An unusual association of von Hippel–Lindau disease and posterior nutcracker syndrome: Management of neurological and urological aspects in a young male

Abstract

Von Hippel–Lindau disease is a complex and systemic entity that can be discovered by neurological complications. Its association with other serious malformative disease like Nutcracker syndrome was not described, and its complex presentation must not make some other serious problem easily missed or neglected. Case Report: We present the case of 27 year-old male patient with 3 intramedullary hemangioblastoma of 6 months of insidious evolution complicated by medullary compression. The lesions were completely removed with excellent neurological postoperatory outcome. Thinking of von Hippel-Lindau (VHL) disease, our investigations were expanded and revealed multiple vermian and cerebellar hemangioblastomas, renal masses, and multiple pancreatic cysts. In addition, an incidentally found of posterior NCS during the abdominal computed tomography (CT) done for VHL disease investigations. Our attitude to both neurosurgical and urological problems (the serious presentation of VHL disease with multiple locations of hemangioblastoma and right renal carcinomas) and for the missed symptomatic posterior Nutcracker syndrome were discussed. Conclusion: The complex presentation of the VHL disease must not makesome other serious problems easily missed or neglected like renal carcinoma or Nutcracker syndrome as seen in our case