Response assessment in pediatric neurooncology (RAPNO) criteria revisited: a practical navigation guide for neuroradiologists.

The Response Assessment in Pediatric Neuro-Oncology (RAPNO) Working Group is an international, collaborative network of experts dedicated to pediatric central nervous system (CNS) tumors that was created in 2011. Since then, six RAPNO articles with imaging guidelines for response assessment in diverse pediatric tumor subgroups have been published, namely: 1) medulloblastomas and leptomeningeal seeding tumors (2018), 2) pediatric high-grade gliomas (2020), 3) pediatric low-grade gliomas (2020), 4) diffuse intrinsic pontine gliomas (2020), 5) pediatric intracranial ependymomas (2022) and 6) pediatric craniopharyngiomas (2023). The purpose of this article is to review all current available RAPNO criteria using a systematized and comparative approach centered on the role of neuroradiologists and supported by neuroimaging examples.

Prenatal Imaging of Supratentorial Fetal Brain Malformation.

This review article provides a comprehensive overview of fetal MR imaging in supratentorial cerebral malformations. It emphasizes the importance of fetal MR imaging as an adjunct diagnostic tool used alongside ultrasound, improving the detection and characterization of prenatal brain abnormalities. This article reviews a spectrum of cerebral malformations, their MR imaging features, and the clinical implications of these findings.

Incomplete partition type II in its various manifestations: isolated, in association with EVA, syndromic, and beyond; a multicentre international study.

Purpose: Incomplete partition type II (IP-II) is characterized by specific histological features and radiological appearance. It may occur in isolation or in association with an enlarged vestibular aqueduct (EVA). Among those with IP-II and EVA, a subset has a diagnosis of Pendred syndrome.

ACR Appropriateness Criteria® Orbital Imaging and Vision Loss-Child.

Orbital disorders in children consist of varied pathologies affecting the orbits, orbital contents, visual pathway, and innervation of the extraocular or intraocular muscles. The underlying etiology of these disorders may be traumatic or nontraumatic. Presumed location of the lesion along with the additional findings, such as eye pain, swelling, exophthalmos/enophthalmos, erythema, conjunctival vascular dilatation, intraocular pressure, etc, help in determining if imaging is needed, modality of choice, and extent of coverage (orbits and/or head).

Classification and neuroimaging of ependymal tumors.

Ependymal tumors arise from the ependymal cell remnants of the cerebral ventricles, the central canal of the spinal cord, or the filum terminale or conus medullaris, although most pediatric supratentorial ependymomas do not exhibit clear communication or abutment of the ventricles. In this article, we discuss the classification, imaging characteristics, and clinical settings of these tumors. The WHO 2021 classification system has categorized ependymal tumors based on histopathologic and molecular features and location, in which they are grouped as supratentorial, posterior fossa (PF), and spinal.

Isolated Sixth Nerve Palsies in a Child With Familial Hemophagocytic Lymphohistiocytosis Type 2.

A previously healthy 2-year-old boy presented with a left sixth cranial nerve palsy. There was a family history of multiple sclerosis and optic neuritis. Neuroimaging showed multiple foci of T2/FLAIR hyperintense signal abnormality in both cerebral hemispheres and in the brainstem.

Imaging of pediatric brain tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee/ASPNR White Paper.

Tumors of the central nervous system are the most common solid malignancies in children and the most common cause of pediatric cancer-related mortality. Imaging plays a central role in diagnosis, staging, treatment planning, and response assessment of pediatric brain tumors. However, the substantial variability in brain tumor imaging protocols across institutions leads to variability in patient risk stratification and treatment decisions, and complicates comparisons of clinical trial results.

Fetal Brain Anatomy.

"Fetal brain development has been well studied, allowing for an ample knowledge of the normal changes that occur during gestation. Imaging modalities used to evaluate the fetal central nervous system (CNS) include ultrasound and MRI. MRI is the most accurate imaging modality for parenchymal evaluation and depiction of developmental CNS anomalies.

Simultaneously Acquired MRI Arterial Spin-Labeling and Interictal FDG-PET Improves Diagnosis of Pediatric Temporal Lobe Epilepsy.

Background And Purpose: Interictal FDG-PET scans are a routine diagnostic technique for the identification of epileptogenic foci in the presurgical work-up of medically refractory pediatric epilepsy. With the advent of PET/MR imaging, it has become possible to simultaneously acquire FDG-PET and arterial spin-labeling perfusion data. The objective of this study was to evaluate whether the incorporation of arterial spin-labeling data with interictal FDG-PET could improve the diagnostic performance metrics of FDG-PET for identification of epileptogenic foci.

Attention-guided deep learning for gestational age prediction using fetal brain MRI.

Magnetic resonance imaging offers unrivaled visualization of the fetal brain, forming the basis for establishing age-specific morphologic milestones. However, gauging age-appropriate neural development remains a difficult task due to the constantly changing appearance of the fetal brain, variable image quality, and frequent motion artifacts. Here we present an end-to-end, attention-guided deep learning model that predicts gestational age with R score of 0.

Neuroradiological Mimics of Periventricular Leukomalacia.

Aim: Periventricular leukomalacia (PVL) is a term reserved to describe white matter injury in the premature brain. In this review article, the authors highlight the common and rare pathologies mimicking the chronic stage of PVL and propose practical clinico-radiological criteria that would aid in diagnosis and management.

Methods And Results: The authors first describe the typical brain MRI (magnetic resonance imaging) features of PVL.

Neuroimaging manifestations of epidermal nevus syndrome.

Epidermal nevus syndrome (ENS) represents a diverse group of rare neurocutaneous diseases associated with the presence of characteristic epidermal nevi (EN) in the skin and extracutaneous manifestations in the eyes, skeletal, urogenital and central nervous systems. We present a case series of 7 children with ENS, with specific attention to the neuroradiological characteristics of this entity.

Magnetic resonance imaging of the fetal musculoskeletal system.

Diagnosing musculoskeletal pathology requires understanding of the normal embryological development. Intrinsic errors of skeletal development are individually rare but are of paramount clinical importance because anomalies can greatly impact patients' lives. An accurate assessment of the fetal musculoskeletal system must be performed to provide optimal genetic counseling as well as to drive therapeutic management.

Imaging phenotype correlation with molecular and molecular pathway defects in malformations of cortical development.

The increase in understanding of molecular biology and recent advances in genetic testing have caused rapid growth in knowledge of genetic causes of malformations of cortical development. Imaging diagnosis of malformations of cortical development can be made prenatally in a large subset of fetuses based on the presence of specific deviations from the normal pattern of development, characteristic imaging features, and associated non-central-nervous-system (CNS) abnormalities. In this review the authors discuss the role of four key cell molecules/molecular pathways in corticogenesis that are frequently implicated in complex prenatally diagnosed malformations of cortical development.

Characteristic Cochlear Hypoplasia in Patients with Walker-Warburg Syndrome: A Radiologic Study of the Inner Ear in α-Dystroglycan-Related Muscular Disorders.

Background And Purpose: Walker-Warburg syndrome, muscle-eye-brain disease, and Fukuyama congenital muscular dystrophy are α-dystroglycan-related muscular disorders associated with brain malformations and eye abnormalities in which no structural inner ear abnormality has been described radiologically. We collected patients from 6 tertiary pediatric hospitals and reported the radiologic features and frequency of inner ear dysplasias.

Materials And Methods: Patients previously diagnosed clinicoradiologically with Walker-Warburg syndrome, muscle-eye-brain disease, or Fukuyama congenital muscular dystrophy were included.

Diagnostic accuracy of colposcopic examination in patients with oral dysplastic lesions.

Objective: Colposcopy is a direct microscopic method and is the gold standard tool to detect early cervical dysplastic lesions. In the past, many attempts have been made to use gynecologic methods to examine the oral mucosa. The aim of this study was to detect the diagnostic accuracy of oral colposcopy in diagnosing oral dysplastic lesions in comparison with microscopic evaluation based on biopsy and compare Reid's Colposcopic Index (RCI) and the Swede scoring system in diagnosing oral dysplastic lesions.

Rare Saposin A deficiency: Novel variant and psychosine analysis.

Saposin A is a post-translation product of the prosaposin (PSAP) gene that serves as an activator protein of the galactocerebrosidase (GALC) enzyme, and is necessary for the degradation of certain glycosphingolipids. Deficiency of saposin A leads to a clinical picture identical to that of early-infantile Krabbe disease caused by GALC enzyme deficiency. Galactosylsphingosine, also known as psychosine, is a substrate of the GALC enzyme that is known to be elevated in classic Krabbe disease.

Imaging of temporal bone inflammations in children: a pictorial review.

Purpose: Understanding the underlying pathophysiology and the patterns of disease spread is crucial in accurate image interpretation. In this pictorial review, the common and important inflammatory processes of the temporal bone in children will be discussed, and key computed tomography (CT) and magnetic resonance imaging (MRI) features described.

Methods: Inflammatory processes are categorized by anatomical location: the petrous apex and the inner, middle and outer ear.

Pediatric Central Nervous System Imaging of Nonaccidental Trauma: Beyond Subdural Hematomas.

Infants and children under 2 years of age are at greatest risk for devastating neurologic complications following nonaccidental trauma. While a subdural hematoma (SDH) is the most common finding and is often enough to raise suspicion for abuse, no single injury is pathognomonic for abusive head trauma (AHT). Rather, the combination of imaging and physical findings and the clinical presentation help confirm the diagnosis of AHT.

Lobular capillary hemangioma of the mandible: A case report.

Lobular capillary hemangiomas are acquired benign vascular neoplasms which typically affect the skin and mucous membranes. While these lesions commonly involve the head and neck, particularly the oral cavity, there are no reports in the literature of lobular capillary hemangioma arising from the mandible. The diagnosis of such a rare entity can therefore be challenging, especially as it may mimic more aggressive lesions, including malignancy.

Pretransplant functional imaging and outcome in pediatric patients with relapsed/refractory Hodgkin lymphoma undergoing autologous transplantation.

Background: Pretransplant functional imaging (FI), particularly a negative positron emission tomography (PET), is a strong predictor of outcome in adults with relapsed or refractory Hodgkin lymphoma (HL), but data in pediatrics are limited.

Methods: The medical records of 49 consecutive pediatric patients, who received autologous transplant at a single institution, were retrospectively analyzed. All patients had either gallium or PET scan before transplant and were conditioned with carmustine, etoposide, cytarabine, and melphalan (BEAM).