Anesthetic Management of a Patient with Von Hippel-Lindau Syndrome: A Case Report

Abstract

Von Hippel-Lindau (VHL) syndrome poses significant challenges in anesthetic management due to its diverse clinical manifestations, including vascular tumors and endocrine abnormalities. We present a case of a 25-year-old male with VHL syndrome undergoing pancreatic lesion and right adrenal mass resection. Preoperative optimization involves α and β blockers to manage potential catecholamine-induced hypertensive crises. Anesthesia induction comprised of fentanyl, propofol, and vecuronium, with lignocaine to blunt intubation response. Maintenance of anesthesia with oxygen-nitrous oxide-isoflurane with an activated epidural infusion. Intraoperative monitoring focused on detecting catecholamine surges, with vasodilators and vasopressors administration as required. Postoperative care emphasizes the monitoring of persistent hypertension and hemodynamic instability. The patient's perioperative course was uncomplicated, highlighting the importance of meticulous preoperative assessment and intraoperative vigilance. This case underscores the need for tailored anesthesia strategies and multidisciplinary collaboration to optimize outcomes in VHL syndrome surgical interventions, addressing the unique challenges posed by this complex condition.