Not all ventriculomegaly is created equal: diagnostic overview of fetal, neonatal and pediatric ventriculomegaly

Abstract

Fetal ventriculomegaly refers to a condition in which there is enlargement of the ventricular spaces, typically on prenatal ultrasound. It can be associated with other CNS or extra-CNS abnormalities, and this relationship is crucial to understand as it affects overall neonatal outcome. Isolated ventriculomegaly has been described in the literature with variable clinical outcome. Typically, outcome is based on the etiology and degree of ventriculomegaly. When associated with a pathologic condition, ventriculomegaly can be a result of hydrocephalus. While initial diagnosis is usually made on prenatal ultrasound, fetal magnetic resonance imaging is preferred to further elucidate any associated CNS malformations. In this paper, the authors aim to provide a comprehensive review of the diagnosis, associated etiologies, prognosis, and treatment options related to fetal, neonatal, and pediatric ventriculomegaly and hydrocephalus. In addition, preliminary data is provided from our institutional cohort of patients with a prenatal diagnosis of ventriculomegaly followed through the perinatal period.

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Abbreviations

CNS:

Central nervous system

ICP:

Intracranial pressure

GA:

Gestational age

MRI:

Magnetic resonance imaging

CT:

Computed tomography

FeMRI:

Fetal magnetic resonance imaging

CSF:

Cerebrospinal fluid

CAS:

Congenital aqueductal stenosis

ETV:

Endoscopic third ventriculostomy

PHH:

Post-hemorrhagic hydrocephalus

VAD:

Ventricular access device

VSG:

Ventriculosubgaleal shunt

LP:

Lumbar puncture

ACC:

Agenesis of the corpus callosum

CC:

Corpus callosum

CIM:

Chiari I malformation

MPS:

Mucopolysaccharidoses

HPE:

Holoprosencephaly

VGM:

Vein of Galen malformation

MMC:

Myelomeningocele

CPC:

Choroid plexus cauterization

DTI:

Diffusion tensor imaging

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Patel, S.K., Zamorano-Fernandez, J., Nagaraj, U. et al. Not all ventriculomegaly is created equal: diagnostic overview of fetal, neonatal and pediatric ventriculomegaly. Childs Nerv Syst 36, 1681–1696 (2020). https://doi.org/10.1007/s00381-019-04384-w

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Keywords

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