Genetic Syndromes: General Approach

Considerations

 

  • Potentially difficult airway​

    • Many patients with genetic syndromes abnormal airway findings 

    • Special attention is needed for syndromes with mandibular hypoplasia (e.g. Pierre Robin, Treacher Collins, Goldenhar) as well as patients with cleft lip/palate 

    • Careful airway physical exam & review of previous anesthetics is warranted 

  • Congenital cardiac defects​

    • Syndromes associated with cardiac defects include:  VACTERL, CHARGE, trisomy 13, 18, and 21, and velocardiofacial syndromes

    • Imperative to conduct thorough physical exam, review Echocardiogram, & understand cardiac anatomy/pathophysiology 

  • Neuro-Cognitive abnormalities​

    • Potentially anxious & uncooperative patient 

    • Possible cognitive deficits & increased sensitivity to effects of anesthetics

  • Positioning & vascular access issues​

    • In presence of limb abnormalities, positioning & vascular access may be difficult 

  • Orthopedic abnormalities ​

    • Scoliosis, hip dysplasia, & limb contractures are common in this patient population