Achondroplasia in an adult for prone lumbar decompression38-year-old man, 52 kg, 128 cm, with achondroplasia, for elective L3–L5 decompression in the prone position

The patient

The examiner asks

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1. Consider

   What are your considerations?

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   • A 38-year-old man with achondroplasia, for elective prone lumbar decompression. Patient
   • His foramen magnum may be narrow, so his neck is never hyperextended. Airway
   • Big tongue, flat nose and large jaw make his mask seal hard; a short neck makes intubation hard.
   • His tube is chosen by his weight, not his age, and is often smaller than expected.
   • Snoring and a small stiff chest suggest sleep apnoea and restrictive lung disease. Disease
   • Those raise his risk of pulmonary hypertension.

2. Prepare

   How will you assess him?

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   • I ask about neck pain, arm symptoms and pauses in breathing at night: cord or brainstem compression. Airway
   • Echo for pulmonary hypertension before this major spinal operation. Disease
   • Sleep study and lung function; his restrictive disease shapes my ventilation plan.
   • Venous access may be hard through thick soft tissue, so ultrasound is ready.
   • A cuff that fits his short arm, or an arterial line if it won't.
   • Neuraxial anaesthesia would be technically difficult, so GA is my plan. Surgery

3. Plan

   What is your plan for his airway and the case?

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   • Videolaryngoscope or fibreoptic intubation with his neck neutral. Airway
   • A range of tube sizes, including smaller ones, ready on the trolley.
   • Drug doses by his actual weight, 52 kg; he responds normally to anaesthetic drugs. Disease
   • Prone with head neutral, eyes and pressure points padded, chest and abdomen free. Surgery
   • Extubated awake, with difficult-airway kit at hand. After surgery

4. Crisis

   After induction and relaxant you can't get a seal; two-handed ventilation barely moves his chest. SpO2 92% and falling. What do you do?

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   • DAS difficult facemask ventilation: oxygenation comes first. Crisis
   • Call for help; two-person technique with the VE grip and an oral or nasal airway.
   • Full neuromuscular block confirmed; it makes mask ventilation easier.
   • Best first attempt: videolaryngoscope, neck neutral, smaller tube. Airway
   • If that fails: second-generation SAD to oxygenate him. Crisis
   • If SAD and a final mask attempt fail: can't intubate, can't oxygenate, front-of-neck access.

5. Crisis

   Surgery goes well. An hour after extubation he is drowsy, RR 8, SpO2 90% on 2 L, PaCO2 62 mmHg. What do you do?

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   • Hypoventilation with CO2 retention: opioid on top of restrictive lungs and sleep apnoea. Crisis
   • Stimulate him, sit him up and check for residual block with a nerve stimulator.
   • If opioid is the cause, naloxone 40 mcg at 2-minute intervals, to a 200 mcg total.
   • CPAP or NIV for his obstruction and CO2; reintubation if he tires.
   • ICU or HDU for monitoring, not an unmonitored ward. After surgery