Associating a standardized reporting tool for chest radiographs with clinical complications in pediatric acute chest syndrome.

BACKGROUND: Acute chest syndrome (ACS) is an important cause of morbidity in sickle cell disease (SCD). A standardized tool for reporting chest radiographs in pediatric SCD patients did not previously exist. OBJECTIVE: To analyze the interobserver agreement among pediatric radiologists' interpretations for pediatric ACS chest radiographs utilizing a standardized reporting tool. We also explored the association of radiographic findings with ACS complications. METHODS: This was a retrospective cohort study of pediatric ACS admissions from a single institution in 2019. ICD-10 codes identified 127 ACS admissions. Two radiologists independently interpreted the chest radiographs utilizing a standardized reporting tool, a third radiologist adjudicated discrepancies, and κ analysis assessed interobserver agreement. Clinical outcomes were correlated with chest radiograph findings utilizing Pearsons' χ2 , t tests, and Mann-Whitney U tests. Odds ratios (ORs) with 95% confidence intervals (CIs) were calculated. RESULTS: Interobserver agreement was moderate to near-perfect across variables, with κ analysis showing near-perfect agreement for opacity reported in the right upper lobe (0.84), substantial agreement for right lower lobe (0.63), and vertebral bony changes (0.72), with moderate agreement for all other reported variables. On the initial chest radiograph, an opacity located in the left lower lobe (LLL) correlated with pediatric intensive care unit transfer (p = .03). Pleural effusion on the initial chest radiograph had a 3.98 OR (95% CI: 1.35-11.74) of requiring blood products and a 10.67 OR (95% CI: 3.62-31.39) for noninvasive ventilation. CONCLUSION: The standardized reporting tool showed moderate to near-perfect agreement between radiologists. LLL opacity, and pleural effusion were associated with increased risk of ACS complications.

Clinical Improvement in Early Onset Interstitial Lung Disease Using Rituximab in Children With Antimelanoma Differentiation-Associated Gene 5-Positive Juvenile Dermatomyositis.

OBJECTIVE: Children with juvenile dermatomyositis (JDM) and antibodies to antimelanoma differentiation-associated gene 5 (anti-MDA5) are at increased risk of severe disease complications, including interstitial lung disease (ILD). Data regarding treatment of disease complications in this patient population are limited. In this study, we examined the disease course of children with JDM and anti-MDA5 antibodies before and after treatment with rituximab (RTX). METHODS: Patients aged 2-21 years and seen at the Children's Hospital at Montefiore between July 2012 and August 2021, with a diagnosis of JDM, positive anti-MDA5 antibodies, and evidence of ILD, and who were treated with RTX were eligible for inclusion. Retrospective clinical and laboratory data were reviewed. RESULTS: Five of 8 patients with positive anti-MDA5 antibodies had evidence of ILD (62.5%). Four patients had data available for review. All patients received at least 5 courses of RTX infusions, with discontinuation of steroids by an average of 12 months after starting RTX and a decrease to fewer than 2 concurrent medications by the fifth course of RTX. Indicators of ILD on high-resolution computed tomography and pulmonary function tests either improved or fully resolved over the course of RTX treatment for all patients. Patients also demonstrated resolution of active cutaneous manifestations and musculoskeletal disease activity. CONCLUSION: To our knowledge, this is the first study to examine the use of RTX in children with JDM and anti-MDA5 antibodies, with notable improvements in ILD, cutaneous, and musculoskeletal manifestations. Further studies are needed to better understand the efficacy of RTX for JDM disease-related complications.

Imaging findings in acute pediatric coronavirus disease 2019 (COVID-19) pneumonia and multisystem inflammatory syndrome in children (MIS-C).

The two primary manifestations of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in children are acute coronavirus disease 2019 (COVID-19) pneumonia and multisystem inflammatory syndrome (MIS-C). While most pediatric cases of acute COVID-19 disease are mild or asymptomatic, some children are at risk for developing severe pneumonia. In MIS-C, children present a few weeks after SARS-CoV-2 exposure with a febrile illness that can rapidly progress to shock and multiorgan dysfunction. In both diseases, the clinical and laboratory findings can be nonspecific and present a diagnostic challenge. Thoracic imaging is commonly obtained to assist with initial workup, assessment of disease progression, and guidance of therapy. This paper reviews the radiologic findings of acute COVID-19 pneumonia and MIS-C, highlights the key distinctions between the entities, and summarizes our understanding of the role of imaging in managing SARS-CoV-2-related illness in children.

A comparison of pulmonary embolism in pediatric and adult patients with acute COVID-19.

BACKGROUND: COVID-19 is associated with pulmonary embolism (PE) in adults. However, the rate of PE in pediatric patients with acute COVID-19 evaluated by CT pulmonary angiography (CTPA) has not been evaluated. OBJECTIVE: Determine PE rate in pediatric patients with acute COVID-19 and compare to adults. MATERIALS AND METHODS: A retrospective review of CTPA studies, performed between March 2020 and January 2021 on pediatric patients with acute COVID-19, but not MIS-C, was performed. CTPAs performed on an adult cohort of acute COVID-19 patients during April 2020 were reviewed for comparison. Pediatric and chest radiologists independently reviewed CTPAs of pediatric and adult patients, respectively. RESULTS: Of the 355 acute COVID-19 pediatric patients treated during the study period, 14 (16.6 ± 4.8y, median-18.5y, 64% female) underwent CTPA. Of the 1868 acute COVID-19 adults treated during two weeks in April 2020, 50 (57.2 ± 17.0y, median-57.0y, 42% female) underwent CTPA. The PE rate was 14% in the pediatric group (2 patients) and 18% in the adult group (9 patients) (p = 1.0). Both pediatric patients with PE were obese, over 18y, and had asthma, diabetes mellitus, or hypertension. No child<18y with acute COVID-19 had PE. In the adult cohort, higher alanine-aminotransferase and D-dimer levels were associated with PE (p = 0.04 and p = 0.004, respectively). CONCLUSION: Despite similar PE rates in pediatric and adult patients, PE occurred in acute COVID-19 pediatric patients who were >18y, obese, and had at least 1 comorbidity. Children <18y with COVID-19 did not have PE.

Review of COVID-19, part 2: Musculoskeletal and neuroimaging manifestations including vascular involvement of the aorta and extremities.

The coronavirus disease 2019 (COVID-19) pandemic caused by the novel severe acute respiratory syndrome coronavirus (SARS-CoV-2) has affected almost every country in the world resulting in severe morbidity, mortality and economic hardship, altering the landscape of healthcare forever. Its devastating and most frequent thoracic and cardiac manifestations have been well reported since the start of the pandemic. Its extra-thoracic manifestations are myriad and understanding them is critical in diagnosis and disease management. The role of radiology is growing in the second wave and second year of the pandemic as the multiorgan manifestations of COVID-19 continue to unfold. Musculoskeletal, neurologic and vascular disease processes account for a significant number of COVID-19 complications and understanding their frequency, clinical sequelae and imaging manifestations is vital in guiding management and improving overall survival. The authors aim to provide a comprehensive overview of the pathophysiology of the virus along with a detailed and systematic imaging review of the extra-thoracic manifestation of COVID-19. In Part I, abdominal manifestations of COVID-19 in adults and multisystem inflammatory syndrome in children will be reviewed. In Part II, manifestations of COVID-19 in the musculoskeletal, central nervous and vascular systems will be reviewed.

Review of COVID-19, part 1: Abdominal manifestations in adults and multisystem inflammatory syndrome in children.

The coronavirus disease 2019 (COVID -19) pandemic caused by the novel severe acute respiratory syndrome coronavirus (SARS-CoV-2) has affected almost every country in the world, resulting in severe morbidity, mortality and economic hardship, and altering the landscape of healthcare forever. Although primarily a pulmonary illness, it can affect multiple organ systems throughout the body, sometimes with devastating complications and long-term sequelae. As we move into the second year of this pandemic, a better understanding of the pathophysiology of the virus and the varied imaging findings of COVID-19 in the involved organs is crucial to better manage this complex multi-organ disease and to help improve overall survival. This manuscript provides a comprehensive overview of the pathophysiology of the virus along with a detailed and systematic imaging review of the extra-thoracic manifestation of COVID-19 with the exception of unique cardiothoracic features associated with multisystem inflammatory syndrome in children (MIS-C). In Part I, extra-thoracic manifestations of COVID-19 in the abdomen in adults and features of MIS-C will be reviewed. In Part II, manifestations of COVID-19 in the musculoskeletal, central nervous and vascular systems will be reviewed.

Imaging Findings in Multisystem Inflammatory Syndrome in Children (MIS-C) Associated With Coronavirus Disease (COVID-19).

BACKGROUND. A multisystem inflammatory syndrome in children (MIS-C) associated with coronavirus disease (COVID-19) has recently been described. OBJECTIVE. The purpose of our study was to evaluate the imaging findings of MIS-C associated with COVID-19. METHODS. Imaging studies and medical records of patients (age range, 0-20 years) admitted with MIS-C between April 22 and May 21, 2020, were retrospectively reviewed. Thoracic imaging studies were evaluated for parenchymal, mediastinal and hilar, and cardiovascular abnormalities. Abdominal imaging studies were evaluated for abnormalities of solid viscera, hollow viscera, and the peritoneum as well as the mesentery and retroperitoneum. Studies were reviewed independently by two radiologists, and disagreements were resolved by a third senior radiologist. RESULTS. Sixteen patients (10 male and six female patients; age range, 20 months-20 years) were included in this study. All 16 patients presented with fever. Other presenting signs and symptoms included the following: vomiting (12/16, 75%), abdominal pain (11/16, 69%), rash (10/16, 63%), conjunctivitis (8/16, 50%), diarrhea (7/16, 44%), headache (6/16, 38%), and sore throat (5/16, 31%). Shortness of breath and cough were each present in one patient. Chest radiography showed cardiomegaly (10/16, 63%), congestive heart failure or cardiogenic pulmonary edema (9/16, 56%), atelectasis (9/16, 56%), pleural effusions (7/16, 44%), acute respiratory distress syndrome (2/16, 13%), and pneumonia (1/16, 6%). Eight patients (50%) were evaluated for pulmonary embolism (PE) (six [75%] by CT angiography [CTA] and two [25%] by ventilation-perfusion scintigraphy). In two of the eight patients (25%), CTA showed a segmental PE. Abdominal imaging findings (ultrasound, CT, and radiography) included small-volume ascites (6/16, 38%), hepatomegaly (6/16, 38%), echogenic kidneys (5/16, 31%), bowel wall thickening (3/16, 19%), gallbladder wall thickening (3/16, 19%), mesenteric lymphadenopathy (2/16, 13%), splenomegaly (1/16, 6%), and bladder wall thickening (1/16, 6%). The frequencies of findings based on all the reviewed modalities were as follows: cardiomegaly (12/16, 75%), pleural effusion (10/16, 63%), and atelectasis (10/16, 63%). Absolute interobserver agreement was 0.69-1 for thoracic findings and 0.17-1 for abdominal findings. Fifteen patients (94%) were discharged from the hospital (length of hospital stay: range, 3-20 days), and one patient remained in the hospital at the end of the study period. There were no mortalities. CONCLUSION. MIS-C associated with COVID-19 is characterized predominantly by cardiovascular abnormalities, although solid visceral organ, gallbladder, and bowel abnormalities as well as ascites are also seen, reflecting a multisystemic inflammatory process. CLINICAL IMPACT. The constellation of imaging findings in the setting of COVID-19 may alert radiologists to the diagnosis of MIS-C before rapid deterioration of patients.

Tailored Optimization of Pediatric Body MR Angiography for Successful Outcomes in Thoracic Applications.

OBJECTIVE. The purpose of this article is to describe optimized techniques for successful thoracic MR angiography (MRA) in children and to review examples of commonly encountered conditions. CONCLUSION. Successful MRA in children relies on considering the specific child and condition being evaluated and making appropriate choices about the most suitable modality, the use of anesthesia, and the best MRA techniques to determine a diagnosis.

Thoracic Imaging Findings of Multisystem Inflammatory Syndrome in Children Associated with COVID-19: What Radiologists Need to Know Now.

The coronavirus disease 2019 (COVID-19) global pandemic is an ongoing public health emergency, with over 4 million confirmed cases worldwide. Due to the novel nature of this coronavirus and our evolving understanding of its pathophysiology, there is continued uncertainty surrounding diagnosis and management of COVID-19, especially in pediatric patients. In addition, a new febrile hyperinflammatory Kawasaki-like syndrome (also known as multisystem inflammatory syndrome in children, or MIS-C) has emerged in pediatric patients with temporal association to COVID-19 infection. This review article aims to provide an up-to-date review of the clinical and imaging findings of pediatric MIS-C associated with COVID-19, compared with typical acute pediatric COVID-19 infection, with an emphasis on thoracic imaging findings. Supplemental material is available for this article. © RSNA, 2020.

Hepatic Imaging in Neonates and Young Infants: State of the Art.

Neonatal liver disease is an important source of morbidity in the pediatric population. The manifestation of liver disease in young infants may be different than in older patients, and there are a number of diagnoses that are unique to this age group. Familiarity with these entities is important as imaging plays a key role in the diagnostic workup, and prompt diagnosis is necessary to prevent complications. This article reviews the spectrum of liver pathologies that can manifest in the first 6 months of life and is intended to educate the general radiologist who may be faced with interpretation of neonatal liver imaging. Categories of disease that will be reviewed include cholestatic diseases, tumors, vascular anomalies, and acquired diseases. The authors will also review optimization of ultrasonography (US) and magnetic resonance imaging of the liver and present a systematic method for interpretation of neonatal liver US findings in the context of clinical and laboratory findings. © RSNA, 2017.

Three-dimensional ultrasound of the neonatal brain: technical approach and spectrum of disease.

Brain pathology is an important cause of morbidity and mortality in neonates, especially in the premature population. While conventional two-dimensional neurosonography is traditionally used for screening, diagnosis and monitoring of brain disorders such as germinal matrix hemorrhage, periventricular leukomalacia and hydrocephalus, three-dimensional ultrasonography has gained popularity in a variety of clinical applications in recent years. Three-dimensional ultrasonography is not yet widely utilized in pediatric imaging but is a potentially powerful tool for evaluating the neonatal brain. Three-dimensional neurosonography allows imaging of the entire brain in a single volumetric sweep and offers the capability of reconstructing images in the axial plane and performing volumetric analyses that are unavailable in conventional two-dimensional neurosonography. The purpose of this article is two-fold: (1) to present the technical aspects of three-dimensional neurosonography and (2) to illustrate the potential applications of three-dimensional neurosonography in the context of commonly encountered neonatal neuropathology.

Accuracy of Point-of-care Lung Ultrasonography for Diagnosis of Acute Chest Syndrome in Pediatric Patients with Sickle Cell Disease and Fever.

OBJECTIVES: The objective was to determine the test performance characteristics for point-of-care lung ultrasonography (LUS) performed by pediatric emergency medicine (PEM) physicians compared with radiographic diagnosis of acute chest syndrome (ACS) in patients with sickle cell disease (SCD) and fever. METHODS: This was a prospective study of patients up to 21 years with SCD and fever requiring chest X-ray (CXR) evaluation for ACS. Before obtaining CXR, a blinded PEM physician performed LUS using a standardized scanning protocol. Positive LUS for ACS was defined as lung consolidation. All patients received CXR and follow-up. The criterion standard for ACS was consolidation on CXR as determined by a blinded radiologist. LUS clips were reviewed by a blinded expert PEM sonologist. RESULTS: A total of 116 febrile events from 91 patients with a median age of 5.7 years were enrolled by 15 PEM sonologists. CXR was positive for ACS in 15 (13%) patients, and LUS was positive for ACS in 19 (16%) patients. Positive LUS had a sensitivity of 87% (95% confidence interval [CI] = 62% to 96%), specificity of 94% (95% CI = 88% to 97%), positive likelihood ratio of 14.6 (95% CI = 6.5 to 32.5), and negative likelihood ratio of 0.14 (95% CI = 0.04 to 0.52) for ACS. The interobserver agreement (kappa) was 0.77. There were two missed cases of ACS on LUS. CONCLUSIONS: LUS may be sensitive and specific for diagnosis of ACS in pediatric patients with SCD and fever. LUS may reduce the need for routine CXR and associated ionizing radiation exposure in this population.

Three-Dimensional Sonography of Biliary Tract Disorders.

In this pictorial essay, we review the 3-dimensional sonographic appearance of the normal gallbladder and biliary tree, as well as the appearance of congenital and acquired biliary tract disorders. Using examples, we demonstrate the advantage of 3-dimensional scanning compared with 2-dimensional sonography and, even in certain cases, magnetic resonance cholangiopancreatography.

Imaging appearances of musculoskeletal developmental variants in the pediatric population.

Variations in musculoskeletal development in children are commonly encountered. These variants often have a confusing appearance on imaging and may simulate pathologic conditions. However, in many instances, these normal variants have certain features that allow for confident determination of the benign nature of these entities. An awareness of the characteristic imaging features is therefore important for radiologists. In this review, we focus on 4 specific categories of variants in the development: (1) variations in the normal ossification of skeletal structures, (2) the appearance of tendinous and ligamentous insertions in the developing skeleton, (3) overlapping lines that can be confused with fractures or other pathologic conditions, and (4) variant orientation of normal bones. We review the etiology and imaging appearance of these entities and also describe methods of differentiating these benign entities from pathologic lesions. Although in certain cases, correlation with clinical parameters is needed to confidently diagnose the lesion as benign, in many cases, an appreciation of the characteristic imaging features alone would suffice and prevent a potentially costly workup.

Bedside upper gastrointestinal series in critically ill low birth weight infants.

BACKGROUND: The upper gastrointestinal (UGI) series is the preferred method for the diagnosis of malrotation. A bedside UGI technique was developed at our institution for use in low birth weight, critically ill neonates to minimize the risks of transportation from the neonatal intensive care unit (NICU) such as hypothermia and dislodgement of support lines and tubes. OBJECTIVE: To determine the ability of a bedside UGI technique to identify the position of the duodenojejunal junction (DJJ) in low birth weight, critically ill infants in the NICU. MATERIALS AND METHODS: We retrospectively reviewed bedside UGI examinations performed in premature infants weighing less than 1,500 g from 2008 to 2013 and correlated the findings with clinical data, imaging studies and surgical findings. RESULTS: Of 27 patients identified (weight range: 633-1,495 g), 21 (78%) bedside UGI series were diagnostic. Twenty of 27 cases (74%) demonstrated normal intestinal rotation. One case demonstrated malrotation with midgut volvulus, which was confirmed at surgery. In six cases (22%), the position of the DJJ could not be accurately determined. No cases of malrotation with midgut volvulus were missed. None of the patients with normal bedside UGI studies was found to have malrotation based on clinical follow-up (mean: 20 months), surgical findings or further imaging. CONCLUSION: The bedside UGI is a useful technique to exclude malrotation in critically ill neonates and minimizes potential risks of transportation to the radiology suite. Pitfalls that may preclude a diagnostic examination include incorrect timing of radiographs, patient rotation, suboptimal enteric tube position and bowel distention. In cases of diagnostic uncertainty, a follow-up study should be performed.

Do clinical outcomes suffer during transition to an ultrasound-first paradigm for the evaluation of acute appendicitis in children?

OBJECTIVE: The objective of our study was to examine whether the rate of complicated appendicitis and the hospital length of stay (LOS) increased during the transition to an ultrasound-first paradigm for the imaging evaluation of acute appendicitis. MATERIALS AND METHODS: All pediatric patients with surgically proven appendicitis from 2005 to 2011 were identified by searching the hospital billing database for the discharge diagnosis codes for simple appendicitis (ICD-9 [International Classification of Diseases, 9th revision] code 540.9) and complicated appendicitis (ICD-9 codes 540.0 and 540.1). Annual trends of the proportions of these patients who underwent ultrasound and CT were determined and plotted for the study period. Correlation of complicated appendicitis and median hospital LOS with calendar year was assessed using the Spearman (ρ) rank correlation test. RESULTS. Eight hundred four patients met the inclusion criteria. The percentage of patients who underwent CT only showed a moderate downward association with year (ρ = -0.32, p < 0.01), and the percentage of patients who underwent ultrasound first showed a moderate upward trend (ρ = 0.44, p < 0.01). The percentage of patients with ultrasound as the only study performed before appendectomy increased moderately over the 7-year study period (ρ = 0.33, p < 0.01). The percentage of patients with complicated appendicitis and the median hospital LOS did not increase significantly over the study duration (ρ = -0.01, p = 0.74 and ρ = -0.04, p = 0.25, respectively). CONCLUSION: The transition to an ultrasound-first pathway for the imaging workup of acute appendicitis in children occurred without evidence of a corresponding increase in the proportion of patients with complicated appendicitis or in the median hospital LOS.

The role of CT angiography in the evaluation of pediatric renovascular hypertension.

Historically, the evaluation of renovascular hypertension has been accomplished by US, renal scintigraphy and digital subtraction angiography. Based on its high accuracy reported in adults renal CT angiography (CTA) with pediatric-appropriate low radiation dose techniques has become an important tool in the workup of renovascular hypertension in children. Renal CTA has several advantages over more conventional imaging modalities, including rapid and non-invasive acquisition, high resolution and easy reproducibility. Additionally, in our experience high-quality renal CTA can be performed using low-dose radiation exposures and can be acquired without sedation in most instances. This article illustrates by examples the usefulness of renal CTA for diagnosis of childhood renovascular hypertension and provides an overview of renal CTA findings in the most common childhood renovascular diseases.

Comparison of ultrasound and CT in the evaluation of pneumonia complicated by parapneumonic effusion in children.

OBJECTIVE: The purpose of our study was to compare chest ultrasound and chest CT in children with complicated pneumonia and parapneumonic effusion. MATERIALS AND METHODS: We retrospectively compared chest ultrasound and chest CT in 19 children (nine girls and 10 boys; age range, 8 months-17 years) admitted with complicated pneumonia and parapneumonic effusion between December 2006 and January 2009. Images were evaluated for effusion, loculation, fibrin strands, parenchymal consolidation, necrosis, and abscess. In the subset of patients who underwent surgical management, imaging findings were correlated with operative findings. RESULTS: Eighteen of 19 patients had an effusion on both chest ultrasound and chest CT. The findings of effusion loculation as well as parenchymal consolidation and necrosis or abscess were similar between the two techniques. Chest ultrasound was better able to visualize fibrin strands within the effusions. Of the 14 patients who underwent video-assisted thoracoscopy, five had surgically proven parenchymal abscess or necrosis. Preoperatively, chest ultrasound was able to show parenchymal abscess or necrosis in four patients, whereas chest CT was able to show parenchymal abscess or necrosis in three. CONCLUSION: In our series, chest ultrasound and chest CT were similar in their ability to detect loculated effusion and lung necrosis or abscess resulting from complicated pneumonia. Chest CT did not provide any additional clinically useful information that was not also seen on chest ultrasound. We suggest that the imaging workup of complicated pediatric pneumonia include chest radiography and chest ultrasound, reserving chest CT for cases in which the chest ultrasound is technically limited or discrepant with the clinical findings.

Killer cell immunoglobulin-like receptors (KIR) of the African-origin sabaeus monkey: evidence for recombination events in the evolution of KIR.

Killer cell immunoglobulin (Ig)-like receptors (KIR) were characterized in the West African sabaeus monkey (Chlorocebus sabaeus) to elucidate the mechanism by which diversity evolves in this family of molecules. Complementary DNA encoding four forms of KIR molecules, including KIR3DL, KIR2DL4, KIR2DL5, and KIR3DH forms, were identified in two unrelated sabaeus monkeys. A novel hybrid form showing features found in both KIR2DL5 and KIR3DH was also identified. Both the KIR3DL and KIR3DH forms from the sabaeus monkey were considerably more polymorphic than any KIR form identified in great apes or humans. The polymorphic residues of the three Ig-like domains were frequently located in structural loops, indicating that point mutations have occurred in these regions. The three Ig-like domains of the KIR3D forms of six primate species were found to have different patterns of clustering in phylogenetic trees, suggesting that each Ig-like domain has a distinct phylogenetic history. This variation in relationships suggests that repeated recombination events have occurred between the Ig-like domains during the evolution of the KIR family in primates. Recombination between individual Ig-like domains, in addition to point mutations, provides a mechanism for generating the diversity of the KIR genes.