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Fetal ventriculomegaly: Diagnosis, treatment, and future directions

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.

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Abbreviations

AD:

Atrial diameter

AS:

Aqueductal stenosis

CSF:

Cerebrospinal fluid

MRI:

Magnetic resonance imaging

US:

Ultrasound

VA:

Ventriculo-amniotic fluid

VM:

Ventriculomegaly

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Acknowledgements

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

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Correspondence to Jared M. Pisapia.

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Pediatric Hydrocephalus Foundation Research Grant.

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

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The authors declare that they have no conflicts of interest.

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Pisapia, J.M., Sinha, S., Zarnow, D.M. et al. Fetal ventriculomegaly: Diagnosis, treatment, and future directions. Childs Nerv Syst 33, 1113–1123 (2017). https://doi.org/10.1007/s00381-017-3441-y

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Keywords

  • Fetal ventriculomegaly
  • Hydrocephalus
  • Fetus
  • Fetal magnetic resonance imaging