Neurofibromatosis Type 1 Related Hydrocephalus

Jonathan Roth*, Shlomi Constantini

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review


The prevalence of hydrocephalus among patients with neurofibromatosis type I (NF1) is estimated to be between 1 and 13%. Aqueductal webs, chiasmatic-hypothalamic tumors, and thalamic mass effect related to NF changes are the common causes of NF1-related hydrocephalus. Brain tumors and moyamoya syndrome may mimic the clinical presentation of hydrocephalus in children with NF1, and should be ruled out while evaluating children with headaches. Treatment of NF1-related hydrocephalus should be personally tailored, including shunts, endoscopic procedures such as septostomy and third ventriculostomy, and tumor resection or debulking. Despite these personalized treatments, many of the primary treatments (including shunts and endoscopic procedures) fail, and patients should be screened and followed accordingly. In the current manuscript, we review the causes of NF1-related hydrocephalus, as well as treatment options.

Original languageEnglish
Pages (from-to)S368-S371
JournalNeurology India
Issue number8
StatePublished - 1 Nov 2021


  • Endoscopic third ventriculostomy
  • and tumor resections. Patients should be screened for recurrence of the hydrocephalic process.
  • endoscopic third ventriculostomies
  • hydrocephalus
  • neurofibromatosis 1
  • optic pathway glioma
  • shunt


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