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Medical and endocrine disease · Core01 / 30

Undiagnosed phaeochromocytoma unmasked at laryngoscopy34-year-old woman, 62 kg, for elective right shoulder arthroscopy in the beach-chair position

Catecholamine crisis: recognising phaeochromocytoma, blockade before surgery and pressor storm

From memory: the diagnosis and the notes are hidden. Answer each question aloud, then press Show.

Know this first

  • Attacks of high BP with a racing heart and palpitations point to a phaeochromocytoma.
  • A non-selective beta-blocker before alpha-blockade leaves alpha effects unopposed and causes a hypertensive crisis.
  • Plasma-free metanephrines are the most sensitive test when suspicion is high.
  • After the tumour is out, hypotension is the commonest cause of early death and hypoglycaemia follows.

The patient

34-year-old woman, 62 kg, for elective right shoulder arthroscopy in the beach-chair position. Two years of attacks of headache, sweating and palpitations; her BP has been labile but never investigated. No regular drugs. Vitals: BP 158/96, HR 96, SpO2 99% on air, RR 14, glucose 162 mg/dL.

The examiner asks

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  1. Consider

    What concerns you before you start?

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    • A 34-year-old for an elective shoulder arthroscopy, with uninvestigated attacks of headache, sweats and palpitations. Patient
    • Labile hypertension with palpitations makes me suspect a phaeochromocytoma until it's excluded. Disease
    • Her raised glucose fits: excess catecholamine drives glycogenolysis and suppresses insulin release.
    • Unprepared, her induction, laryngoscopy or positioning could each trigger a catecholamine crisis. Stem trigger
    • This shoulder is elective, so I'd postpone and investigate her rather than proceed today. Surgery
  2. Crisis

    The list goes ahead. At laryngoscopy her BP is 240/130 and HR 135, with multifocal ventricular ectopics. What do you do?

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    • A catecholamine surge; with her history, an untreated phaeochromocytoma heads my list. Crisis
    • I call for help, put her on 100% oxygen and deepen the anaesthetic.
    • I rule out the others fast: light anaesthesia, hypoxia, hypercarbia, MH and thyroid storm.
    • Nitroprusside 5–25 mcg boluses, then 0.5–5 mcg/kg/min, or phentolamine 2–5 mg IV.
    • Magnesium 2–4 g IV as an adjunct; it blunts catecholamine release and is antiarrhythmic.
    • For her ventricular ectopy, lidocaine 1–1.5 mg/kg IV; no beta-blocker until alpha-blockade is on board.
    • Arterial line now, and the shoulder is abandoned. Surgery
  3. Crisis

    Ten minutes later her SpO2 is 86% on 100% oxygen, airway pressure has risen and pink froth fills the tube. BP 190/110, HR 125. What now?

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    • Acute pulmonary oedema, likely catecholamine cardiomyopathy and an afterload crisis in her. Crisis
    • Suction the tube, keep FiO2 1.0 and add PEEP; it offloads a failing left ventricle.
    • Bring her afterload down: nitroprusside or nitroglycerin infusion at 0.5–5 mcg/kg/min.
    • Bedside echo for her ventricle: dilated, hypertrophic or outflow-obstructed forms all occur.
    • She stays intubated and goes to ICU with arterial monitoring. After surgery
  4. Plan

    She settles in ICU. How do you take her forward?

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    • Plasma-free metanephrines to confirm, then CT or MRI to find the tumour. Disease
    • Alpha-blockade first, phenoxybenzamine or doxazosin, for 3 to 14 days or more.
    • A beta-blocker only after that, if her heart rate stays high.
    • Ready when her BP is normal, her attacks stop and ECG changes and arrhythmias resolve.
    • At adrenalectomy I fill her before vein ligation; post-resection hypotension is the main early killer. Surgery
    • Her glucose is checked 1–2 hourly over the first 6–24 hours: rebound hypoglycaemia is expected. After surgery

Fatal mistakes

  • Giving a beta-blocker first for her tachycardia, before any alpha-blockade.
  • Carrying on with an elective shoulder in the middle of a catecholamine crisis.
  • Missing a phaeochromocytoma in a patient with classic attacks and labile BP.

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

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