Contrast-enhanced ultrasound of adrenal hemorrhage: a helpful problem solving tool.

AIMS: A focal lesion in the region of the adrenal gland in a newborn often requires further assessment. Ultrasound (US) is the initial imaging method of choice in young children as it does not use radiation or require sedation and it has excellent spatial resolution. In this case series, we present contrast-enhanced ultrasound (CEUS) as a problem-solving tool in the evaluation of neonatal adrenal lesions. MATERIAL AND METHODS: The imaging and medical records of five patients with adrenal lesions were retrospectively reviewed. All patients underwent US as an initial examination and all had US follow-up. Additionally, two patients had MRI examinations. CEUS was performed in all patients as a follow up examination. The enhancement characteristics of the adrenal masses on CEUS were analyzed with the use of VueBox software. In addition, qualitative analysis of the cine loops for the presence of vascularization within the lesions was performed by consensus between two radiologists. RESULTS: The presence of an adrenal hematoma was correctly detected and characterized by CEUS in all five cases using VueBox perfusion analysis. Adrenal hematomas had no internal perfusion and flat time intensity curves. CONCLUSION: The quantitative and qualitative CEUS assessment of the mass can distinguish hemorrhage from a malignant lesion. Based on our findings, CEUS could serve as an alternative diagnostic tool to magnetic resonance imaging in the diagnosis of slowly resolving NAH lesions.

Recurrent or persistent salivary gland enlargement in children: When is it Sjögren's?

OBJECTIVES: To describe characteristic features in children with recurrent or persistent salivary gland enlargement and to propose a diagnostic algorithm with specific consideration for Sjögren's disease (SD). METHODS: In this single-center, prospective study, 45 patients < 18 years, with recurrent or persistent salivary gland enlargement of unknown etiology were enrolled from 2006 to 2019. We collected detailed clinical information to characterize this group of patients including specific details of their major salivary gland signs and symptoms. We compared clinical, laboratory and radiological parameters between 4 groups based on the results of labial salivary gland biopsy (LSGB) and between patients who met existing SD criteria or not. RESULTS: 44 patients, with a mean age of 6.8 years and female to male ratio 21:23 were observed over a mean of 3.8 years. Characteristics of salivary gland swelling episodes varied considerably between individuals, but the majority experienced ≤5 episodes per year, lasting ≤ 1 week, with swelling affecting either or both glands. Ocular and oral dryness symptoms were observed only in 25% and 59% patients, respectively. The majority were positive for ANA, but negative for SD-specific antibodies. A total of 75% patients fulfilled at least one of the existing SD criteria. CONCLUSION: SD is a major cause of recurrent salivary gland enlargement in children. For children meeting adult criteria, the diagnosis of SD is clear. However, for the many children without dryness symptoms, objective dryness, or SD-specific antibodies, further workup including a combination of salivary gland imaging and histopathological examination can help establish the diagnosis of SD.

Bilateral Ovarian Germ Cell Tumor in a 46,XX Female with Nijmegen Breakage Syndrome and Hypergonadotropic Hypogonadism

Nijmegen breakage syndrome (NBS) is a rare autosomal recessive disease, affecting mainly patients of Slavic origin. It is caused by a defect in the NBN gene, resulting in defective nibrin protein formation. This leads to chromosomal instability, which predisposes to cancer, with lymphoid malignancies predominating. Nibrin is also involved in gonadal development and its disfunction in females with NBS frequently results in a pure gonadal dysgenesis (PGD) causing hypergonadotropic hypogonadism. However, only a few ovarian tumors in NBS patients have been reported to date. We describe the first case of a girl with NBS with PGD, who developed metachronous bilateral ovarian germ cell tumors (dysgerminoma and gonadoblastoma). Pathogenesis of PGD, neoplastic transformation and therapeutic approach in females with NBS are discussed.

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.

European Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB): An Update on the Pediatric CEUS Registry on Behalf of the "EFSUMB Pediatric CEUS Registry Working Group".

The European Federation of Ultrasound in Medicine and Biology (EFSUMB) created the "EFSUMB Pediatric Registry" (EFSUMB EPR) with the purpose of collecting data regarding the intravenous application of pediatric contrast-enhanced ultrasound (CEUS). The primary aim was to document the current clinical practice and usefulness of the technique and secondarily to assess CEUS safety in children. We issue the preliminary results of this database and examine the overall practice of CEUS in children in Europe.

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.

Response to Comment on Jaworska, J. et al. Consensus on the Application of Lung Ultrasound in Pneumonia and Bronchiolitis in Children. Diagnostics 2020, 10, 935.

Thank you for the opportunity to respond to the issues raised by Nenna et al [...].

Consensus on the Application of Lung Ultrasound in Pneumonia and Bronchiolitis in Children.

This evidence-based consensus aims to establish the role of point-of-care lung ultrasound in the management of pneumonia and bronchiolitis in paediatric patients. A panel of thirteen experts form five Polish tertiary pediatric centres was involved in the development of this document. The literature search was done in PubMed database. Statements were established based on a review of full-text articles published in English up to December 2019. The development of this consensus was conducted according to the GRADE (Grading of Recommendations, Assessment, Development and Evaluations)-adopted and Delphi method. Initially, 22 proposed statements were debated over 3 rounds of on-line discussion and anonymous voting sessions. A total of 17 statements were agreed upon, including four statements referring to general issues, nine referring to pneumonia and four to bronchiolitis. For five statements experts did not achieve an agreement. The evidence supporting each statement was evaluated to assess the strength of each statement. Overall, eight statements were rated strong, five statements moderate, and four statements weak. For each statement, experts provided their comments based on the literature review and their own experience. This consensus is the first to establish the role of lung ultrasound in the diagnosis and management of pneumonia and bronchiolitis in children as an evidence-based method of imaging.

Ultrasound Curricula of Student Education in Europe: Summary of the Experience.

Background Despite the increasing role of ultrasound, structured ultrasound teaching is only slowly being integrated into the curricula of medical schools and universities all over Europe. Aim To survey the current situation at European universities regarding the integration of ultrasound in student medical education and to report on models of student ultrasound training from selected European universities. Methods A questionnaire survey focusing on the implementation of curricular ultrasound education was sent out to the 28 presidents of the national ultrasound societies of the European Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB), who were asked to distribute the questionnaires to the medical universities of their countries. Results Overall, 53 questionnaires were returned from 46 universities in 17 European countries. In most of the universities (40/46 universities, 87%), the theoretical background of ultrasound is taught. However, in only a minority of universities is ultrasound integrated in anatomy courses (8/46 universities, 17%) or basic science courses (16/46 universities, 35%). Practical skills in ultrasound are taught in 56% of the universities (26/46 universities) and tested in a practical exam in seven of the responding universities (15%). The number of hours in which ultrasound was taught ranged from one to 58 (mean, seven). The respondents reported that lack of time and limited faculty funding were major hurdles. Conclusion According to our survey, only a minority of European universities has integrated ultrasound into the preclinical curriculum thus far. Future EFSUMB initiatives will continue to promote the introduction of ultrasound as an integrative part of the core curriculum of student medical education, and the preparation of proper teaching material.

Recommendations for Lung Ultrasound in Internal Medicine.

A growing amount of evidence prompts us to update the first version of recommendations for lung ultrasound in internal medicine (POLLUS-IM) that was published in 2018. The recommendations were established in several stages, consisting of: literature review, assessment of literature data quality (with the application of QUADAS, QUADAS-2 and GRADE criteria) and expert evaluation carried out consistently with the modified Delphi method (three rounds of on-line discussions, followed by a secret ballot by the panel of experts after each completed discussion). Publications to be analyzed were selected from the following databases: Pubmed, Medline, OVID, and Embase. New reports published as of October 2019 were added to the existing POLLUS-IM database used for the original publication of 2018. Altogether, 528 publications were systematically reviewed, including 253 new reports published between September 2017 and October 2019. The new recommendations concern the following conditions and issues: pneumonia, heart failure, monitoring dialyzed patients' hydration status, assessment of pleural effusion, pulmonary embolism and diaphragm function assessment. POLLUS-IM 2020 recommendations were established primarily for clinicians who utilize lung ultrasound in their everyday clinical work.

Consensus of the Study Group for Point-of-Care Lung Ultrasound in the intensive care management of COVID-19 patients.

Respiratory failure is a dominating medical issue in the severe course of COVID-19. Both at the stage of diagnostics prior to admission to the intensive care unit and during the monitoring of lesion evolution, diagnostic imaging techniques may significantly influence clinical decisions. Although computed tomography remains the gold standard for diagnosing lung diseases, its usefulness for infected, critically ill patients has been largely limited during the pandemic. Reports from those countries in which the healthcare systems were most seriously overloaded with patients with COVID-19-induced pneumonia stress the key role of point-of-care lung ultrasound performed by clinicians first during preliminary diagnostics and then while monitoring disease dynamics. This consensus, worked out by an interdisciplinary team of specialists forming the Study Group for Point-of-Care Lung Ultrasound in the Intensive Care Management of COVID-19 Patients, presents a broad spectrum of aspects regarding the analysed issue. Its concise form is meant to serve clinicians who perform ultrasound as a straightforward and informative guide.

Sonographic appearance of rhabdomyolysis - a systematic review of the literature.

Rhabdomyolysis is the process of striated muscle cell lysis, during which proteins and microelements such as myoglobin are released into the bloodstream. It is important to diagnose rhabdomyolysis as soon as possible and start the treatment according to severity, as it is a state that significantly increases the mortality of the patients. The current gold standard of rhabdomyolysis diagnosis is the creatine kinase plasma concentration test, but it can be also diagnosed with imaging techniques, such as ultrasound (US). This review aims to gather previously published information regarding sonographic appearance of rhabdomyolysis. We searched through PubMed and ScienceDirect databases for studies using designed queries. After the selection process we were left with 13 studies containing a description of US appearance of rhabdomyolysis confirmed with a CK plasma level test. Findings described in the majority of the cases were muscle thickening, ground glass opacity, traits of edema and anechoic areas. Other than these, there were several less often reported findings. As a conclusion, rhabdomyolysis seems to have its own US appearance, but for now it cannot be precisely specified and needs further research for clarification.

Use of bedside ultrasound to assess fluid status: a literature review.

The assessment of a patient's body fluid status is a challenging task for modern clinicians. Ultrasonography has numerous advantages, the most important being reproducibility and bedside monitoring of the patient. The examination is quick and has a significant diagnostic value. We reviewed the literature to assess the possibility of using ultrasound methods for evaluating body fluid status. The search of PubMed and Medline databases was performed up to February 2019. Data from published reports and clinical observations show that the quick and noninvasive ultrasound examination facilitates the assessment of intravascular volume status and that the results correlate with other modalities, including invasive methods. Ultrasound enables physicians to determine the baseline status of hydration and to monitor the patient during fluid therapy. Additionally, it allows an assessment of asymptomatic patients, patients who are well adapted to chronic oxygen deficiency, and those who develop pulmonary congestion secondary to congestive heart failure or chronic kidney disease. The development of a protocol for an ultrasound assessment of the volume status would significantly facilitate the everyday practice of internal medicine specialists.

Polish recommendations for lung ultrasound in internal medicine (POLLUS-IM).

The aim of this study was to establish recommendations for the use of lung ultrasound in internal medicine, based on reliable data and expert opinions. Methods: The bibliography from the databases (Pubmed, Medline, OVID, Embase) has been fully reviewed up to August 2017. Members of the expert group assessed the credibility of the literature data. Then, in three rounds, a discussion was held on individual recommendations (in accordance with the Delphi procedure) followed by secret voting. Thirty-eight recommendations for the use of lung ultrasound in internal medicine were established as well as discussed and subjected to secret voting in three rounds. The first 31 recommendations concerned the use of ultrasound in the diagnosis of the following conditions: pneumothorax, pulmonary consolidation, pneumonia, atelectasis, pulmonary embolism, malignant neoplastic lesions, interstitial lung lesions, cardiogenic pulmonary edema, interstitial lung diseases with fibrosis, dyspnea, pleural pain and acute cough. Furthermore, seven additional statements were made regarding the technical conditions of lung ultrasound examination and the need for training in the basics of lung ultrasound in a group of doctors during their specialization programs and medical students. The panel of experts established a consensus on all 38 recommendations.

Bacterial meningitis in neonates and infants - the sonographic picture.

Bacterial meningitis is a major diagnostic and therapeutic problem among children and neonates, with severe, rapidly progressing course and potentially life-threatening complications. Early antibacterial treatment is essential for the patient's favorable prognosis. Cerebral imaging plays an important role in the diagnostic process alongside physical examination and laboratory tests. Magnetic resonance imaging is the gold standard for diagnosing bacterial meningitis. Because of limited availability of magnetic resonance imaging, cranial ultrasound is the first imaging procedure to be performed (if the anterior fontanelle is preserved providing an adequate acoustic window). The safety and reliability of ultrasound examination, possibility to perform the examination at bedside without the need for sedation make cranial ultrasound a useful tool both for preliminary diagnostic investigation and for the monitoring of both treatment and long-term complications. Sonographic findings in patients with bacterial meningitis and possible complications are diverse. Changes can be seen on the surface of the brain, in the extra-axial space, in the ventricular system and in brain tissue. In some cases they can also be visible in the lumbosacral segment of the spinal cord. This paper presents ultrasound characteristics of lesions associated with bacterial meningitis in neonates and infants, based on the authors' own material and data from the available literature.

Langerhans cell histiocytosis in children - a disease with many faces. Recent advances in pathogenesis, diagnostic examinations and treatment.

Langerhans cell histiocytosis is a rare clonal disease characterized by the proliferation of CD1a-positive immature dendritic cells. The purpose of this article was to present an updated review of recent advances in the pathogenesis, clinical features, imaging and treatment of this disease. The discovery of oncogenic BRAF mutations and the presence of proinflammatory cytokines and chemokines confirmed the unusual characteristics of this disease. Currently, children with organ involvement who do not have a good response to chemotherapy and have neurodegeneration or diabetes insipidus are the most problematic patients. Further research is needed to improve the results of treatment.

Is a linear probe helpful in diagnosing diseases of pulmonary interstitial spaces?

In a lung ultrasound examination, interstitial lung lesions are visible as numerous B-line artifacts, and are best recorded with the use of a convex probe. Interstitial lung lesions may result from many conditions, including cardiogenic pulmonary oedema, non-cardiogenic pulmonary oedema, or interstitial lung disease. Hence difficulties in the differential diagnostics of the above clinical conditions. This article presents cases of patients suffering from interstitial lung lesions discovered in the course of lung ultrasound examination. The patients were examined with a 3.5-5.0 MHz convex probe and a 7.0-11.0 MHz linear probe. Ultrasound images have been analysed, and differences in the imaging with both probes in patients with interstitial lung lesions have been detailed. The use of a linear probe in patients with interstitial lung lesions (discovered with a convex or a micro-convex probe) provides additional information on the source of the origin of the lesions.

Role of Contrast-Enhanced Ultrasound (CEUS) in Paediatric Practice: An EFSUMB Position Statement.

The use of contrast-enhanced ultrasound (CEUS) in adults is well established in many different areas, with a number of current applications deemed "off-label", but the use supported by clinical experience and evidence. Paediatric CEUS is also an "off-label" application until recently with approval specifically for assessment of focal liver lesions. Nevertheless there is mounting evidence of the usefulness of CEUS in children in many areas, primarily as an imaging technique that reduces exposure to radiation, iodinated contrast medium and the "patient-friendly" circumstances of ultrasonography. This position statement of the European Federation of Societies in Ultrasound and Medicine (EFSUMB) assesses the current status of CEUS applications in children and makes suggestions for further development of this technique.