Referral guidelines for medical imaging in children: an ESR-EuroSafe Imaging survey on availability, awareness and use in clinical practice among European radiologists.

OBJECTIVES: Justification of medical exposures from medical imaging is fundamental to radiation protection. Referral guidelines are intended to help physicians decide when an imaging study is justified. For two decades, referral guidelines have been a legally binding requirement for European Union member states. Recently, the European Society of Radiology (ESR) developed iGuide tool, which provides evidence-based referral guidance for imaging inclusive of children. The aim of this survey was to assess the availability, use and familiarity of referral guidelines for medical imaging in children and knowledge about the availability of ESR iGuide among ESR member radiologists. METHODS: Over a 2-month period (15 September-15 November 2019), 33,257 ESR member radiologists were invited to respond to an anonymised web-based questionnaire, which consisted of 12 multiple-choice questions. RESULTS: In total, 2067/33,257 responses (6.3%) were received from 52 countries. A total of 1068 out of 2067 (51.7%) respondents were aware that imaging referral guidelines are a legal requirement. One thousand five (48.6%) of all respondents did not know whether dedicated guidelines for imaging in children were available, and only 653 (31.2%) were aware of the mainstays of the available guidelines. Similarly, just 746 (36.1%) of all respondents were aware of ESR iGuide availability and features. CONCLUSIONS: The information gathered confirms that effective and widespread adoption of imaging referral guidelines is lacking, especially in children. Further work is required to improve uptake and awareness. KEY POINTS: • Justification of medical exposures is fundamental to radiation protection and evidence-based referral guidelines are crucial for practical implementation of this principle. • About half of survey respondents are aware that the availability of imaging referral guidelines is a legal requirement, despite this being mandated since 1997. • The information gathered from this survey confirms that, especially in children, an effective and widespread adoption of imaging referral guidelines is lacking.

Nano Superconducting QUantum Interference Device sensors for magnetic nanoparticle detection.

In this paper, Superconducting QUantum Interference Devices (SQUIDs) based on single layer Nb nanobridge Josephson junctions are described. Devices, with loop area ranging from 4 to 0.5 microm2, have been patterned by Electron Beam Lithography (EBL) in a 20 nm thick Nb layer, achieving a responsivity of about 30 microA/phi0. Magnetization measurements have been performed via switching current measurements at a temperature T = 4.2 K. Preliminary detection of Silica-magnetite (Fe3O4-SiO2) core/shell nanoparticle cluster has been proven.

European consensus on patient contact shielding.

Patient contact shielding has been in use for many years in radiology departments in order to reduce the effects and risks of ionising radiation on certain organs. New technologies in projection imaging and CT scanning such as digital receptors and automatic exposure control (AEC) systems have reduced doses and improved image consistency. These changes and a greater understanding of both the benefits and the risks from the use of shielding have led to a review of shielding use in radiology. A number of professional bodies have already issued guidance in this regard. This paper represents the current consensus view of the main bodies involved in radiation safety and imaging in Europe: European Federation of Organisations for Medical Physics, European Federation of Radiographer Societies, European Society of Radiology, European Society of Paediatric Radiology, EuroSafe Imaging, European Radiation Dosimetry Group (EURADOS), and European Academy of DentoMaxilloFacial Radiology (EADMFR). It is based on the expert recommendations of the Gonad and Patient Shielding (GAPS) Group formed with the purpose of developing consensus in this area. The recommendations are intended to be clear and easy to use. They are intended as guidance, and they are developed using a multidisciplinary team approach. It is recognised that regulations, custom and practice vary widely on the use of patient shielding in Europe and it is hoped that these recommendations will inform a change management program that will benefit patients and staff.

Multislice CT in congenital bronchopulmonary malformations in children.

Congenital bronchopulmonary malformations encompass a wide spectrum of pathologies involving the lungs, trachea and bronchi, pulmonary vessels, and oesophagus. These developmental lesions are often isolated, but the association of two or more anomalies is not infrequent. Contrast-enhanced multidetector computed tomography (MDCT), thanks to multiplanar and 3D reconstructions, allows for detailed studies of these malformations, achieving better accuracy compared with conventional techniques such as chest X-ray, fluoroscopy, ventilation and perfusion scintigraphy and ultrasonography. MDCT is characterised by fast data acquisition and does not require sedation in the majority of cases. The main drawbacks of MDCT are the use of ionising radiation and - in many cases -contrast media. Recently, improved CT scanners and optimised CT protocols have made available to children all the benefits of MDCT, thanks to a significant reduction in radiation dose and an improved risk-benefit ratio. The aim of our paper was to evaluate MDCT in children with bronchopulmonary malformations by reporting our experience (about 2,400 studies in 30 months with a 64-slice MDCT scanner) and comparing it with the available literature.

Shifting from open surgical cut down to ultrasound-guided percutaneous central venous catheterization in children: learning curve and related complications.

PURPOSE: Tunneled indwelling central venous catheters (CVC) are essential in the management of children with cancer, hematological, nephrological disorders and for parenteral nutrition. The aim of this study is to present the experience of a single center of the transition from traditional open surgical cut down procedure (OSC) to ultrasound (US)-guided percutaneous CVC insertion, focusing on learning curve and related complications. METHODS: All CVCs inserted between April 2008 and November 2009 in children at the Gaslini Children Hospital were revised, and data on methods of cannulation, intraoperative and device-related complications and re-intervention were recorded. RESULTS: 194 CVCs were positioned in 188 patients. 128 out of 194 CVCs were positioned through an OSC technique, whereas the remaining 66 CVCs were inserted percutaneously with US guidance. Of the 27 recorded complications, 15 were mechanical events, 7 cases developed infection, whereas the remaining 5 (2.6%) were classified as intraoperative complications. A second surgical procedure was described in 23 (11.8%) cases. CONCLUSION: Shifting from OSC to US-guided percutaneous CVC insertion inevitably involves a challenging learning curve which is generally associated with high complication rates. Complications progressively decrease once a good experience in US guidance and percutaneous technique has been obtained.

Conservative management of chylous ascites after oncological surgery for peripheral neuroblastic tumors in pediatric patients.

Chylous ascites may complicate the postoperative course of abdominal surgery mainly due to the iatrogenic disruption of the lymphatic channels during extensive retroperitoneal dissection. Sparse data are available regarding treatment; however, in many cases a recommended first-line treatment approach is by way of enteral feeding, consisting of a formula high in medium-chain triglycerides (MCTs) together with a complete total parenteral nutrition teamed with somatostatin (or an equivalent). Nonetheless, the ligation of chylous fistulae, together with the application of Fibrin glue, as well as the creation of peritoneal-venous shunts have also been documented. The aims of this study are to document incidence of postoperative chylous ascites following resection of abdominal peripheral neuroblastic tumors, evaluate efficacy of the management of chylous ascites, and investigate the main risk factors. A survey was carried out over a span of six years, from March 2010 to March 2016 at Giannina Gaslini Children's Hospital involving seventy-seven children with resections of peripheral neuroblastic tumors. Incidence rate of postoperative chylous ascites following a normal diet was 9% (n=7). Treatment using total parenteral nutrition with octreotide resulted in a complete recovery from chylous ascites within a 20 day period without recurrence. Length of operative time, nephrectomy, and the extension of lymphadenectomy were all significantly associated with a higher incidence of postoperative chylous ascites (p<0.05) which also lengthened hospital stay (p<0.05) and possibly delayed beginning adjuvant chemotherapy.

Rare viral infections in children receiving hemopoietic stem cell transplant.

Viral infections are a rare complication in autologous hemopoietic stem cell transplant (HSCT) recipients but represent a frequent cause of disease after allogeneic HSCT. In the last years, there has been an increase in the number of viral diseases observed in these patients. This fact may be at least partially due to an improvement in diagnostic facilities, but the increasing number of transplant procedures and the more severe immunosuppression may also have played an important role.

Invasive mycoses in children receiving hemopoietic SCT.

Invasive mycoses represent a rare but severe complication following hemopoietic SCT (HSCT) in children. Their incidence is related to the type of donor, being higher after allogeneic transplant, especially from alternative donors. Moreover, the incidence of invasive mycoses varies in the different post transplant phases. Neutropenia, lymphopenia, GvHD, high-dose steroids or other immunosuppressive drugs represent well-known risk factors. The clinical features of invasive mycoses after HSCT in children are similar to those observed in adults, and the diagnostic tools, including Aspergillus galactomannan antigen detection, are feasible also in pediatrics. Mortality due to invasive mycoses after HSCT in children is high.

Bethlem myopathy: early-onset benign autosomal dominant myopathy with contractures. Description of two new families.

Bethlem myopathy is an apparently rare early-onset benign autosomal dominant limb-girdle myopathy with contractures of the fingers. To determine whether this disorder is unrecognized rather than rare we used muscle computerized tomography (CT) and isokinetic muscle testing and assiduously sought contractures of the fingers in relatives of two patients with the disease. CT showed that muscle impairment was mild but more diffuse than clinically apparent and showed an unexpected progressive degeneration of lumbar paravertebral muscles. The isokinetic test showed that the quadriceps was more involved that the hamstrings. In addition we found that contractures of the last four fingers progressed centripetally with age from the distal interphalangeal joints to the wrist. As a result we proved that 15 of the 21 examined subjects had the disease, 7 of them being unaware that anything was amiss. Careful examination may reveal that Bethlem myopathy is more common than is now thought.

Spine surgery in spinal muscular atrophy: long-term results.

Fifteen patients with spinal muscular atrophy operated on for scoliosis were reviewed and interviewed. Age at time of surgery averaged 16 yr. Follow-up averaged more than 5 yr. Eleven patients underwent posterior spinal fusion with Luque instrumentation. Four had Harrington instrumentation, with segmental wiring in three. Three out of the 15 also had anterior fusion with Dwyer instrumentation. Patients were immobilized in cast or brace for 6 months, on average, after surgery. The average curve correction was 50%. There was a loss of vital capacity after the operation, which in most cases was regained with time. In functional terms, there were improvements and deteriorations both in the short and long term. Esthetic appearance improved in all patients, as did the perceived well-being. Retrospectively all but one would have the operation again.

Isokinetic muscle testing (IMT) in neuromuscular diseases. Preliminary report.

Isokinetic muscle testing (IMT) allows precise and reliable measurement of the force produced by the skeletal muscle during exercise at constant velocity and accommodating resistance. This study reports on some clinical situations that illustrate the difference between manual muscle testing (MMT) and IMT in neuromuscular patients. IMT was performed by a special method (continuous passive motion plus gravity compensation) which allowed the measurement of very weak forces, such as in the four patients described in this study. It is important to note that for the same MMT grading the corresponding isokinetic force values were very different. Therefore there is an obvious correspondence between the isokinetic measurement of muscle strength and the morphological change in the muscle on the CT scan of the thigh, which did not show up on MMT. IMT could be extremely important for research into neuromuscular disorders, where the detection of even the tiniest variations in strength is relevant.

Epidemiology of neuromuscular disorders in the under-20 population in Bologna Province, Italy.

A retrospective epidemiological survey of the principal neuromuscular disorders was undertaken in the population under the age of 20 yr in Bologna city and province. The incidence of Duchenne muscular dystrophy (DMD) proved to be 25.8 x 10(-5), that is, comparable with the outcome of neonatal screening studies. The incidence of spinal muscular atrophy (SMA) was very high (11.2 x 10(-5), probably the result of our greater awareness and recognition of this disorder. The prevalence of neuromuscular disorders in the 0-19 yr population is 1.5 times that in the general population; that of DMD is 28 x 10(-5) vs 6.3 x 10(-5) and that of the SMA 6.5 x 10(-5) vs 1.2 x 10(-5). Our results indicate that neuromuscular disorders, particularly DMD and SMA I and II, have a much higher prevalence in the first two decades of life than is generally thought. Awareness of the higher prevalence of neuromuscular disorders among the under-20s is essential to the adequate planning of medical services.

Long-term results of spine surgery in Duchenne muscular dystrophy.

We report the long-term orthopaedic and functional results of segmental spinal instrumentation and fusion in 30 Duchenne patients. Twenty-nine had a mean 59% correction of scoliosis with post-operative immobilization in a brace of only three months on average and with a very limited loss of correction over time. One died after cardiac arrest. The mean vital capacity preoperatively was 57 +/- 17% with a decrease to 34 +/- 13% at 3.9 +/- 2 yr after surgery. The sitting position, aesthetic improvement and the quality of life after spinal fusion have been positively evaluated by the large majority of the patients and their parents. Head control was lost in the 14 patients who developed a more severe extension contracture of the neck measured as a significantly longer chin-sternum distance. More than 90% would have the operation or would give their consent again for their son having the operation.

Bronchioloalveolar carcinoma arising in congenital cystic adenomatoid malformation in a child: a case report and review on malignancies originating in congenital cystic adenomatoid malformation.

A type I congenital cystic adenomatoid malformation (CCAM) in the left lower lobe was removed from a 11-year-old boy with a 3-month history of recurrent pneumonia. As incidental finding, a bronchioloalveolar carcinoma (BAC) was found in the lung parenchyma adjacent to the cyst. A left lower lobectomy was performed. At 18 months after surgery the patient is well and free of neoplastic disease. To the best of our knowledge, this association has not been reported previously in a pediatric patient. Malignancies complicating CCAM are rarely seen, but have been reported in adults. Including our case, eight cases of BAC and five cases of rhabdomyosarcoma (RMS) in association with CCAM have been reported so far. As CCAM can host metaplastic mucous cells, primitive mesenchymal cells and differentiated but poorly organized striated muscle fibers, it has been proposed that CCAM may act as a predisposing condition for oncogenesis. Our experience adds further support that CCAM can act as a premalignant lesion. Previous reports of both BAC and RMS in asymptomatic CCAM suggest prompt resection shortly after diagnosis.

Medium-term results after cholecystectomy in patients younger than 10 years.

BACKGROUND: Gallbladder stones are a well-known and widely studied problem in children. Hematological disorders are the most common diseases that can cause cholelithiasis. However, in the last few years, the proportion of children with idiopathic cholelithiasis has increased 50%. Herein, we present a prospective study on laparoscopic cholecystectomy in a selected group of patients aged < 10 years. METHODS: Fifty-eight patients aged < 10 years underwent laparoscopic cholecystectomy for stones in the period 1992-99. The female/male ratio was 1.5, the mean age was 8 years (range, 2-10), and the mean weight was 30 kg. In all patients, parenteral nutrition, fasting state, and prolonged use of antibiotics had been suspended for > or = 6 months, and conservative treatment had been tried for > or = 12 months in the absence of symptoms of stone migration. All the patients were followed up after surgery: clinically at 1,6,12, and 36 months and by ultrasound at 1, 12, and 36 months. Liver function and hematological tests were performed in case of symptoms or if hemolytic disorders were the cause of stones. RESULTS: The mean operative time was 63 min (range, 30-120) in children undergoing cholecystectomy alone and 150 min in children undergoing associated splenectomy. There were no major complications or reoperations. Minor complications included bleeding from accessory cystic artery (n = 3) and insufflation of the omentum (n = 2). One case was converted to an open procedure due to technical problems. All the children were followed up and no complications were observed. CONCLUSION: We consider the laparoscopic approach the gold standard for cholecystectomy in children. This procedure does not have a complication rate any higher than open cholecystectomy, and patient follow-up is as good as that of open surgery. Previous abdominal surgery is not a contraindication to laparoscopy.

Rigid spine syndrome and rigid spine sign in myopathies.

We studied eight patients with rigid spine syndrome aged 8 to 20 years at the time of first examination. Muscle weakness, rigid spine, and flexion contracture of elbows and ankles were noted in the first 6 years of age. Radiological study of the cervical spine revealed considerable reduction not only of flexion, but also of extension, of the neck. The "alligator sign" was demonstrated. Progression of scoliosis and of contractures reduced the functional capacity in six patients. A restrictive ventilatory syndrome was observed in all, while central apneas with oxygen desaturation were shown in two. Cardiac arrhythmias were observed in four. We suggest that a distinction should be made between myopathic rigid spine and rigid spine syndrome. Myopathic rigid spine is a clinical sign which is rarely sought but is present in several types of well-defined myopathies, meaning the myopathic involvement of the extensor muscles of the spine. Rigid spine syndrome is an axial myopathy with peculiar clinical and radiographic signs and a characteristic natural history.

Spinal muscular atrophy: natural history and orthopaedic treatment of scoliosis.

To study the natural history of scoliosis in spinal muscular atrophy (SMA), 63 spinal radiographs of affected patients who attended the Muscle Clinic of Rizzoli Orthopaedic Institute between 1974 and 1988 were reviewed. All but one of the intermediate SMA patients, and all of the mild SMA patients who stopped walking had a scoliosis that ranged from 10 degrees to 165 degrees. Out of the 19 mild SMA patients still able to walk, 12 had a scoliosis ranging from 10 degrees to 45 degrees. Mean age at the onset of scoliosis was 4 years 4 months in intermediate SMA, and 9 years 10 months in mild SMA. Data on characteristics of the scoliotic curve are reported. The effectiveness of orthopaedic treatment in the prevention of scoliosis is discussed.

Pancreatitis caused by duodenal duplication.

The authors present the investigations and surgical treatment of two cases of duodenal cystic duplication. Abdominal pain and gastroesophageal reflux were the most important symptoms and signs associated with an history of recurrent acute pancreatitis. Computed tomography scan, ultrasound examination, and cholangiography confirmed preoperatively the diagnosis, and a transduodenal surgical approach was carried out in both children. A simple marsupialization of the cyst was performed in the former, and a sphincterotomy with papillosphincteroplasty was associated in the latter. The diagnosis was confirmed by microscopy, and both the children are asymptomatic after a 14 and 18 months of follow-up. This report focuses on the importance of the cholangiopancreatography for every child presenting with recurrent, unexplained bouts of acute pancreatitis, and underlines the technical surgical aspects on the basis of the anatomic identification of the malformation.

Treatment of vesico-ureteric reflux in children with neuropathic bladder: a comparison of surgical and endoscopic correction.

BACKGROUND/PURPOSE: Vesico-ureteric reflux (VUR) is a common problem in children with neuropathic bladder. Lesser-degree VUR may be manageable by intermittent catheterization or by anticholinergics, but higher grades usually require surgical treatment. If left untreated, two thirds of such patients may experience deterioration of the upper renal tracts. The aim of this study was to compare the results of the STING (Subureteric Teflon Injection) technique with surgical ureteric reimplantation as treatment for VUR in neuropathic bladder. METHODS: From January 1981 to December 1996, 58 children with NB (81 ureters) were treated for VUR. STING and Cohen ureteroneocystotomy were performed in 40 and 41 ureters, respectively. Mean age was 4.5 years (STING) and 5.1 years (Cohen). RESULTS: Twenty-nine of 40 refluxing ureters (72.5%) were cured by STING, whereas Cohen eradicated reflux in 39 of 41 ureters (95.5%). No complications were observed in either group. All the ureters in which STING failed were treated successfully by Cohen ureteroneocystostomy. The 2 ureters still refluxing after surgical reimplantation were cured successfully by a single STING. The mean follow-up was 6.8 years in the Cohen group and 4.8 years in the STING group. During follow-up, no recurrence was observed in patients cured by open reimplantation. In the STING group, 2 previously cured ureters showed recurrence of VUR: both were treated successfully by a further STING. CONCLUSIONS: Open ureteral reimplantation is more effective than STING in correcting VUR in children with neuropathic bladder dysfunction. Nevertheless, the good success rate, the relative technical simplicity, outpatient nature, and rapid recovery point to STING as a safe and effective procedure for the initial treatment of VUR. Failure of STING does not preclude a successful open operation.