MON-532 - A Storm after Retrieval: Pheochromocytoma Crisis Provoked by Oocyte Retrieval in A Patient with Neurofibromatosis Type 1

A 33-year-old G1P1 woman with neurofibromatosis type 1 (NF1), migraines, anxiety, and infertility presented with severe nausea, vomiting, and epigastric pain five hours after an uncomplicated transvaginal oocyte retrieval. In the emergency department, she was hypertensive (BP 167/98 mmHg) with frequent premature ventricular contractions. Labs showed leukocytosis (WBC 25.7 × 10³/µL) and metabolic acidosis (pH 7.229, HCO₃ 14 mmol/L). Computed tomography revealed a 5.2-cm heterogeneous right adrenal mass.
Her condition rapidly deteriorated with flash pulmonary edema and acute hypoxemic respiratory failure. After receiving intravenous labetalol for rate control, she developed pulseless electrical activity arrest requiring intubation and resuscitation. Bedside echocardiography displayed severe global hypokinesis with apical hypercontractility and a left ventricular ejection fraction of 10–15%. She required support with VA-ECMO (venoarterial extracorporeal membrane oxygenation) and an Impella device. In the setting of hypertensive crisis and high clinical suspicion for pheochromocytoma crisis, nitroprusside infusion was initiated followed by alpha-blockade with phentolamine initially and carefully titrated doxazosin (up to 5 mg twice daily). Metoprolol was later introduced for management of tachycardia after adequate alpha-blockage. Biochemical evaluation confirmed markedly elevated catecholamine levels with 24-hour urinary metanephrines of 21,458 µg/24 hr (n: 36-209 µg/24 hr) and 24-hour urinary normetanephrines of 12,149 µg/24 hr (n: 131-612 µg/24 hr).
After hemodynamic stabilization, she was extubated and underwent right adrenalectomy 12 days post-presentation with intraoperative esmolol infusion for rate control. Her postoperative course was uncomplicated, with repeat echocardiography showing cardiac function recovery. Surgical pathology confirmed pheochromocytoma.

Discussion:
This case highlights oocyte retrieval as a novel trigger for pheochromocytoma crisis. While routine screening is not recommended in asymptomatic NF1, this patient's history of anxiety and migraines may have indicated an increased pre-test probability. This case underscores the critical importance of early recognition and the dangers of beta-blockade before adequate alpha-blockade in suspected pheochromocytoma. Successful management required deferring adrenalectomy until complete hemodynamic stability and optimized alpha-blockade, emphasizing the necessity of a coordinated multidisciplinary approach to minimize perioperative risk and optimize outcomes in these high-risk patients.

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