Imaging urolithiasis: complications and interventions in children.

Urolithiasis affects people in all age groups, but over the last decades there has been an increasing incidence in children. Typical symptoms include abdominal or flank pain with haematuria; in acute cases dysuria, fever or vomiting also occur. Ultrasound is considered the modality of choice in paediatric urolithiasis because it can be used to identify most clinically relevant stones. Complementary imaging modalities such as conventional radiographs or non-contrast computed tomography should be limited to specific clinical situations. Management of kidney stones includes dietary, pharmacological and urological interventions, depending on stone size, location or type, and the child's condition. With a very high incidence of underlying metabolic abnormalities and significant recurrence rates in paediatric urolithiasis, thorough metabolic evaluation and follow-up examination studies are of utmost importance.

Imaging of anorectal malformations: where are we now? Abdominal imaging task force of the European Society of Paediatric Radiology.

Anorectal and cloacal malformations are a broad mix of congenital abnormalities related to the distal rectum and anus. Confusion exists between all the forms in this large and heterogeneous group. The spectrum includes everything from anal stenosis, ventral anus, anal atresia (with and without fistula) and the full spectrum of cloacal malformations. Imaging in these conditions is done through the whole armamentarium of radiologic modalities, with very different imaging strategies seen across the centres where these conditions are managed. In 2017, the European Society of Paediatric Radiology (ESPR) abdominal imaging task force issued recommendations on the imaging algorithm and standards for imaging anorectal malformations. This was followed by further letters and clarifications together with an active multispecialty session on the different imaging modalities for anorectal malformations at the 2018 ESPR meeting in Berlin. Through this paper, the abdominal task force updates its guidelines and recommended imaging algorithm for anorectal malformations.

Surveillance of Fontan-associated liver disease: current standards and a proposal from the European Society of Paediatric Radiology Abdominal Task Force.

Since Francis Fontan first introduced the eponymous technique, the Fontan procedure, this type of surgical palliation has allowed thousands of children affected by specific heart malformations to reach adulthood. Nevertheless, abdominal, thoracic, lymphatic and neurologic complications are the price that is paid by these patients. Our review focuses on Fontan-associated liver disease; the purpose is to summarize the current understanding of its physiopathology, the aim of follow-up and the specific radiologic follow-up performed in Europe. Finally, we as members of the Abdominal Task Force of the European Society of Paediatric Radiology propose a consensus-based imaging follow-up algorithm.

Radiologic follow-up in Fontan-associated liver disease in Europe: European Society of Paediatric Radiology survey demonstrates the need for a consensus protocol.

Fontan surgery is a life-saving procedure for newborns with complex cardiac malformations, but it originates complications in different organs. The liver is also affected, with development of fibrosis and sometimes cirrhosis and hepatocellular carcinoma. There is no general agreement on how to follow-up these children for the development of liver disease. To understand the current practice on liver follow-up, we invited members of the European Society of Paediatric Radiology (ESPR) to fill out an online questionnaire. The survey comprised seven questions about when and how liver follow-up is performed on Fontan patients. While we found some agreement on the use of US as screening tool, and of MRI for nodule characterization, the discrepancies on timing and the lack of a shared protocol make it currently impossible to compare data among centers.

Practical approach for the diagnosis of biliary atresia on imaging, part 2: magnetic resonance cholecystopancreatography, hepatobiliary scintigraphy, percutaneous cholecysto-cholangiography, endoscopic retrograde cholangiopancreatography, percutaneous liver biopsy, risk scores and decisional flowchart.

We aim to present a practical approach to imaging in suspected biliary atresia, an inflammatory cholangiopathy of infancy resulting in progressive fibrosis and obliteration of extrahepatic and intrahepatic bile ducts. Left untreated or with failure of the Kasai procedure, biliary atresia progresses to biliary cirrhosis, end-stage liver failure and death within the first years of life. Differentiating biliary atresia from other nonsurgical causes of neonatal cholestasis is difficult as there is no single method for diagnosing biliary atresia and clinical, laboratory and imaging features of this disease overlap with those of other causes of neonatal cholestasis. In this second part, we discuss the roles of magnetic resonance (MR) cholecystopancreatography, hepatobiliary scintigraphy, percutaneous biopsy and percutaneous cholecysto-cholangiography. Among imaging techniques, ultrasound (US) signs have a high specificity, although a normal US examination does not rule out biliary atresia. Other imaging techniques with direct opacification of the biliary tree combined with percutaneous liver biopsy have roles in equivocal cases. MR cholecystopancreatography and hepatobiliary scintigraphy are not useful for the diagnosis of biliary atresia. We propose a decisional flowchart for biliary atresia diagnosis based on US signs, including elastography, percutaneous cholecysto-cholangiography or endoscopic retrograde cholangiopancreatography and liver biopsy.

Practical approach to imaging diagnosis of biliary atresia, Part 1: prenatal ultrasound and magnetic resonance imaging, and postnatal ultrasound.

We present a practical approach to imaging in suspected biliary atresia, an inflammatory cholangiopathy of infancy resulting in progressive fibrosis and obliteration of extrahepatic and intrahepatic bile ducts. Left untreated or with failure of the Kasai procedure, biliary atresia progresses towards biliary cirrhosis, end-stage liver failure and death by age 3. Differentiation of biliary atresia from other nonsurgical causes of neonatal cholestasis is challenging because there is no single method for diagnosing biliary atresia, and clinical, laboratory and imaging features of this disease overlap with those of other causes of neonatal cholestasis. Concerning imaging, our systematic literature review shows that ultrasonography is the main tool for pre- and neonatal diagnosis. Key prenatal features, when present, are non-visualisation of the gallbladder, cyst in the liver hilum, heterotaxy syndrome and irregular gallbladder walls. Postnatal imaging features have a very high specificity when present, but a variable sensitivity. Triangular cord sign and abnormal gallbladder have the highest sensitivity and specificity. The presence of macro- or microcyst or polysplenia syndrome is highly specific but less sensitive. The diameter of the hepatic artery and hepatic subcapsular flow are less reliable. When present in the context of acholic stools, dilated intrahepatic bile ducts rule out biliary atresia. Importantly, a normal US exam does not rule out biliary atresia. Signs of chronic hepatopathy and portal hypertension (portosystemic derivations such as patent ductus venosus, recanalised umbilical vein, splenomegaly and ascites) should be actively identified for - but are not specific for - biliary atresia.

The importance of FDG PET/CT in the diagnostic process of the middle aortic syndrome in a 15-year-old boy patient with suspected systemic vasculitis and final diagnosis of Williams-Beuren syndrome.

The differential diagnosis in children with the systemic vasculopathy is still a challenge for clinicians. The progress in vascular imaging and the latest recommendations improve the diagnostic process, but only single reports describe the use of new imaging tests in children. The publication aims to demonstrate the important role of 18F-fluoro-2-deoxy-D-glucose (FDG) positron emission tomography combined with anatomical computed tomography angiography (PET/CTA) imaging in the case of a 15-year-old boy with chest pain, intermittent claudication, hypertension and features of middle aortic syndrome in computed tomography angiography (CTA). The patient was suspected to have Takayasu arteritis, but was finally diagnosed with Williams-Beuren syndrome. The case indicates that the FDG PET/CT imaging might be essential in the diagnostic process of middle aortic syndrome in children. We suggest that this imaging technique should be considered in the diagnostic process of systemic vasculopathy particularly in children.

Intracavitary contrast-enhanced ultrasonography in children: review with procedural recommendations and clinical applications from the European Society of Paediatric Radiology abdominal imaging task force.

Contrast-enhanced ultrasonography (US) has become an important supplementary tool in many clinical applications in children. Contrast-enhanced voiding urosonography and intravenous US contrast agents have proved useful in routine clinical practice. Other applications of intracavitary contrast-enhanced US, particularly in children, have not been widely investigated but could serve as a practical and radiation-free problem-solver in several clinical settings. Intracavitary contrast-enhanced US is a real-time imaging modality similar to fluoroscopy with iodinated contrast agent. The US contrast agent solution is administered into physiological or non-physiological body cavities. There is no definitive list of established indications for intracavitary US contrast agent application. However, intracavitary contrast-enhanced US can be used for many clinical applications. It offers excellent real-time spatial resolution and allows for a more accurate delineation of the cavity anatomy, including the internal architecture of complex collections and possible communications within the cavity or with the surrounding structures through fistulous tracts. It can provide valuable information related to the insertion of catheters and tubes, and identify related complications such as confirming the position and patency of a catheter and identifying causes for drainage dysfunction or leakage. Patency of the ureter and biliary ducts can be evaluated, too. US contrast agent solution can be administered orally or a via nasogastric tube, or as an enema to evaluate the gastrointestinal tract. In this review we present potential clinical applications and procedural and dose recommendations regarding intracavitary contrast-enhanced ultrasonography.

Save Your Maximum Tolerated Dose: How to Diagnose Procedure-Related Spinal Cord Lesions After Lumbar Intrathecal Bolus Administration of Oligonucleotides in Cynomolgus Monkeys.

Many potential drugs for treatment of neurodegenerative diseases, particularly antisense oligonucleotides (ASOs), are administered via lumbar intrathecal injection, because these drugs do not cross the blood-brain barrier. Intrathecal injection is a well-established method in cynomolgus monkeys, a species that is used in preclinical safety assessment when other nonrodent species cannot be used. The authors completed intrathecal ASO administration in over 30 preclinical safety studies (>1000 animals and >4500 dose administrations) during which we observed 3 cases of procedure-related spinal cord necrosis (incidence <0.1%). We describe clinical symptoms, diagnostic approaches, morphological features, and prognosis of this rare injury, and compare these findings with typical drug-related findings of ASOs dosed by intrathecal injection. The low incidence of procedure-related and dose-limiting lesions confines this analysis to a small sample set. The pattern of effects is similar across all monkeys despite differences in age, body weight, and intrathecal injection site. All 3 cases presented a combination of the following findings: blood in cerebrospinal fluid at time of injection, clinical signs that increase in severity within a day of dosing, lameness of both hind limbs, reduced muscle tone, and loss of patellar, foot grip, and/or anal reflexes. In all cases, magnetic resonance imaging (MRI) showed a linear hyperintense lesion in the lumbar spinal cord. In 2 cases, this hyperintensity was associated with evidence of spinal cord edema. We conclude that a pattern of in-life and pathology findings, including noninvasive MRI assessment, is indicative of procedure-related effects.

Intraoperative contrast-enhanced urosonography during endoscopic treatment of vesicoureteral reflux in children.

BACKGROUND: There are many controversies surrounding the effectiveness of endoscopic treatment of vesicouretheral reflux (VUR) in children, thus it is of highest priority to analyze factors influencing the outcome of therapy and to search for new methods that would increase the success rate and reduce the number of reinjections. OBJECTIVE: The aim of the study was to analyze whether intraoperative contrast-enhanced urosonography (ce-US) may increase the effectiveness of endoscopic anti-reflux therapy. MATERIALS AND METHODS: Intraoperative contrast-enhanced urosonography (ce-US) with SonoVue® was performed in 17 patients (25 ureteral units) undergoing endoscopic treatment of VUR. Ce-US was performed in the operating room before the procedure and after injection of the bulking material. When VUR persisted, the operator repeated the injection, which was followed by ce-US. The results were compared with those obtained from a control group (15 patients; 22 ureteral units). RESULTS: A repeat injection during a single endoscopic treatment was required in 24% of cases. The overall success rate confirmed at 6-12 months' follow-ups was 84%. The success rate was significantly higher in comparison to the control group (success: 64%). CONCLUSION: Intraoperative ce-US performed during endoscopic treatment of VUR enables immediate monitoring of outcome and provides the opportunity for repeat injection during the same procedure, thus increasing the efficacy of the procedure and reducing the number of reinjections.

Ischemic Stroke in the Course of COVID-19 in a 16-Year-Old Boy.

The SARS-CoV-2 virus that causes COVID-19 disease is still evolving and, despite the end of the acute phase of the pandemic, still poses a risk to public health. One of the very rare complications, occurring in less than 1% of children, is multisystem inflammatory syndrome in children (MISC). Due to the risk of thromboembolic complications as well as cardiac problems, MISC carries a number of life-threatening complications. We report a case of a 16-year-old boy who was hospitalized due to general weakness, fever, conjunctivitis, vomiting and diarrhoea. In view of the mother's positive result of the SARS-CoV-2 test, the teenager underwent numerous laboratory tests. Taking into account the critical condition of the patient, anticoagulant and antipyretic treatment, steroids and IVIG were added. During hospitalisation, alarming symptoms occurred, including dysarthria, drooping corner of the mouth and muscle weakness on the right side. The magnetic resonance imaging showed changes characteristic of ischemic stroke. Further studies are needed to assess possible thrombotic complications in children after SARS-CoV-2 infection, and specialists should be more vigilant in paediatric patients presenting with such symptoms.

Nonspecific Gastrointestinal Symptoms as the First Sign of Ganglioneuroblastoma Intermixed-Case Report and Literature Review.

Nonspecific gastrointestinal symptoms remain a problem for pediatricians because, out of a thousand trivial cases, there are rare diseases that require in-depth diagnostics and extensive knowledge to identify them. These complaints may be caused by a neoplastic process. We present the case of a 5-year-old boy whose diagnostic pathway lasted about 3 months. He was admitted to hospital due to severe abdominal pain. Physical examination revealed a bloated, hard, and painful abdomen. In the standing X-ray, the features of intestinal obstruction were visualized. An ultrasound examination showed a possible malignant lesion in the location of the left adrenal gland. After the surgical removal of the pathological mass and histopathological examination, the diagnosis of ganglioneuroblastoma intermixed was made. This tumor, along with neuroblastoma, ganglioneuroma, and ganglioneuroblastoma nodular, belongs to neuroblastic tumors (NTs), which originate from primitive cells of the sympathetic nervous system. NTs are quite rare, but they are still the majority of extracranial solid tumors in children, and their symptoms often appear relatively late when the neoplastic process is already advanced. The purpose of this review is to present current information about ganglioneuroblastoma, with a special emphasis on nonspecific gastrointestinal symptoms as first sign of this tumor and its diagnostics.

Chronic Appendicitis-From Ambiguous Clinical Image to Inconclusive Imaging Studies.

A six-year-old boy visits a general practitioner due to diarrhea and abdominal pain with a moderate fever of up to 39 °C for 2 days. Treatment is initiated; however, the recurrence of abdominal pain is observed. Physical examination of the child at the emergency department reveals abdominal guarding and visible, palpable, painful intestinal loops in the left iliac and hypogastric regions-this is referred to as an 'acute abdomen'. An X-ray shows single levels of air and fluid indicative of bowel obstruction. Ultrasound reveals distended, fluid-filled intestinal loops with diminished motility. The intestinal wall is swollen. Laboratory tests indicate increased inflammatory indices. Contrast-enhanced computed tomography examination of the abdominal cavity and lesser pelvis shows intestinal dilation. The loops were filled with liquid content and numerous collections of gas. The patient is qualified for a laparotomy. An intraoperative diagnosis of perforated gangrenous appendicitis with autoamputation was made. In addition, numerous interloop and pelvic abscesses, excessive adhesions, signs of small intestine micro-perforation, and diffuse peritonitis are found. The patient's condition and laboratory parameters significantly improve during the following days of hospitalization. Despite the implementation of multidirectional, specialized diagnostics in the case of acute abdomen, in everyday practice we still encounter situations where the final diagnosis is made intraoperatively only.

Epilepsy in Pediatric Patients-Evaluation of Brain Structures' Volume Using VolBrain Software.

Epilepsy is one of the most frequent serious brain disorders. Approximately 30,000 of the 150,000 children and adolescents who experience unprovoked seizures are diagnosed with epilepsy each year. Magnetic resonance imaging is the method of choice in diagnosing and monitoring patients with this condition. However, one very effective tool using MR images is volBrain software, which automatically generates information about the volume of brain structures. A total of 57 consecutive patients (study group) suffering from epilepsy and 34 healthy patients (control group) who underwent MR examination qualified for the study. Images were then evaluated by volBrain. Results showed atrophy of the brain and particular structures-GM, cerebrum, cerebellum, brainstem, putamen, thalamus, hippocampus and nucleus accumbens volume. Moreover, the statistically significant difference in the volume between the study and the control group was found for brain, lateral ventricle and putamen. A volumetric analysis of the CNS in children with epilepsy confirms a decrease in the volume of brain tissue. A volumetric assessment of brain structures based on MR data has the potential to be a useful diagnostic tool in children with epilepsy and can be implemented in clinical work; however, further studies are necessary to enhance the effectiveness of this software.

Quantitative assessment of urethral vascularity in nulliparous females using high-frequency endovaginal ultrasonography.

PURPOSE: To assess the vascular parameters in the urethra of nulliparous females and to compare the vascularity among various parts of the urethra, using high-frequency endovaginal ultrasonography (EVUS). METHODS: Twenty-two nulliparous women, mean age 27 years, underwent EVUS using a biplane transducer at 12 MHz frequency. Color Doppler examinations of the urethra were recorded and further evaluated off-line using special software (Pixel Flux) for quantitative assessment of the vascularity. The urethra was divided into four regions of interest (ROIs) in the midsagittal plane and three ROIs in the axial plane. The following parameters were measured: velocity (V), perfused area (A), perfusion intensity (I), pulsatility index (PI), and resistance index (RI). Interobserver and intraobserver reproducibility analysis was also performed. RESULTS: In midsagittal plane, the midurethra presented the highest value of V and lowest value of A. The intramural part showed the lowest value of I and the highest values of RI and PI. In the distal urethra, the highest value of I and the lowest value of RI was seen. In the axial plane, the values of V, A, and I were statistically significantly higher in the external part of the midurethra compared with the internal part. Excellent interobserver and intraobserver reproducibility was shown in the majority of parameters for the entire urethra. CONCLUSIONS: Vascularity differs in different parts of the urethra. Pixel Flux is a valuable tool for providing reproducible quantitative analysis of vascular parameters for the entire urethra.

3D/4D contrast-enhanced urosonography (ceVUS) in children - is it superior to the 2D technique?

BACKGROUND: By now, two-dimensional contrast-enhanced voiding urosonography (ceVUS) has become a well-established method for the diagnosis and treatment monitoring of vesicoureteral reflux in children, particularly after the recent approval for this application in children in the USA and in Europe. The introduction of three-dimensional static (3D) and real-time (4D) techniques with ultrasound contrast agents opens up new diagnostic opportunities for this imaging modality. OBJECTIVE: To analyze whether 3D and 4D ceVUS is a superior technique compared to standard 2D ceVUS in diagnosing vesicoureteral reflux in children. MATERIAL AND METHODS: The study included 150 patients (mean age 3.7 years) who underwent 2D and 3D/4D ceVUS for the diagnosis and grading of vesicoureteral reflux. RESULTS: 2D ceVUS and 3D/4D ceVUS diagnosed the same number of vesicoureteral refluxes, however, there was a statistically significant difference in grading between the two methods. Performing 3D/4D ceVUS resulted in changing the initial grade compared to 2D ceVUS in 19 out of 107 refluxing units (17.76%) diagnosed. The 4D technique enabled a more conspicuous visualization of vesicoureteral reflux than the 3D technique. CONCLUSIONS: 2D ceVUS and 3D/4D ceVUS diagnosed the same number of vesicoureteral refluxes, however, there was a statistically significant difference in grading between the two methods. Thus 3D/4D ceVUS appears at least a valid, if not even a more conspicuous technique compared to 2D ceVUS.

Two-dimensional (2D), three-dimensional static (3D) and real-time (4D) contrast enhanced voiding urosonography (ceVUS) versus voiding cystourethrography (VCUG) in children with vesicoureteral reflux.

BACKGROUND: Two-dimensional (2DUS) contrast enhanced voiding urosonography has been used in the diagnosis and treatment monitoring of the vesicoureteral reflux in children for over 15 years. The opportunity of performing this examination with the use of three-dimensional static (3DUS) and real-time (4DUS) techniques opens up new diagnostic horizons. OBJECTIVE: To analyze if 3DUS/4DUS bring additional information leading to an increased detection rate or change in the grading of reflux compared to 2DUS and voiding cystouretrography. MATERIAL AND METHODS: We evaluated 69 patients (mean 4.1 years) who underwent 2DUS/3DUS/4DUS contrast enhanced voiding urosonography (ceVUS) and voiding cystourethrography (VCUG) for the diagnosis and grading of vesicoureteral reflux. RESULTS: 2DUS and 3DUS/4DUS urosonography diagnosed 10 more refluxes (7.25%) than cystourethrography and in 3 refluxes (2.17%) detected a higher grade. In 9 refluxes (6.52%) 3DUS/4DUS urosonography and cystourethrography diagnosed a higher grade than 2DUS. There was a statistically significant difference between cystourethrography and 3DUS/4DUS urosonography when the number of detected refluxes and differences in grading were compared. 4DUS enabled a better visualization of reflux than 3DUS. CONCLUSIONS: 3DUS/4DUS techniques bring additional information leading to a change in reflux grading compared to 2DUS and a detect higher number of refluxes compared to cystourethrography.

Standards of the Polish Ultrasound Society - update. Ultrasound examination of the kidneys, ureters and urinary bladder.

The paper presents the principles of performing proper ultrasound examinations of the urinary tract. The following are discussed: preparation of patients, type of optimal apparatus, technique of examination and conditions which its description should fulfill. Urinary track examination in adults and in children constitutes an integral part of each abdominal examination. Such examinations should be performed with fasting patients or several hours after the last meal, with filled urinary bladder. APPARATUS: Ultrasound examinations in children and infants are performed using transducers with the frequency of 5.0-9.0 MHz and in adults - with the frequency of 2.0-6.0 MHz. Doppler options are desirable since they improve diagnostic capacity of sonography in terms of differentiation between renal focal lesions. SCANNING TECHNIQUE: Renal examinations are performed with the patients in the supine position. The right kidney is examined in the right hypochondriac region using the liver as the ultrasound "window." The left kidney is examined in the left hypochondriac region, preferably in the posterior axillary line. Ultrasound examinations of the upper segment of the ureters are performed after renal examination when the pelvicalyceal system is dilated. A condition necessary for a proper examination of the perivesical portion of the ureter is full urinary bladder. The scans of the urinary bladder are performed in transverse, longitudinal and oblique planes when the bladder is filled. DESCRIPTION OF THE EXAMINATION: The description should include patient's personal details, details of the referring unit, of the unit in which the examination is performed, examining physician's details, type of ultrasound apparatus and transducers as well as the description proper.

Errors in the ultrasound diagnosis of the kidneys, ureters and urinary bladder.

The article presents the most frequent errors made in the ultrasound diagnosis of the urinary system. They usually result from improper technique of ultrasound examination or its erroneous interpretation. Such errors are frequent effects of insufficient experience of the ultrasonographer, inadequate class of the scanner, insufficient knowledge of its operation as well as of wrong preparation of patients, their constitution, severe condition and the lack of cooperation during the examination. The reasons for misinterpretations of ultrasound images of the urinary system may lie in a large polymorphism of the kidney (defects and developmental variants) and may result from improper access to the organ as well as from the presence of artefacts. Errors may also result from the lack of knowledge concerning clinical and laboratory data. Moreover, mistakes in ultrasound diagnosis of the urinary system are frequently related to the lack of knowledge of the management algorithms and diagnostic possibilities of other imaging modalities. The paper lists errors in ultrasound diagnosis of the urinary system divided into: errors resulting from improper technique of examination, artefacts caused by incorrect preparation of patients for the examination or their constitution and errors resulting from misinterpretation of ultrasound images of the kidneys (such as their number, size, fluid spaces, pathological lesions and others), ureters and urinary bladder. Each physician performing kidney or bladder ultrasound examination should possess the knowledge of the most frequent errors and their causes which might help to avoid them.