Changes to pediatric brain tumors in 2021 World Health Organization classification of tumors of the central nervous system.

New tumor types are continuously being described with advances in molecular testing and genomic analysis resulting in better prognostics, new targeted therapy options and improved patient outcomes. As a result of these advances, pathological classification of tumors is periodically updated with new editions of the World Health Organization (WHO) Classification of Tumors books. In 2021, WHO Classification of Tumors of the Central Nervous System, 5th edition (CNS5), was published with major changes in pediatric brain tumors officially recognized including pediatric gliomas being separated from adult gliomas, ependymomas being categorized based on anatomical compartment and many new tumor types, most of them seen in children. Additional general changes, such as tumor grading now being done within tumor types rather than across entities and changes in definition of glioblastoma, are also relevant to pediatric neuro-oncology practice. The purpose of this manuscript is to highlight the major changes in pediatric brain tumors in CNS5 most relevant to radiologists. Additionally, brief descriptions of newly recognized entities will be presented with a focus on imaging findings.

Isolated Right Common Carotid Artery Arising from the Right Pulmonary Artery.

Isolated aortic arch vessels arising anomalously from the pulmonary arterial system are rare congenital anomalies. Case reports of isolated arch vessels are often associated with 22q11 deletion, CHARGE syndrome, or right aortic arch. Isolation of the carotid artery may lead to cerebral steal phenomenon and ischemia or to pulmonary overcirculation. The authors report what is, to their knowledge, the first case of isolated right common carotid artery arising from the right pulmonary artery, associated with 22q11 deletion, and describe the challenging multimodality image evaluation. Keywords: Congenital, Anatomy, Carotid Arteries © RSNA, 2022.

A unique cardiovascular presentation of Marfan syndrome.

We report a neonate with severe Marfan syndrome (MS), prenatally identified to have persistent atrial tachycardia, biventricular dysfunction, and an unusual structure within the atria. Detailed postnatal echocardiographic evaluation and cross-sectional imaging confirmed congenital pseudoaneurysm of the mitral-aortic intervalvular fibrosa. Emergent testing by next-generation sequencing identified a FBN1 pathological variant, key to establishing goals of care. To our knowledge, this is the first reported case of a congenital pseudoaneurysm of the mitral-aortic intervalvular fibrosa in MS.

Magnetic resonance imaging of children following esophageal button battery removal: What are we looking for?

Despite society recommendations that cross-sectional imaging be obtained following removal of ingested button batteries, there is no published consensus on how it effectively guides clinical management. This single institution survey demonstrates a lack of uniformity by clinicians regarding which imaging findings impact management decisions, highlighting the need for further guidelines.

Pearls and Pitfalls of Thoracic Manifestations of Abuse in Children.

Child abuse is a broad term that includes, but is not limited to, physical or emotional harm, neglect, sexual abuse, and exploitation. In 2018 in the United States, there were nearly 700,000 victims of such maltreatment, of which 1700 children died. The majority of deaths occur in infants and toddlers under 3 years of age. While clinical signs and symptoms may raise suspicion for inflicted injury, such as bruising in young infants, imaging often plays a central role in identifying and characterizing nonaccidental trauma. The purpose of this article is to discuss the array of inflicted traumatic injuries to the thorax in children. Rib fractures are among the most common and telling features of physical abuse, especially in infants. The locations of such fractures and differences in appearance while healing will be presented, along with potential mimics and pitfalls. Less typical fractures seen in abuse will also be reviewed, including those of the sternum, clavicle, spine, and scapula. Finally, uncommon injuries to the lungs, heart and esophagus will also be considered.

The current and future roles of artificial intelligence in pediatric radiology.

Artificial intelligence (AI) is a broad and complicated concept that has begun to affect many areas of medicine, perhaps none so much as radiology. While pediatric radiology has been less affected than other radiology subspecialties, there are some well-developed and some nascent applications within the field. This review focuses on the use of AI within pediatric radiology for image interpretation, with descriptive summaries of the literature to date. We highlight common features that enable successful application of the technology, along with some of the limitations that can inhibit the development of this field. We present some ideas for further research in this area and challenges that must be overcome, with an understanding that technology often advances in unpredictable ways.

Computed tomography of pulmonary veins: review of congenital and acquired pathologies.

Newer-generation CT scanners with ultrawide detectors or dual sources offer millisecond image acquisition times and significantly decreased radiation doses compared to historical cardiac CT and CT angiography. This technology is capable of nearly freezing cardiac and respiratory motion. As a result, CT is increasingly used for diagnosing and monitoring cardiac and vascular abnormalities in the pediatric population. CT is particularly useful in the setting of pulmonary vein evaluation because it offers evaluation of the entire pulmonary venous system and lung parenchyma. In this article we review a spectrum of congenital and acquired pulmonary venous abnormalities, including potential etiologies, CT imaging findings and important factors of preoperative planning. In addition, we discuss optimization of CT techniques for evaluating the pulmonary veins.

Accuracy and Reliability of 4D-CT and Flexible Laryngoscopy in Upper Airway Evaluation in Robin Sequence.

OBJECTIVES: To evaluate the performance of 4-dimensional computed tomography (4D-CT) in assessing upper airway obstruction (UAO) in patients with Robin sequence (RS) and compare the accuracy and reliability of 4D-CT and flexible fiber-optic laryngoscopy (FFL). STUDY DESIGN: Prospective survey of retrospective clinical data. SETTING: Single, tertiary care pediatric hospital. METHODS: At initial and 30-day time points, a multidisciplinary group of 11 clinicians who treat RS rated UAO severity in 32 sets of 4D-CT visualizations and FFL videos (dynamic modalities) and static CT images. Raters assessed UAO at the velopharynx and oropharynx (1 = none to 5 = complete) and noted confidence levels of each rating. Intraclass correlation and Krippendorff alpha were used to assess intra- and interrater reliability, respectively. Accuracy was assessed by comparing clinician ratings with quantitative percentage constriction (QPC) ratings, calculated based on 4D-CT airway cross-sectional area. Results were compared using Wilcoxon rank-sum and signed-rank tests. RESULTS: There was similar intrarater agreement (moderate to substantial) with 4D-CT and FFL, and both demonstrated fair interrater agreement. Both modalities underestimated UAO severity, although 4D-CT ratings were significantly more accurate, as determined by QPC similarity, than FFL (-1.06 and -1.46 vs QPC ratings, P = .004). Overall confidence levels were similar for 4D-CT and FFL, but other specialists were significantly less confident in FFL ratings than were otolaryngologists (2.25 and 3.92, P < .0001). CONCLUSION: Although 4D-CT may be more accurate in assessing the degree of UAO in patients with RS, 4D-CT and FFL assessments demonstrate similar reliability. Additionally, 4D-CT may be interpreted with greater confidence by nonotolaryngologists who care for these patients.

Pediatric radiologic manifestations of COVID-19.

PURPOSE: While full description of pediatric COVID-19 manifestations is evolving, children appear to present less frequently, and often display a less severe disease phenotype. There is correspondingly less data regarding pediatric radiologic findings. To describe the imaging findings of pediatric COVID-19, we evaluated the radiologic imaging of the initial patient cohort identified at our institution. METHODS: In this IRB approved study, all patients at our institution aged 0-21 with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) based on PCR or immunoglobulin testing were identified. Imaging was reviewed by the co-authors and presence of abnormalities determined by consensus. Pre-existing comorbidities and alternative diagnoses were recorded. Rates of each finding were calculated. Findings were compared to published data following review of the available literature. RESULTS: Out of 130 Covid-19 positive patients, 24 patients underwent imaging, including 21 chest radiographs and 4 chest CT scans. Chest x-rays were normal in 33%. Patchy or streaky opacities were the most common radiographic abnormality, each seen in 38% of patients. CT findings included ill-defined or geographic ground glass opacities, dense opacities, septal thickening and crazy paving, and small pleural effusions. Results are similar to those reported in adults. Multiple COVID-19 positive children presented for symptoms due to an additional acute illness, including appendicitis and urinary infection. CONCLUSIONS: Radiologic findings of COVID-19 in pediatric patients range from normal to severe ARDS type appearance. During this ongoing pandemic, these radiographic signs can be useful for the evaluation of disease status and guiding care, particularly in those with comorbidities. PRECIS: Radiologic findings of COVID-19 in pediatric patients are similar to those seen in adults, and may range from normal to severe ARDS type appearance.

Severe COVID-19 initially presenting as mesenteric adenopathy.

Coronavirus disease 2019 (COVID-19) can present with abdominal pain in children and adults. Most imaging findings have been limited to characteristic lung findings, as well as one report of bowel-ischemia-related findings in adults. We report a case of COVID-19 in a healthy teenager who initially presented with isolated mesenteric adenopathy, typically a self-limited illness, which progressed to severe illness requiring intensive care before complete recovery. The boy tested negative for COVID-19 twice by polymerase chain reaction (PCR) from upper respiratory swabs before sputum PCR resulted positive. A high index of suspicion should be maintained for COVID-19 given the continued emergence of new manifestations of the disease.

Optimization of Pediatric Body CT Angiography: What Radiologists Need to Know.

OBJECTIVE. Pediatric CT angiography (CTA) presents unique challenges compared with adult CTA. Because of the ionizing radiation exposure, CTA should be used judiciously in children. The pearls offered here are observations gleaned from the authors' experience in the use of pediatric CTA. We also present some potential follies to be avoided. CONCLUSION. Understanding the underlying principles and paying meticulous attention to detail can substantially optimize dose and improve the diagnostic quality of pediatric CTA.

Imaging Assessment of Complications from Transplantation from Pediatric to Adult Patients: Part 1: Solid Organ Transplantation.

End-stage organ failure is commonly treated with transplantation of the respective failing organ. Although outcomes have progressively improved over the decades, early and late complications do occur, and are often diagnosed by imaging. Given the increasing survival rates of transplant patients, the general radiologist may encounter these patients in the outpatient setting. Awareness of the normal radiologic findings after transplantation, and imaging findings of the more common complications, is therefore important. We review and illustrate the imaging assessment of complications from lung, liver, and renal transplantation, highlighting the key similarities and differences between pediatric and adult patients.

Imaging Assessment of Complications from Transplantation from Pediatric to Adult Patients: Part 2: Hematopoietic Stem Cell Transplantation.

Hematopoietic stem cell transplantation is an intravenous transfusion of pluripotent stem cells to repopulate the marrow and restore immunocompetence. However, before transplantation, the patient undergoes a conditioning regimen to eradicate the underlying disease, subsequently resulting in an immunocompromised state. Serious and some life-threatening complications involving any organ can occur. Currently, with advances in hematopoietic stem cell transplantation techniques and posttransplant management, more pediatric patients are now living longer and into their adulthood. The goal of this review article is to discuss the common neurologic, pulmonary, and abdominal complications associated with hematopoietic stem cell transplantation with emphasis on their imaging characteristics.

Applications of Pediatric Body CT Angiography: What Radiologists Need to Know.

OBJECTIVE. Pediatric CT angiography (CTA) can be useful for assessing numerous congenital and acquired disorders. This article discusses common pediatric applications of thoracoabdominal CTA, including for congenital pulmonary airway malformation, sequestration, vascular rings, aortic coarctation, pulmonary embolism, nontraumatic hemorrhage, abdominal transplant evaluation, and several vascular disorders, and highlights key clinical and imaging features. CONCLUSION. With appropriate use, CTA can play a fundamental role in diagnostic and preprocedural assessment in a variety of pediatric conditions.

The continuous lure of pediatric radiology.

Pediatric radiology is an immensely rewarding career choice. Eight pediatric radiologists, enthusiastic for their profession, were asked six questions about their career choice. Their responses illustrate the common virtues of pediatric radiology and also demonstrate the diverse paths and activities that pediatric radiologists take and pursue.

Diagnostic performance of Liver Imaging Reporting and Data System (LI-RADS) v2017 in predicting malignant liver lesions in pediatric patients: a preliminary study.

BACKGROUND: Liver Imaging Reporting and Data System (LI-RADS) has standardized the evaluation of hepatic lesions in adults at risk of developing hepatocellular carcinoma (HCC). There is no accepted imaging algorithm for diagnosing HCC in the pediatric population. OBJECTIVE: The aim of our study was to evaluate the diagnostic accuracy and inter-rater reliability of LI-RADS version 2017 (v2017) for diagnosing HCC in a pediatric cohort. MATERIALS AND METHODS: This retrospective, Institutional Review Board-approved study involved review of all abdominal dynamic contrast-enhanced imaging at a tertiary children's hospital during a 10-year period, yielding 151 liver lesions in patients <18 years. Cases with active extrahepatic malignancy or an inadequate reference standard were excluded. Two readers independently evaluated all included hepatic lesions using LI-RADS criteria. Pathology and imaging follow-up were used as reference standards. RESULTS: A total of 41 lesions in 41 patients met criteria for evaluation (3 HCCs, 8 non-HCC malignancies, 30 benign lesions). A LI-RADS designation of definite HCC had high sensitivity (Reader 1/Reader 2: 100%, 95% confidence interval [CI] 31-100%) and high specificity (Reader 1: 84%, 95% CI: 68-93%; Reader 2: 97%, 95% CI: 85-100%) for predicting HCC. However, positive predictive value was only 33% (95% CI: 9-69%) and 75% (95% CI: 22-99%) for Reader 1 and Reader 2, respectively. For predicting any type of hepatic malignancy, a LI-RADS designation of definitely or likely malignant (i.e. not necessarily HCC) had a sensitivity of 100% (95% CI: 74-100%) and 90% (95% CI: 61-100%) for Reader 1 and Reader 2, respectively, and a negative predictive value (NPV) of 100% (95% CI: 81-100%) and 96% (95% CI: 83-99%) for Reader 1 and Reader 2, respectively. Interobserver agreement was substantial for the overall LI-RADS category (weighted κ=0.62; 95% CI: 0.38-0.86). CONCLUSION: The positive predictive value of LI-RADS v2017 for diagnosing HCC was limited by the low frequency of HCC among pediatric patients. However, a LI-RADS designation of definitely or likely malignant had high sensitivity and NPV for any type of hepatic malignancy and may serve to direct clinical management by selecting patients for tissue sampling.