Aortic arch malformations

Although anomalies of the aortic arch and its branches are relatively uncommon malformations, they are often associated with congenital heart disease. Isolated lesions may be clinically significant when the airways are compromised by a vascular ring. In this article, the development and imaging appearance of the aortic arch system and its various malformations are reviewe

Effects of Pulmonary Regurgitation on Distensibility and Flow of the Branch Pulmonary Arteries in Tetralogy of Fallot

Significant pulmonary regurgitation (PR) after repair of tetralogy of Fallot (TOF) may affect flow in the pulmonary artery (PA) side branches. We sought to assess flow changes and distensibility of the PA side branches in vivo and test correlation with the degree of PR and right-ventricular (RV) dilatation. Thirty patients after TOF repair and 16 controls underwent cardiovascular magnetic resonance for quantification of RV volumes and measurement of flow in the PA side branches. RV volumes and function, blood flow volumes, and cross-sectional area of the main, left (LPA), and right (RPA) PA were measured and regurgitant volumes and distensibility calculated. Results were compared between the LPA and the RPA and between patients and controls. Median regurgitation fraction of PR was 41% (range 22-60%). Regurgitant fraction was greater in the LPA (40%) than in the RPA (29%), resulting in lower net flow into the LPA (p<0.001). LPA area was significantly greater than that of the RPA (303.9 vs. 232.7mm2/m2) (p<0.0001). The LPA showed lower distensibility than the RPA (39 vs. 44%). PA side branch distensibility correlated with MPA regurgitant volume (p=0.001), MPA regurgitant fraction (p=0.001), and RV end-diastolic volume (p=0.03). PA side branches have greater distensibility in patients with PR than in normal subjects. Significant PR leads to changes in flow profile and distensibility of the PA side branches. The LPA shows greater regurgitant volume and greater area but lower distensibility than the RP

In utero diffusion tensor imaging of the fetal brain: a reproducibility study

Our purpose was to evaluate the within-subject reproducibility of in utero diffusion tensor imaging (DTI) metrics and the visibility of major white matter structures. Images for 30 fetuses (20-33. postmenstrual weeks, normal neurodevelopment: 6 cases, cerebral pathology: 24 cases) were acquired on 1.5 T or 3.0 T MRI. DTI with 15 diffusion-weighting directions was repeated three times for each case, TR/TE: 2200/63 ms, voxel size: 1 ∗ 1 mm, slice thickness: 3-5 mm, b-factor: 700 s/mm(2). Reproducibility was evaluated from structure detectability, variability of DTI measures using the coefficient of variation (CV), image correlation and structural similarity across repeated scans for six selected structures. The effect of age, scanner type, presence of pathology was determined using Wilcoxon rank sum test. White matter structures were detectable in the following percentage of fetuses in at least two of the three repeated scans: corpus callosum genu 76%, splenium 64%, internal capsule, posterior limb 60%, brainstem fibers 40% and temporooccipital association pathways 60%. The mean CV of DTI metrics ranged between 3% and 14.6% and we measured higher reproducibility in fetuses with normal brain development. Head motion was negatively correlated with reproducibility, this effect was partially ameliorated by motion-correction algorithm using image registration. Structures on 3.0 T had higher variability both with- and without motion correction. Fetal DTI is reproducible for projection and commissural bundles during mid-gestation, however, in 16-30% of the cases, data were corrupted by artifacts, resulting in impaired detection of white matter structures. To achieve robust results for the quantitative analysis of diffusivity and anisotropy values, fetal-specific image processing is recommended and repeated DTI is needed to ensure the detectability of fiber pathways

Assessment of lung density in pediatric patients using three-dimensional ultrashort echo-time and four-dimensional zero echo-time sequences.

PURPOSE Lung magnetic resonance imaging (MRI) using conventional sequences is limited due to strong signal loss by susceptibility effects of aerated lung. Our aim is to assess lung signal intensity in children on ultrashort echo-time (UTE) and zero echo-time (ZTE) sequences. We hypothesize that lung signal intensity can be correlated to lung physical density. MATERIALS AND METHODS Lung MRI was performed in 17 children with morphologically normal lungs (median age: 4.7 years, range 15 days to 17 years). Both lungs were manually segmented in UTE and ZTE images and the average signal intensities were extracted. Lung-to-background signal ratios (LBR) were compared for both sequences and between both patient groups using non-parametric tests and correlation analysis. Anatomical region-of-interest (ROI) analysis was performed for the normal cohort for assessment of the anteroposterior lung gradient. RESULTS There was no significant difference between LBR of normal lungs using UTE and ZTE (p < 0.05). Both sequences revealed a LBR age-dependency with a high negative correlation for UTE (Rs =  - 0.77; range 2.98-1.41) and ZTE (Rs =  - 0.82; range 2.66-1.38)). Signal-to-noise (SNR) and contrast-to-noise ratios (CNR) were age-dependent for both sequences. SNR was higher for children up to 2 years old with 3D UTE Cones while for the rest it was higher with 4D ZTE. CNR was similar for both sequences. Posterior lung areas exhibited higher signal intensity compared to anterior ones (UTE 9.4% and ZTE 12% higher), both with high correlation coefficients (R2UTE = 0.94, R2ZTE = 0.97). CONCLUSION The ZTE sequence can measure signal intensity similarly to UTE in pediatric patients. Both sequences reveal an age- and gravity-dependency of LBR

Arterial spin labelling MRI for assessment of cerebral perfusion in children with moyamoya disease: comparison with dynamic susceptibility contrast MRI

Introduction: This study seeks to evaluate the diagnostic accuracy of cerebral perfusion imaging with arterial spin labelling (ASL) MR imaging in children with moyamoya disease compared to dynamic susceptibility contrast (DSC) imaging. Methods: Ten children (7 females; age, 9.2 ± 5.4years) with moyamoya disease underwent cerebral perfusion imaging with ASL and DSC on a 3-T MRI scanner in the same session. Cerebral perfusion images were acquired with ASL (pulsed continuous 3D ASL sequence, 32 axial slices, TR = 5.5s, TE = 25ms, FOV = 24cm, matrix = 128 × 128) and DSC (gradient echo EPI sequence, 35 volumes of 28 axial slices, TR = 2,000ms, TE = 36ms, FOV = 24cm, matrix = 96 × 96, 0.2ml/kg Gd-DOTA). Cerebral blood flow maps were generated. ASL and DSC images were qualitatively assessed regarding perfusion of left and right ACA, MCA, and PCA territories by two independent readers using a 3-point-Likert scale and quantitative relative cerebral blood flow (rCBF) was calculated. Correlation between ASL and DSC for qualitative and quantitative assessment and the accuracy of ASL for the detection of reduced perfusion per territory with DSC serving as the standard of reference were calculated. Results: With a good interreader agreement (κ = 0.62) qualitative perfusion assessment with ASL and DSC showed a strong and significant correlation (ρ = 0.77; p < 0.001), as did quantitative rCBF (r = 0.79; p < 0.001). ASL showed a sensitivity, specificity and accuracy of 94%, 93%, and 93% for the detection of reduced perfusion per territory. Conclusion: In children with moyamoya disease, unenhanced ASL enables the detection of reduced perfusion per vascular territory with a good accuracy compared to contrast-enhanced DS

Transplantation of infant en bloc kidneys into paediatric recipients

En bloc renal transplantation (EBT) from infant donors is an option for children with end-stage renal failure. Owing to potential complications, EBT is not performed in all paediatric nephrology centres. We evaluated the perioperative and long-term course of five children undergoing EBT. Primary diagnosis was atypical (diarrhoea-negative) haemolytic uraemic syndrome (n=2), interstitial nephropathy (two siblings) and branchio-oto-renal syndrome (n=1). Recipient and donor ages ranged between 5.9 and 11.1 years and 0.3 and 2.5 years, respectively. Follow-up time after EBT was 2.1-13.2 years. Perioperative complications included (1) a renal artery thrombosis, with immediate intraoperative reconstruction and primary non-functioning of the graft, with recovery after 10 days, and (2) a vesico-ureteric obstruction, successfully managed with temporary insertion of a JJ-catheter. All grafts had good long-term function. Absolute glomerular filtration rate (GFR; millilitres/minute) increased in all patients, whereas relative GFR (millilitres/minute per 1.73m2 body surface area) remained stable during the follow-up period in all but one. Kidney size increased significantly, with maximal growth during the first year after EBT; magnetic resonance imaging (MRI) showed normal structure and vasculature. EBT is a safe and effective option for young children with end-stage renal failure. Absolute GFR and graft size increase and adapt to the children's growing body mas

Mediastinal widening on chest radiography caused by combined aortic valve disease and primary large B-cell lymphoma

Poststenotic aortic root dilatation in patients with aortic valvular stenosis may result in mediastinal widening on chest radiograph. Main differential diagnosis of mediastinal widening is a tumour. In fact, besides atypical chest pain or dyspnoea at exertion secondary to compression of intrathoracic structures in the latter, symptoms may be absent. This article reports a case of combined aortic valve disease and additional primary large B-cell lymphoma.peer-reviewe

Management of Orofacial Manifestations of Juvenile Idiopathic Arthritis: Interdisciplinary Consensus-Based Recommendations

Involvement of the temporomandibular joint (TMJ) is common in juvenile idiopathic arthritis (JIA). TMJ arthritis can lead to orofacial symptoms, orofacial dysfunction, and dentofacial deformity with negative impact on quality of life. Management involves interdisciplinary collaboration. No current recommendations exist to guide clinical management. We undertook this study to develop consensus-based interdisciplinary recommendations for management of orofacial manifestations of JIA, and to create a future research agenda related to management of TMJ arthritis in children with JIA. Recommendations were developed using online surveying of relevant stakeholders, systematic literature review, evidence-informed generation of recommendations during 2 consensus meetings, and Delphi study iterations involving external experts. The process included disciplines involved in the care of orofacial manifestations of JIA: pediatric rheumatology, radiology, orthodontics, oral and maxillofacial surgery, orofacial pain specialists, and pediatric dentistry. Recommendations were accepted if agreement was >80% during a final Delphi study. Three overarching management principles and 12 recommendations for interdisciplinary management of orofacial manifestations of JIA were outlined. The 12 recommendations pertained to diagnosis (n = 4), treatment of TMJ arthritis (active TMJ inflammation) (n = 2), treatment of TMJ dysfunction and symptoms (n = 3), treatment of arthritis-related dentofacial deformity (n = 2), and other aspects related to JIA (n = 1). Additionally, a future interdisciplinary research agenda was developed. These are the first interdisciplinary recommendations to guide clinical management of TMJ JIA. The 3 overarching principles and 12 recommendations fill an important gap in current clinical practice. They emphasize the importance of an interdisciplinary approach to diagnosis and management of orofacial manifestations of JIA