ABSTRACT
Pheochromocytoma and paraganglioma are neuroendocrine tumors that occur less commonly among children compared to adults. The excess catecholamines secreted by the tumor cells result in hypertension, tachycardia, excess sweating, and headache. Other symptoms include abdominal pain or distension caused by the adrenal mass. Here, we report a case of pheochromocytoma arising from the left adrenal medulla in a 14-year-old boy, which was exclusively secreting norepinephrine, as suggested by elevated plasma and 24-hour urinary norepinephrine and its metabolite normetanephrine. The epinephrine and its metabolite metanephrine were within normal limits. He presented with abdominal pain, recurrent vomiting, and headache and was noted to have elevated blood pressure. He underwent adrenalectomy after controlling his blood pressure with an alpha-blocker Prazosin. His blood pressure remained stable after surgery, and his plasma-free metanephrines returned to normal limits. He tested negative for hereditary paraganglioma-pheochromocytoma gene panel.
ABSTRACT
OBJECTIVE: There are limited data on the outcomes of children receiving delayed (≥7 days) extracorporeal membrane oxygenation after cardiac surgery. The primary aim of this project is to identify the aetiology and outcomes of extracorporeal membrane oxygenation in children receiving delayed (≥7 days) extracorporeal membrane oxygenation after cardiac surgery. PATIENTS AND METHODS: We conducted a retrospective review of all children ≤18 years supported with delayed extracorporeal membrane oxygenation after cardiac surgery between the period January, 2001 and March, 2012 at the Arkansas Children's Hospital, United States of America, and Royal Children's Hospital, Australia. The data collected in our study included patient demographic information, diagnoses, extracorporeal membrane oxygenation indication, extracorporeal membrane oxygenation support details, medical and surgical history, laboratory, microbiological, and radiographic data, information on organ dysfunction, complications, and patient outcomes. The outcome variables evaluated in this report included: survival to hospital discharge and current survival with emphasis on neurological, renal, pulmonary, and other end-organ function. RESULTS: During the study period, 423 patients undergoing cardiac surgery were supported with extracorporeal membrane oxygenation at two institutions, with a survival of 232 patients (55%). Of these, 371 patients received extracorporeal membrane oxygenation <7 days after cardiac surgery, with a survival of 205 (55%) patients, and 52 patients received extracorporeal membrane oxygenation ≥7 days after cardiac surgery, with a survival of 27 (52%) patients. The median duration of extracorporeal membrane oxygenation run for the study cohort was 5 days (interquartile range: 3, 10). In all, 14 patients (25%) received extracorporeal membrane oxygenation during active cardiopulmonary resuscitation with chest compressions. There were 24 patients (44%) who received dialysis while being on extracorporeal membrane oxygenation. There were eight patients (15%) who had positive blood cultures and four patients (7%) who had positive urine cultures while being on extracorporeal membrane oxygenation. There were nine patients (16%) who had bleeding complications associated with extracorporeal membrane oxygenation runs. There were 10 patients (18%) who had cerebrovascular thromboembolic events associated with extracorporeal membrane oxygenation runs. Of these, 19 patients are still alive with significant comorbidities. CONCLUSIONS: This study demonstrates that mortality outcomes are comparable among children receiving extracorporeal membrane oxygenation ≥7 days and <7 days after cardiac surgery. The proportion of patients receiving extracorporeal membrane oxygenation ≥7 days is small and the aetiology diverse.
