Aqueductal Stenosis in Neurofibromatosis

Neurofibromatosis. 1988;1(5-6):312-7.


Macrocrania (i.e., macrocephaly) is a common manifestation of von Recklinghausen neurofibromatosis (NF-1), occurring in approximately 30% of patients with this disorder. Megalencephaly is the usual basis for NF-1-associated macrocrania, although obstructive hydrocephalus secondary to a brain tumor or cranial vault dysplasia may occur. We describe 2 patients with NF-1 and aqueductal stenosis documented by CT scan. The incidence of aqueductal stenosis among our patients with NF-1 is 1%.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Brain Diseases / complications*
  • Brain Diseases / diagnostic imaging
  • Cerebral Aqueduct / diagnostic imaging*
  • Child, Preschool
  • Humans
  • Male
  • Neurofibromatosis 1 / complications*
  • Tomography, X-Ray Computed