Anesthetic management of endovascular embolization in a patient of neurofibromatosis with associated aortic and multisystemic vasculopathy: A case report

Patients with neurofibromatosis undergo multiple surgeries during their lifetime, presenting as challenging scenarios to the perioperative team. We present the case report of a young male patient with type 1 neurofibromatosis scheduled for endovascular embolization of thoracic spinal arteriovenous malformation under general anesthesia. Associated vasculopathy included stented descending thoracic aortic coarctation, an unrepaired saccular aortic aneurysm (AA) distal to the stent suggestive of a type 1 endoleak, bilateral segmental pulmonary arterial thrombosis, bilateral subclavian artery stenosis, and a retroperitoneal neurofibroma compressing the coeliac artery. Our primary concerns were a possible periprocedural AA rupture becoming a surgical emergency, difficult vascular access, and perioperative thromboembolism. Periprocedural hemodynamic surges were avoided using continuous hemodynamic monitoring, adequate anesthetic depth, and analgesia. After an uneventful procedure, the patient was extubated with a smooth postoperative course. An in-depth knowledge about underlying pathophysiology, anticipation and preparedness for potential complications, and interdisciplinary coordinated teamwork serve to preserve patient outcomes.