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Child's Nervous System
Erschienen in: Child's Nervous System 8/2020

25.05.2020 | Focus Session

Fetal brain damage in congenital hydrocephalus

verfasst von: Maria Florencia Varela, Marcos M. Miyabe, Marc Oria

Erschienen in: Child's Nervous System | Ausgabe 8/2020

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Abstract

Background

Congenital hydrocephalus (HCP) is a developmental brain disorder characterized by the abnormal accumulation of cerebrospinal fluid within the ventricles. It is caused by genetic and acquired factors that start during early embryogenesis with disruption of the neurogerminal areas. As might be expected, early-onset hydrocephalus alters the process of brain development leading to irreparable neurological deficit. A primary alteration of the ependyma/neural stem cells (affecting vesicle trafficking and abnormal cell junctions) leads to its loss or denudation and translocation of neural progenitor cells (NPCs) and neural stem cells (NSCs) into the cerebrospinal fluid (CSF). Under these abnormal conditions, morphological and functional processes, underlying the concept of astroglial reaction, are initiated in an attempt to recover homeostasis in the periventricular zone. This astroglial reaction includes astrocyte hypertrophy, hyperplasia, and development of a new layer with reorganized functional features that resemble the ependyma. Despite decades of research, there is a lack of information concerning the biological basis of the brain abnormalities that are associated with HCP.

Discussion

The present review of current literature discusses the neuropathological changes during gestation following the onset of congenital hydrocephalus and the unanswered questions into the pathophysiology of the disease. A better understanding of those missing points might help create novel therapeutic strategies that can reverse or even prevent the ultimate neurological impairment that affects this population and improve long-term clinical outcome.
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Metadaten
Titel
Fetal brain damage in congenital hydrocephalus
verfasst von
Maria Florencia Varela
Marcos M. Miyabe
Marc Oria
Publikationsdatum
25.05.2020
Verlag
Springer Berlin Heidelberg
Erschienen in
Child's Nervous System / Ausgabe 8/2020
Print ISSN: 0256-7040
Elektronische ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-020-04657-9

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