A 56-year-old woman from home alone presented to the emergency department with vomiting and chest tightness. On arrival to hospital, she was found to have significant biochemical derangement with pH <6.8, potassium 8.6 mmol/L, lactate 12 mmol/L, glucose 42 mmol/L, ketones 4.8 mmol/L and serum osmolality 356 mmol/L. ECG demonstrated ventricular tachycardia and bedside echocardiogram showed severe left ventricular dysfunction. Systolic blood pressure was initially >200 mmHg and she required intubation for acute pulmonary oedema and renal replacement therapy for acute kidney injury. Following intubation, her blood pressure dropped significantly requiring inotropic support, however she had periods of significant blood pressure spikes with systolic blood pressure intermittently >200 mmHg treated with glyceryl trinitrate and hydralazine, and required sedation with dexmedetomidine. During an attempted tracheostomy and bronchoscopy under sedation, her systolic blood pressure ranged from 60 to 190 mmHg and the procedure had to be aborted.
CT chest/abdomen/pelvis performed in the setting of fever and undifferentiated cause for multi-organ failure demonstrated a 3.5cm heterogeneously enhancing left adrenal mass, which was inconsistent with a simple adenoma on MRI. Further investigations demonstrated plasma normetanephrine 15506 pmol/L (<900), metanephrine 6126 pmol/L (<500), and 3-methoxytyramine 1007 pmol/L (<110). She was diagnosed with a phaeochromocytoma, however management was complex due to intermittent hypotension even prior to commencement of alpha-blockade. She was commenced on low-dose prazosin, which was changed to phenoxybenzamine once her clinical status had stabilised, and later commenced on beta-blockade with metoprolol. Following adequate alpha-blockade, she underwent elective surgical excision of her phaeochromocytoma without complication.
Phaeochromocytoma crisis is a rare presentation of phaeochromocytoma, characterised by haemodynamic instability (including either transient or sustained hypotension) and end-organ dysfunction. Management is complex due to difficulties with alpha-blockade if hypotension is a feature. Surgical timing and the role of emergency adrenalectomy is controversial.