The Expected and the Unexpected Difficult Airway

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The Expected and the Unexpected Difficult Airway

• Lei Wang, MD

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Case 1

• J.N is a 12 yo boy S/F dental restoration
• PMH Congenital-Hunters syndrome
• 1. Partial airway obstruction occ. sleep apnea.
• 2. History of heart murmur with normal cardiac
  function
• 3. Extremity weakness partial contracture
  unable to sit up.
• 4. Severe developmentally delayed.

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Hunters Syndrome
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Case 1 (cont)

• PSH MRI in 1997 under GA with fiber optic
  intubation. Pt was intubated for three days
  postop for upper airway edema.
• Allergies NKDA
• Meds Benadryl PRN

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Physical Exam

• BP 95/56 P 74 RR 20 SpO2 99 28kg
• Airway macroglossia with protruding tongue.
  Patient has copious drooling.
• Neck short neck. Unable to exam ROM.
• Pulm coarse upper airway noises.
• Cardiac RRR
• Extremities partial contracture UE and LE
• Neuro developmental delay.

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Planned Anesthetic Management

• A. Peripheral IV placed preop.
• B. Anesthetized nasal fiberoptic intubation with
  spontaneous ventilation.

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Anesthetic Management-cont.

• A. Peripheral IV placed preop.
• B. Anesthetized nasal fiberoptic intubation with
  spontaneous ventilation.
• C. DL reviewed grade IV view.
• D. Patient stable during procedure.
• E. Patient extubated awake after placing a tube
  changer in the trachea.

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Anesthetic Management-cont.

• F. Patient was able to maintain patent upper
  airway.
• G. Patient discharged to home on postop day1.

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Hunters syndrome

• AKA mucopolysaccharidosis II
• Etiology X-linked hereditary disorder of
  lysosomal enzyme, iduronate sulfatase, resulting
  accumulation of incompletely metabolized
  mucopolysaccharide, dermatan sulfate and heparan
  sulfate, in tissues through out the body.

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Hunters syndrome

• Incidence 1/30,000 for mucopolysaccharidosis in
  U.S.
• Natural history gradual decline in growth rate
  from 2 to 6 years of age.
• Deafness at 2 to 3 years of age.
• Death common prior to 15 years due to cardiac and
  pulmonary failure.

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Hunters syndrome

• Growth deficiency onset 2 to 6 years of age.
• Neurologic mental and neurologic deterioration
  at 2 to 5 years partial contracture joints occ.
  Hydrocephalus and seizure disorder.
• Craniofacial Macrocephaly coarsening of facial
  features.

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Hunters syndrome

• Airway macroglossia small mouth opening
  relative micrognathia airway obstruction
  possible altanto-axial subluxation.
• Cardiovascular possible cardiomyopathy valvular
  disease coronary occlusion, and CHF (older
  adolescent patients)

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Hunters syndrome

• Anesthetic considerations
• 20 perioperative mortality
• Difficult airway 25 difficult intubation
• Cardiac instability
• Difficult IV placement
• Difficult padding and positioning

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Case 2

• J.L. is a 4 year-old boy S/F palatoplasty
• PMH Trisomy 22
• Highly arched palate
• Polycystic kidneys
• Left ear malformation
• Developmental delay, but catching up
• Denies cardiac or pulmonary disease

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Case 2, cont.

• PSH none
• Allergies NKDA
• Meds none
• Labs Hct 39.6 K 4.1 BUN 8 Cr 0.4

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Case 2 Physical Exam

• Vitals BP 102/61 P 88 RR 25 SpO2 97 Ht
  99cm Wt 13 kg
• Airway not evaluated
• HEENT microcephaly mild micrognathia high
  arched palate left ear malformation
• Neck FROM
• CV RRR
• Lungs CTA
• Neuro normal mental development

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Anesthetic Management

• Inhalation induction- smooth
• IV placed Vecuronium 0.1 mg/kg administered
• Easy mask ventilation

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Anesthetic Management

• Inhalation induction- smooth
• IV placed Vecuronium 0.1 mg/kg administered
• Easy mask ventilation
• DLx6 with different blades grade IV view

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Anesthetic Management

• Inhalation induction- smooth
• IV placed Vecuronium 0.1 mg/kg administered
• Easy mask ventilation
• DLx6 with different blades grade IV view
• Fiberoptic via oral airway unsuccessful due to
  excessive soft tissue and bleeding

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Anesthetic Management

• Inhalation induction- smooth
• IV placed Vecuronium 0.1 mg/kg administered
• Easy mask ventilation
• DLx6 with different blades grade IV view
• Fiberoptic via oral airway unsuccessful due to
  excessive soft tissue and bleeding
• Light wand unsuccessful

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Anesthetic Management

• LMA 2 placed. Trachea intubated under fiberoptic
  guidance using LMA as a conduit
• Patient stable during surgery
• Patient extubated awake at the end of case
• Patient D/C home on postop day 2

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Trisomy 22

• A very rare congenital chromosomal disorder. Most
  patient die a few days after birth
• Anomalies includes growth retardation,
  psychomotor delay, microcephaly, micrognathia,
  broad nasal bridge, low-set malformed ears,
  highly arched palate, hypertelorism, congenital
  heart disease.

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ASA difficult airway algorithm
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ASA difficult airway algorithm
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ASA difficult airway algorithm