Phaeochromocytoma Crisis: Two Cases of Undiagnosed Phaeochromocytoma Presenting after Elective Nonrelated Surgical Procedures

P C Johnston,K R Mullan,J A Silversides,A Hutchinson,P A Farling,S J Hunter,F Eatock,H Wallace

doi:10.1155/2013/514714

Abstract

Phaeochromocytoma is a catecholamine producing tumour and an uncommon cause of hypertension. We present two cases of relatively asymptomatic individuals, in which previously undiagnosed phaeochromocytoma was unmasked by elective nonadrenal surgical procedures, manifesting as postoperative hypertensive crisis and subsequent cardiogenic shock. The initial management in intensive care is discussed, in addition to the clinical and biochemical diagnostic challenges present. Successful adrenalectomy was performed in each case.

Highlights

Phaeochromocytomas are rare catecholamine secreting tumours arising from chromaffin cells of the adrenal medulla and extra adrenal paragangliomas [1]
We considered further therapeutic options: emergent adrenalectomy under cardiopulmonary bypass, mechanical circulatory support with extracorporeal membrane oxygenation (ECMO), or left ventricular assist device (LVAD)
In the setting of a hypertensive crisis, a tako-tsubo type, inverse tako-tsubo type, or an acute dilated cardiomyopathy can occur; systolic anterior motion with dynamic outflow obstruction or coronary artery spasm may lead to cardiogenic shock [6, 7]

Summary

Introduction

Phaeochromocytomas are rare catecholamine secreting tumours arising from chromaffin cells of the adrenal medulla and extra adrenal paragangliomas [1]. Secretion of catecholamines in phaeochromocytoma can be episodic or biochemically silent and can present difficulty in diagnosis; failure to diagnose phaeochromocytoma can have fatal consequences. Palpitations, sweats, and sustained or paroxysmal hypertension, as a result of elevated catecholamine production [2]; presentation can be varied. A “hypertensive crisis” as the initial manifestation of undiagnosed phaeochromocytoma is rare and can have a high mortality rate. Presentation in the perioperative setting in the context of a nonadrenal surgical procedure is infrequent, anaesthetists, intensivists, surgeons, and others who care for such patients must have an awareness of this condition in order to provide timely and appropriate management [3,4,5]

Case 1

Case 2

Discussion