Describe the clinical features associated with a difficult airway, including those of syndromes and congenital abnormalities such as Pierre Robin, mucopolysaccaridoses and Treacher Collins

Difficult airway

the clinical situation in which a conventionally trained anaesthetist experiences difficulty with facemask ventilation, tracheal intubation or both. Difficult laryngoscopy ~1.35% of paediatric procedures

Most children with difficult aiways are ID preop as most commonly have face malformations

Pierre Robin and Treacher Collins

Klipperl-Feil

Beckwith-wiedeman

Pierre Robin and Treacher Collins

• Short mandible

• Micrognathia

• Ear derformities

Klipperl-Feil

• Cervical fusion → limited neck extension

Beckwith-wiedeman

• Limited MO

• Restricted TMJ

• Large tongue

Sx and Si of airway obstruction

Hx

• Apnoeic episodes

• Daytime tiredness

• Irritability

• Hyperactivity

Ex

• Noisy breathing

• Stridor

• Snoring

• WOB

• Side on assess MO

• Dentition (overbite)

Syndromes assoc with difficult airway

o Pierre Robin sequence

o Mucopolysaccaridoses

o Treacher Collins

o Down’s syndrome

These sydromes airway improves with age:

• Pierre robin – micrognathia (jaw size increases)

• Goldenhar – asymmetrical micrognathia (jaw size increases)

These syndromes worsen with age

o Treacher Collins (micrognathia, small mouth, funnel shaped larynx)

o Apert (midface anomalies, cervical fusion)
o Hunter and Hurler (mucopolysaccharides in tongue and larynx)
o Beckwith-Wiedemann (macroglossia)
o Freeman-Sheldon syndrome (circumoral fibrosis and microstomia)

o Fibrodysplasia ossificans

Pierre Robin

Clinical triad

Intubation technique

Mucopolysaccaridosis (e.g. Hunter’s or Hurler’s syndrome)

In what way is airway mx difficult

Treacher-Collins Syndrome

Goldenhar syndrome

Apert Syndrome

Beckwith-Wiedemann Syndrome

Omphalocoele - organs grow outside the body

Trisomy 21

Implications for Airway

Most common 1:600-800

Airway

• Short neck

• Macroglossia

• Microdontia

• Mid-facial and mandibular hypoplasia (underdeveloped)

• Tonsillar hypertrophy

• Atlanto-axial instability

• Vertebral ligamentous abnormalities

• Higher incidence of congenital sib-glottic or tracheal stenosis

OSA too