Down Syndrome 

 

Considerations

 

  • Potential for atlanto-axial (AAI) or atlanto-occipital instability & neurologic injury with neck manipulation

  • Potential for difficult bag mask ventilation (but not usually intubation):

    • Large tongue,↑ oropharyngeal soft tissue, small mouth, subglottic stenosis, high arched palate

    • Tonsillar & adenoid hypertrophy

  • Possible obstructive sleep apnea:  

    • Pulmonary hypertension/RV dysfunction 

    • Sensitive to sedatives/hypnotics 

  • Developmental delay/cooperation problems 

  • Associated congenital heart disease (up to ½):

    • Cushion defect (AVSD) = #1

    • VSD, ASD, TOF

    • Pulmonary hypertension 

  • Other medical issues:

    • Obesity

    • Accelerated coronary disease & valvulopathy (MVP, AI)

    • Hypothyroidism

    • GI: duodenal atresia or stenosis, TEF, Hirschsprungs, celiac disease

    • Heme: polycythemia, leukemia, immune deficiency

    • Joint laxity (careful with positioning)

    • Early Alzheimer’s dementia

 

 

Goals

  • Rule out & manage AAI: neurologic exam/history & flexion-extension views of c-spine

  • Airway adjuncts such as a video laryngoscope

  • Skin topicalization for IV start 

  • Sedation (midazolam may be paradoxical)

  • Arrange post-op monitoring

  • Overnight oximetry useful

 

 

Conflicts

  • Unstable C-spine vs uncooperative patient with developmental delay:

    • Consider IM/PO sedation to facilitate IV placement

      • Ketamine 5 mg/kg IM, 7 mg/kg PO

      • Midazolam 0.5 mg/kg PO (max dose 20 mg)

    • Inhalational induction may be problematic in an adult with DS due to obesity, OSA, uncooperative nature

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