Genetic Syndromes: General Approach
Considerations
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Potentially difficult airway
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Many patients with genetic syndromes abnormal airway findings
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Special attention is needed for syndromes with mandibular hypoplasia (e.g. Pierre Robin, Treacher Collins, Goldenhar) as well as patients with cleft lip/palate
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Careful airway physical exam & review of previous anesthetics is warranted
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Congenital cardiac defects
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Syndromes associated with cardiac defects include: VACTERL, CHARGE, trisomy 13, 18, and 21, and velocardiofacial syndromes
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Imperative to conduct thorough physical exam, review Echocardiogram, & understand cardiac anatomy/pathophysiology
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Neuro-Cognitive abnormalities
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Potentially anxious & uncooperative patient
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Possible cognitive deficits & increased sensitivity to effects of anesthetics
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Positioning & vascular access issues
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In presence of limb abnormalities, positioning & vascular access may be difficult
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Orthopedic abnormalities
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Scoliosis, hip dysplasia, & limb contractures are common in this patient population
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