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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]

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/).)

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.)