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Medical and endocrine disease04 / 30

Down syndrome, type 1 diabetes and consent for cataract surgery26-year-old man, 78 kg, with Down syndrome and mild intellectual disability

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Know this first

  • If he can decide, the consent is his; only when he can't does a guardian decide, and only in his interest.
  • Atlantoaxial instability: 10–30% have it without symptoms; about 1% have symptoms.
  • Mask ventilation and intubation are both likely to be hard; his tube may need to be smaller than predicted.
  • Under anaesthesia, hypoglycaemic signs are easily blamed on something else; treat with dextrose 25 g IV.

The patient

26-year-old man, 78 kg, with Down syndrome and mild intellectual disability, for elective right cataract extraction under GA. Type 1 diabetes on glargine and meal-time aspart; loud snoring with witnessed pauses; VSD closed in childhood, recent echo normal. Lives with his mother, who helps with his insulin; he tells you he wants to see better. Vitals: BP 118/72, HR 70, SpO2 97% on air, RR 14, glucose 142 mg/dL.

The examiner asks

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  1. Consider

    What are your considerations?

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    • A 26-year-old with Down syndrome, type 1 diabetes and likely sleep apnoea, for elective cataract under GA. Patient
    • He's an adult: if he has capacity for this decision, the consent is his, not his mother's.
    • I assess his capacity for this specific decision, using simple words, time and his mother's help.
    • If he lacks capacity, his legal guardian consents in his interest, with a psychiatric report documented.
    • Atlantoaxial instability is more common in Down syndrome, so his neck stays neutral. Airway
    • Big tongue, snoring and possible subglottic narrowing: a hard mask, a hard intubation and a smaller tube.
  2. Prepare

    How do you prepare him?

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    • I ask about neck pain, arm weakness or a change in walking; any of these needs neurosurgical advice. Airway
    • I confirm his closed VSD has left no shunt, heart failure or pulmonary hypertension. Disease
    • He goes first on the morning list to shorten his fast.
    • Basal glargine continues; his morning aspart is held.
    • Glucose on arrival, then every 1–2 hours while he's on insulin and fasting.
  3. Plan

    How do you anaesthetise him?

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    • Calm induction with his mother beside him; propofol titrated within 1–2.5 mg/kg. Patient
    • No neck extension at any point; if I must intubate, in-line stabilisation and a fibreoptic or video view. Airway
    • A smaller tube than his size predicts is ready.
    • Minimal opioid: with his sleep apnoea, a high opioid need means monitored recovery. After surgery
    • Glucose checked during the case and before he leaves recovery. Disease
  4. Crisis

    Two minutes after the LMA is removed in recovery, he snores, then his chest moves with no air flow. SpO2 84%. What do you do?

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    • Upper airway obstruction: his tongue and sleep apnoea, or laryngospasm. Crisis
    • Help called; 100% oxygen by facemask, jaw thrust and CPAP at 5–15 cmH2O.
    • Jaw thrust without head extension, to protect his upper cervical spine. Airway
    • If CPAP fails: an oral or nasal airway, or the LMA back in. Crisis
    • Laryngospasm not breaking with CPAP: suxamethonium 0.1–1 mg/kg IV, then reintubate.
    • If that fails, I reintubate him, neck held in line, using a smaller tube. Airway
    • Afterwards, continuous oximetry overnight. After surgery
  5. Crisis

    Forty minutes later he is drowsy and sweaty, HR 104, SpO2 96%. His glucose reads 41 mg/dL. How do you manage this?

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    • Hypoglycaemia: glucose under 55 mg/dL with sweating, a fast pulse and drowsiness. Crisis
    • Dextrose 25 g IV, 50 mL of 50%, now.
    • If his cannula is lost, glucagon 1 mg IM.
    • Recheck shortly, then dextrose running until he eats and drinks.
    • These signs pass for emergence or pain after anaesthesia, which is why glucose is measured, not guessed.
    • He stays overnight in a monitored bed, with the diabetes team adjusting his insulin. After surgery

Fatal mistakes

  • Taking his mother's signature as consent for a capable adult.
  • Extending his neck for laryngoscopy without asking about cord symptoms.
  • Giving insulin with no glucose monitoring around a fast.

Checked against: Stoelting 8e · Miller 10e · Saudi Guidelines for Informed Consent, First Edition 1440H (2019) · Barash 9e

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