pot’l resp difficulties hyperthermia – no inhalational
induction. Trigger free anesthetic
Klippel-Feil multiple skeletal abN’s spinal stenosis, scoliosis BMV usu OK
syndrome incl fused C-spine, ±CVS (usu VSD) Difficult DL b/c of neck rigidity &
abN±unstable GU (may have renal instability
craniocervical junction insufficiency) FOI ± LMA has been successful
Associated with cleft palate
Trisomy 21 (most Small nasopharynx OSA BMV & DL may be challenging;
common chromosomal Lg tongue; CL±CP CHD (endocardial cushion smaller ETTs available
abN) Subglottic stenosis defects, TOF, PDA) C-spine instability
atlantoaxial subluxation Tendency to obesity Consider postopICU esp post AW
Sx
Cleft Lip & Palate
Occurs in 1/750 births
May be isolated or as part of broader syndrome (Pierre
Robin/ Velocardiofacial) with 20% associated with CHD
Airway management is usually straightforward though
potential for tongue or laryngoscope blade to fall into cleft
and impair ventilation or view of larynx
Down’s Syndrome
Reviewed elsewhere (macroglossia, high arched palate,
subglottic stenosis, A/O instability)
Mucopolysaccharidosis
Infiltration of mucopolysaccharides results in
macroglossia associated with cardiomyopathy &
accelerated CAD, aortic insufficiency
Tonsillar hypertrophy
Odontoid hypoplasia and Atlantoaxial Subluxation
Achondroplasia
Potential for Hydrocephalus
Odontoid hypoplasia with atlantoaxial instability
CHARGE/VA(C)TER(L)
Coloboma; Heart disease (TOF, PDA..) choanal Atresia,
Retarded growth, Genital anomalies (hypogonadism) and
Ear anomalies