SMARCA4-deficient primary bone sarcoma with "teratoid" features in a rhabdoid tumor predisposition syndrome patient.

SMARCA4 is a catalytic subunit of the SWItch/sucrose non-fermentable (SWI/SNF) complex. Truncating SMARCA4 germline pathogenic variants (PVs) lead to rhabdoid tumor predisposition syndrome type 2 (RTPS2), associated with small cell carcinoma of ovary hypercalcemic type (SCCOHT) and pediatric rhabdoid tumors. To our knowledge, no primary bone neoplasm with SMARCA4 loss is reported in the literature.

Low Pretreatment Bone Mineral Density in Gender Diverse Youth.

Gender diverse adolescents have low pretreatment bone mineral density (BMD), with variable changes in BMD after initiation of gender-affirming treatment. We aimed to assess factors associated with low BMD in gender diverse youth. Sixty-four patients were included in our analysis (73% assigned male at birth).

Emerging Trends in Radionuclide Imaging of Infection and Inflammation in Pediatrics: Focus on FDG PET/CT and Immune Reactivity.

The most common indication for F-FDG PET/CT is tumor imaging, which may be performed for initial diagnosis, staging, therapeutic response monitoring, surveillance, or suspected recurrence. In the routine practice of pediatric nuclear medicine, most infectious, inflammatory, and autoimmune processes that are detected on F-FDG PET/CT imaging - except for imaging in fever or inflammation of unknown origin - are coincidental and not the main indication for image acquisition. However, interpreting these "coincidental" findings is of utmost importance to avoid erroneously attributing these findings to a neoplastic process.

Radioiodine treatment of pediatric Graves disease: a multicenter review.

Background: There is no standardized approach to iodine-131 (I-131) therapy of hyperthyroidism in pediatric Graves disease. This prevents systematic study of outcomes.

Objective: To characterize current radioiodine dosing and define therapeutic outcomes at multiple institutions that use ultrasound to measure thyroid size to guide I-131 ablation of Graves disease.

Hemorrhagic Transformation Following Childhood Cardioembolic Stroke Is Not Increased in Anticoagulated Patients.

Objective: To characterize the risk of hemorrhagic transformation following cardioembolic stroke in childhood, and whether anticoagulation impacts that risk.

Methods: Ninety-five children (1 month-18 years) with cardioembolic arterial ischemic stroke between January 1, 2009, and December 31, 2019, at 2 institutions were identified for retrospective chart review. Neuroimaging was reviewed to assess for hemorrhagic transformation.

Management of Pediatric Graves Disease: A Review.

Importance: The incidence of Graves disease (GD) is rising in children, and adequate care of these patients requires a multidisciplinary approach. Whether patients are seen in the context of endocrinology, nuclear medicine, or surgery, it is important to know the nuances of the therapeutic options in children.

Observations: Given the rarity of GD in children, it is important to recognize its various clinical presenting signs and symptoms, as well as the tests that may be important for diagnosis.

Cerebral Visual Impairment Characterized by Abnormal Visual Orienting Behavior With Preserved Visual Cortical Activation.

Purpose: Children with cerebral visual impairment (CVI) often have abnormal visual orienting behaviors due to impaired or damaged visual cortex. Alternatively, visual-cortical function is intact but visual information is not transformed downstream into an appropriate oculomotor output (visuomotor dysfunction). We examined visual, anatomic, and oculomotor assessments to distinguish visuomotor dysfunction from CVI associated with severely reduced visual-cortical response.

Pediatric hyperparathyroidism: review and imaging update.

Hyperparathyroidism, due to increased secretion of parathyroid hormones, may be primary, secondary or tertiary. Most pediatric patients with sporadic primary hyperparathyroidism will be symptomatic, presenting with either end-organ damage or nonspecific symptoms. In younger patients with primary hyperparathyroidism, there is a higher prevalence of familial hyperparathyroidism including germline inactivating mutations of the calcium-sensing receptor genes that result in either neonatal severe hyperparathyroidism or familial hypocalciuric hypercalcemia.

Management of Hydrocephalus in Children: Anatomic Imaging Appearances of CSF Shunts and Their Complications.

This article addresses the management of hydrocephalus and the CSF shunts used to treat this entity. CSF shunts have a high failure rate. Imaging plays a pivotal role in assessing CSF shunt failure and determining the need for surgical revision.

Deep medullary vein engorgement and superficial medullary vein engorgement: two patterns of perinatal venous stroke.

Perinatal venous stroke has classically been attributed to cerebral sinovenous thrombosis with resultant congestion or thrombosis of the small veins draining the cerebrum. Advances in brain MRI, in particular susceptibility-weighted imaging, have enabled the visualization of the engorged small intracerebral veins, and the spectrum of perinatal venous stroke has expanded to include isolated congestion or thrombosis of the deep medullary veins and the superficial intracerebral veins. Congestion or thrombosis of the deep medullary veins or the superficial intracerebral veins can result in vasogenic edema, cytotoxic edema or hemorrhage in the territory of disrupted venous flow.

Complications of CSF Shunts in Pediatrics: Functional Assessment With CSF Shunt Scintigraphy-Performance and Interpretation.

The purpose of this article is to review the performance method and criteria for interpretation of CSF shunt scintigraphy studies. Interpretation of CSF shunt scintigraphy studies requires an in-depth understanding of hydrocephalus, the functioning of CSF shunts and their components, and the mechanisms of failure of such devices. Application of strict interpretive criteria when evaluating CSF shunt scintigraphy studies improves diagnostic yield, providing valuable functional information to the neurosurgical team who manages patients with shunted hydrocephalus.

Imaging of Horner syndrome in pediatrics: association with neuroblastoma.

Neuroblastoma is the most common neoplasm associated with pediatric Horner syndrome. The laboratory and imaging evaluation of isolated pediatric Horner syndrome is controversial. We review the literature published in the last several decades and present the rationale for the imaging work-up in this patient cohort.

Rapid Magnetic Resonance Imaging of the Spine in Neonates with Spinal Dysraphism.

Background: The use of nonsedated T2-weighted Half-Fourier Acquisition Single-shot Turbo spin Echo magnetic resonance imaging (MRI) sequences in screening for spinal cord syrinx in neonates with spinal dysraphism has not been reported in the literature. We sought to review our experience using T2-weighted Half-Fourier Acquisition Single-shot Turbo spin Echo imaging of the spine (i.e.

Dual-energy X-ray absorptiometry bone densitometry in pediatrics: a practical review and update.

The assessment of pediatric bone mineral content and density is an evolving field. In this manuscript we provide a practical review and update on the interpretation of dual-energy X-ray absorptiometry (DXA) in pediatrics including historical perspectives as well as a discussion of the recently published 2019 Official Position Statements of the International Society of Clinical Densitometry (ISCD) that apply to children.

Clarifying radiology's role in safety events: a 5-year retrospective common cause analysis of safety events at a pediatric hospital.

Background: Common cause analysis of hospital safety events that involve radiology can identify opportunities to improve quality of care and patient safety.

Objective: To study the most frequent system failures as well as key activities and processes identified in safety events in an academic children's hospital that underwent root cause analysis and in which radiology was determined to play a contributing role.

Materials And Methods: All safety events involving diagnostic or interventional radiology from April 2013 to November 2018, for which the hospital patient safety department conducted root cause analysis, were retrospectively analyzed.

PET with F-Fluorodeoxyglucose/Computed Tomography in the Management of Pediatric Sarcoma.

The role for PET with fludeoxyglucose F 18 (F-FDG PET)/computed tomography (CT) in the management of pediatric sarcomas continues to be controversial. The literature supports a role for PET/CT in the staging and surveillance of certain specific pediatric sarcoma subtypes; however, the data are less clear regarding whether PET/CT can be used as a biomarker for prognostication. Despite the interest in using this imaging modality in the management of pediatric sarcomas, most studies are limited by retrospective design and small sample size.

Reliability of Measuring Insertion Depth in Cochlear Implanted Infants and Children Using Cochlear View Radiography.

Objectives: Cochlear implant depth of insertion affects audiologic outcomes and can be measured in adults using plain films obtained in the "cochlear view." The objective of this study was to assess interrater and intrarater reliability of measuring depth of insertion using cochlear view radiography.

Study Design: Prospective, observational.

Initial treatment of pediatric differentiated thyroid cancer: a review of the current risk-adaptive approach.

Differentiated thyroid cancer in children is a rare disease, accounting for only 1.4% of all pediatric malignancies. The diagnosis, biological behavior and treatment of differentiated thyroid cancer in children is different from that in adults.

Utility of 18F-FDG PET/CT in Infantile Myofibromatosis.

Infantile myofibromatosis, a rare, nonmalignant disease seen almost exclusively in the pediatric population, can involve skin, muscle, soft tissues, bone, or viscera in either solitary or multicentric pattern. Although nonmalignant, visceral involvement in infantile myofibromatosis is a key prognostic indicator, which is associated with mortality in 75% of patients. Those with pulmonary involvement have a particularly poor outcome.

Glial injury in neurotoxicity after pediatric CD19-directed chimeric antigen receptor T cell therapy.

Objective: To test whether systemic cytokine release is associated with central nervous system inflammatory responses and glial injury in immune effector cell-associated neurotoxicity syndrome (ICANS) after chimeric antigen receptor (CAR)-T cell therapy in children and young adults.

Methods: We performed a prospective cohort study of clinical manifestations as well as imaging, pathology, CSF, and blood biomarkers on 43 subjects ages 1 to 25 who received CD19-directed CAR/T cells for acute lymphoblastic leukemia (ALL).

Results: Neurotoxicity occurred in 19 of 43 (44%) subjects.

Pediatric Musculoskeletal Imaging: The Indications for and Applications of PET/Computed Tomography.

The use of PET/computed tomography (CT) for the evaluation and management of children, adolescents, and young adults continues to expand. The principal tracer used is 18F-fluorodeoxyglucose and the principal indication is oncology, particularly musculoskeletal neoplasms. The purpose of this article is to review the common applications of PET/CT for imaging of musculoskeletal issues in pediatrics and to introduce the use of PET/CT for nononcologic issues, such as infectious/inflammatory disorders, and review the use of 18F-sodium fluoride in trauma and sports-related injuries.

Two signs indicative of successful access in nuclear medicine cerebrospinal fluid diversionary shunt studies.

Background: Successful shunt access is the first step in a properly performed nuclear medicine cerebrospinal fluid (CSF) shunt study.

Objective: To determine the significance of the radiotracer configuration at the injection site during initial nuclear medicine CSF shunt imaging and the lack of early systemic radiotracer activity as predictors of successful shunt access.

Materials And Methods: With Institutional Review Board approval, three nuclear medicine physicians performed a retrospective review of all consecutive CSF shunt studies performed in children at our institution in 2015.