Accuracy of Chest Computed Tomography in Distinguishing Cystic Pleuropulmonary Blastoma From Benign Congenital Lung Malformations in Children.

Importance: The ability of computed tomography (CT) to distinguish between benign congenital lung malformations and malignant cystic pleuropulmonary blastomas (PPBs) is unclear. Objective: To assess whether chest CT can detect malignant tumors among postnatally detected lung lesions in children. Design, Setting, and Participants: This retrospective multicenter case-control study used a consortium database of 521 pathologically confirmed primary lung lesions from January 1, 2009, through December 31, 2015, to assess diagnostic accuracy. Preoperative CT scans of children with cystic PPB (cases) were selected and age-matched with CT scans from patients with postnatally detected congenital lung malformations (controls). Statistical analysis was performed from January 18 to September 6, 2020. Preoperative CT scans were interpreted independently by 9 experienced pediatric radiologists in a blinded fashion and analyzed from January 24, 2019, to September 6, 2020. Main Outcomes and Measures: Accuracy, sensitivity, and specificity of CT in correctly identifying children with malignant tumors. Results: Among 477 CT scans identified (282 boys [59%]; median age at CT, 3.6 months [IQR, 1.2-7.2 months]; median age at resection, 6.9 months [IQR, 4.2-12.8 months]), 40 cases were extensively reviewed; 9 cases (23%) had pathologically confirmed cystic PPB. The median age at CT was 7.3 months (IQR, 2.9-22.4 months), and median age at resection was 8.7 months (IQR, 5.0-24.4 months). The sensitivity of CT for detecting PPB was 58%, and the specificity was 83%. High suspicion for malignancy correlated with PPB pathology (odds ratio, 13.5; 95% CI, 2.7-67.3; P = .002). There was poor interrater reliability (κ = 0.36 [range, 0.06-0.64]; P < .001) and no significant difference in specific imaging characteristics between PPB and benign cystic lesions. The overall accuracy rate for distinguishing benign vs malignant lesions was 81%. Conclusions and Relevance: This study suggests that chest CT, the current criterion standard imaging modality to assess the lung parenchyma, may not accurately and reliably distinguish PPB from benign congenital lung malformations in children. In any cystic lung lesion without a prenatal diagnosis, operative management to confirm pathologic diagnosis is warranted.

Childhood interstitial lung disease more prevalent in infancy: a practical review.

Childhood interstitial lung disease (chILD) is a heterogeneous group of uncommon, mostly chronic pediatric pulmonary disorders characterized by impaired gas exchange and diffuse abnormalities on imaging. A subset of these diseases occurs more frequently in infants and young children than in older children and teenagers. Some of these disorders occur in certain clinical scenarios and/or have typical imaging features that can help the radiologist recognize when to suggest a possible diagnosis and potentially spare a child a lung biopsy. We review the clinical, histopathological and computed tomography features of chILD more prevalent in infancy, including diffuse developmental disorders, growth abnormalities, specific conditions of undefined etiology, and surfactant dysfunction mutations and related disorders, to familiarize the pediatric radiologist with this group of disorders.

Pulmonary lymphoproliferative disorders in children: a practical review.

Pulmonary lymphoproliferative disorders represent an uncommon spectrum of proliferation of lymphoid tissue in the lung parenchyma ranging from benign hyperplasia to malignancy. They tend to occur in certain clinical situations and have typical imaging features that together can be used by the radiologist to suggest these entities as part of the differential diagnosis. We review key clinical, histopathological and computed tomography features of pulmonary lymphoproliferative disorders in children including follicular bronchiolitis, lymphoid interstitial pneumonia, granulomatous-lymphocytic interstitial lung disease, lymphoma and post-transplant lymphoproliferative disorder to familiarize the pediatric radiologist with this group of disorders.

Proadrenomedullin Predicts Severe Disease in Children With Suspected Community-acquired Pneumonia.

BACKGROUND: Proadrenomedullin (proADM), a vasodilatory peptide with antimicrobial and anti-inflammatory properties, predicts severe outcomes in adults with community-acquired pneumonia (CAP) to a greater degree than C-reactive protein and procalcitonin. We evaluated the ability of proADM to predict disease severity across a range of clinical outcomes in children with suspected CAP. METHODS: We performed a prospective cohort study of children 3 months to 18 years with CAP in the emergency department. Disease severity was defined as mild (discharged home), mild-moderate (hospitalized but not moderate-severe or severe), moderate-severe (eg, hospitalized with supplemental oxygen, broadening of antibiotics, complicated pneumonia), and severe (eg, vasoactive infusions, chest drainage, severe sepsis). Outcomes were examined using proportional odds logistic regression within the cohort with suspected CAP and in a subset with radiographic CAP. RESULTS: Among 369 children, median proADM increased with disease severity (mild: median [IQR], 0.53 [0.43-0.73]; mild-moderate: 0.56 [0.45-0.71]; moderate-severe: 0.61 [0.47-0.77]; severe: 0.70 [0.55-1.04] nmol/L) (P = .002). ProADM was significantly associated with increased odds of developing severe outcomes (suspected CAP: OR, 1.68; 95% CI, 1.2-2.36; radiographic CAP: OR, 2.11; 95% CI, 1.36-3.38) adjusted for age, fever duration, antibiotic use, and pathogen. ProADM had an AUC of 0.64 (95% CI, .56-.72) in those with suspected CAP and an AUC of 0.77 (95% CI, .68-.87) in radiographic CAP. CONCLUSIONS: ProADM was associated with severe disease and discriminated moderately well children who developed severe disease from those who did not, particularly in radiographic CAP.

Factors Associated With Antibiotic Prescribing and Outcomes for Pediatric Pneumonia in the Emergency Department.

OBJECTIVES: Chest radiographs (CXRs) are often performed in children with respiratory illness to inform the decision to prescribe antibiotics. Our objective was to determine the factors associated with clinicians' plans to treat with antibiotics prior to knowledge of CXR results and the associations between preradiograph plans with antibiotic prescription and return to medical care. METHODS: Previously healthy children aged 3 months to 18 years with a CXR for suspected pneumonia were enrolled in a prospective cohort study in the emergency department. Our primary outcomes were antibiotic prescription or administration in the emergency department and medical care sought within 7 to 15 days after discharge. Inverse probability treatment weighting was used to limit bias due to treatment selection. Inverse probability treatment weighting was included in a logistic regression model estimating the association between the intention to give antibiotics and outcomes. RESULTS: Providers planned to prescribe antibiotics prior to CXR in 68 children (34.9%). There was no difference in the presence of radiographic pneumonia between those with and without a plan for antibiotics. Children who had a plan for antibiotics were more likely to receive antibiotics than those without (odds ratio [OR], 6.39; 95% confidence interval [CI], 3.7-11.0). This association was stronger than the association between radiographic pneumonia and antibiotic receipt (OR, 3.49; 95% CI, 1.98-6.14). Children prescribed antibiotics were more likely to seek care after discharge than children who were not (OR, 1.85; 95% CI, 1.13-3.05). CONCLUSIONS: Intention to prescribe antibiotics based on clinical impression was the strongest predictor of antibiotic prescription in our study. Prescribing antibiotics may lead to subsequent medical care after controlling for radiographic pneumonia.

Pediatric Myocardial T1 and T2 Value Associations with Age and Heart Rate at 1.5 T.

The objective of the study was to determine normal global left ventricular reference values for T1 and T2 in children. This is a retrospective study that included healthy subjects, age 5-19 years, who underwent CMR for the indication of pectus excavatum from 2018 to 2019. Linear regression models were used to determine associations of native T1 and T2 values to heart rate, age, and other CMR parameters. 102 patients with a mean age of 14.0 ± 2.4 years were included (range 5.4-18.8). 87 (85%) were males and 15 (15%) were females. The mean global T1 was 1018 ± 25 ms and the mean T2 was 53 ± 3 ms. T1 was negatively correlated with age (r = - 0.39, p < 0.001) and positively correlated with heart rate (r = 0.32, p < 0.001) by univariate analysis. Multivariable analysis showed that age and heart rate were independently associated with T1. T2 demonstrated a weak negative correlation with age (r = - 0.20, p = 0.047) and no correlation with heart rate. There was no difference in T1 (p = 0.23) or T2 (p = 0.52) between genders. This study reports normal pediatric T1 and T2 values at a 1.5 Tesla scanner. T1 was dependent on age and heart rate, while T2 was less dependent on age with no correlation with heart rate.

Breath-hold and free-breathing quantitative assessment of biventricular volume and function using compressed SENSE: a clinical validation in children and young adults.

BACKGROUND: Although the breath-hold cine balanced steady state free precession (bSSFP) imaging is well established for assessment of biventricular volumes and function, shorter breath-hold times or no breath-holds are beneficial in children and severely ill or sedated patients. METHODS: Clinical cardiovascular magnetic resonance (CMR) examinations from September 2019 to October 2019 that included breath-hold (BH) and free-breathing (FB) cine bSSFP imaging accelerated using compressed sensitivity encoding (C-SENSE) factor of 3 in addition to the clinical standard BH cine bSSFP imaging using SENSE factor of 2 were analyzed retrospectively. Patients with structurally normal hearts who could perform consistent BHs were included. Aortic flow measured by phase contrast acquisition was used as a reference for the left ventricular (LV) stroke volume. Comparative analysis was performed for evaluation of biventricular volumes and function, imaging times, quantitative image quality, and qualitative image scoring. RESULTS: There were 26 patients who underwent all three cine scans during the study period (16.7 ± 6.4 years, body surface area (BSA) 1.6 ± 0.4 m2, heart rate 83 ± 7 beats/min). BH durations of 8 ± 1 s with C-SENSE = 3 were significantly shorter (p < 0.001) by 33% compared to 12 ± 1 s with SENSE = 2. Actual scan time for BH SENSE (4.9 ± 1.2 min) was comparable to that with FB C-SENSE (5.2 ± 1.5 min; p= NS). Biventricular stroke volume and ejection fraction, and LV mass computed using all three sequences were comparable. There was a small but statistically significant (p < 0.05) difference in LV end-diastolic volume (- 3.0 ± 6.8 ml) between BH SENSE and FB C-SENSE. There was a small but statistically significant (p < 0.005) difference in end-diastolic LV (- 5.0 ± 7.7 ml) and RV (- 6.0 ± 8.5 ml) volume and end-systolic LV (- 3.2 ± 4.3 ml) and RV(- 4.2 ± 6.8 ml) volumes between BH C-SENSE and FB C-SENSE. The LV stroke volumes from all three sequences had excellent correlations (r = 0.96, slope = 0.98-1.02) with aortic flow, with overestimation by 2.7 (5%) to 4.6 (8%) ml/beat. The image quality score was Excellent (16 of 26) to Good (10 of 26) with BH SENSE, Excellent (13 of 26) to Good (13 of 26) with BH C-SENSE, and Excellent (3 of 26) to Good (21 of 26) to Adequate (2 of 26) with FB C-SENSE. CONCLUSIONS: Image quality and ventricular volumetric and functional indices using either BH or FB C-SENSE cine bSSFP imaging were comparable to standard BH SENSE cine bSSFP imaging while maintaining nominally identical spatio-temporal resolution. This accelerated image acquisition provides an alternative to accommodate patients with impaired BH capacity.

Interpretation of pediatric chest radiographs by non-radiologist clinicians in Botswana using World Health Organization criteria for endpoint pneumonia.

BACKGROUND: In low- and middle-income countries, chest radiographs are most frequently interpreted by non-radiologist clinicians. OBJECTIVE: We examined the reliability of chest radiograph interpretations performed by non-radiologist clinicians in Botswana and conducted an educational intervention aimed at improving chest radiograph interpretation accuracy among non-radiologist clinicians. MATERIALS AND METHODS: We recruited non-radiologist clinicians at a referral hospital in Gaborone, Botswana, to interpret de-identified chest radiographs for children with clinical pneumonia. We compared their interpretations with those of two board-certified pediatric radiologists in the United States. We evaluated associations between level of medical training and the accuracy of chest radiograph findings between groups, using logistic regression and kappa statistics. We then developed an in-person training intervention led by a pediatric radiologist. We asked participants to interpret 20 radiographs before and immediately after the intervention, and we compared their responses to those of the facilitating radiologist. For both objectives, our primary outcome was the identification of primary endpoint pneumonia, defined by the World Health Organization as presence of endpoint consolidation or endpoint effusion. RESULTS: Twenty-two clinicians interpreted chest radiographs in the primary objective; there were no significant associations between level of training and correct identification of endpoint pneumonia; concordance between respondents and radiologists was moderate (κ=0.43). After the training intervention, participants improved agreement with the facilitating radiologist for endpoint pneumonia from fair to moderate (κ=0.34 to κ=0.49). CONCLUSION: Non-radiologist clinicians in Botswana do not consistently identify key chest radiographic findings of pneumonia. A targeted training intervention might improve non-radiologist clinicians' ability to interpret chest radiographs.

Biomarkers and Disease Severity in Children With Community-Acquired Pneumonia.

BACKGROUND: Host biomarkers predict disease severity in adults with community-acquired pneumonia (CAP). We evaluated the association of the white blood cell (WBC) count, absolute neutrophil count (ANC), C-reactive protein (CRP), and procalcitonin with the development of severe outcomes in children with CAP. METHODS: We performed a prospective cohort study of children 3 months to 18 years of age with CAP in the emergency department. The primary outcome was disease severity: mild (discharged from the hospital), mild-moderate (hospitalized but not moderate-severe or severe), moderate-severe (eg, hospitalized with receipt of intravenous fluids, supplemental oxygen, complicated pneumonia), and severe (eg, intensive care, vasoactive infusions, chest drainage, severe sepsis). Outcomes were examined within the cohort with suspected CAP and in a subset with radiographic CAP. RESULTS: Of 477 children, there were no statistical differences in the median WBC count, ANC, CRP, or procalcitonin across severity categories. No biomarker had adequate discriminatory ability between severe and nonsevere disease (area under the curve [AUC]: 0.53-0.6 for suspected CAP and 0.59-0.64 for radiographic CAP). In analyses adjusted for age, antibiotic use, fever duration, and viral pathogen detection, CRP was associated with moderate-severe disease (odds ratio 1.12; 95% confidence interval, 1.0-1.25). CRP and procalcitonin revealed good discrimination of children with empyema requiring chest drainage (AUC: 0.83) and sepsis with vasoactive infusions (CRP AUC: 0.74; procalcitonin AUC: 0.78), although prevalence of these outcomes was low. CONCLUSIONS: WBC count, ANC, CRP, and procalcitonin are generally not useful to discriminate nonsevere from severe disease in children with CAP, although CRP and procalcitonin may have some utility in predicting the most severe outcomes.

Antibiotic Use and Outcomes in Children in the Emergency Department With Suspected Pneumonia.

BACKGROUND AND OBJECTIVES: Antibiotic therapy is often prescribed for suspected community-acquired pneumonia (CAP) in children despite a lack of knowledge of causative pathogen. Our objective in this study was to investigate the association between antibiotic prescription and treatment failure in children with suspected CAP who are discharged from the hospital emergency department (ED). METHODS: We performed a prospective cohort study of children (ages 3 months-18 years) who were discharged from the ED with suspected CAP. The primary exposure was antibiotic receipt or prescription. The primary outcome was treatment failure (ie, hospitalization after being discharged from the ED, return visit with antibiotic initiation or change, or antibiotic change within 7-15 days from the ED visit). The secondary outcomes included parent-reported quality-of-life measures. Propensity score matching was used to limit potential bias attributable to treatment selection between children who did and did not receive an antibiotic prescription. RESULTS: Of 337 eligible children, 294 were matched on the basis of propensity score. There was no statistical difference in treatment failure between children who received antibiotics and those who did not (odds ratio 1.0; 95% confidence interval 0.45-2.2). There was no difference in the proportion of children with return visits with hospitalization (3.4% with antibiotics versus 3.4% without), initiation and/or change of antibiotics (4.8% vs 6.1%), or parent-reported quality-of-life measures. CONCLUSIONS: Among children with suspected CAP, the outcomes were not statistically different between those who did and did not receive an antibiotic prescription.

Structural lung abnormalities on computed tomography correlate with asthma inflammation in bronchoscopic alveolar lavage fluid.

Objective: Image scoring systems have been developed to assess the severity of specific lung abnormalities in patients diagnosed with various pulmonary diseases except for asthma. A comprehensive asthma imaging scoring system may identify specific abnormalities potentially linking these to inflammatory phenotypes.Methods: Computed tomography (CT) images of 88 children with asthma (50 M/38 F, mean age 7.8 ± 5.4 years) acquired within 12 months of bronchoscopic alveolar lavage fluid (BALF) sampling that assessed airway inflammation cell types were reviewed along with CT images of 49 controls (27 M/22 F, mean age 3.4 ± 2.2 years). Images were scored using a comprehensive scoring system to quantify bronchiectasis (BR), bronchial wall thickening (BWT), ground glass opacity, mucus plugging (MP), consolidations, linear densities (LD), and air trapping (AT). Each category was scored 0-2 in each of six lobar regions (with lingula separated from left upper lobe).Results: Absolute average overall scores of the controls and children with asthma were 0.72 ± 1.59 and 5.39 ± 5.83, respectively (P < 0.0001). Children with asthma scored significantly higher for BR (N = 20, 0.33 ± 0.80, P = 0.0002), BWT (N = 28, 0.72 ± 1.40, P < 0.0001), MP (N = 28, 0.37 ± 1.12, P = 0.0052), consolidation (N = 31, 0.67 ± 1.22, P < 0.0001), LD (N = 58, 1.12 ± 1.44, P < 0.0001), and AT (N = 52, 1.78 ± 2.31, P < 0.0001). There was a significant difference between the BR score of children with positive inflammatory response in BALF (N = 53) and those who were negative for airway inflammation cells (0.14 ± 0.36, P = 0.040).Conclusions: Significant lung structural abnormalities were readily identified on CT of children with asthma, with image differentiation of those with an inflammatory response on BALF. Chest imaging demonstrates potential as a noninvasive clinical tool for additional characterization of asthma phenotypes.

Feeding Tolerance, Intestinal Motility, and Superior Mesenteric Artery Blood Flow in Infants with Gastroschisis.

OBJECTIVE: The aim of this study was to determine the relationship of superior mesenteric artery (SMA) blood flow and intestinal motility with feeding tolerance in infants with gastroschisis. STUDY DESIGN: This was a prospective observational cohort study of 23 infants with gastroschisis. Magnetic resonance images were obtained at abdominal wall closure, initiation of feeds, and full feeds. Motility and SMA flow data were correlated with feeding tolerance. RESULT: All infants had abnormal motility, and most continued with abnormal motility despite achieving full feeds. Increased SMA flow at the time of abdominal wall closure was found to be significantly related to the earlier achievement of full feeds (ρ = -0.45, p = 0.05) and trended towards earlier initiation of feeds (ρ = -0.36, p = 0.13), shorter parenteral nutrition days (ρ = -0.42, p = 0.07), and earlier discharge (ρ = -0.41, p = 0.08). CONCLUSION: Increased SMA blood flow at the time of abdominal wall closure is positively correlated with feeding tolerance, suggesting the importance of initial intestinal perfusion in the pathophysiology for feeding intolerance and intestinal dysmotility in gastroschisis.

Pharmacologic stress cardiovascular magnetic resonance in the pediatric population: A review of the literature, proposed protocol, and two examples in patients with Kawasaki disease.

Pharmacologic stress cardiovascular magnetic resonance (PSCMR) is a well-established and reliable diagnostic tool for evaluation of coronary artery disease in the adult population. Stress imaging overall and PSCMR in particular is less utilized in the pediatric population with limited reported data. In this review, we highlight the potential use of PSCMR in specific pediatric cohorts with congenital and acquired heart disease, and we review the reported experience. A suggested protocol is presented in addition to two case examples of patients with Kawasaki disease where PSCMR aided decision making.

mDixon ECG-gated 3-dimensional cardiovascular magnetic resonance angiography in patients with congenital cardiovascular disease.

BACKGROUND: Cardiovascular magnetic resonance (CMR) angiography (CMRA) is an important non-invasive imaging tool for congenital heart disease (CHD) and aortopathy patients. The conventional 3D balanced steady-state free precession (bSSFP) sequence is often confounded by imaging artifacts. We sought to compare the respiratory navigated and electrocardiogram (ECG) gated modified Dixon (mDixon) CMRA sequence to conventional non-gated dynamic multi-phase contrast enhanced CMRA (CE-CMRA) and bSSFP across a variety of diagnoses. METHODS: We included 24 patients with CHD or aortopathy with CMR performed between September 2017 to December 2017. Each patient had undergone CE-CMRA, followed by a bSSFP and mDixon angiogram. Patients with CMR-incompatible implants or contraindications to contrast were excluded. The studies were rated according to image quality at a scale from 1 (poor) to 4 (excellent) based on diagnostic adequacy, artifact burden, vascular border delineation, myocardium-blood pool contrast, and visualization of pulmonary and systemic veins and coronaries. Contrast-to-noise ratio (CNR), signal-to-noise ratio (SNR) and quantitative vascular measurements were compared between the two gated sequences. Bland-Altman plots were generated to compare paired measures. RESULTS: All scans were diagnostically adequate. Mean (SD) quality scores were 3.4 (0.7) for the mDixon, 3.2 (0.5) for the bSSFP and 3.4 (0.5) for the CE-CMRA. Qualitatively, the intracardiac anatomy and myocardium-blood pool definition were better in the bSSFP; however, mDixon images showed enhanced vessel wall sharpness with less blurring surrounding the anatomical borders distally. Coronary origins were identified in all cases. Pulmonary veins were visualized in 92% of mDixon sequences, 75% of bSSFP and 96% of CE-CMRA. Similarly, neck veins were identified in 92, 83 and 96% respectively. Artifacts prevented vascular measurement in 6/192 (3%) and 4/192 (2%) of total vascular measurements for the mDixon and bSSFP, respectively. However, the size of signal void and field distortion were significantly worse in the latter, particularly for flow and metal induced artifacts. CONCLUSION: In patients with congenital heart disease, ECG gated mDixon angiography yields high fidelity vascular images including better delineation of head and neck vasculature and pulmonary veins and fewer artifacts than the comparable bSSFP sequence. It should be considered as the preferred strategy for successful CHD imaging in patients with valve stenosis, vascular stents, or metallic implants.

Conservative gadolinium administration to patients with Duchenne muscular dystrophy: decreasing exposure, cost, and time, without change in medical management.

Cardiac MR (CMR) is increasingly used to assess for cardiac involvement in patients with Duchenne muscular dystrophy (DMD). The frequent use of gadolinium based contrast agents (GBCAs) has been called into question with reports of intracranial gadolinium deposition in patients receiving multiple administrations. We adopted a conservative GBCA administration policy, limiting the frequency of GBCA exposure in patients with previously documented late gadolinium enhancement. The aim of our study was to evaluate the clinical effects of this policy change. Data were retrospectively reviewed on 405 consecutive patients with DMD who underwent CMR evaluation. Patients were grouped into conservative GBCA administration or historical control. CMR reports were evaluated and clinical reports were reviewed to determine actionable changes. Ohio Medicaid reimbursements were used to estimate costs. A total of 187 patients comprised the conservative GBCA group and 218 patients the historical cohort. The conservative GBCA group had lower contrast administration rates (84% vs. 99%, p < 0.0001), shorter scan times (35.2 vs. 39.0 min, p < 0.0001), and lower estimated medical costs ($339 vs. $351/study). There was no change regarding the initial presence of first-time late gadolinium enhancement, and no difference in actionable change. Contrast administration substantially decreased 7 months post-policy change (65%) compared to the initial 7 months (96%, p < 0.0001). In the current era with unclear concern for intracranial gadolinium deposition, thoughtful GBCA administration is warranted in patients anticipated to undergo multiple CMRs. Our updated approach has resulted in fewer patients receiving contrast, shorter scan times, and less medical costs, without appreciable changes to patient management.

Case 262: Isolated Left Ventricular Apical Hypoplasia.

History A 17-year-old white male adolescent was re-evaluated for a withheld cardiac condition initially detected during prenatal imaging at an outside institution. He had previously experienced intermittent episodes of shortness of breath, chest pain, and palpitations with exertion, but more recently he had been asymptomatic. He had no other comorbidities and no family history of congenital heart disease, cardiomyopathy, arrhythmia, or sudden cardiac death. At physical examination, the patient had a grade II/IV rumbling systolic murmur best heard at the left upper sternal border. Otherwise, cardiovascular and other physical examination findings were normal. An electrocardiogram showed sinus bradycardia and nonspecific T wave changes. A graded exercise stress test was normal. Cardiac MRI was performed without and with gadolinium-based contrast material. Multiple echocardiograms obtained since birth, cardiac MR images obtained 2 and 4 years earlier, and nongated CT images obtained with iodinated contrast material to evaluate trauma 8 years prior showed findings similar to those of the current examination. Prior chest radiographs (not shown) were normal, and angiography performed when the patient was 4 years old revealed normal coronary arteries.

Inter-radiologist agreement using Society of Abdominal Radiology-American Gastroenterological Association (SAR-AGA) consensus nomenclature for reporting CT and MR enterography in children and young adults with small bowel Crohn disease.

PURPOSE: To assess inter-radiologist agreement using the Society of Abdominal Radiology-American Gastroenterological Association (SAR-AGA) consensus recommendations for reporting CT/MR enterography exams in pediatric and young adult small bowel Crohn disease (CD). METHODS: Institutional review board approval was obtained for this HIPAA-compliant retrospective investigation; the requirement for informed consent was waived. 25 CT and 25 MR enterography exams performed in children and young adults (age range: 6-23 years) between January 2015 and April 2017 with a distribution of ileal CD severity (phenotype) were identified: normal or chronic CD without active inflammation (40%), active inflammatory CD (20%), stricturing CD (20%), and penetrating CD (20%). Five fellowship-trained pediatric radiologists, blinded to one another, documented key imaging findings and standardized impressions based on SAR-AGA consensus recommendations. Inter-radiologist agreement was evaluated using Fleiss' multi-rater kappa statistic (κ) with 95% confidence intervals (CI). RESULTS: Inter-radiologist agreement was moderate for all key imaging findings except presence of ulcerations (κ 0.37 [95% CI 0.28-0.46]) and sacculations (κ 0.31 [95% CI 0.23-0.40]). Agreement for standardized impressions was substantial for stricturing disease (κ 0.79 [95% CI 0.70-0.87]) and moderate for presence of inflammation (κ 0.49 [95% CI 0.44-0.56]) and penetrating disease (κ 0.58 [95% CI 0.49-0.67]). No significant difference in agreement was found between CT and MRI. CONCLUSIONS: Agreement among five pediatric radiologists was moderate to substantial for SAR-AGA standardized impressions and fair to moderate for key imaging findings of pediatric and young adult CD.

Non-contrast three-dimensional gradient recalled echo Dixon-based magnetic resonance angiography/venography in children.

Magnetic resonance imaging (MRI) has been considered a valuable diagnostic tool for noninvasive imaging of the vasculature in children and adults for more than two decades. While a variety of non-contrast MRI methods have been described for imaging of both arteries and veins (e.g., time-of-flight, phase contrast, and balanced steady-state free precession imaging), contrast-enhanced magnetic resonance angiography/venography are the most commonly employed vascular imaging techniques due to their high spatial and contrast resolutions and general reliability. In this technical innovation article, we describe a novel 3-D respiratory-triggered gradient recalled echo Dixon-based MR angiography/MR venography technique that provides high-resolution anatomical imaging of the vasculature of the neck, body and extremities without the need for intravenous contrast material or breath-holding.

Case 262.

History A 17-year-old white male adolescent was re-evaluated for a withheld cardiac condition initially detected during prenatal imaging at an outside institution. He had previously experienced intermittent episodes of shortness of breath, chest pain, and palpitations with exertion, but more recently he had been asymptomatic. He had no other comorbidities and no family history of congenital heart disease, cardiomyopathy, arrhythmia, or sudden cardiac death. At physical examination, the patient had a grade II/IV rumbling systolic murmur best heard at the left upper sternal border. Otherwise, cardiovascular and other physical examination findings were normal. An electrocardiogram showed sinus bradycardia and nonspecific T wave changes. A graded exercise stress test was normal. Cardiac MRI was performed without and with gadolinium-based contrast material ( Figs 1 - 3 ). Multiple echocardiograms obtained since birth, cardiac MR images obtained 2 and 4 years earlier ( Fig 4 ), and nongated CT images obtained with iodinated contrast material to evaluate trauma 8 years prior ( Fig 5 ) showed findings similar to those of the current examination. Prior chest radiographs (not shown) were normal, and angiography performed when the patient was 4 years old revealed normal coronary arteries. [Figure: see text][Figure: see text][Figure: see text][Figure: see text][Figure: see text][Figure: see text][Figure: see text].

Three-dimensional printing and virtual surgery for congenital heart procedural planning.

Complex unrepaired congenital heart disease requires extensive planning to determine the optimal procedural approach. Conventional noninvasive diagnostic imaging initially provides only two-dimensional (2D) representations of the complex, three-dimensional cardiovascular anatomy. With the expansion of 3D visualization techniques in imaging, a paradigm shift has occurred in complex congenital heart disease surgical planning using digital and 3D printed heart models. There has been early success in demonstrating the benefit of these models in interdisciplinary communication and education. The future goal of this work is to demonstrate a clinical outcome benefit using digital and 3D printed models to plan both surgical and catheterization-based interventional procedures. Ultimately, the hope is that advanced procedural planning with virtual surgery and 3D printing will enhance decision-making in complex congenital heart disease cases resulting in improved perioperative performance by reducing operative times, complications, and reoperations.