Abstract
Von Hippel-Lindau (VHL) is a rare autosomal dominant disease with high penetration. Hemangioblastomas of the central nervous system are located in the cerebellum, brainstem and spinal cord.
Their clinical significance is mainly determined by the location and dynamics of growth. Multiple renal cysts are found in more than 2/3 of patients.
Unfortunately, they have a strong tendency to develop kidney cancer, which, along with cerebral hemangioblastomas, is a major cause of mortality in VHL. Repeated operations in the area of the posterior cranial pit require careful closure of the dura mater to prevent postoperative complications.
If it is difficult to obtain autologous diaper replacement in the wound area during repeated operations, it is possible to use an artificial dura or to take a graft from another location. In the care of patients with VHL, a common complication is associated with renal impairment with concomitant dialysis.