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Images from Duenas-Arias JE, Aguilar-Medina M, Arambula-Meraz E, et al. J Med Case Rep. 2007;1:94. [Open access.] PMID: 17880714, PMCID: PMC2040152.

Neonatal and Infant Brain Imaging Evaluation

José Luiz de Oliveira Schiavon, MD, MSc; Daphine Centola Grassi, MD | October 3, 2017 | Contributor Information

These magnetic resonance images (MRIs) of the head were obtained in a 5-day old male with 47,XXY/48,XXXY/49,XXXXY mosaic and several congenital anomalies, including hydrocephaly. The left and center images depict a supratentorial noncommunicating hydrocephalus. The right image reveals a slightly hypoplastic corpus callosum.

Imaging studies have a very important role in detecting cerebral pathology in neonates and infants. The neonatal brain undergoes a complex and unique anatomic development in utero, including that of the fetal cerebral vascular system.[1,2] Clinicians should be aware that differences exist between the normal brains of preterm and term infants, including their anatomy and structure, and the types and degree of cerebral damage that may occur.[1-3]

Image from Stolp HB, Liddelow SA, Sa-Pereira I, Dziegielewska KM, Saunders NR. Front Integr Neurosci. 2013;7:61. [Open access.] PMID: 23986663, PMCID: PMC3750212.

Neonatal and Infant Brain Imaging Evaluation

José Luiz de Oliveira Schiavon, MD, MSc; Daphine Centola Grassi, MD | October 3, 2017 | Contributor Information

This image is a schematic view of the neurologic niches (yellow) at the germinal matrix in the developing brain and its rich vascular supply. CSF = cerebrospinal fluid.

The neurologic tissue of the developing brain is one of the most sensitive organs when subjected to hypoxia, primarily due its rich vascular supply at the germinal matrix.[2] As the fetus grows in utero, the germinal matrix disappears (typically by 35-40 weeks of gestation); thus, the germinal matrix is only seen in the brains of premature infants, and these brains are particularly affected by hypoxia.[1,2] A reduction in the cerebral oxygen supply can lead to a reactive increase in blood flow, causing hemorrhage (germinal matrix hemorrhage-intraventricular hemorrhage [GMH-IVH]).[1-3]

Image from Kapellou O, Counsell SJ, Kennea N, et al. PLoS Med. 2006;3(8):e265. [Open access. PMID: 16866579, PMCID: PMC1523379.

Neonatal and Infant Brain Imaging Evaluation

José Luiz de Oliveira Schiavon, MD, MSc; Daphine Centola Grassi, MD | October 3, 2017 | Contributor Information

These serial MRIs depict brain growth in a female born at 25 weeks' gestational age through 39 weeks' gestational age. Straight lines connect the measured values for cerebral volume (triangles) and cortical surface area (circles) to the relevant image pairs. Some images have been omitted for graphical clarity. The inset scatter plot graph shows a linear relationship in log-log coordinates of the cortical surface area and the cerebral volume (diamonds), indicating power law scaling of the cortical surface area relative to the cerebral volume in this infant.

Normal Anatomy of the Premature Brain

One of the main challenges of the neonatal brain evaluation is the normal premature anatomy. The premature brain typically has prominent CSF spaces and ventricles, a smooth cortex, and a thinner cortical mantle.[1,2,4] Normal myelination does not reach maturity until approximately age 2 years.[1,4]

Adapted image from Radiopaedia/Dr Frank Gaillard.

Neonatal and Infant Brain Imaging Evaluation

José Luiz de Oliveira Schiavon, MD, MSc; Daphine Centola Grassi, MD | October 3, 2017 | Contributor Information

This axial T1-weighted MRI reveals normal myelination in a neonate.

Normal Cerebral Anatomy of the Term Infant

The normal brain of term infants is symmetric and typically shows an increase in cortical folding, with complex sulci and numerous gyri, as well as an increase in cortical gray matter volume and a decrease in brain water content.[1,4] The sylvian fissure is closed.[1,4]

Following myelination in utero, myelination continues in the posterior limb of the internal capsule[4-6] as well as in the brainstem, cerebellum, perirolandic region, and optic tract in term newborns.[6] This process normally takes place progressively from central to peripheral, caudal to rostral, dorsal to ventral, and sensory to motor regions.[7]

Adapted image from Radiopaedia/Dr Frank Gaillard.

Neonatal and Infant Brain Imaging Evaluation

José Luiz de Oliveira Schiavon, MD, MSc; Daphine Centola Grassi, MD | October 3, 2017 | Contributor Information

Normal myelination in a 5 month old is shown on this axial T1-weighted MRI.

On T1-weighted MRIs, in which gray matter is darker than white matter, myelination of the anterior limb of the internal capsule can be seen between ages 2 and 3 months, followed by the splenium of the corpus callosum between ages 3 and 4 months, and then the genu of the corpus callosum from ages 4 to 6 months.[6]

On T2-weighted MRIs, in which there is a gray-matter shift from hyperintense to hypointense, myelination of the anterior limb of the internal capsule is present between ages 7 and 11 months, whereas that of the splenium of the corpus callosum is between ages 4 and 6 months, and that the genu of the corpus callosum is at ages 5 to 8 months.[6]

The last area for myelination to mature around age 2 years is the peritrigonal region.[6]

Image from Erik Beek and Floris Groenendaal, via Radiology Assistant.

Neonatal and Infant Brain Imaging Evaluation

José Luiz de Oliveira Schiavon, MD, MSc; Daphine Centola Grassi, MD | October 3, 2017 | Contributor Information

Traditionally, ultrasonography, mainly via the transfontanelle approach, has been the primary imaging modality to evaluate the brain of neonates, as no sedation is required, there is no exposure to ionizing radiation, and it can be performed at the bedside.[2,8-10] It currently remains the most common method for evaluating newborn brains, particularly in premature neonates who are more susceptible to intracranial pathology than their term counterparts.[2]

However, MRI is gaining increasing interest and utility for neonatal neuroimaging, mainly due to its greater sensitivity and specificity for detecting subtle and/or diffuse neurologic pathology.[3,4,8-10] In 2016, researchers reported on their design of a dedicated neonatal brain imaging system that has the potential for providing advanced, high-resolution images with minimal artifacts,[11] and the United States Food and Drug Administration (FDA) cleared the first MRI device specifically for imaging of the neonatal brain and head in neonatal intensive care units (NICUs).[12]

Images from Auckland District Health Board.[13]

Neonatal and Infant Brain Imaging Evaluation

José Luiz de Oliveira Schiavon, MD, MSc; Daphine Centola Grassi, MD | October 3, 2017 | Contributor Information

These standard transfontanelle ultrasonography scans were obtained in a 30-week-old infant with normal-appearing anatomy.[13]

The left image is a coronal view of the anterior horns of the lateral ventricles, with the transducer angled back.[13] Note the dark CSF in the lateral ventricles. In preterm infants, the lateral ventricles and, often, the cavum septum pellucidum, are larger than those seen in term infants. Asymmetry between the lateral ventricles may be a normal variant. The corpus callosum appears above the cavum.[13]

The right image is an oblique parasagittal view, in which the shape of the lateral ventricle is the key landmark.[13] The caudate nucleus lies below the floor of the frontal horn of the lateral ventricle, whereas the thalamus lies behind and below it. Choroid plexus fills the occipital horn of the lateral ventricle. The choroid tucks up in the caudothalamic groove in the floor of the lateral ventricle and may be echogenic.[13]

Adapted image from Radiopaedia/Dr Frank Gaillard.

Neonatal and Infant Brain Imaging Evaluation

José Luiz de Oliveira Schiavon, MD, MSc; Daphine Centola Grassi, MD | October 3, 2017 | Contributor Information

This sonogram reveals a grade I left subependymal GMH-IVH. Note the echogenic material anterior to the caudothalamic groove. This groove is the most common site of GMH.[9,14,15]

Subependymal Germinal Matrix Hemorrhages

GMH and IVH are the most common and most important neurologic injuries in preterm neonates,[1-3,14] thus, all infants younger than 30 weeks' gestation should undergo routine screening cranial ultrasonography between ages 7 and 14 days (noncontrast computed tomography [CT] scanning is preferred in term infants).[15]

As noted earlier, the brain of a premature infant is unable to autoregulate cerebral blood pressure; consequently, fluctuations in cerebral blood pressure and flow can rupture the primitive germinal matrix vessels or lead to infarction of the metabolically active germinal matrix.[1-3,14] Extension into the periventricular white matter can occur, resulting in significant neurologic sequelae (eg, cerebral palsy, mental retardation, seizures). Injury to the germinal matrix has substantial mortality and morbidity rates.[1-3,14]

Adapted image from Radiopaedia/Dr Frank Gaillard.

Neonatal and Infant Brain Imaging Evaluation

José Luiz de Oliveira Schiavon, MD, MSc; Daphine Centola Grassi, MD | October 3, 2017 | Contributor Information

This sonogram reveals a grade III GMH-IVH with marked ventriculomegaly. Note the echogenic material anterior to the caudothalamic groove.

Grade I GMH-IVH is confined to the germinal matrix (at the caudothalamic groove), whereas grade II hemorrhage extends into the lateral ventricles without the presence of hydrocephalus.[14,15]

Grade III hemorrhage involves the ventricles with hydrocephalus, and grade IV hemorrhage involves the parenchyma, often with a mass effect.[15] Grade III and IV hemorrhages are generally associated with neurologic deficits or learning disability.[15]

Adapted image from Radiopaedia/Dr Dalia Ibrahim.

Neonatal and Infant Brain Imaging Evaluation

José Luiz de Oliveira Schiavon, MD, MSc; Daphine Centola Grassi, MD | October 3, 2017 | Contributor Information

This axial fluid-attenuated inversion recovery (FLAIR) MRI sequence reveals hypoxic-ischemic brain injury (arrows) in a full-term infant with a history of severe birth asphyxia and delayed mental and motor milestones.

Asphyxia and Hypoxic-Ischemic Encephalopathy

The lack of oxygen to the brain, especially during the birth of term children, can lead to asphyxia. On ultrasonography, this is characterized by a bilateral increase of the echogenicity of the subcortical/white matter (other potential findings include brain swelling).[16] A change in the ultrasonographic resistive index (RI) of the cerebral arteries (such as a low RI, < 0.6) due to the loss of autoregulation may be a predictor for prolonged asphyxia with intracranial hemorrhage and/or cerebral edema.[2]

Asphyxia is also associated with hypoxic-ischemic encephalopathy (HIE), in which term infants demonstrate clinical and laboratory evidence of acute or subacute brain injury due to asphyxia that is primarily caused by systemic hypoxemia and/or reduced cerebral blood flow.[16,17]

Images from Radiopaedia/Dr Mahmoud Yacout Alabd.

Neonatal and Infant Brain Imaging Evaluation

José Luiz de Oliveira Schiavon, MD, MSc; Daphine Centola Grassi, MD | October 3, 2017 | Contributor Information

This axial FLAIR sequence was taken in a preterm neonate whose MRI findings indicated a moderate degree of HIE.

On MRI, asphyxia can be observed in a FLAIR sequence as a hyperintense signal. The most common acute asphyxia injury pattern on MRI following acute fetal distress during labor and delivery is central cortico-subcortical involvement, with associated lesions in the basal ganglia, thalami, brain stem, hippocampus, and the corticospinal tracts around the central fissure.[16]

Severe partial prolonged asphyxia frequently results in encephalomalacia/HIE. This condition is characterized by bilateral subcortical low density and cystic formation of the white matter (hypointense on T1-weighted MRI; hyperintense on T2-weighted MRI) and often leads to neurodevelopmental delay.[16]

Images courtesy of Drs Jose Luiz de Oliveira Schiavon and Daphine Centola Grassi.

Neonatal and Infant Brain Imaging Evaluation

José Luiz de Oliveira Schiavon, MD, MSc; Daphine Centola Grassi, MD | October 3, 2017 | Contributor Information

These images depict schizencephaly type II. Note the widely separately lips of the cleft walls (arrows).

Schizencephaly

Schizencephaly is an uncommon disorder of neuronal migration characterized by a CSF-filled cleft that is lined by gray matter emerging from the ventricular surface (ependymal) to its periphery (pial surface).[18] The cleft may be unilateral or bilateral, with closed (type I) or open walls (type II).

Image courtesy of Medscape/Dr Ken Close.

Neonatal and Infant Brain Imaging Evaluation

José Luiz de Oliveira Schiavon, MD, MSc; Daphine Centola Grassi, MD | October 3, 2017 | Contributor Information

This is an axial T2-weighted MRI in a patient with unilateral closed-lip (type I) schizencephaly. The cleft is lined by gray matter and extends from the pial surface to the lateral ventricle.

The presentation and outcome vary, depending on the extent of the clefting and the presence/absence of other cerebral anomalies.[18] In general, patients present with seizures, hemiparesis, and developmental deficits.

Images courtesy of Drs Jose Luiz de Oliveira Schiavon and Daphine Centola Grassi.

Neonatal and Infant Brain Imaging Evaluation

José Luiz de Oliveira Schiavon, MD, MSc; Daphine Centola Grassi, MD | October 3, 2017 | Contributor Information

Leigh Syndrome

Leigh syndrome, or subacute necrotizing encephalopathy, is a rare genetic neurometabolic condition mostly associated with pyruvate dehydrogenase complex deficiency, one of the most common neurodegenerative mitochondrial metabolic disorders.[19] This condition typically presents in infancy but may also appear in the late neonatal period and, rarely, in teens or adults. Leigh syndrome is characterized by developmental arrest and psychomotor regression, eventually leading to death (by age 2-3 years) if left untreated.

High T2-weighted MRI signal intensities appear in the putamen and caudate heads (shown).[19] However, abnormal signal intensities may also be seen in the brainstem, periaqueductal gray matter, medulla, midbrain, corpus striatum, subthalamic nuclei, substantia nigra, and thalami.[20] These altered signals are a result of demyelination, spongiform degeneration, gliosis, spongiform degeneration, and/or capillary proliferation.

Images courtesy of Drs Jose Luiz de Oliveira Schiavon and Daphine Centola Grassi.

Neonatal and Infant Brain Imaging Evaluation

José Luiz de Oliveira Schiavon, MD, MSc; Daphine Centola Grassi, MD | October 3, 2017 | Contributor Information

Brain Tumors

Pediatric brain tumors are the most common solid tumor of childhood in the United States and the second leading type of malignancy in US children aged 0 to 19 years.[21]

Medulloblastoma

Medulloblastoma is the predominant malignant pediatric brain tumor.[22] It is a highly cellular embryonal infratentorial neoplasm that is usually spherical and located in the midline, but variants may also appear as nodular clusters. Medulloblastoma arises from the roof of the fourth ventricle, usually from the cerebellar vermis, and it almost always promotes hydrocephalus.[22]

Four subgroups of medulloblastoma are recognized by the 2016 update of the World Health Organization (WHO) CNS tumor classifications: WNT (wingless), SHH (sonic hedgehog), group 3, and group 4.[23] Although the presentation and prognosis vary with the subgroup and mutations involved, the most common signs/symptoms include ataxia and signs of increased intracranial pressure (ICP).[22]

Images courtesy of Drs Jose Luiz de Oliveira Schiavon and Daphine Centola Grassi.

Neonatal and Infant Brain Imaging Evaluation

José Luiz de Oliveira Schiavon, MD, MSc; Daphine Centola Grassi, MD | October 3, 2017 | Contributor Information

Pilomyxoid astrocytoma

Pilomyxoid astrocytoma is the more aggressive variant of the pilocytic astrocytoma (WHO grade I tumor).[23] Signs/symptoms of both tumors include increased ICP, delayed development, visual disturbances, and seizures.[24] Although pilomyxoid astrocytoma was previously designated a WHO grade II neoplasm, the 2016 update of the WHO CNS tumor classifications indicates further study of the behavior of these lesions is required.[23]

Pilomyxoid astrocytomas, like pilocytic astrocytomas, are well circumscribed tumors associated with no or little edema.[24] The lesions strongly enhance (arrow) and are usually suprasellar; unlike pilocytic astrocytomas, CSF dissemination is common for pilomyxoid astrocytomas. Pilocytic astrocytomas arise mainly from the cerebellum but may also arise from the optic chiasm or adjacent to the third ventricle and brainstem.[24] These lesions grow slowly, with accommodation of the mass effect, and typically have a very good prognosis.

Images courtesy of Drs Jose Luiz de Oliveira Schiavon and Daphine Centola Grassi.

Neonatal and Infant Brain Imaging Evaluation

José Luiz de Oliveira Schiavon, MD, MSc; Daphine Centola Grassi, MD | October 3, 2017 | Contributor Information

Embryonal tumor with multilayered rosettes

Traditionally, the term "CNS primitive neuroectodermal tumor (CNS PNET)" included the histologic subtypes CNS neuroblastoma, ganglioneuroblastoma, medulloepithelioma (MEP), ependymoblastoma (EPB), and embryonal tumor with abundant neuropil and true rosettes (ETANTR).[24] "Supratentorial PNETs (S-PNETs)" was used to define rare primitive large, ill-defined, and highly cellular, aggressive cerebral embryonal tumors that were hemispheric supratentorial masses with heterogeneous enhancement and restricted MRI diffusion sequences (arrow).[24] The presentation of S-PNETs varied with the tumor origin site and size but often involved seizures, motor deficits, and increased ICP, and the prognosis was poor.

However, the 2016 update of the WHO CNS tumor classifications has eliminated "PNET" and replaced it with "embryonal tumor with multilayered rosettes (ETMRs), C19MC-altered" to encompass tumors that display amplification of the C19MC region on chromosome 19, including those previously known as ETANTR, EPB, and some types of MEP.[23]

Images courtesy of Drs Jose Luiz de Oliveira Schiavon and Daphine Centola Grassi.

Neonatal and Infant Brain Imaging Evaluation

José Luiz de Oliveira Schiavon, MD, MSc; Daphine Centola Grassi, MD | October 3, 2017 | Contributor Information

Desmoplastic infantile ganglioglioma

Desmoplastic infantile gangliogliomas are rare cortical WHO grade 1 tumors in children younger than 2 years.[24] The lesions are characterized by large cysts (often multilobulated) and cortical nodules or plaquelike regions alongside the meninges, and there is usually no restriction on diffusion MRI sequences (arrow). The most common symptoms/signs in infants include macrocephaly, paresis, and seizures; older children present with seizures and focal neurologic deficits.[24] The prognosis is generally good.

Note: Marked enhancement of these tumors to solid areas, as well as their areas of cellular proliferation and cystic necrosis, may occasionally cause desmoplastic infantile gangliogliomas to be misdiagnosed as higher grade tumors.[24]

Images courtesy of Drs Jose Luiz de Oliveira Schiavon and Daphine Centola Grassi.

Neonatal and Infant Brain Imaging Evaluation

José Luiz de Oliveira Schiavon, MD, MSc; Daphine Centola Grassi, MD | October 3, 2017 | Contributor Information

Choroid plexus papilloma

Choroid plexus papilloma is a WHO grade I, slow-growing, benign tumor that primarily arises from the choroid plexus to the atrium of the lateral ventricle as a lobulated mass, but it also may arise in the fourth ventricle and/or the foramina of Luschka and, uncommonly, the roof of the third ventricle.[24] It is the most common brain tumor in children younger than age 1 year. The most common symptoms/signs include signs of ICP, macrocrania, and ataxia.[24]

The lesion is usually isoattenuating or hyperattenuating on noncontrast CT scan; with the addition of contrast medium, intense homogenous enhancement is observed.[24] MRI reveals a well-defined isointense to hypointense lobular mass. The prognosis is excellent with complete surgical resection.[24]

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Pediatric Brain Tumors: Imaging Matters

Primary brain tumors are the second most common group of malignancies affecting children (after leukemia) and are a leading cause of mortality. Learn more about diagnosing these potentially deadly disorders.Slideshows, August 2017
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Germinal Matrix Hemorrhage Imaging

Germinal matrix hemorrhage (GMH) and intraventricular hemorrhage (IVH) are the most common and most important neurologic injuries in preterm neonates. The brain of a premature infant lacks the ability to autoregulate cerebral blood pressure; thus, fluctuations in cerebral blood pressure and flow can rupture the primitive germinal matrix vessels or lead to infarction of the metabolically active germinal matrix.Diseases/Conditions, November 2015
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References