Authors: Muawad R et al.
Cureus 18(2): e104224 10.7759/cureus.104224
Summary
This case report describes a nine-year-old boy with glucose-6-phosphate dehydrogenase (G6PD) deficiency who presented in hypertensive emergency (BP 190/130 mmHg) with blurred vision and papilledema. Imaging revealed a large (5.2 × 3.5 × 5.5 cm) hypervascular left adrenal mass with central necrosis, and markedly elevated urinary vanillylmandelic acid and normetanephrines confirmed pheochromocytoma. Echocardiography demonstrated mild-to-moderate concentric left ventricular hypertrophy.
Preoperative optimization followed established principles: alpha-adrenergic blockade with prazosin (titrated to 4.5 mg TID), sequential beta blockade (initial labetalol, transitioned to atenolol), and intravascular volume expansion (1.5× maintenance fluids preoperatively). Targets were systolic BP <130 mmHg and HR <100 bpm prior to surgery.
The anesthetic strategy combined:
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General anesthesia (propofol, fentanyl, rocuronium)
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Magnesium sulfate loading (50 mg/kg)
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A neuraxial-enhanced technique with intrathecal dexmedetomidine (0.2 mcg/kg) plus preservative-free morphine (5 mcg/kg)
The goal was controlled sympatholysis during tumor manipulation without relying on a dense local anesthetic sympathetic block. Intraoperatively, only brief hypertensive episodes occurred and were controlled with small boluses of labetalol and esmolol; no continuous vasoactive infusions were required. Hemodynamics stabilized promptly after adrenal vein ligation. The postoperative course was uneventful.
The authors argue that combining optimized alpha-beta blockade, magnesium-mediated catecholamine suppression, and neuraxial alpha-2–mediated sympatholysis may provide smoother hemodynamic control in pediatric pheochromocytoma, particularly in high-risk presentations with target-organ involvement.
Key Points
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Pediatric pheochromocytoma remains a high-risk anesthetic scenario due to catecholamine surges during induction and tumor manipulation, and vasoplegia risk after adrenal vein ligation.
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Preoperative alpha blockade followed by beta blockade, plus volume expansion, remains foundational.
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Magnesium sulfate functions as a catecholamine-suppressing adjunct and may blunt intraoperative surges.
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Intrathecal dexmedetomidine (off-label in pediatrics) was used to provide targeted central sympatholysis without dense local anesthetic sympathectomy, theoretically reducing severe post-resection hypotension.
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This is a single case; conclusions are hypothesis-generating and should be limited to experienced centers with invasive monitoring.
What You Should Know
For pediatric pheochromocytoma—rare but potentially catastrophic—this report adds to the evolving discussion around multimodal sympatholytic strategies beyond standard IV agents. From a practical standpoint, the case reinforces:
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Meticulous preoperative preparation remains the most critical determinant of stability.
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Magnesium is a valuable adjunct in high-catecholamine states.
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Neuraxial techniques without local anesthetic (i.e., intrathecal alpha-2 plus opioid) may theoretically blunt sympathetic outflow while preserving enough vascular tone to avoid profound post-resection vasoplegia—but evidence is limited to case-level data.
Given your ongoing interest in hemodynamic control strategies and catecholamine physiology, this one is conceptually interesting, but it does not change standards of care—yet.
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