Abstract: Objective To compare the experience of perioperative management and anesthesia in VHL syndrome and non-VHL patients undergoing pheochromocytoma resection. Methods 50 patients scheduled for surgical removal of pheochromocytoma in PUMC Hospital from 2009-01-01 to 2014-12-31 were included in this retrospective analysis. Among them，12 patients were diagnosed with VHL syndrome，others were non-VLH patients. We focused on the clinical records, especially clinical manifestation, preoperative preparation, intraoperative anesthetic management, operation duration and postoperative hospital stay. Results Comparing with non-VHL patients, VHL syndrome patients undergoing pheochromocytoma resection surgery were much younger, with multiple pheochromocytoma and a significantly increased norepinephrine release. The drug preparation period was much longer, as well as the operative time and hospital stay (P<0.05). But no statistical difference existed in the intraoperative hemodynamic fluctuation and the outcomes of the patients. Conclusions VHL syndrome patients mainly present with multiple pheochromocytoma which has more aggressive function. Since the long operation duration and high risk, the optimization of perioperative management and adequate drug preparation are the key factors to ensure the operation safety.

Key words: VHL syndrome, pheochromocytoma, operative management, anesthesia management