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Paediatric anaesthesia02 / 17

Down syndrome for strabismus surgery7-year-old girl with Down syndrome, 22 kg, for day-case bilateral strabismus correction

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Know this first

  • Atlantoaxial instability is silent in 10–30% of children with Down syndrome; keep neck movement minimal.
  • Narrow subglottis: a tube smaller than age predicts, with a leak below 25 cmH2O.
  • Marked bradycardia on gas induction affects up to half, even with a normal heart.
  • Oculocardiac reflex: trigeminal afferent, vagal efferent; stopping traction comes first.
  • Strabismus surgery is a high-risk procedure for vomiting in children.

The patient

7-year-old girl with Down syndrome, 22 kg, for day-case bilateral strabismus correction. VSD closed at age 1, no echo since. Loud snoring with pauses her mother has seen. Hypothyroid on levothyroxine. No neck pain, normal gait, no change in bladder control. Vitals: HR 92, BP 100/60, SpO2 97% on air, RR 20.

The examiner asks

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  1. Consider

    What are your considerations?

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    • A 7-year-old with Down syndrome, a closed VSD and probable sleep apnoea, for day-case squint surgery. Patient
    • As a child with learning disability, she needs her mother with her and an unhurried induction.
    • Her big tongue and low tone obstruct quickly at induction, and her subglottis is narrow. Airway
    • Atlantoaxial instability is silent in 10–30%; she has no symptoms, but her neck stays neutral.
    • Squint surgery triggers the oculocardiac reflex and frequent vomiting. Surgery
  2. Prepare

    How will you assess her?

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    • Any neck pain, head tilt or change in walking? She has none. Airway
    • No routine neck films without symptoms; minimal neck movement regardless.
    • Echo before surgery: any residual shunt, valve leak or pulmonary hypertension after her VSD closure. Disease
    • Thyroid tests checked, as Down syndrome brings thyroid disease and she's on replacement.
    • Witnessed pauses mean OSA: sparing opioids and an overnight monitored stay, not a day case. After surgery
  3. Plan

    What is your anaesthetic plan?

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    • Gas induction with her mother present, atropine ready, as sevoflurane can slow her heart. Airway
    • A tube smaller than her age predicts, with a leak confirmed below 25 cmH2O.
    • Her neck stays neutral through laryngoscopy and positioning.
    • Vomiting prophylaxis: dexamethasone 0.15 mg/kg plus ondansetron 50–100 mcg/kg. After surgery
    • Paracetamol 15 mg/kg IV, keeping opioids to a minimum.
  4. Crisis

    As the surgeon pulls on the medial rectus, HR falls from 95 to 40, BP 70/40. What now?

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    • PALS bradycardia, vagal cause: the oculocardiac reflex; the surgeon lets go of the muscle now. (Crisis)
    • Check her depth and ventilation; hypoxia, high CO2 and light anaesthesia make it worse.
    • Her rate usually recovers within about 20 seconds of release.
    • Severe or recurring: atropine 10–20 mcg/kg IV, or glycopyrrolate 10 mcg/kg.
    • Still under 60 and poorly perfused after release and atropine: CPR, adrenaline 0.01 mg/kg.
    • Surgery resumes once she recovers; the reflex usually weakens with repeated pulls.
  5. Crisis

    In recovery she has inspiratory stridor and a barking cough; SpO2 92% on air. What do you do?

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    • Post-intubation croup is likely; Down syndrome is a known risk factor. Crisis
    • Calm her on her mother's lap, sitting up, with humidified oxygen; crying makes it worse.
    • Nebulised racemic adrenaline, 0.5 mL of 2.25% in 3–5 mL saline.
    • Dexamethasone 0.4–1 mg/kg (maximum 20 mg) IV, though it takes 4–6 hours to peak.
    • Rebound is possible, so she's watched 4 hours past the final nebuliser, and stays overnight. After surgery

Fatal mistakes

  • Flexing or extending her neck at laryngoscopy.
  • Sizing her tube by the age formula.
  • Letting traction continue while her heart rate falls.
  • Sending her home the same day with stridor and untreated sleep apnoea.

Checked against: Stoelting 8e · Barash 9e · Miller 10e · Mayo review

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