- 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)
- 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)
Brief List of Drugs to Prepare Besides Usual GA Setup
|Nitroprusside||50 mg in 250 mL = 200 mcg/mL||0.5–10 mcg/kg/min|
|Nitroglycerin||50 mg in 250 mL = 200 mcg/mL||0.5–10 mcg/kg/min|
|Nicardipine||25 mg in 250 mL = 100mcg/mL|
- Start at 5mg/h
- Increase as needed by 2.5 mg/h increments up to 15 mg/h
|Esmolol||2.5 g in 250 mL = 10 mcg/mL||5–200 mcg/kg/min|
|Phenylephrine||20 mg in 250 mL = 80 mcg/mL||0.2–1 mcg/kg/min|
|Norepinephrine||4 mg in 250 mL = 16 mcg/mL||0.2–20 mcg/min|
- Pre-induction arterial line
- Central venous ...
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