Neurosurgical aspects of Noonan syndrome

Purpose Noonan syndrome (NS) is a rare neurodevelopmental syndrome characterized by dysmorphic features, congenital heart defects, neurodevelopmental delay, and bleeding diathesis. Though rare, several neurosurgical manifestations have been associated with NS, such as Chiari malformation (CM-I), syr...

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Bibliographic Details
Published in:Child's nervous system 2023-04, Vol.39 (4), p.849-856
Main Authors: Saragosti, Eldad, Fattal-Valevski, Aviva, Levin, Dror, Hausman-Kedem, Moran, Constantini, Shlomi, Mecica, Noa, Zarour, Shiri, Roth, Jonathan
Format: Article
Language:English
Subjects:
Adolescent
Arnold-Chiari Malformation - complications
Arnold-Chiari Malformation - surgery
Blood Coagulation Disorders
Case-based Review
Child
Disease Susceptibility - complications
Humans
Medicine
Medicine & Public Health
Neurosciences
Neurosurgery
Noonan Syndrome - complications
Noonan Syndrome - surgery
Retrospective Studies
Syringomyelia - surgery
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Summary:Purpose Noonan syndrome (NS) is a rare neurodevelopmental syndrome characterized by dysmorphic features, congenital heart defects, neurodevelopmental delay, and bleeding diathesis. Though rare, several neurosurgical manifestations have been associated with NS, such as Chiari malformation (CM-I), syringomyelia, brain tumors, moyamoya, and craniosynostosis. We describe our experience in treating children with NS and various neurosurgical conditions, and review the current literature on neurosurgical aspects of NS. Methods Data were retrospectively collected from the medical records of children with NS who were operated at a tertiary pediatric neurosurgery department, between 2014 and 2021. Inclusion criteria were clinical or genetic diagnosis of NS, age 
ISSN:0256-7040
1433-0350
DOI:10.1007/s00381-023-05888-2