Perioperative Anaesthetic Management of Hereditary Angioedema in the Context of Adrenalectomy for Pheochromocytoma Surgery: About a Case

Abstract

Hereditary angioedema (HAE) is a rare genetic disease which consists of the appearance of sudden bursts of edema of the skin and mucous membranes. Stress is the main trigger for this crisis, which are usually self-limited but at the same time have a poor response to corticosteroids and antihistamines. Pheochromocytoma is an uncommon neuroendocrine tumor, affecting the chromaffin cells of the adrenal medulla and which typically course with a hyperproduction of catecholamines. Moreover, between 5-11% of them can be associated with Takotsubo (TKS) syndrome, a transient cardiomyopathy which is caused by the hipercatecholaminergic state associated to these tumors. We present the case of a patient with type II HAE scheduled for a laparoscopic adrenalectomy to remove a pheochromocytoma, which was diagnosed during the etiological screening for a second episode of TKS triggered by a corticoid infiltration. The necessity for an especially accurate perioperative management of this patient resides in the difficult hemodynamic management related to pheochromocytoma surgeries (which normally require the administration of hypotensors like short-acting vasodilators and beta blockers); in combination with the underlying TKS cardiomyopathy and with the hazard of the associated HAE, a potentially serious pathology that requires being prepared to treat a perioperative crisis.