Management of blood pressure in children with pheochromocytoma and other catecholamine-secreting tumors (CSTs) is unique and challenging. The authors report a single-center experience using sequential a-adrenergic blockade (phenoxybenzamine), increased fluid intake, and b-blockade for presurgical management of 10 CSTs in children. In this retrospective review, mean duration for blood pressure control in preparation for surgery was 4.5AE2.6 weeks. Intraoperative hypertension was noted transiently (<2 hours) in eight patients (80%) and was treated with continuous infusion of short-acting antihypertensive agents. Two (20%) patients required vasopressor medication infusion to manage intraoperative hypotension. Only two (20%) patients developed postoperative hypotension and required vasopressor medication infusion for <24 hours. All antihypertensive medications were discontinued in the immediate (≤4 days) postoperative period in 80% of patients. In conclusion, a systematic and multidisciplinary approach utilizing adrenergic blockade is effective in treating children with CSTs. J Clin Hypertens (Greenwich). 2015;17:720-725. ª 2015 Wiley Periodicals, Inc.Pheochromocytoma (PH) is a catecholamine-secreting neuroendocrine tumor that arises from chromaffin cells in the medulla of the adrenal gland. A total of 5% to 10% originate from the ganglia of the sympathetic or parasympathetic nervous system (extra adrenal) and are known as paraganglioma (PG).1-4 Neuroblastoma (NB) is also a neuroendocrine tumor that originates from the adrenal gland or primitive neural crest elements in the sympathetic nervous system. 5Symptoms of these catecholamine-secreting tumors (CSTs) are secondary to increased catecholamines (dopamine, norepinephrine, epinephrine, and others) that are released into the circulation. Most of the PHs secrete norepinephrine, resulting in hypertension,
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