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  • Tumors of the adrenal medulla that produce, secrete, and store catecholamines
  • Norepinephrine is predominantly secreted along with small amounts of epinephrine, and occasionally dopamine
  • Perioperative mortality has been reported to be as high as 45% from cardiovascular causes, and directly correlates with tumor size and degree of catecholamine secretion. With appropriate management, mortality is very low
  • Surgical exploration is curative in 95% of cases, with reduction in mortality to 3%
  • Rule of 10s: 10% are malignant, 10% are extra-adrenal, 10% are bilateral
  • Occasionally associated with syndromes: MEN IIA, MEN IIB, von Hippel-Lindau disease, or in rare cases, Von Recklinghausen’s disease, tuberous sclerosis, and Sturge-Weber syndrome
  • Typical presentation is a young adult in 30s to 50s with sustained hypertension (or occasionally paroxysmal), tachycardia, palpitations, tremor, sweating, flushing, hyperglycemia (secondary to α-stimulated inhibition of insulin secretion)
  • Cerebral vascular accidents and myocardial infarction are possible
  • In patients with chest pain and dyspnea, catecholamine-induced cardiomyopathy should be ruled out. The cardiomyopathy may be reversible if the catecholamine stimulation is removed early before fibrosis has occurred
  • Diagnosis: elevated plasma levels of free catecholamines and elevated urinary vanillylmandelic acid (VMA) levels along with CT findings
  • MIBG (methyl-iodo-benzyl-guanidine) scan may be needed to locate tumor(s)

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  • Evaluate for signs of end-organ damage and optimize medical treatment to minimize risk
  • Continue α-adrenergic blockade for at least 10 to 14 days before surgery
    • Both noncompetitive blockers (phenoxybenzamine) and selective α1 blockers (prazosin) have been shown to be equally effective in controlling blood pressure
  • Continue beta blocker therapy (usually used in patients with persistent tachycardia or dysrhythmias), but only with concurrent alpha blockade to avoid unopposed α-mediated vasoconstriction
  • Optimization of medical management indicated by the following:
    • No in-hospital blood pressure higher than 165/90 for 48 hours before surgery
    • Presence of orthostatic hypotension but blood pressure on standing no lower than 80/45 (and typically stuffy nose because of vasodilation)
    • ECG free of ST-T changes
    • No more than one premature ventricular contraction present every 5 minutes
  • Preoperative workup should include
    • TTE to evaluate LV function and relaxation
    • Labs: Na, K, and glucose
  • Normalization of intravascular volume and return of hematocrit toward normal is also recommended
  • Have in room
    • Phentolamine (Bolus: 2–5 mg, Infusion: 1–30 mcg/kg/min)
    • Lidocaine (Bolus: 100 mg, usual concentration 20 mg/mL)
    • Amiodarone (Give 150 mg slowly over 10 minutes, usual concentration 50 mg/mL)

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Brief List of Drugs to Prepare Besides Usual GA Setup
MedicationDilutionInfusion range
Nitroprusside50 mg in 250 mL = 200 mcg/mL0.5–10 mcg/kg/min
Nitroglycerin50 mg in 250 mL = 200 mcg/mL0.5–10 mcg/kg/min
Nicardipine25 mg in 250 mL = 100mcg/mL
  • Start at 5mg/h
  • Increase as needed by 2.5 mg/h increments up to 15 mg/h
Esmolol2.5 g in 250 mL = 10 mcg/mL5–200 mcg/kg/min
Phenylephrine20 mg in 250 mL = 80 mcg/mL0.2–1 mcg/kg/min
Norepinephrine4 mg in 250 mL = 16 mcg/mL0.2–20 mcg/min
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  • Pre-induction arterial line
  • Central venous ...

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