Treatment of Hypertension in Children With Catecholamine‐Secreting Tumors: A Systematic Approach

Abstract

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,

Review of Pediatric Pheochromocytoma and Paraganglioma

“…Renal ultrasound as the first modality of choice will miss small adrenal tumors and PGLs due to sensitivity and specificity of 89% (61) and 61% (62), respectively. Hence, CT or MRI of the abdomen and pelvis have been the imaging modality of choice in numerous pediatric cases (4, 17, 19, 20, 36, 63) given similar diagnostic sensitivities (90–100%) (50, 64). Specificities of both are around 70–80% (50, 64).…”

“…Patients are asked to consume a high sodium diet of 6–10 g and fluid intake of at least 1.5 times maintenance a day once on an alpha blocker, to prevent hypotension from its vasodilatory properties (17, 36, 76). High fluid intake aids in expanding the contracted intravascular volume and reduces postoperative hypotension (77).…”

“…Patients are admitted to the hospital 24–36 h prior to surgery and given an alpha blocker, beta blocker, and in some cases a tyrosine hydroxylase inhibitor, the night before surgery (17, 36, 72, 78). Intravenous fluids of normal saline are given at 1.5 times maintenance to prevent hypotension prior to surgery (17, 36).…”

“…Intravenous fluids of normal saline are given at 1.5 times maintenance to prevent hypotension prior to surgery (17, 36). Table 5 provides a list of medication regimens used in pediatric catecholamine-secreting tumors, with further information delineated as follows.…”

“…Numerous small pediatric case studies relate their experiences with preoperative management (4, 17, 36). Romero et al delineated a stepwise approach to managing pediatric patients with neuroendocrine tumors (36).…”