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Hypertension in Pheochromocytoma and Paraganglioma: Evaluation and Management in Pediatric Patients
Meredith L Seamon1, Ikuyo Yamaguchi2
1Division of Pediatric Nephrology, Department of Pediatrics, University of Utah, 81 N. Mario Capecchi Drive, Salt Lake City, UT, 84113, USA. meredith.seamon@hsc.utah.edu.
Insights
Managing blood pressure in pediatric patients with pheochromocytomas and paragangliomas (PPGL) is challenging. Selective alpha-blockers and calcium channel blockers are increasingly used for perioperative care due to better safety and availability.
Area of Science:
- Pediatric Endocrinology
- Cardiovascular Pharmacology
- Oncology
Background:
- Pheochromocytomas and paragangliomas (PPGL) are rare tumors causing secondary hypertension in children.
- Perioperative blood pressure management in pediatric patients with PPGL is a significant clinical challenge.
Purpose of the Study:
- To review antihypertensive treatment options for pediatric patients with PPGL.
- To focus on recent literature regarding perioperative blood pressure management in this population.
Main Methods:
- Literature review focusing on studies from the past several years.
- Analysis of case series and single-center experiences for treatment guidelines.
Main Results:
- Phenoxybenzamine, selective α1-blockers (e.g., doxazosin), and calcium channel blockers have been used successfully.
- Current pediatric practice and international guidelines support preoperative α-adrenergic blockade.
- Selective α1-blockers and calcium channel blockers are gaining favor over phenoxybenzamine due to cost, availability, and side effect profiles.
Conclusions:
- Selective α1-blockers and calcium channel blockers represent viable and often preferred alternatives to phenoxybenzamine for perioperative management of pediatric PPGL.
- Continued adherence to preoperative α-adrenergic blockade is recommended for optimal patient outcomes in pediatric PPGL management.
Purpose Of Review:
The rare catecholamine-secreting tumors, pheochromocytomas and paragangliomas (PPGL), account for a minority of cases of secondary hypertension in pediatrics. As such, perioperative blood pressure (BP) management in pediatric patients presents a distinct challenge. This review will expand the practitioner's knowledge of antihypertensive treatment options for the pediatric patient with PPGL with a focus on literature in the past several years.
Recent Findings:
There continue to be only small case series and single-center experiences to provide guidelines regarding BP management. While phenoxybenzamine has been more routinely used, selective α1-blockers, such as doxazosin, as well as calcium channel blockers, have also been utilized with success in pediatric patients. While the concept of obligatory α-adrenergic blockade for adult patients has been recently challenged, international guidelines and current practice patterns among pediatric clinicians continue to support preoperative α-adrenergic blockade to ensure the best possible patient outcomes. Selective α1-blockers and calcium channel blockers are becoming more commonly used given the high cost, limited availability, and undesirable side effect profile of phenoxybenzamine.
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