Anesthetic management of a pediatric patient diagnosed with bilateral hereditary pheochromocytoma: A case report

@#Pheochromocytoma is a rare, catecholamine secreting tumor, accounting for 1 % of pediatric hypertensive patients 1. In children, pheochromocytoma is even more infrequent and are mostly bilateral 2. Perioperative management of bilateral pheochromocytoma requires thorough preparation to reduce the consequences of hypotension, hypertension, hypoglycemia, hyperkalemia, and hyponatremia. Mortality of up to 50 % has been reported in the surgical resection of this tumor 2-5. The challenges of the intraoperative team and how an anxious patient turned cooperative and pleasant shall be discussed.

Bilateral Pheochromocytoma: An anesthetic challenge.

Pheochromocytoma is a rare catecholamine secreting neuroendocrine tumor. It is bilateral in 10% of cases. A 24 year lady with bileteral Pheochromocytoma operated for resection under epidural with general anesthesia. Diagnosis was confirmed with 24 hour urinary VMA levels, USG, CT scan and MIBG scan. Tumor resection was incomplete on left side so patient underwent reexploration. Preoperative BP controlled with Phenoxybenzamine and Prazocine. Intra-operative BP fluctuations managed with sodium nitropruside and esmolol. Post operative hypotension was tackled with higher doses of Dopamine and Noradrenline due to down regulation of adrenergic receptors. Patient was discharged on oral steroids after 3 weeks.

A Case of Neurofibromatosis with Bilateral Pheochromocytoma / 대한내분비학회지

Pheochromocytoma is originated from chromaffin cell of sympathetic nervous system and associated with other disease, such as neurofibromatosis, duodenal carcinoid, medullary thyroid cancer and parathyroid adenoma. Especially, pheochromocytoma is developed more than 50% in neurofibromatosis associated with hypertension. In such cases, several clinical features documented as more frequent bilateral phochromocytoma, more associated with other neuroendocrine tumors and thus more poor prognosis. We can observe the sustained hypertension despite of surgical resection of tumors in pheochromocytoma cases. One of the possible reason of post operative sustained hypertension is the pheochromocytoma originated from minor organ of Zukerkandl that was not resected during operation. Untreated or delayed treated cases with pheochromocytoma were often expired by complication of hypertension such as cerebrovascular hemorrhage, myocardial infarction, etc. Thus, in neurofibromatosis with hypertension, screening of pheochromocytorna is very important for the early detection of tumor and more favorable prognosis. Recently, We experienced a case of neurofibromatosis associated with bilateral pheochromocy-toma expired by cerebral hemorrhage during operation, so we report the case with literature review.

Anesthetic Experiences for Resection of Bilateral Pheochromocytoma: Two cases / 대한마취과학회지

We had experienced anesthetic management of two patients with bilateral pheochromocytoma. They had been treated with phenoxybenzamine for 4 weeks preoperatively. Anesthesia was managed with thiopental sodium for induction,enflurane-N2O-O2 for maintenance, vecuronium for muscle relaxation, and sodium nitroprusside for controlling severe hypertension. After tumor resection, severe hypotension was controlled by rapid transfusion, fluid and dopamine infusion. A tolerable blood pressure and pulse rate were maintained throughout the procedure. Preoperative preparation, sufficient sedation, smooth anesthetic induction, complete analgesia, good muscle relaxation, adequate ventilation and proper cardiovascular control are required in resection of pheochromocytoma.