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Fetal brain damage in congenital hydrocephalus

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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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Data availability

Data supporting the findings of this study are available within the article and its supplementary information.

Abbreviations

CNS:

Central nervous system

CSF:

Cerebrospinal fluid

EEA1:

Early endosomal antigen 1

ETV:

Endoscopic third ventriculostomy

GFAP:

Glial fibrillary acidic protein

HCP:

Hydrocephalus

HTx:

Hydrocephalus Texas

L1CAM:

L1 cell adhesion molecule

NSC:

Neural stem cells

NPC:

Neural progenitor cells

PCCH:

Perspective Classification of Congenital Hydrocephalus

RGM:

Repulsive guidance molecule

SVZ:

Subventricular zone

TNFα:

Tumor necrosis factor alpha

TNFαR1:

Tumor necrosis factor alpha receptor 1

VEGF:

Vascular endothelial growth factor

VZ:

Ventricular zone

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Acknowledgments

We greatly appreciate Prof. Helen Jones for the invaluable help and advice during manuscript editing.

Author information

Authors and Affiliations

  1. Center for Fetal and Placental Research, Cincinnati Children’s Hospital Medical Center (CCHMC), Cincinnati, OH, USA

    Maria Florencia Varela, Marcos M. Miyabe & Marc Oria

  2. Department of Pediatrics, College of Medicine, University of Cincinnati, Cincinnati, OH, USA

    Marc Oria

Authors
  1. Maria Florencia Varela
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  2. Marcos M. Miyabe
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  3. Marc Oria
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Contributions

Study concept and design: M.O.

Acquisition of data: MF.V., M. M, M.O

Analysis and interpretation of data: MF.V., M. M, M.O.

First draft of the manuscript: MF.V.

Drafting of the manuscript: MF.V., M. M, M.O.

Critical revision of the manuscript for important intellectual content: MF.V., M. M, M.O.

Study supervision: M.O.

Corresponding author

Correspondence to Marc Oria.

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The authors declare that they have no competing interests.

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Varela, M.F., Miyabe, M.M. & Oria, M. Fetal brain damage in congenital hydrocephalus. Childs Nerv Syst 36, 1661–1668 (2020). https://doi.org/10.1007/s00381-020-04657-9

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