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Pleuropulmonary blastomas are rare, potentially aggressive embryonal cancers of the lung parenchyma and pleural surfaces that account for 0.25%-0.5% of primary pulmonary malignancies in children. Pleuropulmonary blastomas are classified as cystic (type I), mixed cystic and solid (type II), and solid (type III). Pleuropulmonary blastoma occurs in the same age group (0-6 years) as other more common solid tumors such as neuroblastoma and Wilms tumor. Differential diagnosis includes metastasis from Wilms tumor and macrocystic congenital pulmonary airway malformation (CPAM). A key pathologic and genetic discriminator is the DICER1 germline mutation found in patients with pleuropulmonary blastoma. Imaging, histopathologic, and clinical data are important to use in conjunction in order to determine the diagnosis and risk stratification of pleuropulmonary blastomas. Survival varies from poor to good, depending on type. However, the spectrum of pleuropulmonary blastoma is insufficiently understood due to the variable presentation of this rare disease. We present a current review of the literature regarding pleuropulmonary blastomas in this article.

Penetrating injuries of the thorax and abdomen, such as gunshot and stabbing, are rare in children. We present the case of a pediatric patient with a history of remote gunshot injury presenting with a late aneurysm in the left ventricle. (Level of Difficulty: Intermediate.)

Adnexal torsion is a gynecologic emergency in children and adolescents but remains a challenging diagnosis, with no consistent clinical or radiologic diagnostic criteria. Our objective was to identify risk factors associated with adnexal torsion in premenarchal and menarchal patients with surgically confirmed torsion compared with those without torsion.We conducted a retrospective chart review of all patients who underwent surgery between January 2016 and December 2019 for possible adnexal torsion. Data on demographics, clinical characteristics, radiologic variables, and operative findings were compared using descriptive statistics. Independent predictors of torsion were then examined in multivariate logistic regression models.Of the 291 patients who underwent surgery, 168 (57.7%) had torsion. Patients with torsion were younger than those without torsion (11.9 vs. 14.2 years, P .01). Vomiting was significantly associated with torsion for all patients (P .001). Large adnexal volume and absent arterial Doppler flow were associated with torsion for the total population and menarchal subgroup. A logistic regression model for the total population that controlled for age and menarchal status found that vomiting (adjusted odds ratio [aOR] 5.92, 95% confidence interval [CI] 2.87-12.22), highest adnexal volume category (aOR 4.92, 95% CI 2.25-10.75), and absent arterial Doppler flow (aOR 2.674, 95% CI 1.28-5.60) were associated with torsion.Vomiting, enlarged adnexal volume, and absent arterial Doppler flow were associated with adnexal torsion. However, no single risk factor accurately diagnosed torsion, and multiple factors should be interpreted together.Study of Diagnostic Test, Level II.

Stent angioplasty of patent ductus arteriosus has been shown to be a viable alternative to operative shunt placement in cyanotic neonates. With broader implementation of this strategy, novel complications are bound to arise. We present a series of cases evaluated for ductal stent angioplasty in which a dilated and torturous ductus arteriosus compressed the left mainstem bronchus. After reviewing our recent experience with ductal stenting and isolated Blalock-Taussig shunts, our best estimate of the incidence of bronchial compression by the dilated ductus is 4.6% (3/64, 95% confidence interval 1.0-12.9%). Awareness of the airway and other nonvascular contents of the thorax is an important consideration prior to ductal stenting.

E-cigarette or vaping use-associated lung injury (EVALI) remains a major concern due to ongoing use of nicotine and/or cannabis-containing products and resulting acute lung injury. There are few published reports describing the clinical features, comorbidities, severity of disease, and outcomes of treatment in adolescents. This report describes the experience of a single tertiary care children's hospital in the Delaware Valley and reviews data from all patients diagnosed with EVALI in the emergency department and inpatient setting from July 2019 to June 2021 at the Nemours Children's Hospital in Wilmington, Delaware. Demographic, clinical, therapeutic, diagnostic features, and outcomes are presented. Abstinence and steroids improved outcomes in our population. Obtaining a vaping history, negative infectious testing, elevated inflammatory markers, and characteristic computed tomography findings were key to making the diagnosis of EVALI.

Amid the coronavirus disease 2019 (COVID-19) pandemic, numerous publications of imaging findings in children have surfaced in a very short time. Publications discuss populations of overlapping age groups and describe different imaging patterns. We aim to present an overview of the quantity and type of literature available regarding COVID-19 chest imaging findings in children according to a 2020 publication timeline. We conducted a systematic review using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guideline. We searched terminology related to COVID-19, chest, children and imaging modalities in PubMed and Embase. The included papers were published online in 2020 and described imaging findings specific to children and reported five or more cases. Two researchers reviewed each abstract to determine inclusion or exclusion, and a radiologist reconciled any disagreements. Then we reviewed full articles for the main analysis. Eligible study designs included original articles, case series (≥5 cases), systematic reviews and meta-analyses. We excluded non-English manuscripts, retracted articles, and those without available full text. The remaining articles were distributed to four pediatric radiologists (on the Society for Pediatric Radiology Thoracic Committee), who summarized chest imaging findings. Eighty-two articles were included in the final analysis - 28% in radiology journals and 71% in non-radiology journals; 71% contained original data and 29% were review-style papers. There was a disproportionate contribution of review-style papers in April (55%), considering the paucity of preceding publications with original data in March (5 papers). June had the highest number of publications (n=14), followed by April (n=11) and July (n=11). Most (52%) original papers were from China and most individual pediatric imaging descriptions were from China (57%), while the majority of review papers (83%) were international. Imaging descriptions were available for 2,199 children (1,678 CT descriptions and 780 chest radiography descriptions). Findings included a 25% normal CT scan reports vs. 40% normal chest radiography reports. Ground-glass opacification was the most common CT finding (33%) and was reported in only a minority of chest radiographs (9%). A significant amount of information on pediatric COVID-19 chest imaging has become rapidly available over a short period. Most publications in 2020 were original articles, but they were published more often in non-radiology journals. A disproportionate number of review articles were published early on and were based on little original pediatric imaging data. CT scan reports, which represent the standard, outnumbered radiographic reports and indicated that ground-glass opacification is the main imaging finding and that only a quarter of scans are normal in children with COVID-19.

ObjectiveAlthough PU.1/Spi1 is known as a master regulator for macrophage development and function, we have reported previously that it is also expressed in adipocytes and is transcriptionally induced in obesity. Here, we investigated the role of adipocyte PU.1 in the development of age-associated metabolic syndrome.MethodsWe generated mice with adipocyte specific PU.1 knockout, assessed metabolic changes in young and aged PU.1fl/fl (control) and AdipoqCre PU.1fl/fl(aPU.1KO) mice, including body weight, body composition, energy expenditure and glucose homeostasis. We also performed transcriptional analyses using RNA-Sequencing of adipocytes from these mice.ResultsaPU.1KO mice have elevated energy expenditure at a young age and decreased adiposity and increased insulin sensitivity in later life. Corroborating these observations, transcriptional network analysis indicated the existence of validated, aPU.1-modulated regulatory hubs that direct inflammatory and thermogenic gene expression programs.ConclusionsOur data provide evidence for a previously uncharacterized role of PU.1 in the development of age-associated obesity and insulin resistance.

Objective: To assess the value of lateral neck radiographs in quantifying adenoid hypertrophy to help guide treatment decisions in patients with symptoms of nasal obstruction. Study Design: Retrospective review. Methods: Quantitative radiologic grading of adenoids was correlated with the intraoperative grading to select cases in agreement between the two methods. The percent airway obstruction was calculated as a ratio of adenoid size to the size of the nasopharyngeal airway near the level of the choanae on the lateral neck radiographs for adenoidectomy cases in which radiographic and intraoperative grading of adenoid size were in agreement. Results: A total of 426 adenoidectomy cases with preoperative lateral neck radiographs were reviewed (M:F = 254:172 for age range 9 months to 16 years), and only cases in agreement between radiographic and intraoperative adenoid grading were included in radiographic analysis (N = 234). The percent airway obstruction values were significantly different between “severely obstructive” (N = 137, mean = 94.71, SD = 6.55, range [72.00; 100.00]) and “moderately obstructive” adenoid categories (N = 97, mean = 78.53, SD = 6.91, range [63.67; 98.08]), not only within clinically relevant age groups (1-3 years, 4-7 years, 8-15 years), but also for the entire data set (95% CI [14.41; 17.95], P Conclusion: Lateral neck radiographs can provide useful supplemental information on the degree of nasopharyngeal airway obstruction when other clinical findings do not clearly point toward adenoid hypertrophy as a primary cause of nasal obstruction. In our data set, a 65% nasopharyngeal airway obstruction represents a value two standard deviations below the mean for “moderately” obstructive adenoid category, and can be viewed as a simplified cut-off to indicate that the degree of adenoid enlargement is clinically relevant. This cut-off value can assist in evaluation of patients with symptoms of nasal obstruction. Level of Evidence: 4

To evaluate the safety of power injectors for contrast-enhanced computed tomography (CT) in children, namely: the prevalence and injury severity of contrast extravasations related to power injectors and the factors associated with these events.The need to obtain informed consent was waived for this HIPAA-compliant and IRB approved retrospective study. Around 2429 contrast-enhanced CT performed with a power injector were identified during a 3-year period. Data collected included patient demographic, power injector, and contrast agent information. The patients' symptoms, severity of injury and treatment with contrast extravasation were recorded. Around 1496 cases (823 boys, 673 girls) were included in the analysis. Independent-sample t test and Chi-square were used. For a sub-analysis using the extravasation cases, nonparametric tests were used.The mean age was 9.5 ± 6.1 years. The most common access site, catheter site, and contrast agent used were the antecubital fossa, 22 gauge and Iohexol. The mean peak pressure was 68.9 ± 62.3 psi and the flow rate was 1.7 ± 0.9 mL/s. Eighteen cases of contrast extravasation were identified with a mean age of 11.2 ± 6.2 years. There were seven mild, six moderate, and five severe. Cases with extravasation had significantly higher peak pressure (p0.001) and flow rate (p0.001) compared to those without extravasation. Patients who received Iohexol-350 had significantly more contrast extravasation compared to those who used Iohexol-300 (p = 0.03). However, after post-hoc correction, only peak pressure (p0.01) and flow rate (p = 0.01) remained significant.The use of power injectors in children undergoing contrast-enhanced CT is associated with a low rate of extravasation and of long-term injury.

Partial liquid ventilation is proposed as an alternative ventilation strategy to reduce surface tension, increase alveolar recruitment, and decrease inflammation. Studied in acute respiratory distress and other indications, liquid ventilation is being revisited for infants with bronchopulmonary dysplasia. Perfluorooctyl bromide used for liquid ventilation is radiopaque, allowing radiographic visualization of lung liquid ventilation patterns that may provide additional insight into pulmonary pathophysiology. Current protocols utilize reduced liquid dosing, resulting in unique imaging features. We discuss optimal radiographic technique and report initial ultrasound evaluation results. With renewed interest in partial liquid ventilation, it may be helpful for pediatric radiologists to familiarize themselves with the clinical use and radiographic appearance of liquid ventilation material.

To describe the technique and report on our initial experience with the use of intrahepatic dynamic contrast magnetic resonance lymphangiography (IH-DCMRL) for evaluation of the lymphatics in patients with hepatic lymphatic flow disorders. This is a retrospective review of the imaging and clinical findings in six consecutive patients undergoing IH-DCMRL. The technique involves injection of a gadolinium contrast agent into the intrahepatic lymphatic ducts followed by imaging of the abdomen and chest with both heavily T2-weighted imaging and dynamic time-resolved imaging. In six consecutive patients, IH-DCMRL was technically successful. There were four patients with protein-losing enteropathy (PLE) and two with ascites in this study. In the four patients with PLE, IH-DCMRL demonstrated hepatoduodenal connections with leak of contrast into the duodenal lumen not seen by conventional lymphangiography. In one patient with ascites, IH-DCMRL demonstrated lymphatic leakage into the peritoneal cavity not seen by intranodal lymphangiography. In the second patient with ascites, retrograde lymphatic perfusion of mesenteric lymphatic networks and nodes was seen. Venous contamination was seen in two patients. No biliary contamination was identified. There were no short-term complications. IH-DCMRL is a cross-sectional technique which successfully evaluated hepatic lymphatic flow disorders and warrants further investigation. • Intrahepatic dynamic contrast magnetic resonance lymphangiography (IH-DCMRL) is a new imaging technique to evaluate hepatic lymphatic flow disorders such as protein-losing enteropathy. • In comparison to conventional liver lymphangiography, IH-DCMRL offers a 3D imaging technique and better distal lymphatic contrast distribution and does not use ionizing radiation.

The fetal circulation is characterized by the presence of three physiological vascular shunts - the ductus arteriosus, the foramen ovale and the ductus venosus. Acting in concert, these shunts preferentially stream blood flow in a pattern that maximizes efficiency of blood oxygenation by the maternofetal unit. Shortly following the transition to extrauterine life, a quick and predetermined succession of events results in closure of these embryological structures with consequent establishment of postnatal vascular flow patterns. While this transition is often seamless, the physiological shunts of the fetus occasionally fail to regress. Such failure to regress can occur in isolation or in association with other congenital malformations. This failed regression challenges the circulatory physiology of the neonate and might have implications for the optimum functioning of several organ systems. When symptomatic, these shunts are treated. Interventions, when undertaken, might be medical, endovascular or surgical. The radiologist's role continues to expand in the assessment of these shunts, in providing a roadmap for treatment and in prompt identification of treatment-related complications. This review is to familiarize radiologists with the embryology, pre- and post-treatment imaging appearances, and associated complications of persistent fetal vascular shunts.

Midaortic syndrome (MAS) is characterized by narrowing of the descending aorta between the distal aortic arch and the aortic bifurcation. We present the case of a 4-year-old male presenting with a murmur and diagnosed with MAS. He was treated with a thoracoabdominal bypass graft.

Acquired aortopulmonary fistula (APF) in the setting of repaired congenital heart disease is extremely rare but potentially fatal, so timely diagnosis and treatment are critical. We present a case of an 8-year-old female with a history of complex Taussig-Bing anomaly, who underwent an arterial switch procedure with LeCompte maneuver and ventricular septal defect closure early in life. The patient developed neopulmonary stenosis and branch pulmonary artery (PA) stenosis, for which she underwent patch augmentation and balloon dilatation of the left PA. The patient presented with a fistula between the ascending aorta and the left branch PA, confirmed by echocardiography and cardiac catheterization. She underwent repair of the APF with a homograft patch reconstruction of the ascending aorta.

We report on 2 different types of left pulmonary artery sling (LPAS), types IA and IIB, diagnosed by fetal magnetic resonance imaging (MRI). We suggest that fetal MRI is an effective tool for accurately diagnosing LPAS and helping guide its perinatal management. Fetal MRI is relatively unaffected by the conditions that limit visualization by echocardiography. When prenatal ultrasound detects either a possible anomalous origin of the left pulmonary artery or a tracheobronchial anomaly, fetal MRI may provide additional information to confirm the LPAS diagnosis and classify its type. To our knowledge, these are the first reports of prenatally diagnosed LPAS by fetal MRI.

A 16-year-old girl presented to the emergency department with orthopnea and exercise intolerance for 1 week. Electrocardiogram at that time showed rightward deviation of the cardiac axis, with nonspecific T-wave inversion in the lateral precordial leads and flattening in the inferior leads. She was discharged from the emergency department with cardiology follow-up. Subsequent echocardiogram demonstrated that "the heart is in a funny position in the chest," and computed tomography of the chest was requested to evaluate for a mediastinal mass causing leftward displacement of the heart.

Generalized arterial calcification of infancy (GACI) is a rare genetic disease that affects the circulatory system and the large- and medium-sized arteries throughout the body. GACI usually occurs during fetal development. Babies with GACI are diagnosed early, generally soon after birth and in some cases before birth by fetal ultrasound.

To assess the diagnostic efficiency of CT angiography (CTA) to detect pulmonary vein stenosis in children. We retrospectively identify patients between 0 and 3 years old with confirmed pulmonary vein stenosis with conventional angiography or surgery and available CTA. Patients without confirmed stenosis of the pulmonary veins were included as controls. We excluded patients with previous surgery involving the pulmonary veins, exclusively right-heart conventional angiography or insufficient data in the operation note to confirm the status of the pulmonary veins. Two pediatric radiologists evaluated and determine the presence of stenosis and the pulmonary veins affected. Disagreement between the readers were solved by consensus with a third reader. A pediatric cardiologist reviewed the available angiographic images to determine the presence of stenosis. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy were calculated. Kappa statistics was performed. 26 patients (15 boys, 11 Girls) were included. Conventional angiography (n = 20) and cardiothoracic surgery (n = 6) confirmed the diagnosis of pulmonary vein stenosis in 13 children. The diagnostic performance at patient level showed the sensitivity, specificity, PPV, and NPV were 84.6%, 92.3%, 91.6%, 87.5%, and 88.4%, respectively. The interobserver was k = 0.76. The performance at pulmonary vein level showed the sensitivity, specificity, PPV, and NPV were 63.3%, 97.4%, 90.4%,85.7% and 87.9%, respectively. The interobserver agreement was k = 0.62. Computed Tomography Angiography is an excellent and reliable image technique for ruling in pulmonary vein stenosis in young children.

BACKGROUND: The Fontan operation is performed for surgical palliation of single ventricle physiology. This operation is usually preceded by a superior cavopulmonary connection (SCPC); lymphatic abnormalities after SCPC may be demonstrated at MRI and prior to the Fontan operation. PURPOSE: To determine if the degree of neck and thoracic lymphatic abnormalities at T2-weighted MRI in patients after superior cavopulmonary connection (SCPC) correlated with surgical outcomes from the Fontan procedure. MATERIALS AND METHODS: Patients for whom SCPC was performed for palliation of single ventricle disease who underwent chest MRI between July 2012 and May 2015 at a single institution were retrospectively reviewed. T2-weighted images were scored as lymphatic type 1 (little or no T2 mediastinal and supraclavicular signal) to type 4 (T2 signal into both the mediastinum and the lung parenchyma). Fontan takedown, duration of post-Fontan hospitalization and pleural effusion, postoperative plastic bronchitis, need for transplant, and mortality were tabulated. The relationship between lymphatic type and clinical outcomes was evaluated by using analysis of variance (ANOVA), the Kruskal-Wallis H test, and the Fisher exact test. RESULTS: A total of 83 patients (mean age, 7.9 years ± 2.6) were evaluated. Among these 83 patients, 53 (64%) were classified with type 1 or 2 lymphatic abnormalities, 17 (20%) with type 3, and 12 (16%) with type 4. The rate of failure of Fontan completion was higher in patients with type 4 than in type 1 or 2 (54% vs 2%, respectively; P = .004). Need for cardiac transplant (one of 13 [8%]) and death (three of 13 [23%]) occurred only in type 4. Median postoperative length of stay was longer for patients with type 4 than for those with types 1 or 2 (29 days vs 9 days, respectively; P < .01). CONCLUSION: Greater MRI-based severity of lymphatic abnormalities in patients prior to planned Fontan procedure was associated with failure of Fontan completion and longer postoperative stay. © RSNA, 2019 Online supplemental material is available for this article.

Objectives Neonates in the neonatal intensive care unit often require considerable support with endotracheal tubes, umbilical arterial and venous catheters, and peripherally inserted central venous catheters. Support device evaluation with radiography exposes neonates to ionizing radiation. This study evaluated the effectiveness of sonographic localization for endotracheal tubes, umbilical arterial and venous catheters, and peripherally inserted central venous catheters. Methods This blinded prospective Institutional Review Board-approved, Health Insurance Portability and Accountability Act-compliant study with informed consent compared sonography to radiography for endotracheal tube, umbilical arterial and venous catheter, and peripherally inserted central venous catheter localization. Participants were consecutively recruited NICU patients of any weight, gestation, and chronologic age who had an endotracheal tube, umbilical arterial catheter, umbilical venous catheter, or peripherally inserted central venous catheter placed or adjusted and had subsequent radiographic confirmation within 24 hours. Sonographic evaluation was obtained as soon as possible, without prior review of the radiograph, and results were compared. Results Thirty sonographic studies were performed in 25 patients (14 male and 11 female), for a total of 50 lines and tubes: 18 umbilical venous catheters, 12 umbilical arterial catheters, 11 peripherally inserted central venous catheters, and 9 endotracheal tubes. Forty-nine support devices (98%) were visualized with sonography, all concordant with radiography. Forty-four were correctly positioned, and 6 were malpositioned. Sonography identified the location of umbilical venous catheters in all 18 cases (100%), umbilical arterial catheters in all 12 (100%), peripherally inserted central venous catheters in 10 (91%), and endotracheal tubes in 9 (100%). Conclusions The effectiveness of sonography was excellent for evaluation of umbilical arterial and venous catheters, endotracheal tubes, and peripherally inserted central venous catheters. These results support the goal of further point-of-care training and accreditation to use sonography as a primary modality for complete evaluation of NICU support devices.

Evaluate the feasibility and determinants of image quality of ECG-triggered High-Pitch Dual-Source Computed Tomography Angiography (CTA) for cardiovascular assessment in Children.All children that underwent ECG-triggered High-Pitch Dual-Source CTA between August 2014 and September 2017 were identified. Scanner parameters and patients' information were retrieved. Objective image quality was evaluated measuring the Hounsfield units (HU) and standard deviation of regions of interests in the left ventricle, ascending and descending aorta. Signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) were calculated. Qualitative image quality was recorded independently by two pediatric radiologist blinded using a three-point scale: 1 - good image quality, 2 - mild artifacts, 3 - poor image quality. Continuous variables were presented as mean ± standard deviation. The interobserver agreement and non-parametric test were used.93 patients (mean age 5.6 ± 7.1 years) were selected. Average cardiovascular attenuation, SNR and CNR were 406.2 ± 146.3 HU, 24.2 ± 16.0 HU and 52.1 ± 38.6 HU, respectively. Average image quality was 1.51 ± 0.48 and the inter-observer agreement was excellent (k = 0.8). Worse subjective quality scores were associated with lower age, height, weight, BSA, lower contrast dose and slower injection rates (p0.05). Higher heart rate was associated with high attenuation (p0.05), however, SNR and CNR did not show an association with heart rate (p = 0.80).ECG-triggered High-Pitch Dual-Source cardiac CTA is feasible and provides good or excellent image quality for the evaluation of cardiovascular diseases in children.

Assess image quality and radiation dose of ECG-triggered High-Pitch Dual-Source CTA for the evaluation central vascular stents in children. We included all children ≤ 21 years old with one or more central vascular stents and available prospective ECG-triggered High-Pitch Dual-Source CTA performed at our institution between January 2015 and August 2017. Demographic and scanner information was retrieved. Two board-certified pediatric radiologists blinded to the clinical data, independently reviewed and scored each case using a four-point quality score. Scores 1, 2 and 3 were considered of diagnostic image quality. Inter-observer agreement and non-parametric test were used. 18 patients (10 girls, 8 boys) with a mean age of 9.47 ± 7.38 years (mean ± SD) met inclusion criteria. Thirty-two central vascular stents were evaluated. Mean quality score was 2.07 ± 0.94 with 12.5% (4/32) of the cases classified as unevaluable. Interobserver agreement was excellent (k = 0.86). There is no significant difference between quality score and stent location (p = 0.07). There is a significant difference with stent material as all non-diagnostic scores were only seen in covered stents made of platinum-iridium (p 0.001). There was no association between image quality and age, height, weight, BSA, heart rate, radiation dose or stent lumen size (p 0.05). ECG-triggered high-pitch spiral DS-CTA offers appropriate image quality for assessment of central vascular stents in children.