Not all ventriculomegaly is created equal: diagnostic overview of fetal, neonatal and pediatric ventriculomegaly
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Not all ventriculomegaly is created equal: diagnostic overview of fetal, neonatal and pediatric ventriculomegaly Smruti K. Patel 1 & Jorge Zamorano-Fernandez 2 & Usha Nagaraj 3 & Karin S. Bierbrauer 1,2 & Francesco T. Mangano 1,2 Received: 26 August 2019 / Accepted: 20 September 2019 # Springer-Verlag GmbH Germany, part of Springer Nature 2019
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. Keywords Fetal ventriculomegaly . Hydrocephalus . Fetus . Fetal magnetic resonance imaging . Myelomeningocele
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 * Francesco T. Mangano [email protected] 1
Department of Neurosurgery, University of Cincinnati College of Medicine, 3333 Burnet Avenue, MLC 2016, Cincinnati, OH 45229-3026, USA
2
Division of Pediatric Neurosurgery, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Avenue, MLC 2016, Cincinnati, OH 45229-3026, USA
3
Division of Radiology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA
ACC CC CIM MPS HPE VGM MMC CPC DTI
Agenesis of the corpus callosum Corpus callosum Chiari I malformation Mucopolysaccharidoses Holoprosencephaly Vein of Galen malformation Myelomeningocele Choroid plexus cauterization Diffusion tensor imaging
Introduction Ventriculomegaly, or dilatation of the ventricles of the brain, is one of the most commonly diagnosed abnormalities of the pediatric central nervous system (CNS) [1, 2]. The incidence of ventriculomegaly is 0.3 to 2.0 per 1000 pregnancies [3, 4]. The diagnosis of fetal ventriculomegaly is typically made on prenatal ultrasound and is defined by an atrial diameter of the lateral ventricle that is > 10 m
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