Pheochromocytoma Crisis

Causes:

➧ The action of unopposed high circulating levels of catecholamines

- α - receptors: Pressor response

- β - receptors: positive ino- and chrono-topic

Precipitating factors:

➧ Spontaneous

➧ Hemorrhage into pheochromocytoma

➧ Exercise

➧ Pressure on the abdomen

➧ Urination

➧ Drugs: glucagon, naloxone, metoclopramide, ACTH, cytotoxics, tricyclic antidepressants

Clinical picture:

➧ History of poorly controlled Hypertension or accelerated Hypertension.

➧ Hypertension, palpitations, sweating, pallor, pounding headache, anxiety, tremulousness, pulmonary edema, feeling of impending death, hyperhidrosis, nausea and vomiting, abdominal pain, paralytic ileus, hyperglycemia, hypertensive encephalopathy, myocardial infarction, and stroke.

➧ Attacks build up over a few minutes and fade gradually over 15 min or can be more sustained (60 min).

➧ Signs of end-organ damage (Figure 1)

Figure 1: Hypertensive Retinopathy - Grade IV

Biochemical Diagnosis:

➧ 24 h. urine collection for free catecholamines and metanephrines.

Management of Pheochromocytoma Crisis:

-Do not wait for biochemical confirmation of the diagnosis.

-Be aware of postural hypotension.

-Avoid histamine-releasing drugs.

-Surgical resection treatment of choice.

α-adrenergic blockers:

-Treatment with a-antagonists should precede β-antagonist treatment with 48 h. to avoid exacerbation of the crisis.

➧ Phentolamine: (short-acting) 2-5 mg IV repeated/5 min.

➧ Phenoxybenzamine: 1st choice because it’s irreversible and long-acting

10 mg/12h., then 20-40 mg/8 h.

➧ Prazosin, Terazosin & Doxazosin: are selective α1-blockers. Preferable for long term Rx due to favorable S/E

β-blockers:

-Control tachycardia.

-After adequate α-adrenergic blockage to prevent unopposed hypertension.

➧ Non selective b- antagonist: Propranolol: 1-2 mg/5-10 min. 30-60 mg/d. oral.

➧ Esmolol: 500 µg/kg/min. for 1 min., then 100-300 µg/kg/min.

➧ Metoprolol

➧ Labetalol: α and β blocker.

Catecholamine synthesis inhibitors:

➧ Metyrosine

-Inhibit catecholamine synthesis.

-Used when alpha &beta blockers are ineffective or poorly tolerated.

-Combined for difficult resections.

Calcium channel blockers:

-Inhibit norepinephrine mediated calcium transport.

Acute hypertensive crisis (pre/intra-op):

➧ Nitroprusside

➧ Phentolamine

➧ Nicardipine

Surgery and Post-operative care:

➧ Experienced surgeon/anesthetist team.

➧ Last α & β doses on the day of surgery.

➧ Avoid fentanyl, ketamine, morphine, atropine, halothane, and desflurane during surgery.

➧ Treat hypotension post resection with fluids and intermittent doses of vasopressors.

➧ 24 h. urine metanephrine & catecholamine level 1-2 wks post-surgery and annually for life.

➧ Lifelong glucocorticoid and mineralocorticoid therapy for bilateral adrenalectomy.

➧ Radiotherapy, cryoablation, and combination chemotherapy should be considered for malignant pheochromocytoma.

Categories: Endocine Emergencies