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Child's Nervous System

, Volume 33, Issue 7, pp 1113–1123 | Cite as

Fetal ventriculomegaly: Diagnosis, treatment, and future directions

  • Jared M. PisapiaEmail author
  • Saurabh Sinha
  • Deborah M. Zarnow
  • Mark P. Johnson
  • Gregory G. Heuer
Focus Session

Abstract

Fetal ventriculomegaly (VM) refers to the enlargement of the cerebral ventricles in utero. It is associated with the postnatal diagnosis of hydrocephalus. VM is clinically diagnosed on ultrasound and is defined as an atrial diameter greater than 10 mm. Because of the anatomic detailed seen with advanced imaging, VM is often further characterized by fetal magnetic resonance imaging (MRI). Fetal VM is a heterogeneous condition with various etiologies and a wide range of neurodevelopmental outcomes. These outcomes are heavily dependent on the presence or absence of associated anomalies and the direct cause of the ventriculomegaly rather than on the absolute degree of VM. In this review article, we discuss diagnosis, work-up, counseling, and management strategies as they relate to fetal VM. We then describe imaging-based research efforts aimed at using prenatal data to predict postnatal outcome. Finally, we review the early experience with fetal therapy such as in utero shunting, as well as the advances in prenatal diagnosis and fetal surgery that may begin to address the limitations of previous therapeutic efforts.

Keywords

Fetal ventriculomegaly Hydrocephalus Fetus Fetal magnetic resonance imaging 

Abbreviations

AD

Atrial diameter

AS

Aqueductal stenosis

CSF

Cerebrospinal fluid

MRI

Magnetic resonance imaging

US

Ultrasound

VA

Ventriculo-amniotic fluid

VM

Ventriculomegaly

Notes

Acknowledgements

We thank Eo Trueblood, MA, lead medical illustrator at The Children’s Hospital of Philadelphia, for his creation of Figs. 1 and 2.

Compliance with ethical standards

Funding source

Pediatric Hydrocephalus Foundation Research Grant.

Financial disclosure

The authors have no financial relationships relevant to this article to disclose.

Conflict of interest

The authors declare that they have no conflicts of interest.

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Copyright information

© Springer-Verlag Berlin Heidelberg 2017

Authors and Affiliations

  • Jared M. Pisapia
    • 1
    Email author
  • Saurabh Sinha
    • 1
  • Deborah M. Zarnow
    • 2
    • 3
  • Mark P. Johnson
    • 3
  • Gregory G. Heuer
    • 1
    • 3
    • 4
  1. 1.Department of NeurosurgeryUniversity of PennsylvaniaPhiladelphiaUSA
  2. 2.Division of NeuroradiologyChildren’s Hospital of PhiladelphiaPhiladelphiaUSA
  3. 3.The Center for Fetal Diagnosis and TreatmentChildren’s Hospital of PhiladelphiaPhiladelphiaUSA
  4. 4.Division of NeurosurgeryChildren’s Hospital of PhiladelphiaPhiladelphiaUSA

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