Contrast-Enhanced Ultrasound of the Chest in Children and Adolescents: A Pilot Study for Assessment of Added Diagnostic Value.

OBJECTIVES: To determine the added diagnostic value of contrast-enhanced ultrasound (CEUS) in pediatric chest abnormalities by comparing interpretation of CEUS studies and confidence level to conventional US studies. METHODS: CEUS studies in patients with a variety of clinically suspected chest abnormalities performed between 2016 and 2020 were reviewed and compared to same-day conventional US studies. Examinations were independently interpreted by 4 radiologists blinded to clinical and other imaging data. Rater confidence was classified as low, moderate, or high. Diagnostic accuracy was determined by comparing image interpretation to patient outcome as the ground truth. Interobserver agreement was also assessed. RESULTS: Sixteen patients (10 male) with 18 CEUS studies were included. Median rater agreement with ground truth was significantly higher for CEUS (100%) than conventional US (50%; P = .004). Median rater confidence was high (3.0) for CEUS, and low-moderate (1.5) for conventional US (P < .001). CEUS sensitivity (54.6-81.8%) and specificity (63.4-100.0%) were greater than conventional US (45.5-72.7% and 12.5-63.5%, respectively). CEUS false positives (0-4) and false negatives (2-5) were fewer than conventional US (4-7 and 3-6, respectively). Except for one rater pair where agreement was substantial (κ = .78, P < .01), inter-rater agreement for CEUS for all other rater pairs was nonsignificant (κ = .25-0.51, P ≥ .07). Agreement for conventional US was moderate and statistically significant for 3 rater pairs (κ = .55-0.78) and nonsignificant for the remaining 3 rater pairs (P ≥ .06). CONCLUSIONS: CEUS adds diagnostic value to the assessment of a variety of chest abnormalities. The data support further evaluation of the role of CEUS as a non-invasive, problem-solving technique in children.

Physeal injuries of the clavicle: pediatric counterparts to adult acromioclavicular and sternoclavicular joint separations.

The epiphyses at the medial and lateral ends of the clavicle are small, ossify relatively late, and may not fuse until early adulthood. Because of this unique anatomy, pediatric and young adult injuries that involve the clavicle often differ from the patterns typically seen in older adults. Clavicular trauma that affects the acromioclavicular joint laterally or sternoclavicular joint medially often results in a physeal fracture and as such, can go unrecognized or be mistaken for a joint dislocation. Radiographic assessment is challenging, particularly when the epiphysis is not yet ossified. However, MR imaging allows for visualization of the cartilage, periosteum and perichondrium, and ligaments of the affected joints. Lateral clavicle physeal injuries can be categorized by the Dameron and Rockwood system, the pediatric correlate to the Rockwood classification of adult acromioclavicular joint injuries. Medial clavicle physeal fractures, similar to adult sternoclavicular joint dislocations, may result in anterior or posterior displacement. Because of their great ability to heal and remodel, clavicular physeal fractures respond better to conservative management than true acromioclavicular or sternoclavicular joint dislocations. Therefore, it is essential to recognize the true nature of these injuries, as there are implications for successful treatment and appropriate prognosis.

Isolated leukemia cutis as extramedullary relapse of acute myeloid leukemia.

Acute myeloid leukemia (AML) arises from clonal expansion of malignant hematopoietic precursor cells in the bone marrow. In rare instances, AML can recur with prominent extramedullary manifestations (i.e., leukemia cutis or myeloid sarcoma), either simultaneously or preceding marrow involvement, or as a sole site of primary disease relapse.

Advanced imaging of disease unrelated to the coronavirus disease 2019 (COVID-19) during the pandemic: effect of patient demographics in a pediatric emergency department.

BACKGROUND: Coronavirus disease 2019 (COVID-19) disproportionately affected children from underrepresented minorities and marginalized populations, but little is understood regarding the pandemic's effect on non-COVID-19-related illnesses. OBJECTIVE: To examine the effect of the COVID-19 pandemic and related stay-at-home orders on pediatric emergency department (ED) imaging of non-COVID-19-related diseases across patient demographic groups. MATERIALS AND METHODS: We retrospectively reviewed radiology reports from advanced imaging (US, CT, MRI and fluoroscopy) on children in the ED during the month of April for the years 2017, 2018, 2019 and 2020, excluding imaging for respiratory illness and trauma. We used imaging results and the electronic medical record to identify children with positive diagnoses on advanced imaging, and whether these children were admitted to the hospital. Demographic variables included age, gender, race/ethnicity and insurance type. We used multivariable Poisson regression models to report rate ratio (RR) and binomial logistic regression models to report odds ratio (OR) with 95% confidence interval (CI). RESULTS: We included 1,418 ED encounters for analysis. Compared to pre-2020, fewer children underwent ED imaging in April 2020 (RR 0.63, 95% CI 0.52, 0.76). The odds of positive imaging results increased (OR 2.18, 95% CI 1.59, 3.00) overall, and for all racial/ethnic groups except Hispanic patients (OR 0.83, 95% CI 0.34, 2.03). No differences occurred in admission rates for positive imaging results in 2020 compared to pre-2020. CONCLUSION: In April 2020 compared to pre-2020, there were decreased imaging and increased positivity rates for imaging for non-respiratory and non-trauma ED visits. COVID-19 stay-at-home advisories might have resulted in triaging for urgent health care by families or referring clinicians during this month of the pandemic.

A rare case of papular-purpuric "gloves and socks" syndrome associated with influenza.

Papular-purpuric "gloves and socks" syndrome (PPGSS) is a unique, self-limited dermatosis characterized by edema, erythema, and pruritic petechiae and papules in a distinct "gloves and socks" distribution. This is often accompanied by systemic symptoms, including fever, lymphadenopathy, asthenia, myalgia, and arthralgias. PPGSS has also been described as a manifestation of an underlying immunological mechanism that can be triggered by viral or drug-related antigens. A 32-year-old male developed a painful eruption on the bilateral hands and feet after being diagnosed with influenza B. On examination, scattered papular purpura with occasional overlying scale was noted on the bilateral hands, fingers, feet, toes, volar wrists, and ankles. Histopathologic sections showed a mixed pattern of inflammation with interface and spongiotic changes. A parakeratotic scale with overlying basket-weave orthokeratosis was also seen. Within the epidermis, there was intraepidermal vesicles and Langerhans cell microabscess formation with scattered apoptotic keratinocytes. The underlying dermis showed a superficial perivascular lymphocytic infiltrate with mild edematous changes, and extravasation of red blood cells. Clinicopathologic correlation strongly supported a diagnosis of popular-purpuric gloves and socks syndrome. The influenza virus has never been reported in association with PPGSS; thus, this case outlines an important new variant that clinicians should be familiar with.

Emergency pediatric radiology imaging trends for non-COVID-19-related illnesses through different stages of the pandemic.

PURPOSE: To evaluate how the COVID-19 pandemic affected the imaging utilization patterns for non-COVID-19-related illness in a pediatric emergency department (ED). METHODS: We retrospectively reviewed radiology reports for ultrasound, CT, MRI, and fluoroscopy studies performed at a pediatric ED in April from 2017 to 2021, excluding studies for respiratory symptoms and trauma. Radiology reports and medical records were reviewed to determine if patients had a positive radiology diagnosis, the type of diagnosis, and whether it required hospital admission. Results from during the pandemic were compared to predicted rates based on pre-pandemic years. RESULTS: A total of 2198 imaging studies were included. During the COVID-19 pandemic, fewer ED imaging studies were performed compared to predicted. The decrease was greater in April 2020 (RR = 0.56, p < 0.001) than in April 2021 (RR = 0.80, p = 0.038). The odds of positive diagnosis was higher during the pandemic than before, and higher in 2020 (OR 2.53, p < 0.001) than in 2021 (OR 1.38, p = 0.008). The expected numbers of positive diagnoses and hospital admittances remained within the predicted range during the pandemic (p = 0.505-0.873). CONCLUSIONS: Although imaging volumes decreased during the studied months of the pandemic, the number of positive findings was unchanged compared to prior years. No differences were demonstrated in the percentage of patients admitted to the hospital with positive imaging findings. This suggests that, at our institution, the pandemic did not lead to a substantial number of missed diagnoses or severely delay the diagnosis of non-COVID-related conditions. While still lower than expected, imaging volumes increased in April 2021 suggesting a return towards baseline patient behavior as the pandemic conditions improved.

Multifocal Langerhans Cell Histiocytosis: A Unique Presentation of Eosinophilic Granuloma with Intracranial Extension, Massive Lymphadenopathy, and Epstein-Barr Viral Infection in an Adolescent Female.

We report the case of an otherwise healthy 17-year-old female who presented for surgical removal of an enlarging "atypical cyst" on her scalp. During subtotal excision, only friable serosanguinous translucent ribbons of tissue were found. A histopathologic diagnosis of Langerhans cell histiocytosis (LCH) was rendered and imaging studies revealed extradural invasion of the tumor. Within weeks, the patient also developed progressive lymphadenopathy with grossly elevated levels of Epstein-Barr virus viral capsid antigen antibody levels. This report aims to highlight a unique presentation of LCH with discussion of workup, management, and avoidance of potential surgical complications.

Pediatric dermatology emergencies.

Many pediatric skin conditions can be safely monitored with minimal intervention, but certain skin conditions are emergent and require immediate attention and proper assessment of the neonate, infant, or child. We review the following pediatric dermatology emergencies so that clinicians can detect and accurately diagnose these conditions to avoid delayed treatment and considerable morbidity and mortality if missed: staphylococcal scalded skin syndrome (SSSS), impetigo, eczema herpeticum (EH), Langerhans cell histiocytosis (LCH), infantile hemangioma (IH), and IgA vasculitis.

Spaced radiology: encouraging durable memory using spaced testing in pediatric radiology.

Applied memory research in the field of cognitive and educational psychology has generated a large body of data to support the use of spacing and testing to promote long-term or durable memory. Despite the consensus of this scientific community, most learners, including radiology residents, do not utilize these tools for learning new information. We present a discussion of these parallel and synergistic learning techniques and their incorporation into a software platform, called Spaced Radiology, which we created for teaching radiology residents. Specifically, this software uses these evidence-based strategies to teach pediatric radiology through a flashcard deck system.

Tales from the Night:: Emergency MR Imaging in Pediatric Patients after Hours.

Overnight in-house radiology has rapidly become an important part of contemporary practice models, and is increasingly the norm in pediatric radiology. MR imaging is an indispensable first-line and problem-solving tool in the pediatric population. This has led to increasingly complex MR imaging being performed "after hours" on pediatric patients. This article reviews the factors that have led to widespread overnight subspecialty radiology and the associated challenges for overnight radiologists, and provides an overview of up-to-date imaging techniques and imaging findings of the most common indications for emergent MR imaging in the pediatric population.

Pernio as the presenting sign of blast crisis in acute lymphoblastic leukemia.

A previously healthy 5-year-old girl presented with acute onset of blue toes and red spots on the nose and fingers. The striking nature of these lesions, along with the finding of submandibular lymphadenopathy, prompted further evaluation. Laboratory findings were remarkable for anemia, high transaminase levels, and high blast count. Histopathologic findings were consistent with early pernio. Further examination revealed acute B-cell lymphoblastic leukemia. Treatment of the leukemia led to resolution of the pernio.

Analyzing the Genetic Spectrum of Vascular Anomalies with Overgrowth via Cancer Genomics.

Vascular anomalies are variably associated with overgrowth, skeletal anomalies, and abnormalities of the brain, leptomeninges, and eye. We assembled a 16-institution network to determine the range of genetic variants associated with a spectrum of vascular anomalies with overgrowth, ranging from mild to severe. Because of the overlap between cancer-associated variants and previously described somatic variants in vascular overgrowth syndromes, we employed tumor genetic profiling via high-depth next-generation sequencing using a panel to assay affected tissue from a diverse cohort of subjects with vascular anomalies with overgrowth. Seventy-five percent (43/57) harbored pathogenic or likely pathogenic variants in 10 genes. We identified two genes (mTOR, PIK3R1) and several variants previously described in the setting of cancer but that, to our knowledge, have not been described in vascular malformations. All were identified at low variant allele frequency consistent with somatic mosaic etiology. By leveraging somatic variant detection technology typically applied to cancer in a cohort inclusive of broad phenotypic severity, we demonstrated that most vascular anomalies with overgrowth harbor postzygotic gain-of-function mutations in oncogenes. Furthermore, continued interrogation of oncogenes in benign developmental disorders could provide insight into fundamental mechanisms regulating cell growth.

A common intronic variant of PARP1 confers melanoma risk and mediates melanocyte growth via regulation of MITF.

Previous genome-wide association studies have identified a melanoma-associated locus at 1q42.1 that encompasses a ∼100-kb region spanning the PARP1 gene. Expression quantitative trait locus (eQTL) analysis in multiple cell types of the melanocytic lineage consistently demonstrated that the 1q42.1 melanoma risk allele (rs3219090[G]) is correlated with higher PARP1 levels. In silico fine-mapping and functional validation identified a common intronic indel, rs144361550 (-/GGGCCC; r2 = 0.947 with rs3219090), as displaying allele-specific transcriptional activity. A proteomic screen identified RECQL as binding to rs144361550 in an allele-preferential manner. In human primary melanocytes, PARP1 promoted cell proliferation and rescued BRAFV600E-induced senescence phenotypes in a PARylation-independent manner. PARP1 also transformed TERT-immortalized melanocytes expressing BRAFV600E. PARP1-mediated senescence rescue was accompanied by transcriptional activation of the melanocyte-lineage survival oncogene MITF, highlighting a new role for PARP1 in melanomagenesis.

Mucinous hidradenoma in a child-A case report and review of the literature.

Hidradenomas are benign tumors of the sweat glands that are rarely reported in childhood. The presence of mucinous change in hidradenomas, which consists of variable numbers of mucin-rich goblet cells, is occasionally observed in some adult clinical practices. However, it has not been previously reported in the pediatric population. Herein, we present a unique case of a mucinous hidradenoma presenting on the thigh of a 3-year-old boy. The clinical presentation and histological features of the case, along with a literature review of published case reports of pediatric hidradenomas are described. We present this case in order to highlight this variant of hidradenoma and to reinforce its inclusion in the differential diagnosis of neoplasms/conditions of childhood with mucinous differentiation.