Abstract
Pheochromocytoma, (PCC) is a sympathetic paraganglioma of chromaffin cell origin. Individuals with PCC, present with signs and symptoms of sympathetic overstimulation. In this article, we report a case of a 10-year-old male presenting with hypertensive encephalopathy precipitated by bilateral adrenal PCC. A 10 year old, male patient who presented to Emergency Department with history of headache, projectile vomiting, disturbed level of consciousness and two attacks of convulsion for one day. PCC are catecholamine secreting tumors. In the case we are describing, adrenoceptor overstimulation manifested as uncontrolled hypertension which progressed to hypertensive encephalopathy. Only 10% of PCC's cases were found to be bilateral. Though bilateral PCC is more often a part of a familial syndrome, in this case, we report bilateral PCC with no evidence of familial disorders that correlates with this presentation. Preoperative diagnosis is usually made by significantly high levels of catecholamines and their metabolites in blood and urine. In recent studies, using (131-I-MIBG, metaiodobenzylguanidine scan), in combination with platelet normetanephrin, showed 100% sensitivity in detecting PCC. Definitive treatment of PCC is surgical resection of the mass which is considered to be curative in 90% of the cases. Following Cortex sparing Bilateral Adrenalectomy, the patient showed complete resolution of hypertension. PCC should be considered as a possible diagnosis in children presenting with malignant hypertension. Stabilize blood pressure is important part in the preoperative period. Adrenal sparing surgery showed promising outcomes in treating cases of bilateral PCC. (C) 2014 The Authors. Published by Elsevier Inc. All rights reserved.