Magnetic Resonance Imaging Findings Are Associated with Long-Term Global Neurological Function or Death after Traumatic Brain Injury in Critically Ill Children.

The identification of children with traumatic brain injury (TBI) who are at risk of death or poor global neurological functional outcome remains a challenge. Magnetic resonance imaging (MRI) can detect several brain pathologies that are a result of TBI; however, the types and locations of pathology that are the most predictive remain to be determined. Forty-two critically ill children with TBI were recruited prospectively from pediatric intensive care units at five Canadian children's hospitals. Pathologies detected on subacute phase MRIs included cerebral hematoma, herniation, cerebral laceration, cerebral edema, midline shift, and the presence and location of cerebral contusion or diffuse axonal injury (DAI) in 28 regions of interest were assessed. Global functional outcome or death more than 12 months post-injury was assessed using the Pediatric Cerebral Performance Category score. Linear modeling was employed to evaluate the utility of an MRI composite score for predicting long-term global neurological function or death after injury, and nonlinear Random Forest modeling was used to identify which MRI features have the most predictive utility. A linear predictive model of favorable versus unfavorable long-term outcomes was significantly improved when an MRI composite score was added to clinical variables. Nonlinear Random Forest modeling identified five MRI variables as stable predictors of poor outcomes: presence of herniation, DAI in the parietal lobe, DAI in the subcortical white matter, DAI in the posterior corpus callosum, and cerebral contusion in the anterior temporal lobe. Clinical MRI has prognostic value to identify children with TBI at risk of long-term unfavorable outcomes.

Hydrocephalus in Children: A Neuroradiological Perspective.

Concepts about the production, absorption, dynamics, and physiological roles of cerebrospinal fluid (CSF) have dramatically changed over the recent decades. This article will review these new concepts and detail how they must be used for a better assessment and a better understanding of the various aspects of hydrocephalus by using neuroradiological tools.

Deep venous communication in vein of Galen malformations: incidence, Imaging, and Implications for treatment.

BACKGROUND: Failure to appreciate deep venous drainage pathways is a major cause of severe complications in the endovascular treatment of vein of Galen aneurysmal malformations (VOGMs). OBJECTIVE: To report deep venous drainage patterns in patients with VOGM, emphasizing the internal cerebral veins, and to describe the challenges in evaluating these. METHODS: Patients with VOGM presenting to our institute between 2000 and 2018 were retrospectively analyzed. Patients with complete and good quality imaging datasets were included in the study. Three neuroradiologists with expertise in the subject independently analyzed the deep venous drainage patterns on multi-sequence MRI and digital subtraction angiography. Follow-up imaging studies were analyzed for alterations in deep venous drainage patterns that occurred following endovascular treatment. Descriptive statistics were used to report findings. RESULTS: Twenty-three patients had optimal quality MRI imaging and 25 had optimal quality DSA imaging available. In 14/23 (61%) patients, internal cerebral vein (ICV) communication could be reliably identified on MRI and in 8/25 (32%) patients on DSA. Deep venous communication with the VOGM was demonstrated in 8/26 (30.8%) patients. One (3.8%) patient demonstrated ICV communication with the VOGM only on postoperative imaging, while in 2 (8%) patients the ICV drainage route changed from VOGM to alternative pathways after the procedure. Other variant pathways included lateral mesencephalic vein, superior or inferior sagittal sinus, anterior mesencephalic vein, tentorial sinus, deep Sylvian vein, and superior vermian vein. CONCLUSION: ICV communication with the VOGM is not uncommon and requires dedicated preprocedural imaging to identify it. However, there are significant challenges in assessing this communication in the presence of high-flow fistulae, vessel tortuosity and size, and contrast limitations in this population.

Corpus Callosum: Molecular Pathways in Mice and Human Dysgeneses.

The corpus callosum is the largest of the 3 telencephalic commissures in eutherian (placental) mammals. Although the anterior commissure, and the hippocampal commissure before being pushed dorsally by the expanding frontal lobes, cross through the lamina reuniens (upper part of the lamina terminalis), the callosal fibers need a transient interhemispheric cellular bridge to cross. This review describes the molecular pathways that initiate the specification of the cells comprising this bridge, the specification of the callosal neurons, and the repulsive and attractive guidance molecules that convey the callosal axons toward, across, and away from the midline to connect with their targets.

Neuroimaging in Pediatric Hydrocephalus.

The objective of this review is to discuss the role of neuroimaging in evaluating pediatric and fetal hydrocephalus based on possible pathophysiologic mechanisms and in the context of differing etiology. Although conventional brain imaging with ultrasound (US), computed tomography (CT), and magnetic resonance imaging (MRI) is commonly used to assess for ventricular enlargement, however, the underlying mechanisms and management of hydrocephalus is a challenge in pediatric population and fetal hydrocephalus. MRI helps define the possible nature of the obstruction, and provides useful functional and anatomic information. MR imaging, in both pediatric and fetal hydrocephalus, thus may help in better understanding of the possible pathophysiologic mechanisms of the varied causal factors. The article focuses on the usage of MRI sequences in both diagnosis and follow-up of pediatric and fetal hydrocephalus, to be able to investigate all possible etiopathogenesis through the CSF pathway and to assess the efficacy of treatment in a non-invasive standardized manner.

Association of Severe Hydrocephalus With Congenital Zika Syndrome.

Importance: Hydrocephalus is a treatable but potentially fatal complication that has not been previously described in congenital Zika syndrome (CZS). Objective: To describe the clinical features and imaging findings in 24 patients with congenital Zika syndrome (CZS) who developed hydrocephalus. Design, Setting, and Participants: This case series included patients with hydrocephalus who were born in October and November 2015 and followed up until mid-2017 in the 2 largest national referral centers for CZS in Brazil. The participants included consecutively enrolled children with a clinical and laboratorial diagnosis of CZS who developed clinical and/or image findings suggestive of hydrocephalus and who were confirmed to experience increased intracranial hypertension during ventriculoperitoneal shunt procedures. Main Outcomes and Measures: To retrospectively describe clinical and image findings in these 24 patients. Results: This multicenter cohort included 308 patients with CZS; 24 consecutive children were enrolled in this study. These children were aged between 3 to 18 months, and 13 of 24 (54%) were female. All patients presented with at least 1 positive test result for anti-Zika antibodies in cerebrospinal fluid or serum and had classic signs of CZS. At the time of hydrocephalus diagnosis, only 14 of 24 patients (58%) had symptoms and signs suggestive of hydrocephalus (mainly worsening seizures, vomiting, irritability, and/or sudden increase of head circumference percentile). Two of 24 patients (8%) had no symptoms suggestive of hydrocephalus but were found to have reduced brain volume on repeated imaging. Cerebellar or brainstem hypoplasia on baseline imaging were found in 18 of 23 patients (78%). At the second computed tomographic scan, all patients showed a marked increase of ventricular volume, compatible with communicating hydrocephalus, and reduction of brain tissue that was visibly worse than on baseline imaging for the 23 patients with repeated scans. Conclusions and Relevance: We present evidence that hydrocephalus is a complication of CZS in at least a proportion of patients. The clinical spectrum of this condition continues to evolve, but given that presenting signs and symptoms of hydrocephalus can be challenging to recognize in CZS, we provisionally recommend that high suspicion and appropriate monitoring for hydrocephalus should be part of the standard care of patients with CZS.

Pediatric Brain Tumor Genetics: What Radiologists Need to Know.

Brain tumors are the most common solid tumors in the pediatric population. Pediatric neuro-oncology has changed tremendously during the past decade owing to ongoing genomic advances. The diagnosis, prognosis, and treatment of pediatric brain tumors are now highly reliant on the genetic profile and histopathologic features of the tumor rather than the histopathologic features alone, which previously were the reference standard. The clinical information expected to be gleaned from radiologic interpretations also has evolved. Imaging is now expected to not only lead to a relevant short differential diagnosis but in certain instances also aid in predicting the specific tumor and subtype and possibly the prognosis. These processes fall under the umbrella of radiogenomics. Therefore, to continue to actively participate in patient care and/or radiogenomic research, it is important that radiologists have a basic understanding of the molecular mechanisms of common pediatric central nervous system tumors. The genetic features of pediatric low-grade gliomas, high-grade gliomas, medulloblastomas, and ependymomas are reviewed; differences between pediatric and adult gliomas are highlighted; and the critical oncogenic pathways of each tumor group are described. The role of the mitogen-activated protein kinase pathway in pediatric low-grade gliomas and of histone mutations as epigenetic regulators in pediatric high-grade gliomas is emphasized. In addition, the oncogenic drivers responsible for medulloblastoma, the classification of ependymomas, and the associated imaging correlations and clinical implications are discussed. ©RSNA, 2018.

Isolated neonatal bilateral vocal cord paralysis revealing a unilateral medullary defect: a case report.

BACKGROUND: Congenital bilateral vocal cord paralysis is a rare occurrence. Approximately half the cases are associated with a major comorbidity, usually neurological, neuromuscular or malformative. CASE PRESENTATION: In a male newborn, respiratory distress syndrome and stridor were observed immediately following birth. The cause was bilateral vocal cord paralysis in the adducted position. Neuroradiological investigation revealed a unilateral discontinuity between the upper pons and the right medulla oblongata. Hypoplasia of the right posterior hemiarches of C1-C2 and the right exo-occipital bone was observed, as was a small clivus. MR angiography showed the absence of the distal right vertebral artery, with hypoplasia and parietal irregularities of the proximal segments. Respiratory autonomy was not obtained despite endoscopic laser cordotomy, corticosteroid therapy and nasal continuous positive airway pressure. The infant died at the age of 4 weeks after treatment was limited to comfort care. CONCLUSIONS: A medullary lesion is an exceptional cause of congenital bilateral vocal cord paralysis. The strictly unilateral neurological and vascular defect and the absence of associated intracranial or extracranial malformation make this clinical case unique and suggest a disruptive mechanism. This case also highlights the help provided by advanced neuroimaging techniques, i.e. fibre tracking using diffusion tensor imaging, in the decision-making process.

Chiari 1 deformity in children: etiopathogenesis and radiologic diagnosis.

The metamerically associated normal hindbrain and normal posterior fossa are programmed to grow together in such a way that the tonsils are located above the foramen magnum and surrounded by the cerebrospinal fluid (CSF) of the cisterna magna. This allows the pulsating CSF to move freely up and down across the craniovertebral junction (CVJ). A developmental mismatch between the rates of growth of the neural tissue and of the bony posterior fossa may result in the cerebellar tonsils being dislocated across the foramen magnum. The cause of this may be, rarely, an overgrowth of the cerebellum. More commonly, it is due to an insufficient development of the posterior fossa, possibly associated with a malformation of the craniocervical joint. When it is not due to a remediable cause, such a herniation is called a Chiari 1 deformity. This definition is anatomic (descent of the tonsils below the plane of the foramen magnum) and not clinical: many patients with the deformity are and will remain asymptomatic. Most authors consider that a descent of 5 mm or more is clinically significant but other factors, such as the diameter of the foramen magnum and the degree of tapering of the upper cervical "funnel," are likely to be as important. Morphologic markers of severity on magnetic resonance imaging are, beside the degree of descent, the peg-like deformity of the tonsils, the obstruction of the surrounding CSF spaces (at the craniocervical junction and in the whole posterior fossa), a compression of the cord, an abnormal signal of the cord, and a syringomyelia, typically cervicothoracic. The syringomyelia is assumed to be explained by the "Venturi effect" that is associated with the increased velocity of the CSF across the restricted CSF spaces. Radiologically, the etiopathogenic assessment should address the size and morphology of the posterior fossa, and the functional status of the craniocervical flexion joint. The posterior fossa is best evaluated on sagittal cuts by the posterior fossa pentagon proportionality associated with the line of Chamberlain, and on coronal cuts, by showing a possible shallowness of the posterior fossa. The functional status of the craniocervical joint is altered in case of a proatlantal hypoplasia, as this condition results in a cranial shift of the joint that brings the tip of the dens and of the flexion axis in front of the medulla, that is, in a situation of osteoneural conflict. Less commonly, similar conflicts may also occur when an abnormal craniocervical segmentation results in an instability of the joint.

Differential patterns of metastatic dissemination across medulloblastoma subgroups.

OBJECTIVE Metastatic dissemination is a major treatment challenge and cause of death in patients with medulloblastoma. However, the influence of molecular biology on the pattern of metastatic dissemination at diagnosis is not known. In this study, the authors sought to define the location, pattern, and imaging characteristics of medulloblastoma metastases across subgroups at diagnosis. METHODS A consecutive cohort of patients with metastatic medulloblastoma at The Hospital for Sick Children and the University Hospital Motol, who underwent up-front MRI of the craniospinal axis, was assembled and allocated to subgroups using NanoString limited gene-expression profiling. Radiological characteristics (including location, morphology, size, diffusion restriction, and contrast enhancement) were discerned through a retrospective review. RESULTS Forty metastatic medulloblastomas were identified with up-front neuroimaging of the craniospinal axis: 5 sonic hedgehog (SHH), 16 Group 3, and 19 Group 4 metastases. Significant subgroup-specific differences were observed, particularly with respect to tumor location, size, and morphology. Group 3 metastases were most frequently laminar compared with a more nodular pattern in Group 4 (14 of 16 in Group 3 vs 8 of 19 in Group 4; p = 0.0004). Laminar metastases were not observed in patients with SHH medulloblastoma. Suprasellar metastases are highly specific to Group 4 (p = 0.016). Two of the 5 SHH cases had multifocal lesions in the cerebellum, raising the possibility that these were in fact synchronous primary tumors and not true metastases. A minority of patients with Group 4 metastases harbored metastatic deposits that did not enhance on MRI after contrast administration, often in patients whose primary tumor did not enhance. CONCLUSIONS The location, morphology, and imaging characteristics of metastatic medulloblastoma differ across molecular subgroups, with implications for diagnosis and management. This suggests that the biology of leptomeningeal dissemination differs among medulloblastoma subgroups.

Intracranial artery to artery spontaneous revascularization in a child.

INTRODUCTION: Intracranial artery-to-artery antegrade revascularization is a poorly recognized entity, more so when it involves main stem arteries. The etiology, appearance, and significance of this condition are not described in the literature. CASE PRESENTATION: We describe a case of spontaneous revascularization of a chronically occluded middle cerebral arterial branch by collaterals from the proximal segment reconstituting distal flow, mimicking a brain arteriovenous malformation in a 9-year old boy. We discuss the nature of these channels, presumed to be related to artery to artery collaterals that are either dilated adventitial vasa vasorum, or, more likely, leptomeningeal collaterals that are hypertrophied in response to cerebral demand. We review the literature regarding intracerebral vasa vasorum and leptomeningeal collaterals including their imaging. CONCLUSION: Recognizing the tortuous channels associated with this type of vascular abnormality as normal vessels reconsituting distal flow may prevent unnecessary and potentially dangerous treatments.

Longitudinal Study of White Matter Development and Outcomes in Children Born Very Preterm.

Identifying trajectories of early white matter development is important for understanding atypical brain development and impaired functional outcomes in children born very preterm (<32 weeks gestational age [GA]). In this study, 161 diffusion images were acquired in children born very preterm (median GA: 29 weeks) shortly following birth (75), term-equivalent (39), 2 years (18), and 4 years of age (29). Diffusion tensors were computed to obtain measures of fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD), which were aligned and averaged. A paediatric atlas was applied to obtain diffusion metrics within 12 white matter tracts. Developmental trajectories across time points demonstrated age-related changes which plateaued between term-equivalent and 2 years of age in the majority of posterior tracts and between 2 and 4 years of age in anterior tracts. Between preterm and term-equivalent scans, FA rates of change were slower in anterior than posterior tracts. Partial least squares analyses revealed associations between slower MD and RD rates of change within the external and internal capsule with lower intelligence quotients and language scores at 4 years of age. These results uniquely demonstrate early white matter development and its linkage to cognitive functions.

Cerebral hemispheric low-grade glial tumors in children: preoperative anatomic assessment with MRI and DTI.

PURPOSE: The aims of this study are to analyze how the nature and the behavior of low-grade glial tumors (LGGT) in children may correlate with the anatomy of the cerebral hemispheres and to evaluate the consequent impact of diffusion tensor imaging (DTI) techniques in the presurgical assessment. METHODS: This is a combined review of a series of 155 cases of LGGT and of the recent literature on the subject. RESULTS: The cases retrieved from our data bank were divided in central hemispheric tumors (basal ganglia and thalami) (36 cases), glioneuronal cortical-based tumors (49 cases), and glial tumors of the cerebral mantle (70 cases). A close correlation was found in the thalamus between the primary location of the tumor (juxta-ventricular, inferior, lateral, bilateral) and its extension (ventricular lumen, midbrain and mesial temporal, globus pallidus, respectively) which may relate to the connectivity. Among the glioneuronal tumors, most gangliogliomas were located in the temporal lobe and especially in the mesial temporal structures. In addition, the morphologic feature of the ganglioglioma was different there from the neocortical areas. As a complementary approach, DTI data may assist in evaluating the structure and the extension of the LGGT, in addition to planning the surgical strategy. CONCLUSIONS: In the cerebral hemispheres like in the rest of the central nervous system, there is some degree of correlation between the anatomy and the nature, appearance, and behavior of the LGGT in children.

Chiari Type 1 Deformity in Children: Pathogenetic, Clinical, Neuroimaging, and Management Aspects.

Our understanding of cerebellar tonsillar herniation evolved over time and nowadays various pathomechanisms have been proposed. Causes of tonsillar herniation share a discrepancy between content (fore- and hindbrain) and container (supratentorial cranial vault, posterior fossa), may be associated with abnormalities of the craniocervical junction, and may have a developmental or acquired nature. In tonsillar herniation, the hindbrain is not malformed but deformed. Accordingly, "Chiari type 1 deformity," not "Chiari type 1 malformation" is the correct term to characterize primary tonsillar herniation. Chiari type 1 deformity is commonly seen in pediatric neurology, neuroradiology, and neurosurgery and may have various clinical presentations depending on patient age. In addition, Chiari type 1 deformity is increasingly found by neuroimaging studies as an incidental finding in asymptomatic children. An accurate and reliable selection of patients based on clinical and neuroimaging findings is paramount for the success of neurosurgical treatment. Future studies are needed to provide selection criteria with a higher sensitivity and specificity.

Diffusion tensor imaging-based assessment of white matter tracts and visual-motor outcomes in very preterm neonates.

INTRODUCTION: The purpose of this study was to assess the impact of brain injury on white matter development and long-term outcomes in very preterm (VPT) neonates. METHODS: Eighty-five VPT neonates (born <32/40 weeks gestational age (GA)) scanned within 2 weeks of birth were divided into three groups based on the presence of perinatal cerebral injury: (i) no injury, (ii) mild/moderate injury and (iii) severe injury. Diffusion tensor imaging (DTI) was acquired for each neonate and fractional anisotropy (FA), and diffusivity measures were calculated in the posterior limb of the internal capsule (PLIC) and optic radiation (OR). At 2 and 4 years of age, 41 and 44 children were assessed for motor and visual-motor abilities. Analyses determined the relation between GA and DTI measures, injury groups and DTI measures as well as developmental assessments. RESULTS: GA was related to all DTI measures within the PLIC bilaterally, FA in the OR bilaterally and AD in the left OR. The severely injured group had significantly different DTI measures in the left PLIC compared to the other two groups, independent of lateralization of lesions. Group differences in the left OR were also found, due to higher incidence of the white matter injury in the left hemisphere. No differences were found between groups and outcome measures at 2 and 4 years, with the exception of destructive periventricular venous haemorrhagic infarction (PVHI). CONCLUSIONS: DTI measures of the PLIC and OR were affected by injury in VPT neonates. These findings seen shortly after birth did not always translate into long-term motor and visual-motor impairments suggesting compensatory mechanisms.

MR assessment of pediatric hydrocephalus: a road map.

PURPOSE: This study was conducted to design a rational approach to the MR diagnosis of hydrocephalus based on a pathophysiologic reevaluation of its possible mechanisms and to apply it to the different etiological contexts. METHOD: A review of the literature reports describing new physiologic models of production and absorption and of the hydrodynamics of the CSF was made. RESULTS: Besides the secretion of CSF by the choroid plexuses, and its passive, pressure-dependent transdural absorption (arachnoid villi, dural clefts, cranial, and spinal nerve sheaths), water transporters, aquaporins, allow water (if not ions and organic molecules) to exchange freely between the brain parenchyma and the CSF spaces across the ependymal and the pial interfaces (including the Virchow-Robin spaces). Consequently, the CSF bulk flow is not necessarily global, and situations of balanced absorption-secretion may occur separately in different CSF compartments such as the ventricular, intracranial, or intraspinal CSF spaces. This means that rather than from a hypothetical pressure gradient from the plexuses to the dural sinuses, the dynamics of the CSF depend on the force provided in those different compartments by the arterial systolic pulsation of the pericerebral (mostly), intracerebral, and intraventricular (choroid plexuses) vascular beds. CONCLUSION: Using MR imaging, diverse varieties of hydrocephalus may tentatively be explained by applying those concepts to the correspondingly diverse causal diseases. Hopefully, this may have an impact on the choice of the treatment strategies also.

Longitudinal cerebellar growth following very preterm birth.

PURPOSE: To measure cerebellar growth in a longitudinal cohort of very preterm infants to identify early predictors of subsequent brain growth. Although the cerebellum grows rapidly during late gestation, the rate and variability of growth following premature birth, and the effects of associated injury, are largely unknown. MATERIALS AND METHODS: In all, 105 very-preterm born infants (24-32 weeks GA) were imaged using magnetic resonance imaging (MRI) at birth, term-equivalent, 2, and 4 years of age. Cerebellar and total cerebral volumes were estimated from 1 mm isotropic T1 -weighted scans acquired at 1.5T and 3T, using an atlas-based approach. Linear models were used to analyze cerebellar volume as cross-sectional and longitudinal functions of age, clinical, and radiological correlates. Linear models were also used to test for associations between volume and cognitive outcome. RESULTS: Cerebellar volume increased rapidly with age-at-scan during both the preterm (0.7 mL/wk, P < 0.001) and term periods (1.8 mL/wk, P < 0.001). Infants with grade 3 or 4 germinal matrix hemorrhage (GMH) had smaller cerebellar volumes as a percentage of total brain volume starting at birth and continuing to 4 years of age (-0.43%, -0.57%, -1.09% at preterm, term, and 4 years, respectively, P < 0.01). Irrespective of age-at-scan, early cerebellar volume was predictive of volume at 4 years of age (slope = 1.3, P < 0.001). Cerebellar volumes were not found to predict cognitive outcome at 4 years of age; P < 0.2. CONCLUSION: High-grade GMH and small perinatal cerebellar size is predictive of cerebellar development up to 4 years of age. These findings suggest that it is possible to identify individuals at high risk of reduced cerebellar volumes at an early age. J. Magn. Reson. Imaging 2016;43:1462-1473.

Corpus callosum abnormalities: neuroradiological and clinical correlations.

AIM: To study neuroradiological features in pediatric patients with corpus callosum abnormalities, using new functional subtyping for the corpus callosum, and to correlate the features with the clinical presentation. METHOD: We performed a retrospective review of 125 patients with radiologically identified abnormalities of the corpus callosum seen between 1999 and 2012. The study reviewed clinical features, genetic etiology, and chromosomal microarray (CMA) results. We used a new functional classification for callosal abnormalities based on embryological and anatomical correlations with four classes: complete agenesis, anterior agenesis (rostrum, genu, body), posterior agenesis (isthmus, splenium), and complete hypoplasia (thinning). We also studied the presence of extracallosal abnormalities. RESULTS: The new functional callosal subtyping did not reveal significant differences between the various subtypes in association with neurological outcome; however, the presence of cardiac disease was found more frequently in the group with complete agenesis. Thirty-seven per cent (46/125) had identifiable causes: of these, 48% (22/46) had a monogenic disorder, 30% (14/46) had a pathogenic chromosomal copy-number variant detected by CMA or karyotype, and 22% (10/46) had a recognizable clinical syndrome for which no confirmatory genetic test was available (namely Aicardi syndrome/septo-optic dysplasia and Goldenhar syndrome). The diagnostic yield for a significant CMA change was 19%. The presence of Probst bundles was found to be associated with a better neurodevelopmental outcome. INTERPRETATION: The functional classification system alone 'without clinical data' cannot predict the functional outcome. The presence of extracallosal brain abnormalities and an underlying genetic diagnosis predicted a worse neurodevelopmental outcome. This study highlights the importance of CMA testing and cardiac evaluation as part of a routine screen.

Posterior fossa tumors in children: developmental anatomy and diagnostic imaging.

INTRODUCTION: Modern understanding of the relation between the mutated cancer stem cell and its site of origin and of its interaction with the tissue environment is enhancing the importance of developmental anatomy in the diagnostic assessment of posterior fossa tumors in children. The aim of this review is to show how MR imaging can improve on the exact identification of the tumors in the brainstem and in the vicinity of the fourth ventricle in children, using both structural imaging data and a precise topographical assessment guided by the developmental anatomy. RESULTS: The development of the hindbrain results from complex processes of brainstem segmentation, ventro-dorsal patterning, multiple germinative zones, and diverse migration pathways of the neural progenitors. Depending on their origin in the brainstem, gliomas may be infiltrative or not, as well as overwhelmingly malignant (pons), or mostly benign (cervicomedullary, medullo-pontine tegmental, gliomas of the cerebellar peduncles). In the vicinity of the fourth ventricles, the prognosis of the medulloblastomas (MB) correlates the molecular subtyping as well as the site of origin: WNT MB develop from the Wnt-expressing lower rhombic lip and have a good prognosis; SHH MB develop from the Shh-modulated cerebellar cortex with an intermediate prognosis (dependent on age); recurrences are local mostly. The poor prognosis group 3 MB is radiologically heterogeneous: some tumors present classic features but are juxtaventricular (rather than intraventricular); others have highly malignant features with a small principal tumor and an early dissemination. Group 4 MB has classic features, but characteristically usually does not enhance; dissemination is common. Although there is as yet no clear molecular subgrouping of the ependymomas, their sites of origin and their development can be clearly categorized, as most develop in an exophytic way from the ventricular surface of the medulla in clearly specific locations: the obex region with expansion in the cistern magna, or the lateral recess region with expansion in the CPA and prepontine cisterns (cerebellar ependymomas, and still more intra-brainstem ependymomas are rare). Finally, almost all cerebellar gliomas are pilocytic astrocytomas. CONCLUSIONS: A developmental and anatomic approach to the posterior fossa tumors in children (together with diffusion imaging data) provides a reliable pre-surgical identification of the tumor and of its aggressiveness.