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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.Other Information Published in: Pediatric Radiology License: https://creativecommons.org/licenses/by/4.0See article on publisher's website: http://dx.doi.org/10.1007/s00247-022-05395-7

Background Hip involvement predicts severe disease in juvenile idiopathic arthritis (JIA) and is accurately assessed by MRI. However, a child-specific hip MRI scoring system has not been validated. Objective To test the intra- and interobserver agreement of several MRI markers for active and chronic hip changes in children and young adults with JIA and to examine the precision of measurements commonly used for the assessment of growth abnormalities. Materials and methods Hip MRIs from 60 consecutive children, adolescents and young adults with JIA were scored independently by two sets of radiologists. One set scored the same MRIs twice. Features of active and chronic changes, growth abnormalities and secondary post-inflammatory changes were scored. We used kappa statistics to analyze inter- and intraobserver agreement for categorical variables and a Bland–Altman approach to test the precision of continuous variables. Results Among active changes, there was good intra- and interobserver agreement for grading overall inflammation (kappa 0.6–0.7). Synovial enhancement showed a good intraobserver agreement (kappa 0.7–0.8), while the interobserver agreement was moderate (kappa 0.4–0.5). Regarding acetabular erosions on a 0–3 scale, the intraobserver agreement was 0.6 for the right hip and 0.7 for the left hip, while the interobserver agreement was 0.6 for both hips. Measurements of joint space width, caput–collum–diaphyseal angle, femoral neck–head length, femoral width and trochanteric distance were imprecise. Conclusion We identified a set of MRI markers for active and chronic changes in JIA and suggest that the more robust markers be included in future studies addressing clinical validity and long-term patient outcomes.

The aim of this study is to retrospectively assess onset and progression of scoliosis in type II SMA patients not treated with the approved disease modifying treatments. Scoliosis was evaluated by measuring the scoliosis angle on X-ray obtained in the anteroposterior view in sitting position (Cobb's angle method). Eighty-four patients had at least one assessment of scoliosis angle (287 assessments). There was a positive correlation between age and scoliosis angles (p0.001) with a progressive increase of scoliosis with age. When subdividing the population by HFMSE score (10; 11-22;22), there was a progressive increase in scoliosis angles with decreasing HFMSE scores. The difference between HFMSE categories was significant (p0.001). Fifty-four patients had at least two assessments at 6-month distance and were retained for the longitudinal analysis. Using a mixed model, age, functional status and scoliosis angle at baseline were predictive on scoliosis progression. The mean annual rate of increase of scoliosis angle was 5.63 (95%CI: 4.74-6.52). Our results confirm the progression of scoliosis in untreated type II SMA providing details of the progression in relation to different variables. With different therapeutical options being available in many countries, our findings will provide reference data for establishing possible differences in the trajectories of progression with treated type II individuals.

Congenital abnormalities of the kidney and urinary tract include a wide range of malformations ranging from asymptomatic to life-threatening conditions. Although pediatric urogenital system imaging is based on the use of US (pre- and postnatal), voiding cystourethrography and scintigraphic study, magnetic resonance (MR) urography plays a fundamental role in the classification and management of congenital abnormalities of the kidney and urinary tract, giving an overview of the different clinical pictures, thanks to its panoramicity and high anatomical detail. In fact the anomalies of the urinary tract are phenotypically variable because they can affect simultaneously several segments of different embryonic derivation, with complex clinical pictures; they can appear both as isolated phenotypes or as complex malformative conditions, involving renal parenchyma, collecting system and bladder. A deep knowledge of this complex embryogenesis and its possible phenotypic patterns allows a correct interpretation of MR urography images. We describe the embryology and pathophysiology of congenital abnormalities of the kidney and urinary tract as well as MR urography technique and findings. Congenital abnormalities of the kidney and urinary tract are classified into four groups: (1) obstruction (proximal, middle and distal), (2) budding with respect to the Wolffian duct (site and number of ureter), (3) ascent and rotation (ectopia, malrotation and fusion of kidney) and (4) anomaly of metanephric differentiation (dysplasia, megapolicalycosis).

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.

COVID-19 is generally uneventful in children. Only 8% of severe acute respiratory distress syndrome corona virus 2 pediatric patients require intensive care; of these, 1% may need extracorporeal membrane oxygenation. Preexisting medical conditions are an independent risk factor for pediatric intensive care unit admission. We describe the case of an 11-year-old girl with adenosine deaminase 2 deficiency who presented severe COVID-19 acute respiratory distress syndrome, complicated by a massive air leak syndrome. The respiratory failure, refractory to conventional support, required veno-venous extracorporeal membrane oxygenation. To prevent viral diffusion, bicaval double-lumen cannulation was performed percutaneously at the bedside under exclusive echo guidance. Because of pneumomediastinum, pneumothorax, and subcutaneous emphysema, ultrasound visualization of the heart was possible only with transesophageal echo. To our knowledge, this is the first description of a transesophageal echo guided bedside percutaneous bicaval double-lumen extracorporeal membrane oxygenation cannulation in a pediatric patient. Pitfalls of the technique are highlighted.

Background: Urinary tract infection (UTI) is the most common infection in pediatric-age patients. Acute pyelonephritis (PNA) represents a worrying situation in pediatric patients due to the risk of sepsis and long-term cicatricial consequences. The purpose of this retrospective study is to evaluate the diagnostic role of DW-MRI in relation to clinical data, to understand if there are any clinical parameters useful in identifying which patients should undergo it. Methods: According to inclusion and exclusion criteria, we enrolled 51 patients ≤15 years old admitted to our Institute between September 2012 and April 2020 with a febrile UTI who underwent DW-MRI evaluation. Clinical, laboratory and imaging data were collected. Statistical analysis was performed. Results: 34 of 51 patients with an fUTI (66.7%) showed signs of acute parenchymal involvement at DW-MRI evaluation. In 27 of these 34 (79.4%), DW-MRI showed multiple areas of pyelonephritis. A statistically significant relationship (p = 0.0004) between older age at admission and pyelonephritis was demonstrated. No statistically significant relationship was found between the other clinical, anamnestic and laboratory parameters and the outcome of DWI. Only two ultrasound examinations allowed the identification of pathological areas on the renal parenchyma. Conclusions: From these preliminary investigations, we can say that selecting the patients with fUTI on whom to perform a DW-MRI is difficult. Nevertheless, thanks to the low cost, the very rare need for sedation and the accuracy in identifying pyelonephritic areas, the use of DW-MRI in patients with febrile UTI seems recommendable.

Chest imaging plays a fundamental role, as well as in prenatal screening, and it is essential in the diagnosis and follow-up of many thoracic malformations. Over the years, thanks to the introduction of CT and MR, it is also able to provide functional information, useful for defining the surgical indication. In this chapter, we will see the role that each technique currently available has.

During the outbreak of the COVID-19 pandemic, guidelines have been issued by international, national and local authorities to address management and the need for preparedness. Children with COVID-19 differ from adults in that they are less often and less severely affected. Additional precautions required in the management of children address their increased radiosensitivity, need for accompanying carers, and methods for dealing with children in a mixed adult-paediatric institution. In this guidance document, our aim is to define a pragmatic strategy for imaging children with an emphasis on proven or suspected COVID-19 cases. Children suspected of COVID-19 should not be imaged routinely. Imaging should be performed only when expected to alter patient management, depending on symptoms, preexisting conditions and clinical evolution. In order to prevent disease transmission, it is important to manage the inpatient caseload effectively by triaging children and carers outside the hospital, re-scheduling nonurgent elective procedures and managing symptomatic children and carers as COVID-19 positive until proven otherwise. Within the imaging department one should consider conducting portable examinations with COVID-19 machines or arranging dedicated COVID-19 paediatric imaging sessions and performing routine nasopharyngeal swab testing before imaging under general anaesthesia. Finally, regular personal hygiene, appropriate usage of personal protective equipment, awareness of which procedures are considered aerosol generating and information on how to best disinfect imaging machinery after examinations should be highlighted to all staff members. Electronic supplementary material The online version of this article (10.1007/s00247-020-04749-3) contains supplementary material, which is available to authorized users.

Italy has been badly affected by COVID‐19, despite strict quarantine measures. Up to 1 June 2020, 2.1% of the positive cases in Italy were aged 0‐18 years, with no paediatric deaths (1). This paper focuses on the clinical features, laboratory and imaging data and outcomes of 24 patients admitted to the Emergency Department (ED) at IRCCS Gaslini Children’s Hospital, Genoa, Italy, from 24 February to 16 April 2020.

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 scrotal wall may be involved in a variety of pathologic processes. Such lesions may rise primarily from the layers of the scrotum or may be due to a process arising from scrotal content. Imaging is not needed in most cases, but it may be useful for making such differentiations and for evaluation of possible involvement of the testes and epididymides in cases of primary wall abnormalities. This pictorial essay will show the imaging findings observed in a variety of pathologic conditions affecting the scrotal wall, both common and unusual ones, with an emphasis on clinically relevant findings and features that lead to a specific diagnosis.

Background:Whole-body magnetic resonance imaging (WBMRI) is a fast and accurate method to detect diseases throughout the entire body without exposure to ionizing radiation. Possible emerging applications for this technique include rheumatologic field and evaluation of fever of unknown origin (FUO).Objectives:To evaluate the ability of WBMRI to identify significant potential diagnostic clue (PDC) in patients presenting a non specific inflammatory clinical picture.Methods:We retrospectively collected cases of pediatric patients followed in a single pediatric rheumatology center who underwent WBMRI between January 2010 and December 2015 for the following indications: i) FUO (temperature greater than 38.3°C for more than three weeks or failure to reach diagnosis after one week of investigations), iii) recurrent fever (febrile episodes separated by periods of normal temperature), iii) Inflammation of unknown origin, IUO (an illness of at least 3 weeks’ duration, with raised inflammatory markers and fever below 38.3°C).WBMRI studies were acquired with coronal and sagittal planes (slice thickness 5mm) with acquisition of several image sets with automatic direct image realignment after acquisition creating a whole-body scan.Sequences include short τ inversion recovery (STIR) and T1-weighted. All studies have been evaluated twice, the second time according to a predefined checklist, defined by an experienced radiologist, considering systematically single /multifocal bone lesion, bone marrow, joint effusion, soft tissues, adenopathies, parenchymal and vessels looking for PDC. We considered as a Potential Diagnostic Clue each alteration of the examined district that can potentially guide the diagnosis. Each alteration found is a PDC. We retrospectively evaluated patients’ clinical history and final diagnosis and we classified the PDCs identified during both first evaluation and re-evaluation as: Not useful (the identified PDC did not guide the diagnosis and is not coherent with the final diagnosis), consistent (the identified PDC is congruent with the patient’s final diagnosis) or diagnostic (the identification of the considered PDC strongly orient the final diagnosis).Results:We collected 104 patients who underwent WBMRI; 24 (23%) of them presenting FUO, 28 (27%) presenting recurrent fever and 52 (50%) presenting IUO. The mean age of onset symptoms was 6 years and nine months (range: 2 weeks old- 17 years and 6 months). The mean age of execution of WBMRI was 9 years (range: 5 months old- 19 years). After the whole diagnostic work-out a final diagnosis was achieved in 44 patients (42%).PDCs were identified at the first evaluation in 78/104 cases (75%). In 22 cases (21%) the identified PDCs were consistent with the diagnosis, whereas in 9 cases (8.5%) the identified PDCs were considered diagnostic. Globally we can consider that at first evaluation PDCs were somehow contributory to the diagnosis in 31 cases (30%; 6 JIA, 7 systemic infections, 5 monogenic inflammatory diseases, 4 ALPS, 2 Goldbloom’s Syndrome,2 Vasculitis,1 eosinophilic fasciitis, 1 hystiocytosis, 3 oncologic diagnosis).Blind re-evaluation of WBMRI allowed the identification of additional PDCs in 52 patients (12 of them previously negative). In 10 cases the PDC found after re- evaluation were consistent with the final diagnosis (2 JIA, one infectious disease, one neuroblastoma, 3 ALPS, 1 monogenic inflammatory disease, 1Takayasu arteritis, 1 Goldbloom’s syndrome).Conclusion:WBMRI can be a powerful diagnostic tool in patients with FUO. A predefined checklist increases sensitivity of WBMRI in the identification of PDC.Disclosure of Interests:Sara Signa: None declared, Roberta Caorsi: None declared, Giorgio Stagnaro: None declared, Francesca Minoia: None declared, Paolo Picco: None declared, Angelo Ravelli: None declared, Maria Beatrice Damasio: None declared, Marco Gattorno Consultant of: Sobi, Novartis, Speakers bureau: Sobi, Novartis

International audience; Background: Obstructive congenital anomalies of the kidney and urinary tract have a high risk of kidney failure if not surgically corrected. Dynamic renal scintigraphy is the gold standard technique to evaluate drainage curves and split renal function (SRF). Objectives: To compare functional magnetic resonance (MR) urography with dynamic renal scintigraphy in measuring volumetric SRF and in the classification of drainage curves in patients with congenital anomalies of the kidney and urinary tract. Materials and Methods: We retrospectively collected patients with hydroureteronephrosis or pelvicalyceal dilatation at renal ultrasound, who underwent both functional MR urography and dynamic renal scintigraphy (DRS) within 6 months. DRS studies were evaluated by a single nuclear medicine physician with a double reading. Functional MR urography renograms were blind evaluated twice by two radiologists. The functional MR urographyintra-and inter-reading agreements as well as the agreement between the two imaging techniques were calculated. SRF was evaluated by Area Under the Curve and Rutland-Patlak methods. Drainage curves were classified as normal, borderline or accumulation patterns by both the techniques. Results: Fifty-two children were studied, 14 with bilateral involvement. A total of 104 kidney-urinary tracts were considered: 38 normal and 66 dilated. Considering Area Under the Curve and Rutland-Patlak for SRF, the intra-and inter-reader agreements of functional MR urography had excellent and good results, respectively, and the two techniques demonstrated a good concordance (r2: 67% for Area Under the Curve and 72% for Rutland-Patlak). Considering drainage curves, the inter-readers agreement Damasio et al. fMRU vs DRS in Children for functional MR urography and the concordance between the two techniques were moderate (Cohen's k, respectively, 55.7 and 56.3%). Conclusions: According to our results, there are no significant differences between functional MR urography and DRS in measuring volumetric SRF and in the classification of drainage curves in patients with congenital anomalies of the kidney and urinary tract.

Summary Introduction Ureteropelvic junction obstruction (UPJO) is one of the most common urological diseases in children. The etiology can be intrinsic, extrinsic (crossing vessel [CV] or adhesions), or mixed. To date, ultrasonography and scintigraphy are considered gold-standard imaging techniques for the study of UPJO. Functional magnetic resonance urography (fMRU) combines anatomical and functional information and has been recently evaluated for the detection of CVs in UPJO. Objective The objective of the study was to evaluate the concordance between fMRU and surgery in determining the etiology of UPJO and the presence of obstructing/non-obstructing CVs. Study design Patients with unilateral hydronephrosis who underwent surgery after an fMRU were included in the sample. Surgical data regarding the etiology of UPJO were compared with radiological results. The etiology was divided into intrinsic, extrinsic due to CV, extrinsic due to adhesions, and mixed or cicatricial (postoperative). The concordance was calculated by means of the Cohen's kappa coefficient. Results The observed agreement between fMRU and surgical findings regarding the etiology and the presence of CV were 83.2% and 89.4%, respectively (with substantial Cohen's kappa coefficient). The sensitivity and specificity of fMRU were 0.84 and 0.93, respectively; the positive predictive value (PPV) and negative predictive value (NPV) were 0.889 and 0.897, respectively. The observed agreement regarding the type of vessel was 88.3% with a Cohen's kappa coefficient of 0.787 (substantial). Discussion In children with hydronephrosis, it is very important for the surgeon to quantify the extent of dilation, define the etiology of the obstruction, and the presence or absence of CVs. fMRU is a ‘one-stop-shop’ technique which provides both anatomical and functional information showing a high concordance with surgical findings, avoiding radiation exposure. Conclusions fMRU should be considered a valid imaging technique in the study of pediatric UPJO, as it provides the surgeon with important information regarding the etiology of the obstruction for the preoperative planning. Download : Download high-res image (186KB) Download : Download full-size image fMRU image showing the presence of CV as etiologic cause of the obstruction.

Knowing that ureteropelvic junction obstruction is due to a crossing renal vessel is essential in choosing the appropriate surgical treatment. To evaluate the diagnostic accuracy of non-contrast magnetic resonance (MR) angiography in identifying crossing renal vessels in children younger than 4 years old with unilateral hydronephrosis. A retrospective review of preoperative MR urography of children with unilateral hydronephrosis was conducted by two independent readers. The presence or absence of crossing renal vessels was identified and compared with surgical findings. Twenty-nine patients were included. The disagreement between MR angiography with and without contrast enhancement in detecting a crossing renal vessel was 8%. The disagreement between non-contrast-enhanced MR and surgical findings was 17%. The disagreement between contrast-enhanced MR angiography and surgical findings was 25%. The balanced triggered angiography without contrast enhancement had a sensitivity of 70% (95% confidence interval [CI]: 35-93%) and a specificity of 93% (95% CI: 66-100%). Contrast-enhanced MR angiography had a sensitivity of 56% (95% CI: 21-86%) and a specificity of 91%. (95% CI: 59-100%). MR without contrast enhancement may be a reliable, valid and safe alternative to contrast-enhanced MR angiography for identifying crossing renal vessels.

Summary Introduction Ureteropelvic junction obstruction (UPJO) is one of the most frequent urological diseases affecting the pediatric population. It can be due to both intrinsic stenosis of the junction and extrinsic causes such as the presence of crossing vessels (CVs), which can be detected by color Doppler ultrasound (CD-US). Magnetic resonance urography (MRU) is a good alternative, but sedation and infusion of a contrast agent are required. Objective The aim of this study was to analyze the diagnostic accuracy of CD-US and MRU in visualizing CVs in pediatric hydronephrosis, in order to decide the correct diagnostic pathway in the pre-operative phase. Material and methods A retrospective review was performed of medical records for all patients who underwent surgical treatment for hydronephrosis from August 2006 to February 2016. Ultrasound and scintigraphy had been performed on all patients. Data about CD-US and MRU were collected. A high-level technology ultrasound scanner and a 1.5 T MR scanner were used. The presence of CVs at surgery was considered the gold standard. Sensitivity, specificity, positive and negative predictive values (NPV) were calculated and reported for both of the imaging techniques. Results A total of 220 clinical charts were reviewed. Seventy-three CVs were identified at surgery (33.2% of UPJO). The median age was statistically higher in the group with CVs compared to the group without CVs (P Discussion According to the data, CD-US had higher sensitivity and NPV than MRU, resulting in superior detection of CVs. It is important for a surgeon to know that a child has a CV, especially in older children in which the incidence of extrinsic UPJO is higher. The main limitation of this study was the presence of incomplete data, due to the retrospectivity. Conclusions In the pre-operative phase, the CD-US should be considered as the investigation of choice to detect CVs in children with hydronephrosis ( Summary Fig ). Moreover, CD-US has lower costs than MRU, and sedation with infusion of contrast agent is unnecessary. For the future, it could be useful to lead a prospective comparison between the two imaging techniques. Download : Download high-res image (136KB) Download : Download full-size image Summary Fig . Crossing vessel detection over the UPJ with color Doppler ultrasound.

Many children who suffer from congenital, hereditary, or severe acquired renal disease have a substantially diminished number of functioning nephrons. Loss of nephrons cannot be replaced by new units, and recovery is impossible. Consequently, according to the patient’s age, different diseases enter a common pathway of progressive renal dysfunction called chronic renal failure (CRF). Further deterioration is associated with clinical symptoms and loss of metabolic control. End-stage renal disease (ESRD) is reached when survival is possible with only renal replacement therapy. Aside from all medical and psychosocial care during CRF and ESRD, renal transplantation is the ultimate goal to optimize rehabilitation and lifestyle. This chapter is devoted to these children who need lifelong multidisciplinary care and treatment, including pediatric radiology.

Pediatric vasculitides are rare conditions that can represent a diagnostic challenge because symptoms are usually aspecific and variable. Symptoms are related to the size of the involved vessel, extension of disease, and organs affected. The outcome is closely linked to an early diagnosis and proper treatment. Diagnostic imaging allows visualization of the involvement of large-size and medium-size vessels and assesses end-organ changes and response to therapy, thus playing a pivotal role in the diagnosis and treatment. This article explores the general features of pediatric vasculitis and discusses the imaging approach and the most common diagnostic findings.

The use of paediatric multi-slice CT (MSCT) is rapidly increasing worldwide. As technology advances its application in paediatric care is constantly expanding with an increasing need for radiation dose control and appropriate utilization. Recommendations on how and when to use CT for assessment of the paediatric urinary tract appear to be an important issue. Therefore the European Society of Paediatric Radiology (ESPR) uroradiology task force and European Society of Urogenital Radiology (ESUR) paediatric working groups created a proposal for performing renal CT in children that has recently been published. The objective of this paper is to discuss paediatric urinary tract CT (uro-CT) in more detail and depth. The specific aim is not only to offer general recommendations on clinical indications and optimization processes of paediatric CT examination, but also to address various childhood characteristics and phenomena that facilitate understanding the different approach and use of uro-CT in children compared to adults. According to ALARA principles, paediatric uro-CT should only be considered for selected indications provided high-level comprehensive US is not conclusive and alternative non-ionizing techniques such as MR are not available or appropriate. Optimization of paediatric uro-CT protocols (considering lower age-adapted kV and mAs) is mandatory, and the number of phases and acquisition series should be kept as few as possible.

The ESPR Uroradiology Task Force and the ESUR Paediatric Working Group present two new recommendations on imaging in childhood cystic kidney disease and in childhood renal transplantation, and address the presently restricted availability of contrast-enhanced (ce) US in children. New insights into the genetics require an updated classification of paediatric cystic kidney disease along with a new concept of diagnostic imaging. Characteristic imaging features are key to the new classification. Available recommendations for imaging renal transplantation in children are not satisfactory. The following consensus-based algorithm proposes a more effective and more uniform imaging concept, reducing invasiveness, enhancing diagnostic accuracy, and facilitating future multicentre studies and meta-analysis. At present, ce-US in children can only be performed off-license, since the only approved US contrast agent (CA) for children has been taken off the market. Nevertheless, paediatric ce-US is practiced at multiple places using Sonovue (Bracco, Milan, Italy), a generally available agent in Europe. From a medical and scientific perspective, paediatric ce-US should be promoted, and efforts are undertaken to collect data on paediatric US-CA applications. Routine paediatric imaging depends on local expertise and availability of equipment. The imaging recommendations and supportive data are intended to ease the physicians' difficult task of dealing with the specific diagnostic demands of paediatric paediatric cystic kidney disease and transplantation.

The ESPR working groups have addressed intravenous urography (IVU), uro-CT and MR-urography (MRU) in childhood as the last in the series of recommendations for paediatric uroradiology. The aim of this process was again to standardise paediatric uroradiologic imaging and to reduce invasiveness and radiation dose. As for the existing recommendations, the new proposals are consensus-based because evidence is lacking, use and indications have changed, or approaches on how to perform the examination in children differ in the literature (MRU). As in the previous recommendations, a thorough review of the literature and existing guidelines and recommendations has been performed. The proposals were discussed within the group and with non-member experts and colleagues from other partner disciplines. These recommendations aim to serve as a quality measure in order to standardise the procedures and thus grant comparable good quality results throughout different institutions.

Objective. To determine the capability and reliability of dynamic contrast-enhanced MRI (DCE-MRI) in the assessment of disease activity in juvenile idiopathic arthritis (JIA). Methods. DCE-MRI of the clinically more affected wrist or hip joints was undertaken in 21 patients, coupled with standard clinical assessment and biochemical analysis. Synovial inflammation was assessed by computing the maximum level of synovial enhancement (ME), the maximum rate of enhancement (MV) and the rate of early enhancement (REE) from the enhancement curves generated from region of interest independently delineated by two readers in the area of the ME. Correlations between dynamic parameters and clinical measures of disease activity, and static MRI synovitis score were investigated. Results. In patients with wrist arthritis, REE correlated with the wrist swelling score (r s = 0.72), ESR (r s = 0.69), pain assessment scale (r s = 0.63) and childhood HAQ (r s = 0.60). In patients with hip arthritis, ME correlated with the hip limitation of motion (r s = 0.69). Static MRI synovitis score based on post-gadolinium enhancement correlated with MV (r s = 0.63) in patients with wrist arthritis and with ME (r=0.68) in those with hip arthritis. The inter-reader agreement assessed by intra-class correlation coefficient (ICC) for ME, MV and REE (ICC = 0.98, 0.97 and 0.84, respectively) was excellent. Conclusions. DCE-MRI represents a promising method for the assessment of disease activity in JIA, especially in patients with wrist arthritis. As far as we know, this study is the first to demonstrate the feasibility, reliability and construct validity of DCE-MRI in JIA. These results should be confirmed in large-scale longitudinal studies in view of its further application in therapeutic decision making and in clinical trials.

Whole-body magnetic resonance imaging (WBMRI) is a fast and accurate method for detecting and monitoring of diseases throughout the entire body without exposure to ionizing radiation. Among emerging non-oncological potential applications of WBMRI, rheumatological diseases play an important role. Rheumatological WBMRI applications include the evaluation of chronic multifocal recurrent osteomyelitis, dermatomyositis, fever of unknown origin, arthritis, and connective tissue diseases. Aim of this review is to give an overview of the use of WBMRI in rheumatological field.

Renal transplantation is the therapy of choice in children with ESKD. Radiological investigations are required in both pre- and post-transplant assessment, although there is paucity of both consensus-based statements and evidence-based imaging guidelines in pediatric renal transplantation. The phases of pediatric ESKD management that require imaging are pretransplantation recipient assessment and post-transplantation surveillance for detection of potential complications. We present suggestions for imaging algorithms for both pre- and post-transplant assessment in pediatric renal transplant recipients.

Juvenile idiopathic arthritis (JIA) is a heterogeneous condition encompassing all forms of chronic arthritis of unknown origin and with onset before 16 years of age. During the last decade new, potent therapeutic agents have become available, underscoring the need for accurate monitoring of therapeutic response on both disease activity and structural damage to the joint. However, so far, treatment efficacy is based on clinical ground only, although clinical parameters are poor markers for disease activity and progression of structural damage. Not so for rheumatoid arthritis patients where the inclusion of radiographic assessment has been required by FDA to test the disease-modifying potential of new anti-rheumatic drugs.In imaging of children with JIA there has been a shift from traditional radiography towards newer techniques such as ultrasound and MRI, however without proper evaluation of their accuracy and validity. We here summarize present knowledge and discuss future challenges in imaging children with JIA.

Objective To compare whole-body MRI (WB-MRI) with clinical examination in the assessment of disease activity in juvenile dermatomyositis (JDM). Methods WB-MR images were obtained from 41 JDM patients and 41 controls using a 1.5 T MRI scanner and short τ inversion recovery sequences. 18 patients had follow-up WB-MRI. Muscle, subcutaneous tissue and myofascial signal abnormalities were scored in 36 muscular groups and on proximal and distal extremities. WB-MRI and clinical assessments were performed concurrently and results compared. Validation procedures included analysis of feasibility, reliability, construct validity, discriminative ability and responsiveness. Results WB-MRI revealed distal legs (26/41 patients) and forearm (19/41 patients) muscle inflammation undetected during clinical examination and allowed an accurate assessment of subcutaneous (23/41 patients) and myofascial involvement (13/41 patients). 27 patients showed a patchy distribution of muscle inflammation while in seven the abnormal hyperintense areas tended to be homogeneously distributed. The inter-reader agreement for muscular, subcutaneous and myofascial WB-MRI scores was excellent. Correlations between WB-MRI muscle score and disease activity measures were excellent (Manual Muscle Test: rs=−0.84, Childhood Myositis Assessment Scale: rs=−0.81). WB-MRI score was higher in JDM active patients when compared with the control group (p B B =0.004), and showed an excellent responsiveness (standardised response mean=1.65). Follow-up WB-MRI showed resolution of inflammation in nine patients whereas clinical criteria for remission were satisfied in five. Conclusions WB-MRI provides additional information to clinical evaluation and represents a promising tool to estimate total inflammatory burden, tailor treatment and monitor its efficacy.

Paediatric urology often presents challenging scenarios. Magnetic resonance urography (MRU) and laparoscopy are increasingly used. We retrospectively studied children affected by a disease of the upper urinary tract who after MRU were elected for laparoscopic treatment. This pictorial essay draws on our experience; it illustrates some specific MRU findings and highlights the usefulness of MRU for the diagnosis of upper urinary tract pathology in children. It also offers some examples of the potential additional diagnostic information provided by laparoscopy as well as its therapeutic role.

There is sparse knowledge about grading tenosynovitis using MRI. The purpose of this study was to assess the reliability of a tenosynovitis MRI scoring system in juvenile idiopathic arthritis. Children with juvenile idiopathic arthritis and wrist involvement were enrolled in two paediatric centres, from October 2006 to January 2010. The extensor (compartments II, IV and VI) and flexor tendons were assessed for the presence of tenosynovitis on T1-weighted postcontrast fat-saturated MR images and were scored from 0 (normal) to 2 (moderate to severe) by two observers independently. Intra- and interobserver agreement was assessed. Ninety children (age range: 5–18.5 years) were included, of whom 34 had tenosynovitis involving extensors and 28 had tenosynovitis involving flexors. A total of 360 tendon areas were analysed, of which 114 had tenosynovitis (86/270 extensors and 28/90 flexors). Intra-reader 1 agreement was excellent for the extensors (k = 0.82–0.91) and for the flexors (k = 0.85); intra-reader 2 agreement was moderate to good for the extensors (k = 0.51–0.72) and good for the flexors (k = 0.64). Inter-reader agreement was good for the extensors (k = 0.69–0.73) and moderate for the flexors (k = 0.49). The proposed MRI scoring system for the assessment of wrist tenosynovitis in juvenile idiopathic arthritis appears feasible with an observer agreement sufficient for clinical use.

This study was done to analyse colour Doppler ultrasound (CDUS) findings in patients with dual kidney transplantation (DKT) and to compare renal volume and resistive index (RI) values between DKT and single kidney transplantation (SKT).We reviewed the clinical and imaging findings [30 CDUS, five magnetic resonance (MR) and one computed tomography (CT) examination] in 30 patients with DKT (23 men and seven women; median age 65 years; range 55-82). Three patients had clinical signs of graft malfunction. Renal volumes and RI were compared with those of 14 SKT patients and comparable levels of renal function.Three patients had graft dysfunction: one had chronic rejection and two had pathologies involving one kidney only (one encrusted pyeloureteritis of a left graft and one occluded main artery of a left graft). Asymptomatic unilateral pathologies were seen in six cases. In asymptomatic DKT patients, no significant differences in length, volume, cortical echogenicity and RI between the two kidneys were observed; DKTs were smaller (median volume 116.7 vs. 171.6 cc) and had higher RIs (0.76 vs. 0.68) (p0.01) than SKTs.CDUS provides useful information in patients with DKT, allowing detection of clinically unsuspected unilateral diseases. At comparable levels of renal function, DKT patients had higher RI and lower volumes than SKT patients.

Bone marrow oedema (BMO) is included in MRI-based scoring systems of disease activity in adults with rheumatoid arthritis. Similar systems in juvenile idiopathic arthritis (JIA) are lacking. To assess the reproducibility in a multi-centre setting of an MRI BMO scoring system in children with JIA. Seventy-six wrist MRIs were read twice, independently, by two experienced paediatric radiologists. BMO was defined as ill-defined lesions within the trabecular bone, returning high and low signal on T2- and T1-weighted images respectively, with or without contrast enhancement. BMO extension was scored for each of 14 bones at the wrist from 0 (none) to 3 (extensive). The intra-observer agreement was moderate to excellent, with weighted kappa ranging from 0.85 to 1.0 and 0.49 to 1.0 (readers 1 and 2 respectively), while the inter-observer agreement ranged from 0.41 to 0.79. The intra- and inter-observer intraclass correlation coefficients were excellent and satisfactory, respectively. The scoring system was reliable and may be used for grading bone marrow abnormality in JIA. The relatively large variability in aggregate scores, particularly between readers, underscores the need for thorough standardisation.

MRI is a sensitive tool for the evaluation of synovitis in juvenile idiopathic arthritis (JIA).The purpose of this study was to introduce a novel MRI-based score for synovitis in children and to examine its inter- and intraobserver variability in a multi-centre study.Wrist MRI was performed in 76 children with JIA. On postcontrast 3-D spoiled gradient-echo and fat-suppressed T2-weighted spin-echo images, joint recesses were scored for the degree of synovial enhancement, effusion and overall inflammation independently by two paediatric radiologists. Total-enhancement and inflammation-synovitis scores were calculated.Interobserver agreement was poor to moderate for enhancement and inflammation in all recesses, except in the radioulnar and radiocarpal joints. Intraobserver agreement was good to excellent. For enhancement and inflammation scores, mean differences (95 % CI) between observers were -1.18 (-4.79 to 2.42) and -2.11 (-6.06 to 1.83). Intraobserver variability (reader 1) was 0 (-1.65 to 1.65) and 0.02 (-1.39 to 1.44).Intraobserver agreement was good. Except for the radioulnar and radiocarpal joints, interobserver agreement was not acceptable. Therefore, the proposed scoring system requires further refinement.

Gaucher disease (GD) type 1 is the most common lysosomal storage disorder due to beta glucocerebrosidase deficiency leading to an abnormal accumulation of its substrate, glucocerebroside, in the mononuclear phagocyte system. The disease presentation is usually characterized by signs and symptoms related to hypersplenism, such as splenomegaly, anaemia, thrombocytopenia and leucopenia. Skeletal disease may occur later for the infiltration of bone marrow by macrophages infiltration and bone resorption: bone involvement may be heterogeneously manifested by symptoms ranging from bone crisis to avascular necrosis, osteoporosis and defect in remodeling of long bones. Herein, we report a patient in whom the osteoarticular involvement has been the only symptom of the disease stressing that this unusual presentation of GD has prompted a wide differential diagnosis with more common forms of coxitis.

US is a technique particularly suited to the investigation of musculoskeletal disorders in children and adolescents. This review paper describes a range of clinical settings beyond the hip joint where US has a significant role to play, including sports injuries, infectious diseases, inflammatory and degenerative conditions, congenital and developmental disorders, acute trauma of bone and joints, and peripheral nerve injuries. In some circumstances, US can be regarded as the most effective means of diagnostic imaging, whereas in other instances, it is an alternative or supplement to other more comprehensive imaging modalities, like MRI and CT. Although MRI offers superior soft-tissue contrast resolution, US is low-cost, non-invasive and has higher spatial resolution and real-time capability for the assessment of musculoskeletal structures during joint movement and stress manoeuvres.

ObjectivesTo develop and validate a paediatric-targeted MRI scoring system for the assessment of disease activity and damage in juvenile idiopathic arthritis (JIA). To compare the paediatric MRI score with the adult-designed. Outcome Measures in Rheumatology Clinical Trials—Rheumatoid Arthritis MRI Score (RAMRIS), whose suitability for assessing growing joints was tested.MethodsIn 66 patients with JIA the clinically more affected wrist was studied. Thirty-nine patients had a 1-year MRI follow-up. Two readers independently assigned the paediatric score and the RAMRIS to all studies. Validation procedures included analysis of reliability, construct validity and responsiveness to change. A reduced version of the bone erosion score was also developed and tested.ResultsThe paediatric score showed an excellent reproducibility (interclass correlation coefficient >0.9). The interobserver agreement of RAMRIS was moderate for bone erosions and excellent for bone marrow oedema (BMO). The paediatric score and RAMRIS provided similar results for construct validity. The responsiveness to change of the paediatric score was moderate for synovitis and bone erosion, and poor for BMO and did not improve when RAMRIS was applied. The reduced version of the bone erosion was valuable for the assessment of joint damage, and provided time-saving advantages.ConclusionThe results demonstrate that the paediatric MRI score is a reliable and valid method for assessing disease activity and damage in JIA. Unexpectedly, the RAMRIS provides acceptable suitability for use in the paediatric age group. Further work, especially in a longitudinal setting, is required before defining the most suitable MRI scale for assessing growing joints.

Juvenile idiopathic arthritis (JIA) represents a group of heterogeneous diseases characterized by a chronic inflammatory process primarily targeting the synovial membrane. A persistent synovitis is associated with an increased risk of osteocartilaginous damage.With the advent of effective structure-modifying treatment for JIA, it may be possible to significantly reduce or even completely prevent structural damage and associated functional disability. The trend towards early suppression of inflammation, in order to prevent erosive disease, shifts the emphasis away from conventional radiographic detectable structural damage to the slightest traces of early joint damage, and drives the need for alternative imaging techniques more sensitive in detecting early signs of disease activity and damage. In this regard MRI and US are playing an increasing role in the evaluation of arthritic joints.This article will review the key aspects of the current status and recent important advances of imaging techniques available to investigate the child with rheumatic disease, briefly discussing conventional radiography, and particularly focusing on MRI and US. In this era of advancing imaging technology, knowledge of the relative values of available imaging techniques is necessary to optimize the management of children with JIA.

Objective To compare magnetic resonance imaging (MRI), conventional radiography, and ultrasonography in identifying bone erosions in patients with juvenile idiopathic arthritis (JIA), and to determine the validity and reliability of an MRI scale in detecting and grading joint damage. Methods In 26 JIA patients, the clinically more affected wrist was studied with MRI, radiography, and ultrasonography, coupled with standard clinical assessment and biochemical analysis. MR images were assessed independently by 2 readers according to an apposite devised scoring system. Results Of 26 patients, 25 (96.1%) had 1 or more erosions as detected by MRI, whereas conventional radiography and ultrasonography revealed erosions in 13 (50%) of 26 and 12 (50%) of 24 patients, respectively. The ability of MRI to detect erosive changes was significantly higher with respect to conventional radiography (P = 0.002 with Bonferroni correction [PB]) and ultrasonography (PB = 0.0002) in the group of patients with

One of the major challenges in the management of juvenile dermatomyositis (JDM) is to accurately assess disease activity in order to optimize therapeutic strategies. Whole-body(WB)-MRI enables a reliable analysis of the site (skeletal muscle, subcutaneous tissue, myofascia) and magnitude of inflammatory process throughout the entire body. By providing an accurate estimation of the total disease activity load, this technique could be useful to tailor treatment according to disease severity.