Pheochromocytoma is a rare cause of severe secondary hypertension in children, and its perioperative management can be particularly challenging due to catecholamine-mediated hemodynamic instability. We report a case of a nine-year-old male with glucose-6-phosphate dehydrogenase (G6PD) deficiency who presented with blurred vision and papilledema and was found to have a systolic blood pressure of 190 mmHg, consistent with a hypertensive emergency. Imaging revealed a 5.2 × 3.5 × 5.5 cm vascular left adrenal mass with central necrosis, and biochemical testing showed markedly elevated urinary vanillylmandelic acid and normetanephrines, confirming the diagnosis of adrenal pheochromocytoma. Echocardiography demonstrated mild to moderate concentric left ventricular hypertrophy. Preoperative optimization included titrated alpha-adrenergic blockade with prazosin, sequential beta-blockade, and intravascular volume expansion. The anesthetic approach combined general anesthesia with a neuraxial enhanced technique, using intrathecal dexmedetomidine and morphine for sympatholysis, along with magnesium sulfate (50 mg·kg⁻¹) as a catecholamine-suppressing adjunct. Intraoperatively, only brief episodes of hypertension were observed, and these were effectively controlled with small boluses of labetalol and esmolol, without the need for continuous vasoactive infusions. Hemodynamics stabilized promptly after adrenal vein ligation. The postoperative course was uneventful. This report suggests that neuraxial enhanced multimodal sympatholytic strategies may facilitate stable perioperative hemodynamic control in pediatric pheochromocytoma.
Muawad et al. (Wed,) studied this question.