Fetal head and neck tumors.

Imaging plays a leading role in detection and diagnosis of fetal head and neck lesions. These lesions comprise a heterogeneous group of congenital tumors and malformations. Complementary imaging modalities that can be used in prenatal medicine are ultrasound and MRI.

Imaging of Pediatric Hearing Loss.

Temporal bone high-resolution computed tomography (HRCT) and magnetic resonance (MR) imaging are valuable tools in the evaluation of pediatric hearing loss. Computed tomography is important in the evaluation of pediatric conductive hearing loss and is the imaging modality of choice for evaluation of osseous abnormalities. MR imaging is the modality of choice for evaluation of sensorineural hearing loss.

Neurologic challenges in 22q11.2 deletion syndrome.

Children with 22q11.2 deletion syndrome often come to medical attention due to signs and symptoms of neurologic dysfunction. It is imperative to understand the expected neurologic development of patients with this diagnosis in order to be alert for the potential neurologic complications, including cortical malformations, tethered cord, epilepsy, and movement disorders.

N-acetylaspartate supports the energetic demands of developmental myelination via oligodendroglial aspartoacylase.

Breakdown of neuro-glial N-acetyl-aspartate (NAA) metabolism results in the failure of developmental myelination, manifest in the congenital pediatric leukodystrophy Canavan disease caused by mutations to the sole NAA catabolizing enzyme aspartoacylase. Canavan disease is a major point of focus for efforts to define NAA function, with available evidence suggesting NAA serves as an acetyl donor for fatty acid synthesis during myelination. Elevated NAA is a diagnostic hallmark of Canavan disease, which contrasts with a broad spectrum of alternative neurodegenerative contexts in which levels of NAA are inversely proportional to pathological progression.

Risk of optic pathway glioma in children with neurofibromatosis type 1 and optic nerve tortuosity or nerve sheath thickening.

Background/aims: Optic nerve tortuosity and nerve and sheath thickening are observed on MRI in some patients with neurofibromatosis type 1 (NF-1). This study aimed to determine if tortuosity and thickening are associated with the development of optic pathway glioma (OPG) and subsequent vision loss.

Methods: Children with NF-1 who underwent brain MRI between 1992 and 2005, and had at least 1 year of subsequent visual acuity (VA) follow-up, were identified retrospectively.

Morphology of the foramen magnum in syndromic and non-syndromic brachycephaly.

Purpose: The shape and size of the foramen magnum (FM) can be altered in craniosynostoses. However, few studies have investigated these changes. In this paper, we investigate the morphology of the foramen magnum in syndromic and non-syndromic brachycephaly.

Childhood encephalitis: relationship between diffusion abnormalities and clinical outcome.

Introduction: The impact of restricted diffusion on clinical outcome has not been well studied in childhood encephalitis. We hypothesized that the patients with lesions with restricted diffusion (LRD) would have worse clinical outcome.

Methods: We reviewed the MR studies of 83 children with encephalitis for LRD.

Imaging findings of patients with metastatic neuroblastoma to the brain.

Rationale And Objectives: Metastatic involvement of brain is rare in neuroblastoma (NB). We retrospectively evaluated conventional and advanced imaging and clinical findings of seven patients with secondary intra-axial brain NB metastases.

Materials And Methods: Magnetic resonance imaging and computed tomography examinations of patients with metastatic brain NB were reviewed.

Functional outcome measures for NF1-associated optic pathway glioma clinical trials.

Objective: The goal of the Response Evaluation in Neurofibromatosis and Schwannomatosis Visual Outcomes Committee is to define the best functional outcome measures for future neurofibromatosis type 1 (NF1)-associated optic pathway glioma (OPG) clinical trials.

Methods: The committee considered the components of vision, other ophthalmologic parameters affected by OPG, potential biomarkers of visual function, and quality of life measures to arrive at consensus-based, evidence-driven recommendations for objective and measurable functional endpoints for OPG trials.

Results: Visual acuity (VA) assessments using consistent quantitative testing methods are recommended as the main functional outcome measure for NF1-OPG clinical trials.

Unilateral cochlear nerve deficiency in children.

Objective: Cochlear nerve deficiency (CND) is increasingly diagnosed in children with sensorineural hearing loss (SNHL). We sought to determine the prevalence of CND, its imaging characteristics, and correlations with audiologic phenotype in children with unilateral SNHL.

Design: Case series with chart review.

Hemizygous mutations in SNAP29 unmask autosomal recessive conditions and contribute to atypical findings in patients with 22q11.2DS.

Background: 22q11.2 deletion syndrome (22q11.2DS) is the most common microdeletion disorder, affecting an estimated 1 : 2000-4000 live births.

Fetal MRI: head and neck.

Abnormalities of the fetal head and neck may be seen in isolation or in association with central nervous system abnormalities, chromosomal abnormalities, and syndromes. Magnetic resonance imaging (MRI) plays an important role in detecting associated abnormalities of the brain as well as in evaluating for airway obstruction that may impact prenatal management and delivery planning. This article provides an overview of the common indications for MRI of the fetal head and neck, including abnormalities of the fetal skull and face, masses of the face and neck, and fetal goiter.

Magnetic resonance imaging of the pediatric neck: an overview.

Evaluation of neck lesions in the pediatric population can be a diagnostic challenge, for which magnetic resonance (MR) imaging is extremely valuable. This article provides an overview of the value and utility of MR imaging in the evaluation of pediatric neck lesions, addressing what the referring clinician requires from the radiologist. Concise descriptions and illustrations of MR imaging findings of commonly encountered pathologic entities in the pediatric neck, including abnormalities of the branchial apparatus, thyroglossal duct anomalies, and neoplastic processes, are given.

Prenatal MR imaging of Dandy-Walker complex: midline sagittal area analysis.

Objective: To measure the mid-sagittal areas of vermis (VA) and of posterior fossa (PFA) and determine their differences among fetuses with various Dandy-Walker (DW) entities and control subjects.

Methods: We reviewed data in 25 fetal patients with a MR diagnosis of DW complex including hypoplastic vermis (HV), HV with rotation (HVR), and mega cistern magna (MCM), and in 85 fetal controls with normal CNS. PFA and VA of each subject were manually traced on mid-sagittal MR images.

High prevalence of temporomandibular joint arthritis at disease onset in children with juvenile idiopathic arthritis, as detected by magnetic resonance imaging but not by ultrasound.

Objective: To determine the prevalence of temporomandibular joint (TMJ) disease in a cohort of children with new-onset juvenile idiopathic arthritis (JIA), and to compare magnetic resonance imaging (MRI) with ultrasound (US) for the detection of acute and chronic changes of TMJ arthritis.

Methods: Between January 2005 and April 2007, children with newly diagnosed JIA were prospectively evaluated for TMJ arthritis. Prior to imaging, jaw pain and disability were assessed with questionnaires and physical examination.

Imaging findings of neonatal herpes simplex virus type 2 encephalitis.

Introduction: The CT, MR, and diffusion-weighted initial and follow-up imaging findings in neonatal herpes simplex virus type 2 (HSV-2) encephalitis were assessed.

Methods: The clinical, laboratory and imaging findings in 12 patients (eight girls and four boys) with proven neonatal HSV-2 encephalitis with follow-up were retrospectively reviewed. Patterns of brain involvement and distribution of lesions were studied and the contribution of diffusion-weighted imaging to the imaging diagnosis of this disease was explored.

Neuroimaging evaluation of cerebral palsy.

MRI can demonstrate and differentiate the various insults and anomalies that can be responsible for cerebral palsy. Recent advances have resulted in techniques and sequences that allow prompt detection of cytotoxic edema and evaluation of brain perfusion. MRI precisely demonstrates the various patterns of injury, distinguishing insults owing to profound asphyxia, partial prolonged asphyxia, and mixed partial prolonged and profound asphyxia.

3.0 T versus 1.5 T pediatric brain imaging.

This article represents a review of the authors' experience with two 3.0 T Siemens Trio Whole Body MR imaging units, with a cumulative experience of 12 months total imaging time on these scanners, over 1000 cases. The authors were able to identify and review numerous patients who had diagnostic studies both on 1.

Fetal posterior fossa volume: assessment with MR imaging.

Purpose: To retrospectively determine the relationship between posterior fossa volume (PFV) and estimated gestational age (EGA) and/or femur length (FL) during pregnancy for the purpose of developing a normal growth curve.

Materials And Methods: Advance institutional review board approval was obtained for this HIPAA-compliant study, and the need for parent informed consent was waived. A cross-sectional retrospective study was performed to measure PFV on in vivo magnetic resonance (MR) images obtained in 76 fetuses of 18-36 weeks gestation who had a morphologically normal CNS.

Diffusion-weighted MR imaging of early methotrexate-related neurotoxicity in children.

Background And Purpose: Methotrexate is a major cause of treatment-related acute neurotoxicity in children with hematologic malignancies. The purpose of this study was to investigate whether diffusion-weighted MR imaging (DWI) detects acute methotrexate white matter neurotoxicity in this patient population.

Methods: Six children-three female and three male-with hematologic malignancies were studied at time of onset of neurologic dysfunction during the delayed intensification or consolidation phase of therapy, when intensive intrathecal methotrexate is given.

Magnetic resonance evaluation of fetal ventriculomegaly-associated congenital malformations and lesions.

Fetal MRI provides diagnostic quality images of the brain which permit differentiation between the various etiologies of ventriculomegaly: hydrocephalus, congenital malformation, and destructive processes.