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Ovid: Oxford Handbook of Acute Medicine

Editors: Ramrakha, Punit S.; Moore, Kevin P. Title: Oxford Handbook of Acute Medicine, 2nd Edition Copyright ©1997,2004 Oxford University Press (Copyright 1997, 2004 by Punit S Ramrakha and Kevin P Moore) > Table of Contents > Chapter 9 – Endocrine emergencies > Phaeochromocytomas: management Phaeochromocytomas: management Patients are usually volume depleted at presentation, and should be rehydrated prior to initiation of α-blockade, otherwise severe hypotension may occur. β-blockade alone may precipitate a hypertensive crisis, and must never be given prior to adequate α-blockade. Labetalol is predominantly a β-blocker and should not be used alone. Long acting α-blockers prevent escape episodes.

  • Adequate fluid replacement with CVP monitoring.
  • Acute hypertensive crises should be controlled with phentolamine (2–5mg iv bolus, repeated as necessary every 15–30 minutes). Alternatively start an infusion of nitroprusside (0.5–1.5µg/kg/min, typical dose 100µg/min, see P164).
  • Preparation for surgery
    • Initiate oral α-blockade: phenoxybenzamine 10mg daily increasing gradually to 40mg tds. Monitor BP closely. Tumour β-stimulation may produce excessive vasodilatation and hypotension requiring inotropic support. Recent studies have shown that prazosin or doxazosin are equally effective and are being used increasingly
    • When the blood pressure is controlled with phenoxybenzamine, add propranolol 10–20mg tds
    • Invasive monitoring [pulmonary artery (Swan–Ganz) catheter and arterial line] is mandatory.
  • Hypotension commonly occurs intra-operatively when the tumour is removed, and this should be managed with blood, plasma expanders, and inotropes as required. Inotropes should only be used when the patient is appropriately fluid replete. Expansion of intravascular volume 12 hours before surgery significantly reduces the frequency and severity of post-operative hypotension. Angiotensin II should be available as an alternative inotrope for cases of resistant hypotension.

Autosomal dominant conditions with a high risk of developing phaeochromocytoma include

  • Von-Recklinghausen disease [neurofibromata, café au lait spots, Lisch nodules (iris hamartomas), and axillary freckling].
  • Von-Hippel Lindau disease (cerebellar haemangioblastomas, retinal haemangiomas, and other neoplasms including hypernephroma).
  • Multiple endocrine neoplasia types 2a (hyperparathyroidism and medullary thyroid carcinoma) and 2b (medullary thyroid carcinoma, bowel ganglioneuromatosis, and hypertrophied corneal nerves).

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