Perioperative management of hypertensive emergency in a patient with new onset of pheochromocytoma

Abstract

Pheochromocytoma is a rare neuroendocrine tumor in pediatrics. It usually appears in the context of a genetic syndrome and is a potentially curable cause of secondary arterial hypertension. Perioperative management of blood pressure (BP), as well as other clinical aspects, is a challenge for the health team.

Objective: To emphasize therapeutic particularities specific to Intensive Care and perioperative care of patients with pheochromocytoma.

Clinical Case: An 8-year-old schoolboy with a one-year history of headache associated with precordalgia and hyperhidrosis. He was hospitalized for hypertensive emergency secondary to a confirmed diagnosis of right pheochromocytoma. Six weeks later, a successful laparoscopic adrenalectomy was performed after alpha adrenergic blockade with doxazosin associated with propranolol and calcium antagonists. Von Hippel-Lindau disease (type 2C) was confirmed, heterozygous variant c.482G>A in the VHL gene. The patient had an uneventful evolution until the time of follow-up, one year after surgery.

Conclusion: BP control is crucial prior to surgery, with a1 adrenergic blockers and calcium channel antagonists being the fundamental therapeutic measures. b-blockers are reserved for the management of secondary tachycardia. Intraoperatively, it is essential to be prepared for variations in heart rate and BP both during anesthetic induction and tumor manipulation.