SAT-494 - Pheochromocytoma Crisis Following Elective Lithotripsy: Presentation with Cardiogenic Shock and Multiorgan Failure

Introduction: Pheochromocytoma (pheo) crisis (PC) is an uncommon but life-threatening complication, occurring in 7–18% of pheo cases and is associated with a mortality of approximately 13.8%. The most severe presentation, pheo multisystem crisis (PMC), is characterized by shock and multiorgan failure.

Case presentation: A 57-year-old woman, who was recently diagnosed with left adrenal mass and biochemical evidence of pheo, presented to ED with nausea, vomiting, tachycardia, & dyspnea. The day before, she underwent an elective lithotripsy under general anesthesia, but for unclear reasons, she did not receive the required prep for pheo. On presentation, she was febrile, tachycardic, tachypneic, and hypoxic. She then developed respiratory failure requiring intubation, hypertensive crisis and subsequent cardiogenic shock. Labs showed leukocytosis, acute kidney failure, transaminitis, lactic acidosis and elevated troponin. Cardiac catheterization was negative. CT confirmed the adrenal mass and revealed diffuse pulmonary edema. Echo revealed heart failure with a reduced EF of 28%.
A month prior, an MRI revealed a 6.6 × 5.4 cm non–lipid-rich left adrenal lesion. Labs showed markedly elevated 24-hr urinary metanephrines (13,270 µg/24 h), normetanephrines (9,444 µg/24 h), and total metanephrines (22,714 µg/24 h).
Her presentation was consistent with PMC, likely triggered by perioperative anesthetic medications including dexamethasone, midazolam, propofol and fentanyl. Her condition improved after appropriate treatment including initial phentolamine during hypertensive phase, followed by pressors during shock. She was ultimately extubated and was weaned off pressors and discharged on doxazosin. A follow-up echo showed improved EF. She later underwent a scheduled left adrenalectomy, confirming an 8.6 cm pheochromocytoma. She remains clinically stable after that.

Discussion: PMC is diagnosed in patients with PC presenting with multiorgan failure, severe hypertension and/or hypotension, fever and encephalopathy. Nausea and vomiting, both present in our case, are associated with high mortality. Without prior alpha-blockade, certain factors can elicit PMC, including surgical manipulation of the tumor, anesthesia, some medications, and/or stressors such as infection, trauma, or pain. Glucocorticoids (dexamethasone ≥2 mg) have also been implicated. In our case, anesthetic exposure and perioperative glucocorticoids were the most likely trigger.
It is critical that patients with confirmed or suspected pheo be pretreated with alpha-blockers prior to any surgical procedure or exposure to general anesthesia. Management of PMC requires ICU-level care with alpha-blockade and advanced organ support. Definitive treatment is adrenalectomy following adequate prep with alpha-blockade, with emergent surgery reserved for refractory shock or progressive organ failure.

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