Hydrocephalus in Neurofibromatosis. Contribution of Magnetic Resonance Imaging to Its Diagnosis, Control and Treatment

Neurofibromatosis. 1989;2(4):218-26.


Cerebral ventricular dilation and/or frank hydrocephalus unrelated to brain tumors is not a rare feature of von Recklinghausen neurofibromatosis (NF-1). In our experience, it was observed in 23% of patients consulting for neurological problems. From 1984 to 1989, 30 patients with documented NF-1 were referred to for cranial or spinal magnetic resonance imaging (MRI). In 9 of these patients, clinical features or previous computed tomographic scanning revealed hydrocephalus or ventriculomegaly. One patient had biventricular hydrocephalus (enlarged lateral ventricles), 7 had triventricular hydrocephalus (aqueductal stenosis) and 1 had tetraventricular hydrocephalus (obstruction of the foramina of Magendie and Luschka). MRI showed anomalies in all 9 patients. The importance of MRI for the diagnosis, control and treatment of the hydrocephalus as well as for understanding the embryologic pathogenesis of these conditions is emphasized.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Cerebrospinal Fluid Shunts
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Hydrocephalus / diagnosis
  • Hydrocephalus / etiology*
  • Hydrocephalus / surgery
  • Magnetic Resonance Imaging*
  • Male
  • Neurofibromatosis 1 / complications*
  • Neurofibromatosis 1 / surgery