Background: Paragnagliomas and pheochromocytomas are rare neuroendocrine tumors that originate from the paraganglionic cells of the autonomic nervous systems. These tumors may be extra or intra-adrenal in site. The use of antihypertensive medications is usually challenging in these patients. Long acting alpha and beta blockade can be quite useful preoperatively but challenging postoperatively. We present a case of familial paraganglioma that was successfully treated by esmolol and other antihypertensive medications without associated perioperative complications.
Case Report: A 13-year-old girl, known case of bronchial asthma, presented with classic symptoms and signs of Pheo/Paragnaglioma. She had impressive family history, and CT abdomen showed right-sided paravertebral mass, therefore, treated as familial paraganglioma. Prazocin was started but she continued to experience uncontrolled fluctuations of a high blood pressure (BP). She initially developed a serious reaction to atenolol, therefore switched to esmolol that successfully controlled her BP alongside prazocin and intermittent doses of hydralyzine. She then underwent laparoscopic surgery and the diagnosis was confirmed by histopathology and genetic study.
Discussion: Preoperative management using Alpha and beta blockade is crucial to prevent the intraoperative complications in Pheo/Paragnaglioma. Phenoxybenzamine, a long acting non-selective alpha blockade, has been widely used since 1950s. In addition, prazosin, a selective alpha 1 blockade, has been used in favor due to its short action, so it causes fewer side effects postoperatively. Beta blockade are generally used to suppress tachycardia, though they also help in control of BP, after alpha-blockers being started. There is no evidence to support the use of beta 1 blockade such as atenolol over the non- selective beta-blockers, which include propanol. Two previous reports suggested the use of esmolol in adults, yet, not in pediatrics as in our case. Esmolol showed a good effect as adjuvant therapy to alpha-blockers and its very short half-life of approximately 3 minutes helped to avoid post-operative complications due to sudden intravascular volume and pressure changes that usually requires meticulous care and possible need of using presser agents.
Conclusion: Esmolol is titrable, effective and can be weaned rapidly helping to avoid post-operative complications in pediatric Pheo/Paragnaglioma. Therefore, it can be a good alternative to propranolol and atenolol that are routinely used in these cases. Further study on its use is needed to confirm our observation.
19 - 21 Sep 2019
European Society for Paediatric Endocrinology