Anesthesia in a patient with a rare disease: Von Hippel-Lindau syndrome

Von Hippel-Lindau disease is a rare autosomal dominant disorder characterized by capillary hemangioblastoma of the central nervous system or retina. Renal and pancreatic cysts as well as pheochromocytoma may accompany this disease. Tumors may be either cancerous or noncancerous and the signs and the symptoms of the disease can occur throughout life. Non-specific findings and wide age range makes differential diagnosis a complicated issue. The diagnosis of von Hippel-Lindau disease depends on the combination of clinical, radiological, pathological and genetic data. The anesthetic concern during operation for this disease is a huge challenge, usually requiring detailed preoperative evaluation. Here, we presented the surgical resection of a spinal hemangioblastoma in a patient with von Hippel-Lindau disease and discussed the significance of this syndrome.