Liver cirrhosis in children - the role of imaging in the diagnostic pathway.

Liver cirrhosis in children is a rare disease with multifactorial causes that are distinct from those in adults. Underlying reasons include cholestatic, viral, autoimmune, hereditary, metabolic and cardiac disorders. Early detection of fibrosis is important as clinical stabilization or even reversal of fibrosis can be achieved in some disorders with adequate treatment. This article focuses on the longitudinal evaluation of children with chronic liver disease with noninvasive imaging tools, which play an important role in detecting cirrhosis, defining underlying causes, grading fibrosis and monitoring patients during follow-up. Ultrasound is the primary imaging modality and it is used in a multiparametric fashion. Magnetic resonance imaging and computed tomography are usually applied second line for refined tissue characterization, clarification of nodular lesions and full delineation of abdominal vessels, including portosystemic communications.

[Transient Ischemia of One Leg in a Very Low Birthweight Infant]. / Transiente Ischämie eines Beines bei einem Frühgeborenen der 31+0 Schwangerschaftswoche.

Ischemias in the extremities are rather rare in the neonatal period. Both intrauterine and postnatal factors can cause ischemias. We present the diagnostic procedure and the course in a female very low birthweight (VLBW) infant with transient ischemia in the lower extremity.

Compressed SENSE in Pediatric Brain Tumor MR Imaging : Assessment of Image Quality, Examination Time and Energy Release.

PURPOSE: To compare the image quality, examination time, and total energy release of a standardized pediatric brain tumor magnetic resonance imaging (MRI) protocol performed with and without compressed sensitivity encoding (C-SENSE). Recently introduced as an acceleration technique in MRI, we hypothesized that C­SENSE would improve image quality, reduce the examination time and radiofrequency-induced energy release compared with conventional examination in a pediatric brain tumor protocol. METHODS: This retrospective study included 22 patients aged 2.33-18.83 years with different brain tumor types who had previously undergone conventional MRI examination and underwent follow-up C­SENSE examination. Both examinations were conducted with a 3.0-Tesla device and included pre-contrast and post-contrast T1-weighted turbo-field-echo, T2-weighted turbo-spin-echo, and fluid-attenuated inversion recovery sequences. Image quality was assessed in four anatomical regions of interest (tumor area, cerebral cortex, basal ganglia, and posterior fossa) using a 5-point scale. Reader preference between the standard and C­SENSE images was evaluated. The total examination duration and energy deposit were compared based on scanner log file analysis. RESULTS: Relative to standard examinations, C­SENSE examinations were characterized by shorter total examination times (26.1 ± 3.93 vs. 22.18 ± 2.31 min; P = 0.001), reduced total energy deposit (206.0 ± 19.7 vs. 92.3 ± 18.2 J/kg; P < 0.001), and higher image quality (overall P < 0.001). CONCLUSION: C­SENSE contributes to the improvement of image quality, reduction of scan times and radiofrequency-induced energy release relative to the standard protocol in pediatric brain tumor MRI.

A 3D Deep Neural Network for Liver Volumetry in 3T Contrast-Enhanced MRI.

PURPOSE: To create a fully automated, reliable, and fast segmentation tool for Gd-EOB-DTPA-enhanced MRI scans using deep learning. MATERIALS AND METHODS: Datasets of Gd-EOB-DTPA-enhanced liver MR images of 100 patients were assembled. Ground truth segmentation of the hepatobiliary phase images was performed manually. Automatic image segmentation was achieved with a deep convolutional neural network. RESULTS: Our neural network achieves an intraclass correlation coefficient (ICC) of 0.987, a Sørensen-Dice coefficient of 96.7 ± 1.9 % (mean ±â€Šstd), an overlap of 92 ±â€Š3.5 %, and a Hausdorff distance of 24.9 ±â€Š14.7 mm compared with two expert readers who corresponded to an ICC of 0.973, a Sørensen-Dice coefficient of 95.2 ±â€Š2.8 %, and an overlap of 90.9 ±â€Š4.9 %. A second human reader achieved a Sørensen-Dice coefficient of 95 % on a subset of the test set. CONCLUSION: Our study introduces a fully automated liver volumetry scheme for Gd-EOB-DTPA-enhanced MR imaging. The neural network achieves competitive concordance with the ground truth regarding ICC, Sørensen-Dice, and overlap compared with manual segmentation. The neural network performs the task in just 60 seconds. KEY POINTS: · The proposed neural network helps to segment the liver accurately, providing detailed information about patient-specific liver anatomy and volume.. · With the help of a deep learning-based neural network, fully automatic segmentation of the liver on MRI scans can be performed in seconds.. · A fully automatic segmentation scheme makes liver segmentation on MRI a valuable tool for treatment planning.. CITATION FORMAT: · Winther H, Hundt C, Ringe KI et al. A 3D Deep Neural Network for Liver Volumetry in 3T Contrast-Enhanced MRI. Fortschr Röntgenstr 2021; 193: 305 - 314.

The Impact of Unilateral 90Y-radioembolization on Functional Changes in the Contralateral Hepatic Lobe: The Prospective, Open-label RadioEmbolization, Volumetry, and Liver FuncTion Measurements (REVoluTion) Study.

Objectives: To investigate how metabolic function of the contralateral liver lobe is affected by unilateral radioembolization (RE), and to compare the changes in volume and metabolic function. Background: Unilateral RE induces contralateral liver hypertrophy, but it is unknown if metabolic liver function improves in line with volume increases. Methods: This prospective open-label, nonrandomized, therapy-optimizing study included all consecutive patients undergoing right-sided or sequential 90Y-RE for liver malignancies without underlying liver disease or biliary obstruction at a single center in Germany. Magnetic resonance imaging volumetry and hepatobiliary scintigraphy were performed immediately before RE and approximately 6 weeks after RE. Results: Twenty-three patients were evaluated (11 metastatic colorectal cancer, 4 cholangiocellular carcinoma, 3 metastatic breast cancer, 1 each of metastatic neuroendocrine tumor, hepatocellular carcinoma, renal cell carcinoma, oesophageal cancer, pancreatic ductal adenocarcinoma). In the untreated contralateral left liver lobe, mean (SD) metabolic function significantly increased from 1.34 (0.76) %/min/m2 at baseline to 1.56 (0.75) %/min/m2 6 weeks after RE (P = 0.024). The mean (SD) functional volume (liver volume minus tumor volume) of the left liver lobe significantly increased from baseline (407.3 [170.3] mL) to follow-up (499.1 [209.8] mL; P < 0.01), with an equivalent magnitude to the metabolic function increase. There were no reports of grade ≥3 adverse events. Conclusion: This study indicates that unilobar RE produces a significant increase in the metabolic function, and equivalent volume increase, of the contralateral lobe. RE may be a useful option to induce hypertrophy of the future liver remnant before surgical resection of primary or secondary liver malignancies.

[Simultaneous fracture of the acromion and coracoid process : Rare variant of double disruption of the superior shoulder suspensory complex]. / Gleichzeitige Frakturen von Acromion und Korakoid : Seltene Variante der doppelten Unterbrechung des "superior shoulder suspensory complex".

INTRODUCTION: Simultaneous fracture of the acromion and coracoid process represents a double disruption of the superior shoulder suspensory complex (SSSC). To date, the two largest reported series have comprised five and eight cases. The aim of this study was to investigate the functional outcome in patients who sustained this rare injury and to provide a review of the relevant literature. MATERIAL AND METHODS: In this retrospective study, patients who were surgically treated between 2009 and 2014 were identified from medical records and the patients were asked to attend a follow-up examination. Functional results, measured as the Constant score and Disabilities of the Arm, Shoulder and Hand (DASH) score, were the main outcomes of the study. RESULTS: A total of six patients underwent surgery for this type of double disruption of the SSSC. All but one patient suffered a high-impact trauma with other associated injuries. The coracoid fracture was classified as type I according to Ogawa in all cases, and the acromion fracture was type III according to Kuhn in 5 out of 6 cases. At least one of the fracture sites was stabilized. Of the patients one showed a concurrent lateral clavicle fracture, representing a third disruption of the SSSC, and another had a fracture of the medial third of the clavicle, representing a strut fracture. In these cases, two fracture sites were operated on. At an average follow-up period of 47 months all but one fracture had healed. In one patient, asymptomatic non-union of the acromion was found. The average DASH score was 22 points and the average Constant score was 74 points. CONCLUSION: This rare injury pattern is usually caused by high-impact trauma, and concomitant injuries frequently occur. Nevertheless, surgical stabilization of at least one of the fracture sites showed satisfactory functional results.

Initial Assessment of the Efficacy of Irreversible Electroporation in the Focal Treatment of Localized Renal Cell Carcinoma With Delayed-interval Kidney Tumor Resection (Irreversible Electroporation of Kidney Tumors Before Partial Nephrectomy [IRENE] Trial-An Ablate-and-Resect Pilot Study).

OBJECTIVE: To assess the efficacy of irreversible electroporation (IRE) ablation of pT1a renal cell carcinoma (RCC) in the first prospective, monocentric phase 2a pilot ablate-and-resect study (Irreversible Electroporation of Kidney Tumors Before Partial Nephrectomy [IRENE] trial). It has been postulated that focal IRE can bring about complete ablation of soft-tissue tumors with protection of healthy peritumoral tissue and anatomic structures. PATIENTS AND METHODS: The first 7 study patients with biopsy-proven pT1a RCC (15-39 mm) underwent IRE. Percutaneous computed tomography-guided IRE was performed with electrocardiographic triggering under general anesthesia and deep muscle paralysis with 3-6 monopolar electrodes positioned within the renal tumor. Twenty-eight days later, the tumor region was completely resected to confirm tumor destruction pathologically. Individual results for these patients are displayed, described, and discussed. RESULTS: Technical feasibility was attained in all patients, but electrode placement and ablation were complex, with a mean overall procedure time of 129 minutes. There were no major complications. Partial kidney resection was performed in 5 patients, and radical nephrectomy was performed in 2 patients because of central tumor location and ablation areas. Resections revealed by tumor, node, and metastasis classification of the International Union for Cancer Control 2017 no residual tumor as complete ablation in 4 cases (ypT0V0N0Pn0R0) and microscopic residual tumor cells as incomplete ablation in the other 3 cases (ypT1aV0N0Pn0R1). CONCLUSION: Renal percutaneous IRE appears to be a safe treatment for pT1a RCC but requires substantial procedural effort. Resection specimens of the ablation zone revealed a high rate of microscopic incomplete ablation 4 weeks after IRE. According to these initial study results, curative, kidney-sparing ablation of T1a RCC appears possible but needs technical improvement to ensure complete ablation.

[Ultrasound of muscular diseases in children and adolescents]. / Ultraschall bei Muskelerkrankungen im Kindes- und Jugendalter.

BACKGROUND: The increasing number of treatable hereditary neuromuscular diseases in children requires a diagnostic tool that can quickly, safely, and noninvasively identify affected patients directly after birth or when showing initial clinical symptoms. With clinical analysis alone, this is very difficult. IMAGING MODALITY: Near-field sonography of skeletal muscles has gradually become established as a successful method over the last 35 years. METHODOLOGICAL INNOVATIONS: Examination is performed using a strictly standardized protocol in isotopic muscle regions and with standardized sections and application parameters. Interpretation is performed with specific assessment criteria and nomenclature for the description of normal and pathological muscle architecture and echogenicity. This is sonographic tissue characterization. PERFORMANCE: Using case studies, the sonoanatomy and sonopathology of selected myo- and neuropathies, metabolic, inflammatory, and other lesions are illustrated. We present their differential diagnosis by texture and echogenicity analysis. Affected persons are identified in 70% up to 100% of cases, depending on the entity; specificity is less dependent on experience and training. Of the 12 disorders presented in this article, 6 are causally/symptomatically treatable today. ACHIEVEMENTS: Standardized myosonography is the imaging modality of first choice for detection of neuromuscular diseases. PRACTICAL RECOMMENDATIONS: High frequency (8-22 MHz) linear array transducer. Highly standardized examination modality. Simultaneous, paired comparison of affected persons and controls. If necessary, muscle tissue biopsy only after ultrasonic determination of a suitable area.

Validation of VX2 as a Hepatocellular Carcinoma Model: Comparison of the Molecular Reaction of VX2 and HepG2 Tumor Cells to Sorafenib In Vitro.

As there is currently no superior hepatocellular carcinoma (HCC) model with percutaneous vascular access for transarterial treatments available, the VX2 rabbit model is frequently used for in vivo investigations on liver carcinoma. However, the VX2 cell line was derived from a virus-induced skin papilloma that can form carcinosarcoma in liver of rabbits and the transferability of obtained results to HCC treatment remains open. Here we compared the most frequently investigated human HCC model cell line, HepG2, with VX2 cells in vitro in terms of sensitivity towards the broad specificity kinase inhibitor sorafenib and responsiveness to the addition of platelet-derived growth factor AB (PDGF-AB), vascular endothelial growth factor (VEGF) and hepatic growth factor (HGF), as well as insulin and interleukin-1ß (IL1ß). Phosphorylation of protein kinase B (AKT) the mitogen-activated protein kinases (MAPKs) p38 and p42/44 (extracellular signal-regulated kinase, ERK1/2) and inhibitor of kappa light chain gene enhancer alpha (IĸBα) was determined by western blotting as these events are associated with early signaling cascades. Additionally, the inhibition of phosphorylation under sorafenib treatment was investigated. Sorafenib was equally toxic to both cell lines, but only in HepG2 was activation of caspase 3/7 activity, as a sign of apoptosis, observed. VX2 cells exhibited generally more intense phosphorylation signals in response to the growth factors and also serum. In contrast to VX2, HepG2 cells showed no response to PDGF-AB or VEGF as determined by kinase phosphorylation. In both cell lines, sorafenib inhibited growth factor-induced phosphorylation of ERK and p38-MAPK. AKT phosphorylation was only inhibited in VX2 cells and IĸBα phosphorylation was not influenced by this kinase inhibitor in either cell type. Taken together, the two cellular models for HCC share several features related to sorafenib application, but differed in their responsiveness towards growth factors. Therefore, results obtained with the VX2 model cannot be extended to human HCC without appropriate caution.

Modified transarterial chemoembolization with locoregional administration of sorafenib for treating hepatocellular carcinoma: feasibility, efficacy, and safety in the VX-2 rabbit liver tumor model.

PURPOSE: We aimed to assess the feasibility, efficacy and safety of a local application of sorafenib within a conventional transarterial chemoembolization in the VX-2 tumor-bearing rabbit model. METHODS: VX-2 tumors were induced in the left liver lobe of 10 New Zealand White rabbits. After two weeks, growth was verified by contrast-enhanced computed tomography (CT). Five rabbits were treated by transarterial chemoembolization using an emulsion of sorafenib and ethiodized oil (referred to as SORATACE; n=5). Rabbits receiving oral sorafenib for two weeks (n=2) and untreated rabbits (n=3) served as controls. After two weeks, contrast-enhanced CT was performed, followed by animal necropsy. RESULTS: The change in tumor diameter between baseline and follow-up was significantly different in the SORATACE group compared with the other groups; tumor shrinkage was observed in the SORATACE group only (P = 0.016). In both control groups, preserved hypervascularity was seen in the follow-up CT in all but one tumor. All tumors in the SORATACE group were devascularized in the follow-up CT. Importantly, substantial parenchymal damage in nontargeted areas of the tumor-bearing liver lobe was seen in rabbits treated with SORATACE. CONCLUSION: SORATACE demonstrated high efficacy in the treatment of experimental VX-2 liver tumors but was also associated with substantial liver parenchymal toxicity.

Irreversible Electroporation (IRE): Standardization of Terminology and Reporting Criteria for Analysis and Comparison.

BACKGROUND: Irreversible electroporation (IRE) as newer ablation modality has been introduced and its clinical niche is under investigation. At present just one IRE system has been approved for clinical use and is currently commercially available (NanoKnife® system). In 2014, the International Working Group on Image-Guided Tumor Ablation updated the recommendation about standardization of terms and reporting criteria for image-guided tumor ablation. The IRE method is not covered in detail. But the non-thermal IRE method and the NanoKnife System differ fundamentally from established ablations techniques, especially thermal approaches, e.g. radio frequency ablation (RFA). MATERIAL/METHODS: As numerous publications on IRE with varying terminology exist so far - with numbers continuously increasing - standardized terms and reporting criteria of IRE are needed urgently. The use of standardized terminology may then allow for a better inter-study comparison of the methodology applied as well as results achieved. RESULTS: Thus, the main objective of this document is to supplement the updated recommendation for image-guided tumor ablation by outlining a standardized set of terminology for the IRE procedure with the NanoKnife Sytem as well as address essential clinical and technical informations that should be provided when reporting on IRE tumor ablation. CONCLUSIONS: We emphasize that the usage of all above recommended reporting criteria and terms can make IRE ablation reports comparable and provide treatment transparency to assess the current value of IRE and provide further development.

First Delayed Resection Findings After Irreversible Electroporation (IRE) of Human Localised Renal Cell Carcinoma (RCC) in the IRENE Pilot Phase 2a Trial.

INTRODUCTION: It is postulated that focal IRE affords complete ablation of soft-tissue tumours while protecting the healthy peritumoral tissue. Therefore, IRE may be an interesting option for minimally invasive, kidney-tissue-sparing, non-thermal ablation of renal tumours. AIM: With this current pilot study ("IRENE trial"), we present the first detailed histopathological data of IRE of human RCC followed by delayed tumour resection. The aim of this interim analysis of the first three patients was to investigate the ablation efficiency of percutaneous image-guided focal IRE in RCC, to assess whether a complete ablation of T1a RCC and tissue preservation with the NanoKnife system is possible and to decide whether the ablation parameters need to be altered. METHODS: Following resection 4 weeks after percutaneous IRE, the success of ablation and detailed histopathological description were used to check the ablation parameters. RESULTS: The IRE led to a high degree of damage to the renal tumours (1 central, 2 peripheral; size range 15-17 mm). The postulated homogeneous, isomorphic damage was only partly confirmed. We found a zonal structuring of the ablation zone, negative margins and, enclosed within the ablation zone, very small tumour residues of unclear malignancy. CONCLUSION: According to these initial, preliminary study results of the first three renal cases, a new zonal distribution of IRE damage was described and the curative intended, renal saving focal ablation of localised RCC below <3 cm by percutaneous IRE by the NanoKnife system appears to be possible, but needs further, systematic evaluation for this treatment method and treatment protocol.

Traumatically shattered kidney without urine extravasation or vascular amputation.

The American Association for the Surgery of Trauma (AAST) injury scoring scale is commonly used for genitourinary injuries. Normally, grade 4-5 lacerations of the kidney show involvement of the pelvicalyceal system (PCS) with urine extravasation (UE). We present a case of a 41-year-old woman who was hospitalised with macrohaematuria and retroperitoneal haematoma after severe blunt acceleration flank trauma. CT scan showed an extended laceration of the left kidney with separation of upper pole. This is the first case of an extended kidney laceration without UE due to rupture within the dichotomous PCS, which healed up after selective embolisation. If possible, severe renal bleeding should be treated with selective embolisation as an alternative to surgery. Any suspected involvement of the PCS should undergo retrograde ureteropyelography and urinary diversion.

Irreversible electroporation (IRE) fails to demonstrate efficacy in a prospective multicenter phase II trial on lung malignancies: the ALICE trial.

PURPOSE: To assess safety and efficacy of irreversible electroporation (IRE) of lung malignancies. MATERIALS AND METHODS: Patients with primary and secondary lung malignancies and preserved lung function were included in this prospective single arm trial. Primary and secondary endpoints were safety and efficacy. Recruitment goal was 36 subjects in 2 centers. Patients underwent IRE under general anesthesia with probe placement performed in Fluoroscopy-CT. The IRE system employed was NanoKnife® (Angiodynamics). System settings for the ablation procedure followed the manufacturer's recommendations. The Mann-Whitney U test was used to evaluate the correlation of nine technical parameters with local tumor control. Median follow up was 12 months. RESULTS: The expected efficacy was not met at interim analysis and the trial was stopped prematurely after inclusion of 23 patients (13/10 between both centers). The dominant tumor entity was colorectal (n = 13). The median tumor diameter was 16 mm (8-27 mm). Pneumothoraces were observed in 11 of 23 patients with chest tubes required in 8 (35 %). Frequently observed alveolar hemorrhage never led to significant hemoptysis. 14/23 showed progressive disease (61 %). Stable disease was found in 1 (4 %), partial remission in 1 (4 %) and complete remission in 7 (30 %) patients. The relative increase of the current during ablation was significantly higher in the group treated successfully as compared to the group presenting local recurrence (p < 0.05). Needle tract seeding was found in three cases (13 %). CONCLUSIONS: IRE is not effective for the treatment of lung malignancies. We hypothesize that the energy deposition with current IRE probes is highly sensitive to air exposure.

Time-resolved perfusion imaging at the angiography suite: preclinical comparison of a new flat-detector application to computed tomography perfusion.

OBJECTIVES: The objective of this study was to compare the parameter maps of a new flat-panel detector application for time-resolved perfusion imaging in the angiography room (FD-CTP) with computed tomography perfusion (CTP) in an experimental tumor model. MATERIALS AND METHODS: Twenty-four VX2 tumors were implanted into the hind legs of 12 rabbits. Three weeks later, FD-CTP (Artis zeego; Siemens) and CTP (SOMATOM Definition AS +; Siemens) were performed. The parameter maps for the FD-CTP were calculated using a prototype software, and those for the CTP were calculated with VPCT-body software on a dedicated syngo MultiModality Workplace. The parameters were compared using Pearson product-moment correlation coefficient and linear regression analysis. RESULTS: The Pearson product-moment correlation coefficient showed good correlation values for both the intratumoral blood volume of 0.848 (P < 0.01) and the blood flow of 0.698 (P < 0.01). The linear regression analysis of the perfusion between FD-CTP and CTP showed for the blood volume a regression equation y = 4.44x + 36.72 (P < 0.01) and for the blood flow y = 0.75x + 14.61 (P < 0.01). CONCLUSIONS: This preclinical study provides evidence that FD-CTP allows a time-resolved (dynamic) perfusion imaging of tumors similar to CTP, which provides the basis for clinical applications such as the assessment of tumor response to locoregional therapies directly in the angiography suite.