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We present a case of a two-year-old girl in which liver lesions were characterised on contrast-enhanced ultrasound as multifocal focal nodular hyperplasia. This child had previously undergone haematopoietic stem cell transplantation for juvenile myelomonocytic leukaemia and was suspected to have hepatobiliary graft versus host disease. Liver biopsy was performed to confirm the unexpected focal nodular hyperplasia and look for concurrent graft versus host disease. Focal nodular hyperplasia was histologically confirmed on a background of diffuse liver damage in keeping with polypharmacotherapy, steatosis and sepsis. An element of graft versus host disease was not excluded but was not confidently shown in the sample of the lesion. This case report describes and illustrates how contrast-enhanced ultrasound may be of use to further assess hepatic lesions in a complex case of multifactorial hepatic pathology. Radiologists, haematologists and pathologists should be aware that multifocal focal nodular hyperplasia is part of the differential diagnosis of liver lesions in a child with liver damage due to complex disease and treatment. Biopsy remains the gold standard, if there is a concurrent clinical suspicion of graft versus host disease. [ABSTRACT FROM AUTHOR]

Aim: This study aimed to investigate the outcomes and effectiveness of various treatment strategies in patients with hepatic artery stenosis (HAS) after pediatric liver transplantation (pLT)., Methods: This is a single center observational cohort study between January 1st, 2004 and August 1st, 2023, including pLT recipients aged <18 years. The primary outcome was graft and patient survival. The secondary outcomes included incidence of biliary complications, technical success of surgery or endovascular therapy (EVT), and changes in liver function. The cut-off for early and late HAS was 14 days after pLT., Results: Among a total of 327 pLT patients, 4 % (n = 13) developed HAS (n = 3 early; n = 10 late). Treatments included surgical revascularization for one early HAS, conservative management with anticoagulation for one early and four late HAS, and EVT for one early and six late HAS. Over a median follow-up of 28.2 months after the diagnosis of HAS, graft survival was 100 % and 83 % in early and late HAS groups, and patient survival reached 100 % in both groups. One graft loss occurred in the conservative group. Conversely, graft survival in the EVT group was 100 %., Conclusion: The long-term outcomes of HAS after pLT are excellent. Both EVT and conservative management exhibited high graft survival rates for late HAS, with EVT achieving high technical success., Competing Interests: Conflict of interest All authors declare no conflicts of interest or industry support of the article entitled: ‘Hepatic artery stenosis after pediatric liver transplantation: the potential role of conservative management’., (Copyright © 2024 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

Background: Liver transplantation is the state-of-the-art curative treatment for end-stage liver disease. Imaging is a key element in the detection of intraoperative and postoperative complications. So far, only limited data regarding the best radiological approach to monitor children during liver transplantation is available., Objective: To harmonize the imaging of pediatric liver transplantation, the European Society of Pediatric Radiology Abdominal Taskforce initiated a survey addressing the current status of imaging including the pre-, intra- and postoperative phase. This paper reports the responses related to intraoperative imaging., Materials and Methods: An online survey, initiated in 2021, asked European centers performing pediatric liver transplantation 48 questions about their imaging approach. In total, 26 centers were contacted, and 22 institutions from 11 countries returned the survey., Results: Intraoperative ultrasound (US) is used by all sites to assess the quality of the vascular anastomosis in order to ensure optimal perfusion of the liver transplant. Vessel depiction is commonly achieved using color Doppler (95.3%). Additional US-based techniques are employed by fewer centers (power angio mode, 28.6%; B-flow, 19%; contrast-enhanced US, 14.3%). Most centers prefer a collaborative approach, with surgeons responsible for probe handling, while radiologists operate the US machine (47.6%). Less commonly, the intraoperative US is performed by the surgeon alone (28.6%) or by the radiologist alone (23.8%). Timing of US, imaging frequency, and documentation practices vary among centers., Conclusion: Intraoperative US is consistently utilized across all sites during pediatric liver transplantation. However, considerable variations were observed in terms of the US setup, technique preferences, timing of controls, and documentation practices. These differences provide valuable insights for future optimization and harmonization studies., (© 2024. The Author(s).)

Background: Liver transplantation is the state-of-the-art curative treatment for end-stage liver disease. Imaging is a key element in the detection of postoperative complications. So far, limited data is available regarding the best radiologic approach to monitor children after liver transplantation., Objective: To harmonize the imaging of pediatric liver transplantation, the European Society of Pediatric Radiology Abdominal Taskforce initiated a survey addressing the current status of imaging including the pre-, intra-, and postoperative phases. This paper reports the responses related to postoperative imaging., Materials and Methods: An online survey, initiated in 2021, asked European centers performing pediatric liver transplantation 48 questions about their imaging approach. In total, 26 centers were contacted, and 22 institutions from 11 countries returned the survey., Results: All sites commence ultrasound (US) monitoring within 24 h after liver transplantation. Monitoring frequency varies across sites, ranging from every 8 h to 72 h in early, and from daily to sporadic use in late postoperative phases. Predefined US protocols are used by 73% of sites. This commonly includes gray scale, color Doppler, and quantitative flow assessment. Alternative flow imaging techniques, contrast-enhanced US, and elastography are applied at 31.8%, 18.2%, and 63.6% of sites, respectively. Computed tomography is performed at 86.4% of sites when clarification is needed. Magnetic resonance imaging is used for selected cases at 36.4% of sites, mainly for assessment of biliary abnormalities or when blood tests are abnormal., Conclusion: Diagnostic imaging is extensively used for postoperative surveillance of children after liver transplantation. While US is generally prioritized, substantial differences were noted in US protocol, timing, and monitoring frequency. The study highlights potential areas for future optimization and standardization of imaging, essential for conducting multicenter studies., (© 2024. The Author(s).)

Background: Children frequently undergo routine Doppler-ultrasound (DUS) after liver transplantation (LT) for which they are fasted, but this may cause hunger and discomfort., Objective: To determine if DUS measurements, with focus on the portal vein (PV), are affected by prandial changes, and if this affects distress and feasibility of the DUS., Materials and Methods: Children were prospectively included to undergo a pre- and postprandial DUS on the same day at 6 months after LT. Pre- and anastomotic PV peak systolic velocity (PSV), and hepatic artery and hepatic vein DUS measurements were obtained. Pre- and postprandial measurements, and relative postprandial change of PV velocity ratio (VR) compared to PV anastomotic PSV, were compared using paired-sample t-tests and intraclass correlation coefficients (ICC). Obscuration by bowel gas, difficulty of DUS, and impact of fasting were assessed using 5-point rating scales., Results: Twenty-eight children (median age 3.5 years, IQR 1.6-10.8) were included; four were subsequently excluded because they were not fasted (N = 2) or withdrew consent for the second DUS (N = 2). Measurements between pre- and postprandial DUS, and relative postprandial change of VR compared to PV anastomotic PSV, were not significantly different (p > .05). Test consistency was good (ICC = 0.69, 95% CI = 0.29-0.67) for PV anastomotic PSV, and excellent (95% CI = 0.61-0.93) for PV VR. Obscuration by bowel gas or ease of DUS did not change after eating (p > .05). The majority (16/28, 57.2%) found fasting difficult, and several (13/28, 46.4%) got upset when fasted., Conclusion: Children with an LT do not need to be fasted for routine DUS, which may decrease the burden of the examination., (© 2023 The Authors. Pediatric Transplantation published by Wiley Periodicals LLC.)

Background: Liver transplantation is the state-of-the-art curative treatment in end-stage liver disease. Imaging is a key element for successful organ-transplantation to assist surgical planning. So far, only limited data regarding the best radiological approach to prepare children for liver transplantation is available., Objectives: In an attempt to harmonize imaging surrounding pediatric liver transplantation, the European Society of Pediatric Radiology (ESPR) Abdominal Taskforce initiated a survey addressing the current status of imaging including the pre-, intra-, and postoperative phase. This paper reports the responses on preoperative imaging., Material and Methods: An online survey, initiated in 2021, asked European centers performing pediatric liver transplantation 48 questions about their imaging approach. In total, 26 centers were contacted and 22 institutions from 11 countries returned the survey. From 2018 to 2020, the participating centers collectively conducted 1,524 transplantations, with a median of 20 transplantations per center per annum (range, 8-60)., Results: Most sites (64%) consider ultrasound their preferred modality to define anatomy and to plan surgery in children before liver transplantation, and additional cross-sectional imaging is only used to answer specific questions (computed tomography [CT], 90.9%; magnetic resonance imaging [MRI], 54.5%). One-third of centers (31.8%) rely primarily on CT for pre-transplant evaluation. Imaging protocols differed substantially regarding applied CT scan ranges, number of contrast phases (range 1-4 phases), and applied MRI techniques., Conclusion: Diagnostic imaging is generally used in the work-up of children before liver transplantation. Substantial differences were noted regarding choice of modalities and protocols. We have identified starting points for future optimization and harmonization of the imaging approach to multicenter studies., (© 2023. The Author(s).)

The cartilage hair hypoplasia and anauxetic dysplasia (CHH-AD) spectrum encompasses a group of rare skeletal disorders, with anauxetic dysplasia (ANXD) at the most severe end of the spectrum. Biallelic variants in RMRP, POP1, and NEPRO (C3orf17) have previously been associated with the three currently recognized ANXD types. Generally, all types are characterized by severe short stature, brachydactyly, skin laxity, joint hypermobility and dislocations, and extensive skeletal abnormalities visible on radiological evaluation. Thus far, only five patients with type 3 anauxetic dysplasia (ANXD3) have been reported. Here, we describe one additional ANXD3 patient. We provide a detailed physical and radiological evaluation of this patient, in whom we identified a homozygous variant, c.280C > T, p.(Arg94Cys), in NEPRO. Our patient presented with clinically relevant features not previously described in ANXD3: atlantoaxial subluxation, extensive dental anomalies, and a sagittal suture craniosynostosis resulting in scaphocephaly. We provide an overview of the literature on ANXD3 and discuss our patient's characteristics in the context of previously described patients. This study expands the phenotypic spectrum of ANXD, particularly ANXD3. Greater awareness of the possibility of atlantoaxial subluxation, dental anomalies, and craniosynostosis may lead to more timely diagnosis and treatment., (© 2023 The Authors. American Journal of Medical Genetics Part A published by Wiley Periodicals LLC.)

Objectives: Doppler ultrasound (DUS) is the main imaging modality to evaluate vascular complications of pediatric liver transplants (LT). The current study aimed to determine reference values and their change over time., Methods: A consecutive cohort of pediatric patients undergoing an LT were retrospectively included between 2015 and 2020. Timepoints for standardized DUS were intra-operative and postoperative (day 0), days 1-7, months 1 and 3, and years 1 and 2. DUS measurements of the hepatic artery (HA), portal vein (PV), and hepatic vein(s) (HV) were included if there were no complications during 2 years follow-up. Measurements consisted of: peak systolic velocity (PSV) and resistive index (RI) for the HA, PSV for the PV, and venous pulsatility index (VPI) for the HV. Generalized estimating equations were used to analyze change over time., Results: One hundred twelve pediatric patients with 123 LTs were included (median age 3.3 years, interquartile range 0.7-10.1). Ninety-five HAs, 100 PVs, and 115 HVs without complications were included. Reference values for HA PSV and RI, PV PSV, and HV VPI were obtained for all timepoints (4043 included data points in total) and presented using 5 th -95 th percentiles and threshold values. All reference values changed significantly over time (p = 0.032 to p < 0.001)., Conclusions: DUS reference values of hepatic vessels in children after LT are presented, reference values change over time with specific vessel-dependent patterns. Timepoint-specific reference values improve the interpretation of DUS values and may help to better weigh their clinical significance., Key Points: • Doppler ultrasound reference values of pediatric liver transplantations are not static but change over time. Applying the correct reference values for the specific timepoint may further improve the interpretation of the measurements. • The pattern of change over time of Doppler ultrasound measurements differs between the hepatic vessel and measurement; knowledge of these patterns may help radiologists to better understand normal postoperative hemodynamic changes., (© 2023. The Author(s).)

Introduction: Doppler ultrasound of the portal vein peak systolic velocity and hepatic artery peak systolic velocity and resistive index in children is often performed during abdominal ultrasound for the assessment of liver and other abdominal pathology. However, evidence-based reference values are not available. We aimed to determine these reference values and to investigate whether they are age-dependent., Methods: Children who underwent abdominal ultrasound between 2020 and 2021 were retrospectively identified. Patients without hepatic or cardiac abnormalities at the time of ultrasound or during at least 3 months follow-up were eligible for the study. Ultrasound without hepatic hilum portal vein peak systolic velocity and/or hepatic artery peak systolic velocity and resistive index measurements were excluded. Age-dependent changes were analyzed using linear regression. Normal range reference values were described using percentiles for all ages, and for age subgroups., Results: One-hundred ultrasound examinations performed in 100 healthy children aged 0-17.9 years (median 7.8 years, interquartile range 1.1-14.1 years) were included. Ninety-nine portal vein peak systolic velocity and 80 hepatic artery peak systolic velocity and resistive index measurements were obtained. There was no significant association between portal vein peak systolic velocity and age (β = -0.056, p  = 0.68). There were significant associations between age and hepatic artery peak systolic velocity, and between age and hepatic artery resistive index (β = -0.873, p  = 0.04 and β = -0.004, p  < 0.001, respectively). Detailed reference values were provided for all ages, and for age subgroups., Conclusion: Reference values for the hepatic hilum portal vein peak systolic velocity, hepatic artery peak systolic velocity, and hepatic artery resistive index in children were established. Portal vein peak systolic velocity is not age-dependent, whereas hepatic artery peak systolic velocity and hepatic artery resistive index decrease when children get older., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2022.)

Purpose Doppler ultrasound (DUS) is widely used to detect vascular complications after pediatric liver transplantation (LT). This study aimed to assess the moment of first detection of vascular complications with DUS, and to determine the positive predictive value (PPV) of DUS. Materials and Methods Patients aged 0-18 years who underwent LT between 2015 and 2019 were retrospectively included. 92 LTs in 83 patients were included (median age: 3.9 years, interquartile range: 0.7-10.5). Patients underwent perioperative (intra-operative and immediately postoperative) and daily DUS surveillance during the first postoperative week, and at 1, 3, and 12 months. Vascular complications were categorized for the hepatic artery, portal vein, and hepatic veins. DUS findings were compared to surgical or radiological findings during the 1-year follow-up. Results 52 vascular complications were diagnosed by DUS in 35/92 LTs (38%). 15 out of 52 (28.8%) were diagnosed perioperatively, 29/52 (55.8%) were diagnosed on postoperative days 1-7, and 8/52 (15.4%) after day 7. The PPV for all vascular complications diagnosed with DUS was 92.3%. During the 1-year follow-up, 18/19 (94.7%) hepatic artery complications, 19/26 (73.1%) portal vein complications, and 7/7 (100%) hepatic vein complications were diagnosed perioperatively or during the first week. Conclusion The majority of vascular complications during the first year after pediatric LT were diagnosed by DUS perioperatively or during the first week, with a high PPV. Our findings provide important information regarding when to expect different types of vascular complications on DUS, which might improve DUS post-LT surveillance protocols., Competing Interests: Conflict of Interest The authors declare that they have no conflict of interest., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/).)

Computed tomography (CT)-derived body metrics such as skeletal muscle index (SMI), psoas muscle index (PMI), and subcutaneous fat area index (ScFI) are measurable components of sarcopenia, frailty, and nutrition. While these body metrics are advocated in adults for predicting postoperative outcomes after liver transplantation (LT), little is known about their value in pediatric populations. This study assessed the relation between preoperative CT-based body metrics and postoperative short-term outcomes in pediatric LT recipients. Patients aged 0-18 years who underwent a primary LT were retrospectively included (n = 101; median age 0.5 years; range 0.2-17.1). SMI, PMI, and ScFI were derived from preoperative axial CT slices. Postoperative outcomes and complications within 90 days were correlated with the CT-based body metrics. To classify postoperative infections, the Clavien-Dindo (CD) classification was used. Subgroup analyses were performed for age groups (<1, 1-10, and >10 years old). An optimal threshold for test performance was defined using Youden's J-statistic and receiver operating characteristic curve as appropriate. ScFI was significantly (P = 0.001) correlated with moderate to severe postoperative infections (CD grade 3-5) in children aged <1 year, with the optimal ScFI threshold being ≤27.1 cm 2 /m 2 (sensitivity 80.4% and specificity 77.8%). A weak negative correlation between SMI and the total duration of hospital stay (R = -0.3; P = 0.01) and intensive care unit (ICU) stay (R = -0.3; P = 0.01) was observed in children aged <1 year. No other associations between CT-based body metrics and postoperative outcomes were shown. In children aged <1 year with cirrhotic liver disease undergoing LT, preoperative CT-based body metrics were correlated with moderate to severe postoperative infections (ScFI) and with longer duration of hospital and ICU stay (SMI), and thus can be considered important tools for pre-LT risk assessment., (Copyright © 2021 The Authors. Liver Transplantation published by Wiley Periodicals LLC on behalf of American Association for the Study of Liver Diseases.)

We describe a case of an adolescent male with the rare combination of pulmonary arterial hypertension (PAH) and pulmonary arteriovenous malformations (PAVM's) without confirmed hereditary hemorrhagic telangiectasia (HHT). The patient showed clinical deterioration on standard vasodilator therapy, leading us to question our initial diagnosis. Post-mortem evaluation confirmed the presence of pulmonary veno-occlusive disease of which no conclusive signs were recognized at diagnostic work-up. This case demonstrates the heterogeneity in the diseases causing PAH and shows that an unexpected treatment response should alert the physician to question the original diagnosis., (© 2021 The Authors.)

Treatment regimens for pediatric Hodgkin lymphoma (HL) depend on accurate staging and treatment response assessment, based on accurate disease distribution and metabolic activity depiction. With the aim of radiation dose reduction, we compared the diagnostic performance of 18 F-FDG PET/MRI with a 18 F-FDG PET/CT reference standard for staging and response assessment. Methods: Twenty-four patients (mean age, 15.4 y; range, 8-19.5 y) with histologically proven HL were prospectively and consecutively recruited in 2015 and 2016, undergoing both 18 F-FDG PET/CT and 18 F-FDG PET/MRI at initial staging ( n = 24) and at response assessment ( n = 21). The diagnostic accuracy of 18 F-FDG PET/MRI for both nodal and extranodal disease was compared with that of 18 F-FDG PET/CT, which was considered the reference standard. Discrepancies were retrospectively classified as perceptual or technical errors, and 18 F-FDG PET/MRI and 18 F-FDG PET/CT were corrected by removing perceptual error. Agreement with Ann Arbor staging and Deauville grading was also assessed. Results: For nodal and extranodal sites combined, corrected staging 18 F-FDG PET/MRI sensitivity was 100% (95% CI, 96.7%-100%) and specificity was 99.5% (95% CI, 98.3%-99.9%). Corrected response-assessment 18 F-FDG PET/MRI sensitivity was 83.3% (95% CI, 36.5%-99.1%) and specificity was 100% (95% CI, 99.2%-100%). Modified Ann Arbor staging agreement between 18 F-FDG PET/CT and 18 F-FDG PET/MRI was perfect (κ = 1.0, P = 0.000). Deauville grading agreement between 18 F-FDG PET/MRI and 18 F-FDG PET/CT was excellent (κ = 0.835, P = 0.000). Conclusion: 18 F-FDG PET/MRI is a promising alternative to 18 F-FDG PET/CT for staging and response assessment in children with HL., (© 2021 by the Society of Nuclear Medicine and Molecular Imaging.)

During LT screening, children undergo CTA to determine hepatic artery anatomy. However, CTA imparts radiation, unlike MRA. The aim was to compare MRA to CTA in assessing hepatic artery anatomy in pediatric LT recipients. Twenty-one children (median age 8.9 years) who underwent both CTA and fl3D-ce MRA before LT were retrospectively included. Interreader variability between 2 radiologists, image quality, movement artifacts, and confidence scores, were used to compare MRA to CTA. Subgroup analyses for ages <6 years and ≥6 years were performed. Interreader variability for MRA and CTA in children <6 years was comparable (k = 0.839 and k = 0.757, respectively), while in children ≥6 years CTA was superior to MRA (k 1.000 and k 0.000, respectively). Overall image quality and confidence scores of CTA were significantly higher compared to MRA at all ages (2.8/3 vs. 2.3/3, p = .001; and 2.9/3 vs. 2.5/3, p = .003, respectively). Movement artifacts were significantly lower in CTA compared to MRA in children ≥6 years (1.0/3 vs. 1.7/3, p = .010, respectively). CTA is preferred over fl3D-ce MRA for the preoperative assessment of hepatic artery anatomy in children receiving LT, both at ages <6 years and ≥6 years., (© 2021 The Authors. Pediatric Transplantation published by Wiley Periodicals LLC.)

Objective: ADC (Apparent Diffusion Coefficient) derived from Diffusion-Weighted Imaging (DWI) has shown promise as a non-invasive quantitative imaging biomarker in Wilms' tumours. However, many non-Gaussian models could be applied to DWI. This study aimed to compare the suitability of four diffusion models (mono exponential, IVIM [Intravoxel Incoherent Motion], stretched exponential, and kurtosis) in Wilms' tumours and the unaffected contralateral kidneys., Materials and Methods: DWI data were retrospectively reviewed (110 Wilms' tumours and 75 normal kidney datasets). The goodness of fit for each model was measured voxel-wise using Akaike Information Criteria (AIC). Mean AIC was calculated for each tumour volume (or contralateral normal kidney tissue). One-way ANOVAs with Greenhouse-Geisser correction and post hoc tests using the Bonferroni correction evaluated significant differences between AIC values; the lowest AIC indicating the optimum model., Results: IVIM and stretched exponential provided the best fits to the Wilms' tumour DWI data. IVIM provided the best fit for the normal kidney data. Mono exponential was the least appropriate fitting method for both Wilms' tumour and normal kidney data., Discussion: The diffusion weighted signal in Wilms' tumours and normal kidney tissue does not exhibit a mono-exponential decay and is better described by non-Gaussian models of diffusion.

Purpose: Early differentiation of adenosine deaminase deficient severe combined immunodeficiency (ADA-SCID) from other forms of SCID may initiate appropriate treatment interventions with the aim of metabolic detoxification and improved outcome. Our hypothesis was that previously described radiological features (inferior scapular angle squaring and spurring and costochondral cupping) can differentiate ADA-SCID from other forms of SCID., Methods: Chest radiographs at clinical presentation between 2000 and 2017 of children with ADA-SCID were retrospectively included, provided that the radiological features were assessable. Random chest radiographs of children with other forms of SCID were included for comparison. Three paediatric radiologists (2 senior, 1 junior) assessed the radiographs for the specific radiological features and stated their diagnosis (ADA-SCID or non-ADA-SCID). An optimal threshold for test performance was defined using a ROC curve., Results: Thirty-six patients with ADA-SCID and twenty-five patients with non-ADA-SCID were included (median age 3.8 months). The optimal threshold for test performance was at approximately < 7 months old: sensitivity 91.7%, specificity 80.7%, interreader agreement was k = 0.709, AUC 0.862. The positive likelihood ratio for scapular squaring, scapular spur, and costochondral cupping was 4.0, 54.6 and 7.8, respectively. The test was valid when performed by both senior and junior paediatric radiologists., Conclusion: Radiological features such as scapular spurring, scapular squaring and costochondral cupping can reliably differentiate between ADA-SCID and other forms of SCID. This is true for children aged approximately < 7 months, and this is reliable when assessed by both senior and junior paediatric radiologists.

Objectives: Volume of necrosis in Wilms tumour is informative of chemotherapy response. Contrast-enhanced T 1 -weighted MRI (T 1 w) provides a measure of necrosis using gadolinium. This study aimed to develop a non-invasive method of identifying non-enhancing (necrotic) tissue in Wilms tumour., Methods: In this single centre, retrospective study, post-chemotherapy MRI data from 34 Wilms tumour patients were reviewed (March 2012-March 2017). Cases with multiple b value diffusion-weighted imaging (DWI) and T 1 w imaging pre- and post-gadolinium were included. Fractional T 1 enhancement maps were generated from the gadolinium T 1 w data. Multiple linear regression determined whether fitted parameters from a mono-exponential model (ADC) and bi-exponential model (IVIM - intravoxel incoherent motion) (D, D*, f) could predict fractional T 1 enhancement in Wilms tumours, using normalised pre-gadolinium T 1 w (T 1 w norm ) signal as an additional predictor. Measured and predicted fractional enhancement values were compared using the Bland-Altman plot. An optimum threshold for separating necrotic and viable tissue using fractional T 1 enhancement was established using ROC., Results: ADC and D (diffusion coefficient) provided the strongest predictors of fractional T 1 enhancement in tumour tissue (p < 0.001). Using the ADC-T 1 w norm model (adjusted R 2  = 0.4), little bias (mean difference = - 0.093, 95% confidence interval = [- 0.52, 0.34]) was shown between predicted and measured values of fractional enhancement and analysed via the Bland-Altman plot. The optimal threshold for differentiating viable and necrotic tissue was 33% fractional T 1 enhancement (based on measured values, AUC = 0.93; sensitivity = 85%; specificity = 90%)., Conclusions: Combining ADC and T 1 w imaging predicts enhancement in Wilms tumours and reliably identifies and measures necrotic tissue without gadolinium., Key Points: • Alternative method to identify necrotic tissue in Wilms tumour without using contrast agents but rather using diffusion and T 1 weighted MRI. • A method is presented to visualise and quantify necrotic tissue in Wilms tumour without contrast. • The proposed method has the potential to reduce costs and burden to Wilms tumour patients who undergo longitudinal follow-up imaging as contrast agents are not used.

Background: The usefulness of acoustic shadowing as a feature of pediatric kidney stone ultrasound (US) may be underestimated., Objective: The hypothesis was that the majority of stones in children have acoustic shadowing and that its specificity is high (>90%) in pediatric kidney stones., Materials and Methods: Our retrospective observational study included children who had undergone abdominal non-enhanced computed tomography (CT) for kidney stones in a pediatric renal stone referral centre between 2015 and 2016. US examinations prior to CT were retrospectively assessed for US features such as acoustic shadowing, twinkle artifact and stone size. These features were compared to CT as reference standard., Results: Thirty-one patients (median age: 13 years, range: 1-17 years) with 77 suspected kidney stones were included. The median stone size was 5 mm (interquartile range [IQR]: 5 mm). For acoustic shadowing, sensitivity was 70% (95% confidence interval [CI] 56-80%) and specificity was 100% (95% CI 56-100%). All kidney stones with a diameter ≥9 mm demonstrated shadowing. Sensitivity for twinkle artifact was 88% (95% CI 72-96%), but specificity for twinkle artifact could not be calculated due to the lack of true negatives. All false-positive stones on US demonstrated twinkle artifact, but none showed shadowing., Conclusion: Acoustic shadowing was demonstrated in the majority of pediatric kidney stones. Specificity was high, but this was not significant. Twinkle artifact is a sensitive US tool for detecting (pediatric) kidney calculi, but with a risk of false-positive findings.

Objective: To compare three different reconstruction techniques of CT data for the detection of pulmonary nodules in children under 13 years. Secondly to assess the prevalence of perifissural nodular opacities., Materials and Methods: The study consisted of chest CTs of 31 children (median age 6.9 years, range 2.1-12.7), of whom 17 had known extra-thoracic malignancies. Four observers assessed three techniques for the presence of nodules: axial 5 mm maximum intensity projections (MIPs) used in conjunction with 1 mm slices (mode A), 1 mm slices alone (mode B) and 3 mm slices (mode C). All modes were available in 3D. Per mode sensitivities were determined above a certain threshold of reader agreement. Confidence level and reader agreement for identification of an opacity as nodule served as surrogate for quality of nodule characterization., Results: 103 nodules (median size 2.0 mm) were detected. Mode A yielded the highest interreader agreement (κ 0.336) and a superior sensitivity (71%, p = 0.003) compared to mode B and C (κ 0.218, sensitivity 57% and κ 0.247, sensitivity 56%, respectively). Mode B provided the highest confidence level and interreader agreement with respect to nodule identification (mean 4.3/5, κw 0.508). Double reading improved and evened interreader agreement for all modes (κ 0.450), mode A maintained the highest sensitivity (89.1%, p = 0.05-0.08). A median of 1 intrapulmonary lymph node/patient was seen in children with and without malignancy., Conclusion: MIP improves the detection of pulmonary nodules in chest CTs of children, but overall interreader agreement is only fair. Double reading represents a powerful tool to increase diagnostic reliability in chest CTs of children with a malignancy. Nodule characterization is best with 1 mm slices. Intrapulmonary lymph nodes occur in children with and without malignancy., (Copyright © 2018 Elsevier B.V. All rights reserved.)

A case is presented in which fat drops are seen on MRI inside extensor compartment 2 and 3, in conjunction with a radius fracture. The occurrence of traumatic tendon sheath fat-fluid levels has been sparsely reported on CT and MR imaging. This case is the first report of post-traumatic tendon sheath fat drops. Although the clinical relevance of tendon sheath fat drops seems to be limited, it is important to detect and correctly diagnose these fat drops in order to provide an accurate and complete radiologic report.

Objectives: Dementia is foremost a clinical diagnosis. However, in diagnosing dementia, it is advocated to perform at least one neuroimaging study. This has two purposes: to rule out potential reversible dementia (PRD), and to help determine the dementia subtype. Our first goal was to establish if MRI combined with visual rating scales changes the clinical diagnosis. The second goal was to demonstrate if MRI contributes to a geriatrician's confidence in the diagnosis., Methods: The dementia subtype was determined prior to and after MRI. Scoring scales used were: global cortical atrophy (GCA), medial temporal atrophy (MTA), and white matter hyperintensity measured according to the Fazekas scale. The confidence level of the geriatrician was determined using a visual analogue scale., Results: One hundred and thirty-five patients were included. After MRI, the diagnosis changed in 23.7 % (CI 17.0 %-31.1 %) of patients. Change was due to vascular aetiology in 13.3 % of patients. PRD was found in 2.2 % of all patients. The confidence level in the diagnosis increased significantly after MRI (p = 0.001)., Conclusions: MRI, combined with visual rating scales, has a significant impact on dementia subtype diagnosis and on a geriatrician's confidence in the final diagnosis., Key Points: • MRI with visual rating scales changes the dementia subtype diagnosis significantly. • MRI is essential in demonstrating vascular disease as a cause of dementia. • All suspected dementia patients should undergo an MRI with visual rating scales. • MRI improves a geriatrician's confidence in the diagnosis of the dementia subtype. • MRI remains essential during the workup of dementia to exclude reversible causes.