Airway abnormalities associated with congenital heart disease.

Airway abnormalities are important but sometimes overlooked problems in children with congenital heart disease. It is often difficult to separate symptoms related to cardiac disease from those associated with airway or lung disease. Some of the lesions are incidental while others cause significant symptoms and are important in overall functional outcome. Congenital and acquired as well as intrinsic and extrinsic lesions occur and can overlap. We review and illustrate these lesions here. Imaging plays a crucial role in diagnosing and assessing the severity of airway abnormalities and guiding medical and surgical management decisions.

Congenital Absence of the Pericardium: Pearls and Pitfalls.

Congenital absence of the pericardium is a very uncommon condition that may be focal, unilateral or bilateral but most frequently involves the left sided pericardium. Clinical signs and symptoms are often absent or nonspecific, overlapping with many other more common conditions. Imaging findings are distinctive if the features are sought and recognized.

Magnetic resonance imaging for congenital lung malformations.

Congenital lung malformations are most often identified on prenatal US screening. Fetal MRI is often performed to further evaluate these lesions. Although some of these lesions might cause prenatal or early postnatal symptoms that require urgent management, the majority are asymptomatic at birth and might be subtle or invisible on chest radiographs. Postnatal imaging is frequently deferred until 3-6 months of age, when surgery or long-term conservative management is contemplated. High-quality imaging and interpretation is needed to assist with appropriate decision-making. Contrast-enhanced chest CT, typically with angiographic technique, has been the usual postnatal imaging choice. In this review, the author discusses and illustrates the indications and use of postnatal MR imaging for bronchopulmonary malformations as well as some differential diagnoses and the advantages and disadvantages of MR versus CT.

Improved detection of air trapping on expiratory computed tomography using deep learning.

BACKGROUND: Radiologic evidence of air trapping (AT) on expiratory computed tomography (CT) scans is associated with early pulmonary dysfunction in patients with cystic fibrosis (CF). However, standard techniques for quantitative assessment of AT are highly variable, resulting in limited efficacy for monitoring disease progression. OBJECTIVE: To investigate the effectiveness of a convolutional neural network (CNN) model for quantifying and monitoring AT, and to compare it with other quantitative AT measures obtained from threshold-based techniques. MATERIALS AND METHODS: Paired volumetric whole lung inspiratory and expiratory CT scans were obtained at four time points (0, 3, 12 and 24 months) on 36 subjects with mild CF lung disease. A densely connected CNN (DN) was trained using AT segmentation maps generated from a personalized threshold-based method (PTM). Quantitative AT (QAT) values, presented as the relative volume of AT over the lungs, from the DN approach were compared to QAT values from the PTM method. Radiographic assessment, spirometric measures, and clinical scores were correlated to the DN QAT values using a linear mixed effects model. RESULTS: QAT values from the DN were found to increase from 8.65% ± 1.38% to 21.38% ± 1.82%, respectively, over a two-year period. Comparison of CNN model results to intensity-based measures demonstrated a systematic drop in the Dice coefficient over time (decreased from 0.86 ± 0.03 to 0.45 ± 0.04). The trends observed in DN QAT values were consistent with clinical scores for AT, bronchiectasis, and mucus plugging. In addition, the DN approach was found to be less susceptible to variations in expiratory deflation levels than the threshold-based approach. CONCLUSION: The CNN model effectively delineated AT on expiratory CT scans, which provides an automated and objective approach for assessing and monitoring AT in CF patients.

Congenital central pulmonary artery anomalies: Part 2.

There is a broad spectrum of congenital anomalies of the central pulmonary arteries including abnormalities of development, origin, course and caliber. These anomalies incorporate simple lesions such as isolated pulmonary valve stenosis to very complex anomalies with many associated abnormalities. Part 1 and Part 2 of this review describe the range of anatomical variations that are encountered as well as important aspects of anatomy, physiology and surgical correction. The authors summarize and illustrate both well-recognized and more complex anomalies to provide a broad and comprehensive understanding of these lesions and their appearances on CT and MR imaging. In Part 2 the authors review abnormalities in development, origin and course of the central branch pulmonary arteries as well as abnormal pulmonary artery caliber.

Congenital central pulmonary artery anomalies: Part 1.

There is a broad spectrum of congenital anomalies of the central pulmonary arteries including abnormalities of development, origin, course and caliber. These anomalies incorporate simple lesions such as isolated pulmonary valve stenosis to very complex anomalies with many associated abnormalities. Part 1 and Part 2 of this review describe the range of anatomical variations that are encountered as well as important aspects of anatomy, physiology and surgical correction. The authors summarize and illustrate well-recognized as well as more complex anomalies to provide a broad and comprehensive understanding of these lesions and their appearances on CT and MR imaging. Part 1 covers anomalous development or origin of the main pulmonary artery.

Mucus plugging, air trapping, and bronchiectasis are important outcome measures in assessing progressive childhood cystic fibrosis lung disease.

OBJECTIVE: To determine which outcome measures could detect early progression of disease in school-age children with mild cystic fibrosis (CF) lung disease over a two-year time interval utilizing chest computed tomography (CT) scores, quantitative CT air trapping (QAT), and spirometric measurements. METHODS: Thirty-six school-age children with mild CF lung disease (median [interquartile range] age 12 [3.7] years; percent predicted forced expiratory volume in 1 second (ppFEV1 ) 99 [12.5]) were evaluated by serial spirometer-controlled chest CT scans and spirometry at baseline, 3-month, 1- and 2-years. RESULTS: No significant changes were noted at 3-month for any variable except for decreased ppFEV1 . Mucus plugging score (MPS) and QATA1andA2 increased at 1- and 2-years. The bronchiectasis score (BS), and total score (TS) were increased at 2-year. All variables tested with the exception of bronchial wall thickness score, parenchymal score (PS), and ppFEV1 , were consistent with longitudinal worsening of lung disease. Multivariate analysis revealed baseline PS, baseline TS, and 1-year changes in BS and air trapping score were predictive of 2-year changes in BS. CONCLUSIONS: MPS and QATA1-A2 were the most sensitive indicators of progressive childhood CF lung disease. The 1-year change in the bronchiectasis score had the most positive predictive power for 2-year change in bronchiectasis.

Left ventricular retraining in corrected transposition: Relationship between pressure and mass.

BACKGROUND: A subset of patients with corrected transposition of the great arteries (CC-TGA) will require left ventricular (LV) retraining before undergoing a double-switch procedure. LV retraining results in an immediate increase in LV pressure but not in LV mass. The purpose of this study was to evaluate the relationship between LV pressure and mass during LV retraining. METHODS: This was a retrospective review of 36 patients with CC-TGA who were enrolled in LV retraining. The median age at enrollment was 12 months. The majority (82%) had an Ebstenoid tricuspid valve or moderate to severe tricuspid valve regurgitation before pulmonary artery banding. RESULTS: Twenty-seven of the 36 patients underwent LV retraining and a double switch, including 14 patients with a single pulmonary artery band (PAB), 12 patients with 2 PABs, and 1 patient with 3 PABs. There was no early or late mortality associated with these procedures. Ninety percent of the patients demonstrated a linear relationship between LV pressure and mass; however, 3 patients (10%) demonstrated a different LV retraining pattern characterized by excess LV mass compared to LV pressure. Two of these 3 patients are LV retraining failures, whereas the third patient did undergo a double switch. CONCLUSIONS: The data show that the majority of patients enrolled in LV retraining have a predictable relationship between LV mass and pressure. A minority demonstrate an excess accrual of mass with respect to pressure load. These results suggest there may be an important distinction between normal and pathological hypertrophy during the LV retraining process.

Ultrafast pediatric chest computed tomography: comparison of free-breathing vs. breath-hold imaging with and without anesthesia in young children.

BACKGROUND: General anesthesia (GA) or sedation has been used to obtain good-quality motion-free breath-hold chest CT scans in young children; however pulmonary atelectasis is a common and problematic accompaniment that can confound diagnostic utility. Dual-source multidetector CT permits ultrafast high-pitch sub-second examinations, minimizing motion artifact and potentially eliminating the need for a breath-hold. OBJECTIVE: The purpose of this study was to evaluate the feasibility of free-breathing ultrafast pediatric chest CT without GA and to compare it with breath-hold and non-breath-hold CT with GA. MATERIALS AND METHODS: Young (≤3 years old) pediatric outpatients scheduled for chest CT under GA were recruited into the study and scanned using one of three protocols: GA with intubation, lung recruitment and breath-hold; GA without breath-hold; and free-breathing CT without anesthesia. In all three protocols an ultrafast high-pitch CT technique was used. We evaluated CT images for overall image quality, presence of atelectasis and motion artifacts. RESULTS: We included 101 scans in the study. However the GA non-breath-hold technique was discontinued after 15 scans, when it became clear that atelectasis was a major issue despite diligent attempts to mitigate it. This technique was therefore not included in statistical evaluation (86 remaining patients). Overall image quality was higher (P=0.001) and motion artifacts were fewer (P<.001) for scans using the GA with intubation and recruitment technique compared to scans in the non-GA free-breathing group. However no significant differences were observed regarding the presence of atelectasis between these groups. CONCLUSION: We demonstrated that although overall image quality was best and motion artifact least with a GA-breath-hold intubation and recruitment technique, free-breathing ultrafast pediatric chest CT without anesthesia provides sufficient image quality for diagnostic purposes and can be successfully performed both without and with contrast agent in young infants.

Tumor-induced Osteomalacia in a 3-Year-Old With Unresectable Central Giant Cell Lesions.

Tumor-induced osteomalacia (TIO) is a rare cause of hypophosphatemia involving overproduction of fibroblast growth factor 23. TIO has been described largely in adults with small mesenchymal tumors. We report a case of TIO in a child who presented with knee pain and radiographic findings concerning for rickets, and was found to have maxillomandibular giant cell lesions. The patient was treated with oral phosphorus and calcitriol, surgical debulking, and intralesional corticosteroids, which resulted in tumor regression and normalization of serum fibroblast growth factor 23 and phosphorus. This case illustrates the occurrence of this rare paraneoplastic syndrome in children and adds to our knowledge about clinical manifestations and pathologic findings associated with pediatric TIO.

Congenital Variants and Anomalies of the Aortic Arch.

Congenital variants and anomalies of the aortic arch are important to recognize as they may be associated with vascular rings, congenital heart disease, and chromosomal abnormalities, and can have important implications for prognosis and management. The purpose of this article is to review cross-sectional imaging techniques used in the evaluation of the aortic arch, describe the embryology and anatomy of the aortic arch system, discuss aortic arch variants and anomalies, and review other malformations of the aortic arch, including interrupted aortic arch, hypoplastic aortic arch, and aortic coarctation. Aortic arch variants and anomalies will be reviewed in the context of a theoretical double aortic arch system. Arch anomalies can be associated with symptoms, such as dysphagia lusoria in the setting of left aortic arch with aberrant right subclavian artery. Arch variants that form a vascular ring, such as double aortic arch, can result in respiratory distress due to tracheal compression. Certain arch anomalies are strongly associated with congenital heart disease, including right aortic arch with mirror image branching. Other malformations of the aortic arch have important associations, such as type B interrupted aortic arch, which is associated with a locus 22q11.2 microdeletion. Noninvasive imaging at CT angiography and MR angiography allows for comprehensive evaluation of the aortic arch and branch vessels in relation to surrounding structures. Familiarity with the spectrum and imaging appearances of aortic arch variants, anomalies, and malformations is essential for accurate diagnosis and classification and to guide management. Online supplemental material is available for this article. ©RSNA, 2016.

Persistent fifth arch anomalies - broadening the spectrum to include a variation of double aortic arch vascular ring.

Fifth arch anomalies are rare and complex and frequently misdiagnosed or mistaken for other entities. We report a double arch vascular ring that is thought to consist of right fourth arch and left fifth arch components, a previously undescribed persistent fifth arch variant. The currently recognized spectrum and classification of fifth arch vascular anomalies are expanded along with illustrative images to justify the proposed changes. Reviewing and expanding the classification of fifth arch anomalies to include a double arch ring variant will promote recognition, correct diagnosis and appropriate management of these anomalies.

Analysis of Kidney Ultrasound Dimensions by Body Habitus and Position.

PURPOSE: Renal dimensions are an important assessment of the genitourinary tract used to evaluate critical aspects of renal growth and development. Understanding the effect of patient position is important to use and interpret these parameters. In this prospective study we determined the effect of patient position and general body habitus on renal length and parenchymal area in children undergoing renal ultrasound. MATERIALS AND METHODS: Between October 2010 and January 2011 children underwent renal ultrasound while prone and supine. Bilateral renal length and renal parenchymal area were measured. Pearson and Bland-Altman statistical analyses were used to examine correlations, measurement bias and the degree of agreement between methods. RESULTS: Renal length measurements in both positions were complete for 201 right and 196 left kidneys. Parenchymal area measurements were complete for both kidneys in 177 children. When compared individually, supine and prone measures of renal length and parenchymal area highly correlated on Pearson analysis (greater than 0.96 and greater than 0.89, respectively). When compared by method, Bland-Altman analyses of differences vs means showed greater than 50% variance, representing wide limits of agreement with poor interrelation. Neither persistent systematic bias nor body habitus influenced results. CONCLUSIONS: While Pearson analysis showed high correlation for supine and prone renal measurements, Bland-Altman analysis of renal length and parenchymal area demonstrated wide limits of agreement, not allowing interchangeable use of prone and supine measurements. As such, renal ultrasound should specify standardized positions and benchmarks. These results provide guidance to standardize renal ultrasound measurements when renal size is used as an indicator of kidney health.

Perinatal Thoracic Mass Lesions: Pre- and Postnatal Imaging.

Chest masses present a common problem in the perinatal period. Advances in prenatal ultrasound, supplemented by fetal magnetic resonance imaging, now allow early detection and detailed characterization of many thoracic lesions in utero. As such, in asymptomatic infants, assessment with postnatal computed tomography or magnetic resonance imaging can often be delayed for several months until the time at which surgery is being contemplated. Bronchopulmonary malformations comprise most of the thoracic masses encountered in clinical practice. However, a variety of other pathologies can mimic their appearances or produce similar effects such as hypoplasia of a lung or both lungs. Understanding of the key differentiating clinical and imaging features can assist in optimizing prognostication and timely management.

Added Value of Radiologist Consultation for Pediatric Ultrasound: Implementation and Survey Assessment.

OBJECTIVE: The purpose of this study was to determine whether radiologist-parent (guardian) consultation sessions for pediatric ultrasound with immediate disclosure of examination results if desired increases visit satisfaction, decreases anxiety, and increases understanding of the radiologist's role. SUBJECTS AND METHODS: Parents chaperoning any outpatient pediatric ultrasound were eligible and completed surveys before and after ultrasound examinations. Before the second survey, parents met with a pediatric radiologist on a randomized basis but could opt out and request or decline the consultation. Differences in anxiety and understanding of the radiologist's role before and after the examination were compared, and overall visit satisfaction measures were tabulated. RESULTS: Seventy-seven subjects participated, 71 (92%) of whom spoke to a radiologist, mostly on request. In the consultation group, the mean score (1, lowest; 4, highest) for overall experience was 3.8 ± 0.4 (SD), consultation benefit was 3.7 ± 0.6, and radiologist interaction was 3.7 ± 0.6. Demographics were not predictive of satisfaction with statistical significance in a multivariate model. Forty-six of 68 (68%) respondents correctly described the radiologist's role before consultation. The number increased to 60 (88%) after consultation, and the difference was statistically significant (p < 0.001). There was also a statistically significant decrease in mean anxiety score from 2.0 ± 1.0 to 1.5 ± 0.8 after consultation (p < 0.001). Sixty-four of 70 (91%) respondents indicated that they would prefer to speak with a radiologist during every visit. CONCLUSION: Radiologist consultation is well received among parents and associated with decreased anxiety and increased understanding of the radiologist's role. The results of this study support the value of routine radiologist-parent interaction for pediatric ultrasound.

Meconium in perinatal imaging: associations and clinical significance.

An abnormal location, distribution, volume, or appearance of meconium is associated with a spectrum of bowel abnormalities, including atresia, obstruction, perforation, fistula, aganglionosis, immaturity, and absorptive dysfunction. This review discusses the fetal and perinatal imaging of these entities, their differential diagnoses, clinical significance, and appropriate imaging workup. Understanding the spectrum of normal and abnormal, specific and nonspecific appearances of meconium and its associated abnormalities on imaging will provide a practical, useful framework for performing and interpreting imaging studies and guiding clinical management.

Vascular lesions--congenital, acquired, and iatrogenic: imaging in the neonate.

The imaging of vasculitis, vasculopathies, and other vascular disorders in neonates presents unique challenges compared with the vascular imaging of older children or adults. These challenges include small-caliber vessels, the need for sedation or anesthesia, a high rate of vasospasm, higher respiratory rates, and a greater susceptibility to long-term effects of ionizing radiation. Duplex ultrasound (US) is typically the first-line imaging modality; in most cases, US provides key diagnostic information necessary for guiding initial management. Magnetic resonance or computed tomography angiography may be performed following US to further define vascular anatomy and relationships, to achieve a definitive diagnosis, and to guide endovascular or surgical planning when necessary. This review article presents and illustrates an update on the imaging strategies for congenital, acquired, and iatrogenic vasculopathies and vascular disorders in neonates.

Improved quantification and mapping of anomalous pulmonary venous flow with four-dimensional phase-contrast MRI and interactive streamline rendering.

BACKGROUND: Cardiac MRI is routinely performed for quantification of shunt flow in patients with anomalous pulmonary veins, but can be technically-challenging to perform. Four-dimensional phase-contrast (4D-PC) MRI has potential to simplify this exam. We sought to determine whether 4D-PC may be a viable clinical alternative to conventional 2D phase-contrast MR imaging. METHODS: With institutional review board approval and HIPAA-compliance, we retrospectively identified all patients with anomalous pulmonary veins who underwent cardiac MRI at either 1.5 Tesla (T) or 3T with parallel-imaging compressed-sensing (PI-CS) 4D-PC between April, 2011 and October, 2013. A total of 15 exams were included (10 male, 5 female). Algorithms for interactive streamline visualization were developed and integrated into in-house software. Blood flow was measured at the valves, pulmonary arteries and veins, cavae, and any associated shunts. Pulmonary veins were mapped to their receiving atrial chamber with streamlines. The intraobserver, interobserver, internal consistency of flow measurements, and consistency with conventional MRI were then evaluated with Pearson correlation and Bland-Altman analysis. RESULTS: Triplicate measurements of blood flow from 4D-PC were highly consistent, particularly at the aortic and pulmonary valves (cv 2-3%). Flow measurements were reproducible by a second observer (ρ = 0.986-0.999). Direct measurements of shunt volume from anomalous veins and intracardiac shunts matched indirect estimates from the outflow valves (ρ = 0.966). Measurements of shunt fraction using 4D-PC using any approach were more consistent with ventricular volumetric displacements than conventional 2D-PC (ρ = 0.972-0.991 versus 0.929). CONCLUSION: Shunt flow may be reliably quantified with 4D-PC MRI, either indirectly or with detailed delineation of flow from multiple shunts. The 4D-PC may be a more accurate alternative to conventional MRI.

Radiologist compliance with California CT dose reporting requirements: a single-center review of pediatric chest CT.

OBJECTIVE: Effective July 1, 2012, CT dose reporting became mandatory in California. We sought to assess radiologist compliance with this legislation and to determine areas for improvement. MATERIALS AND METHODS: We retrospectively reviewed reports from all chest CT examinations performed at our institution from July 1, 2012, through June 30, 2013, for errors in documentation of volume CT dose index (CTDIvol), dose-length product (DLP), and phantom size. Reports were considered as legally compliant if both CTDIvol and DLP were documented accurately and as institutionally compliant if phantom size was also documented accurately. Additionally, we tracked reports that did not document dose in our standard format (phantom size, CTDIvol for each series, and total DLP). RESULTS: Radiologists omitted CTDIvol, DLP, or both in nine of 664 examinations (1.4%) and inaccurately reported one or both of them in 56 of the remaining 655 examinations (8.5%). Radiologists omitted phantom size in 11 of 664 examinations (1.7%) and inaccurately documented it in 20 of the remaining 653 examinations (3.1%). Of 664 examinations, 599 (90.2%) met legal reporting requirements, and 583 (87.8%) met institutional requirements. In reporting dose, radiologists variably used less decimal precision than available, summed CTDIvol, included only series-level DLP, and specified dose information from the scout topogram or a nonchest series for combination examinations. CONCLUSION: Our institutional processes, which primarily rely on correct human performance, do not ensure accurate dose reporting and are prone to variation in dose reporting format. In view of this finding, we are exploring higher-reliability processes, including better-defined standards and automated dose reporting systems, to improve compliance.