TY - JOUR
T1 - Neurofibromatosis Type 1 Related Hydrocephalus
AU - Roth, Jonathan
AU - Constantini, Shlomi
N1 - Publisher Copyright:
© 2021 Wolters Kluwer Medknow Publications. All rights reserved.
PY - 2021/11/1
Y1 - 2021/11/1
N2 - 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.
AB - 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.
KW - Endoscopic third ventriculostomy
KW - and tumor resections. Patients should be screened for recurrence of the hydrocephalic process.
KW - endoscopic third ventriculostomies
KW - hydrocephalus
KW - neurofibromatosis 1
KW - optic pathway glioma
KW - shunt
UR - http://www.scopus.com/inward/record.url?scp=85122009209&partnerID=8YFLogxK
U2 - 10.4103/0028-3886.332254
DO - 10.4103/0028-3886.332254
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C2 - 35102991
AN - SCOPUS:85122009209
SN - 0028-3886
VL - 69
SP - S368-S371
JO - Neurology India
JF - Neurology India
IS - 8
ER -