Von Hippel Lindau (VHL) disease is a rare autosomal dominant disease with multi organ involvement. A 45yr old female, Known case of VHL with a diagnosis of renal cell carcinoma, pancreatic neuroendocrine tumour and spinal hemangioblastoma was scheduled for open partial nephrectomy. She underwent excision of cerebellar hemangioblastoma with ventriculoperitoneal shunt insertion 2 yrs back. During pre anesthetic check up, the functionality of ventriculoperitoneal shunt was confirmed radiologically and neuroendocrine tumor workup was done to rule out pheochromocytoma. Intraoperatively, the hemodynamic response was attenuated and care was taken during positioning and handling patients as it may have effects on ICP. Post operative pain was managed by continuous erector spinae plane infusion. Knowledge about the disease and associated lesions helped us to effectively manage perioperatively.
Krishnagopal et al. (2026) studied this question.