PEDIATRIC AIRWAY SYNDROMES

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

KEY ANESTHETIC ISSUES

1. Simple maneuvers may relieve airway obstruction – prone positioning, pulling tongue
forward (suture in tongue, oro/nasopharyngeal airway, LMA)
2. Pre-operative assessment of associated organ system involvement (CHD, renal)
3. Don’t forget option of intraop tracheostomy if postop airway management is going to be
very difficult
4. Awareness of the dynamic status of the airway as patient grows or the disease progresses
5. Specific Syndromes
a. Cleft lip and palate
i. associated with Pierre Robin, CHD
ii. L sided cleft may be a difficult laryngoscopy
b. Pierre-Robin syndrome
i. Micrognathia, cleft palate, glossoptosis
ii. CHD
c. Beckwith-Wiedemann syndrome
i. Macrosomia, macroglossia
ii. Hypoglycemia
iii. Associated with omphalocele
iv. Likely easy laryngoscopy but difficult BMV

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

 Pierre Robin syndrome:

 Micrognathia, glossoptosis (abnormal downward or back placement
of the tongue), respiratory obstruction +/- cleft palate and CHD
 Pulm HTN, cor pulmonale, or pulm edema secondary to chronic A/W
obstruction
 Technical specifics:
 May obstruct A/W when supine
 Oral, nasal A/W and prone positioning may facilitate ventilation
 Fiberoptic laryngoscopy often required
 Older patients may be easier to intubate as mandible grows

 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)

 Treacher Collins syndrome (mandibulofacial dysostosis):

 Maxillary, zygomatic and mandibular hypoplasia with high,
arched palate and small mouth
 May be associated with cleft palate and velopharyngeal
incompetence (leading to nasal speech) as well as CHD
 OSA (cor pulmonale) – post op HDU, consider trach
 Associated dysmorphisms include laterally sloping palpebral
fissures, notched lower eyelids, coloboma (split/gap in iris, eyelid,
lens etc.) of eye and hearing loss
 Older children may develop basilar cranial kyphosis
 Technical specifics:
 Often very difficult ventilation and intubation requiring fiberoptic intubation or
tracheostomy

 Hemifacial microsomia (includes Goldenhar syndrome):

 Uni or bilateral mandibular hypoplasia and soft-tissue loss
 May have auricular abnormalities and facial nerve weakness
 Bilateral may be mistaken for Pierre Robin
 Associated with Goldenhar syndrome (which includes normal IQ,
CHD (20%), macrostomia, bony spinal deformity, possible
hydrocephalus)
 Technical specifics:
 Severity of deformity reflects magnitude of A/W challenges
 Instrumentation of A/W may be more difficult after surgery to jaw or TMJ
due to soft tissue contractures
 Goldenhar may have limited neck mobility making BMV and direct
laryngoscopy very difficult

 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

 Down syndrome (trisomy 21):

 See Down syndrome discussion elsewhere
 Technical specifics:
 Atlantoaxial instability requires careful positioning
 Large tongue and narrow nasopharynx may lead to
post-operative obstruction, while small larynx and
cricoid predisposes to acquired subglottic stenosis

 Freeman-Sheldon (Whistling Face) syndrome (craniocarpotarsal dysplasia):

 Myopathic dysplasia leading to facial contractures with circumoral
fibrosis and microstomia
 Associated with camptodactyly (flexion deformity of the finger)
and talipes equinovarus (clubfoot)
 Technical specifics:
 MH RISK.
 Limited mobility may make direct laryngoscopy difficult

 Fibrodysplasia ossificans progressiva:

 Heterotopic ossification of head, neck, spine and shoulder girdle
 Technical specifics
 Limited mobility may make direct laryngoscopy difficult.

 Hurler syndrome (Mucopolysaccharidosis Type 1H)

 Inborn error of metabolism leading to infiltration and
thickening of various AW structures including the tongue,
vocal cords and nasal passages.
 Associated with cardiomyopathy, mental retardation,
gargoyle facies, dwarfism, pectus excavatum,
kyphoscoliosis, and hepatosplenomegaly
 Technical specifics:
 Thick secretions may obscure visualization
 Difficult BMV and intubation requiring possible rigid bronchoscopy or
tracheostomy.
 Patients may get more difficult to intubate with age.

 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.)

 Juvenile onset rheumatoid arthritis:

 Multi-system disease which involves destruction of joints
including TMJ, c-spine and crico-arytenoids
 Technical specifics:
 Mobility problems may complicate proper
positioning.
 Laryngeal involvement may restrict passage of ETT

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

Craniofacial Dysostosis (Apert/ Crouzon/ Pfeiffer)

These patients have craniosynostosis with a degree of
midface hypoplasia & a high arching palate with choanal
stenosis and preferential mouth breathing
Typically the mandible is of normal size
Often bag mask ventilation is difficult with improper fit
due to midface hypoplasia
Usually intubation is easy, however
Apert’s may have CHD, renal anomalies and increased
ICP

Mandibulofacial Dysostosis (Treacher Collins)

These children have maxillary, zygomatic and mandibular
hypoplasia
Other features include laterally sloping palpebral fissures,
coloboma, small mouth opening, and auricular atresia +/-
cleft palate
Difficult BMV and intubation
Tracheostomy is common in this group
Cardiac Defects occasionally present

Hemifacial Microsomia (Goldenhar’s)

Mandibular hypoplasia, Ear abnormalities, Macrostomia
and Vertebral Anomalies
This may be confused with Pierre Robin
Cardiac Defects possible

Klippel Feil Syndrome

Cervical and occipital fusion accompanied by spinal
stenosis and scoliosis
Cardiac and genitourinary anomalies may also occur
Although BMV is typically easy, direct laryngoscopy is
hampered by immobile cervical spine which may also
demonstrate instability
 Beckwith Weidemann
Associated with omphalocele: macgroglossia, giagantism,
hypoglycemia, polycythemia
Chronic airway obstruction may be severe, leading to cor
pulmonale
Suggested to have an assistant hold tongue while
performing DL

Down’s Syndrome
Reviewed elsewhere (macroglossia, high arched palate,
subglottic stenosis, A/O instability)

Whistling Face Syndrome (Freeman Sheldon)

Facial contractures, microstomia and microcephaly:
Reported to have MH RISK
Potential for increased ICP

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

Vertebral anomalies; Anal atresia, (Cardiac), TEF, Renal

anomalies, Limb anomalies
 DiGeorge/Velocardiofacial
Hypoplasia of thymus, parathyroid, micrognathia, cleft
palate, laryngeal web, cardiac defects

Turner’s Sydrome (Noonan’s)

45 X genotype: Short Neck; Micrognanthia; Webbed
neck; Kyphoscoliosis; Aortic Stenosis; Ao Coarct;
Accelerated CAD; Hypothyroidism; Diabetes; Liver
disease/coagulopathy (factor XI deficiency)
(Noonan’s resembles Turners, though has normal karyotype
and pulmonic stenosis and risk of HOCM)