A chance to cut is a chance to cure: complete resection of an atypical neurofibroma prevents further progression to malignancy.

Plexiform neurofibromas are the hallmark of neurofibromatosis type 1 (NF1) and significantly contribute to the overall burden of disease. While surgical excision has long been the only available therapy, the MEK inhibitor (MEKi) selumetinib has been approved as a non-surgical treatment option for these tumors in 2020 (USA) and 2021 (Europe), respectively. However, selumetinib will result in tumor shrinkage only after several months of therapy and might not prevent malignant transformation of a plexiform neurofibroma that occurs with a frequency of 10-15%. Here, we demonstrate that surgical excision might be the therapy of choice in some plexiform neurofibromas despite the availability of MEKi therapy.

Ultrasound of the chest and mediastinum in children, interventions and artefacts. WFUMB review paper (part 3).

Ultrasound (US) is an ideal diagnostic tool for paediatric patients owning to its high spatial and temporal resolution, realtime imaging, and lack of ionizing radiation and bedside availability. The lack of superficial adipose tissue and favourable acoustic windows in children makes US the first line of investigation for evaluation of pleural and chest wall abnormalities.In the first part of the topic the technical requirements were explained and the use of ultrasound in the lung and pleura in paediatric patients were discussed. In the second part lung parenchymal diseases with their subpleural consolidations are reflected. In the third part, the use of ultrasound for chest wall, mediastinum, diaphragmatic diseases, trachea, interventions and artifacts in paediatric patients are summarized.

Lung ultrasound in children, WFUMB review paper (part 2).

Ultrasound (US) is an ideal diagnostic tool for paediatric patients owning to its high spatial and temporal resolution, real-time imaging, and lack of ionizing radiation and bedside availability. In the current World Federation of Societies for Ultra-sound in Medicine and Biology (WFUMB) paper series so far (part I) the topic has been introduced and the technical require-ments explained. In the present paper the use of US in the lung in paediatric patients is analysed. Lung diseases including the interstitial syndrome, bacterial pneumonia and viral infections, CoViD findings, atelectasis, lung consolidation, bronchiolitis and congenital diseases of the respiratory system including congenital pulmonary airway malformation (CPAM) and sequester but also pneumothorax are discussed.

Ultrasound of the pleura in children, WFUMB review paper.

Ultrasound (US) is an ideal diagnostic tool for paediatric patients owning to its high spatial and temporal resolution, real-time imaging, and lack of ionizing radiation and bedside availability. The lack of superficial adipose tissue and favorable acoustic windows in children makes US the first line of investigation for the evaluation of pleural and chest wall abnormali-ties. Lung parenchyma was previously thought to be inaccessible to ultrasound due to the presence of the air and bony thorax. The change in attitude and growing awareness of the diagnostic possibilities has led to lung ultrasound (LUS) being accepted as a valuable point of care method. In addition, the application of LUS has widened with improvements in technology such as higher resolution transducers, harmonic imaging and contrast-enhanced ultrasound. In the current World Federation of Societies for Ultrasound in Medicine and Biology (WFUMB) paper series the topic will be introduced, the technical requirements explained and the use of ultrasound in the lung and pleura in pediatric patients are discussed.

Time Tracking of Standard Ultrasound Examinations in Pediatric Hospitals and Pediatric Medical Practices - A Multicenter Study by the Pediatric Section of the German Society of Ultrasound in Medicine (DEGUM).

PURPOSE: Ultrasonography is the primary imaging modality in pediatrics but still lacks sufficient reimbursement in Germany. In this multicenter study, national data for the duration of standard ultrasound in pediatrics were systematically documented in order to specify the actual time required. MATERIALS AND METHODS: N = 10 hospitals (N = 5 university hospitals, N = 5 non-university hospitals) and N = 3 medical practices in Germany recorded the entire process of an ultrasound examination in a special protocol developed by the Pediatric Section of the DEGUM. The duration of each of seven single steps during ultrasonography (from data input to final discussion of the results) of different organ systems was logged. RESULTS: In total, N = 2118 examinations from different organ systems were recorded. N = 10 organ systems were examined frequently (> 30 times). The total duration of an ultrasound examination was statistically significantly longer in hospitals compared to medical practices (median (IQR) 27 min. (18-38) vs. 12 min. (9-17), p < 0.001). The "hands-on" patient time was approximately one half of the total required time in both settings (49.9 % vs. 48.9 %). Ultrasonography of the abdomen and brain lasted longer in university hospitals than in non-university hospitals (p < 0.001, and p = 0.04, respectively). Cooperation and age did not uniformly correlate with the total duration. CONCLUSION: This study provides novel comprehensive national data for the duration of standardized ultrasound examinations of children and adolescents in Germany. These data are essential for a further evaluation of the economic costs and should support better remuneration in the future.

Cranial Doppler ultrasound in Vein of Galen malformation.

INTRODUCTION: Vein of Galen malformation (VGM) is the severest paediatric neurovascular disease usually requiring multiple staged embolisations. In the high-risk group of children with high-flow arteriovenous shunts, timing of treatment is uncertain. Low Doppler resistance index (RI) is known to be associated with adverse outcome in hypoxic-ischaemic brain injury in children. In this study, we want to present our long-term results of cranial transfontanellar Doppler ultrasound in children with VGM. METHODS: We identified and retrospectively analysed 264 transfontanellar Doppler measurements in 19 endovascular-treated true VGM (five females, 14 males) between 2000 and 2013. The recordings were obtained from the internal carotid arteries (ICA), the anterior cerebral arteries (ACA) and the basilar arteries (BA). Maximal systolic velocity (Vs), end-diastolic velocity (Ved) and the RI were measured before and after embolisation. RESULTS: Untreated, nearly all cases showed pathologic high systolic (up to >1.0 m/s), very high diastolic velocities (up to >0.5 m/s) and low RI (<0.6). There were statistically significant differences (p = 0.012) between the pre-embolisation RI and the post-embolisation RI with pathologic low RI before and nearly normal RI after successful shunt reduction. CONCLUSION: Successful endovascular shunt reduction in VGM leads to significant changes of cranial Doppler RI from pathologic low values to normal values. We propose cranial Doppler ultrasound as an adjunctive technique to other screenings in the management of VGM. Further research is warranted to evaluate the role of the RI in the treatment timing decision.

Caroli disease associated with vein of Galen malformation in a male child.

We report the first case of a male child with both Caroli disease and vein of Galen malformation. The neonate presented to our department with congestive heart failure as a result of the intracranial arteriovenous high-flow shunt. Over time, several endovascular embolizations led to a complete angiographic occlusion of the shunt. Additionally, the diagnosis of Caroli disease was made at the age of 2 months. He developed choledocholithiasis necessitating endoscopic sphincterotomy and stone extraction. As a prolonged medical treatment he received ursodeoxycholic acid and antibiotics. A coincidence of Caroli disease and vein of Galen malformation has not yet been described. Both diseases are very rare, leading to the question of whether there is a link in the pathogenesis. Based on the few previously described underlying mechanisms, we develop hypotheses about the relationship between both rare diseases. We consider overexpression of vascular endothelial growth factor and its receptors as a possible common molecular mechanism in their pathogenesis. We also highlight the critical role of increased expression of the Notch ligand Jagged 1 both in the development of cerebral arteriovenous malformations in general and in the formation of dilated intrahepatic bile ducts (eg, in Caroli disease).

Vein of Galen aneurysmal malformation: combined transvenous and transarterial method using a "kissing microcatheter technique".

INTRODUCTION: Vein of Galen aneurysmal malformation (VGAM) is a severe pediatric neurovascular disease. Children often present with congestive heart failure in the neonatal period. In the last decades, endovascular treatment became the first therapeutic option. The purpose of this study is to report our results in the treatment of VGAM with a combined transvenous and transarterial method in the last ten years. METHODS: In our cohort of 28 patients with VGAM, 22 patients were treated endovascularly between 1992 and 2010. In the last 10 years, a consecutive series of 14 children were treated with a combined transvenous and transarterial method. The therapeutic goal was immediate shunt reduction of the arteriovenous malformation, especially in the neonatal period. Closure of the fistulous connections was achieved by coiling using a combined transvenous and transarterial approach, called "kissing microcatheter technique". RESULTS: Eight of 14 children presented in the neonatal period with severe congestive heart failure. The other six patients presented between the age of 2 and 17 months. One patient died due to a non-procedural complication in another hospital 2 years after the last treatment. Complete or >90% of angiographically confirmed closure of the malformation was documented in 11 of 14 patients. Normal or near-normal outcome was achieved in 9 of 13 surviving children, a non-favorable outcome was observed in four children. Control of heart failure was achieved in all patients. CONCLUSION: Endovascular treatment of VGAM using a combined transvenous and transarterial method is a safe procedure with a low complication rate. The overall outcome can be improved, especially in the high-risk neonatal group with congestive heart failure.