MI3 The Saga

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MI-3?The Saga

• Down Syndrome and Anesthetic Implications

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Could you provide back up for the CT scan??

• 2½ year old female with Down syndrome
• unstable gait (changed from before)
• needs CT scan

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3 days later Scheduled for C1-C2 posterior
spinal fusion

• 2½ year old female with Down syndrome
• C1-C2 subluxation
• s/p ASD and VSD closure
• chronic G-tube feeds
• NKDA, wt10kg, Hct34
• last 3 days pt had been in ICU in head/torso
  harness and sedated

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Operative Course

• Inhaled induction w/ sevo and N2O
• EZ mask ventilation -gt roc for intubation
• laryngoscopy w/ in-line stabilization, MAC 2
  blade, 4.0 ETT, EZ intubation
• prone position, skin to skin 3½ hours
• maintenance sevo/iso, N2O, fentanyl
• pt does not tolerate N2O very well

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Emergence

• pt in head/torso harness
• turned supine, trachea extubated
• good air movement, but desaturation on transfer
  to bed
• no improvement w/ PPV by mask
• reintubation, ET suction and lavage -gt copious
  amounts of secretions -gt O2 sat ?

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Postop Course

• fever, hypotension, septic picture
• treated for sepsis
• trachea extubated after 3-4 days
• transferred to other hospital
• readmitted for cervical wound breakdown
• wound care, abx

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1 mo p initial fusion Scheduled for anesthesia
for new mold for brace

• pt needs to be prone for plaster application to
  back for new mold (5 min)
• then anesthesia (supine) until new mold is ready,
  for this time pt will have no brace
• reapplication of old brace, wake up
• estimated total duration 20 min

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Down syndrome

• 1600 newborns, risk increases with age of
  mother, significant increase if gt35y
• 95 have 47 chromosomes with trisomy 21, 4 of
  sporadic cases have 46 chromosomes with an
  abnormal translocation
• most consistent characteristic is mental
  retardation with IQs between 20 and 80

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Atlanto-occipital instability

• well documented association w/ Down syndrome
• laxity of transverse atlantal ligament and high
  incidence of bony abnormalities
• x-ray findings not predictive
• case reports of postop symptomatic subluxation
• caution w/ airway management and positioning

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Respiratory System-Downs

• high incidence of preop respiratory tract
  infection (up to 23)
• anatomical features causing high incidence of
  sleep apnea and airway obstruction, also poss
  difficult airway
• higher incidence of subglottic stenosis
• more frequent atelectasis and postextubation
  stridor

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Cardiovascular System-Downs

• overall incidence of associated congenital
  cardiac defects about 40, endocardial cushion
  defects are most common
• most of the defects involve L-gtR shunt and
  therefore predispose to pulmonary hypertension
  which develops earlier than in pts w/o Down
  syndrome
• pts w/ Down syndrome have higher perioperative
  risk

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Heme-Onc Immunology

• relative immune deficiency, risk for all
  infections higher, especially respiratory
• higher incidence of HBS Ag
• polycythemia more common
• leukemia (ALL and AML) are about 20x more common

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Endocrine GI

• Down syndrome often associated with organ
  specific autoimmune dysfunction
• increased incidence of hypothyroidism
• associated w/ duodenal atresia
• also increased incidence of Hirschsprungs and
  GERD

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CNS

• mental retardation
• increased incidence of postop agitation
• epilepsy in up to 10
• strabismus more common
• abnormally low muscle tone
• increased sensitivity to atropine (mydriatic and
  ?cardiac response)