Radiology

European Society of Paediatric Radiology Computed Tomography and Dose Task Force: European guidelines on diagnostic reference levels for paediatric imaging Abstract The recent European Council Directive 2013/59/EURATOM requires the establishment of diagnostic reference levels (DRLs) to optimise radiation dose in diagnostic and interventional radiology procedures. At the time this directive was enacted, just a few European countries had already set paediatric DRLs and many of these were outdated. For this reason, the European Commission launched a project addressing European Guidelines on Diagnostic Reference Levels for Paediatric Imaging that was awarded to a consortium led by the European Society of Radiology with the collaboration of the European Society of Paediatric Radiology and other European stakeholders involved in the radiation protection of children. The main aims of this project were to establish European DRLs to be used by countries without their own national paediatric DRLs and to provide a consistent method to establish new DRLs in the future. These European guidelines have been very recently endorsed by the European Commission and published in issue N° 185 of the Radiation Protection series. The purpose of this article is to introduce these guidelines to the wide community of paediatric radiologists.

Magnetic resonance imaging of cerebellar cysts in a neonate with congenital cytomegalovirus infection Abstract Congenital cytomegalovirus infection is one of the most common congenital viral infections in the world. Brain magnetic resonance imaging plays a key role in evaluating brain involvement and establishing prognosis; several characteristic features have been described. We present a description of cerebellar cysts in a neonate with polymerase chain reaction-confirmed cytomegalovirus congenital infection, and discuss the differential diagnosis and potential pathophysiological mechanisms.

Hermes

Idiopathic, heritable and veno-occlusive pulmonary arterial hypertension in childhood: computed tomography angiography features in the initial assessment of the disease Abstract Background In children, idiopathic and heritable pulmonary arterial hypertension present echocardiographic and heart catheterization findings similar to findings in pulmonary veno-occlusive disease. Objective To provide a systematic analysis of CT angiography anomalies in children with idiopathic or heritable pulmonary arterial hypertension, or pulmonary veno-occlusive disease. We also sought to identify correlations between CT findings and patients' baseline characteristics. Materials and methods We retrospectively analyzed CT features of children with idiopathic and heritable pulmonary arterial hypertension or pulmonary veno-occlusive disease and 30 age-matched controls between 2008 and 2014. We compared CT findings and patient characteristics, including gene mutation type, and disease outcome until 2017. Results The pulmonary arterial hypertension group included idiopathic (n=15) and heritable pulmonary arterial hypertension (n=11) and pulmonary veno-occlusive disease (n=4). Median age was 6.5 years. Children with pulmonary arterial hypertension showed enlargement of pulmonary artery and right cardiac chambers. A threshold for the ratio between the pulmonary artery and the ascending aorta of ≥1.2 had a sensitivity of 90% and a specificity of 100% for pulmonary arterial hypertension. All children with pulmonary veno-occlusive disease had thickened interlobular septa, centrilobular ground-glass opacities, and lymphadenopathy. In children with idiopathic and heritable pulmonary arterial hypertension, presence of intrapulmonary neovessels and enlargement of the right atrium were correlated with higher mean pulmonary artery pressure (P=0.011) and pulmonary vascular resistance (P=0.038), respectively. Mediastinal lymphadenopathy was associated with disease worsening within the first 2 years of follow-up (P=0.024). Conclusion CT angiography could contribute to early diagnosis and prediction of severity in children with pulmonary arterial hypertension.

Pediatric ovarian volumes measured at ultrasound after contralateral unilateral oophorectomy Abstract Background Changes that occur in the remaining ovary after contralateral oophorectomy are not well described. Objective To determine average ovarian volume in pediatric patients after contralateral oophorectomy compared to age-matched controls with two normal ovaries. Materials and methods We performed a retrospective review of ultrasound examinations and electronic medical records of patients ages 0–18 years who had unilateral oophorectomy from 2000 to 2017 (n=64). We used 384 consecutive normal age-matched ovaries for comparison, analyzing mean ovarian volumes. Results Higher mean ovarian volume (mL) was observed in patients who had oophorectomy compared to controls in the first decade of life (P<0.003) and second decade of life (P<0.0003). Higher mean ovarian volume was seen in both premenarchal and menstruating patients with prior oophorectomy when compared to controls (P<0.05 and P<0.0001, respectively). When comparing volume during menstrual cycle, we saw higher mean ovarian volumes in the oophorectomy group compared to the control group for the follicular (P<0.0001), pre-ovulatory (P=0.0005) and luteal phases (P<0.0003). We provide an updated reference of normal ovarian volumes for pediatric patients, with values similar to those already reported in the literature. Conclusion Ovarian volume is higher in pediatric patients with one normal ovary following contralateral oophorectomy. The provided normative volumes can be used in evaluating these patients.

ESPR 2019

Comparison of diagnostic accuracy for fistulae at ultrasound and voiding cystourethrogram in neonates with anorectal malformation Abstract Background Recently, it has been reported that anorectal malformation with rectourethral fistula in male neonates can be managed by primary neonatal reconstruction without colostomy. To prevent urethral injury during anorectoplasty, the fistula's location is important. To date, the use of voiding cystourethrograms to determine the presence and location of fistulas in neonates with anorectal malformations has not been studied. Objective To compare the accuracy of ultrasound (US) and voiding cystourethrogram for determining the presence and location of fistulas in neonates with anorectal malformation. Materials and methods We included 21 male neonates with anorectal malformation with rectourethral fistula (n=16), rectovesical fistula (n=1) or no fistula (n=4) who underwent US and voiding cystourethrogram preoperatively on the day of surgery. Fistula imaging was classified into three grades (0–2), and grades 1–2 were considered fistula positive. We compared the imaging-based location of the fistula with surgical findings. Results US performed significantly better than voiding cystourethrogram for determining the presence of fistulas (area under the receiver operating characteristic curve, 0.90 vs. 0.71, respectively; P=0.044) (diagnostic accuracy 85.7%, 95% confidence interval [95% CI] 63.7–97.0% and 52.4%, 95% CI 29.8–74.3%, respectively). In cases with fistulas detected by either modality, the accuracy of locating the fistula by US was 50.0% (95% CI 24.7–75.3%) and by voiding cystourethrogram was 100% (95% CI: 59.0–100%). Conclusion US accurately detected, but did not accurately locate, fistulas in neonates with anorectal malformation. When planning primary neonatal reconstruction of anorectal malformation without colostomy, voiding cystourethrogram could provide additional information about fistula location.

Congenital diaphragmatic hernia sacs: prenatal imaging and associated postnatal outcomes Abstract Background The presence of a hernia sac in congenital diaphragmatic hernia (CDH) has been reported to be associated with higher lung volumes and better postnatal outcomes. Objective To compare prenatal imaging (ultrasound and MRI) prognostic measurements and postnatal outcomes of CDH with and without hernia sac. Materials and methods We performed database searches from January 2008 to March 2017 for surgically proven cases of CDH with and without hernia sac. All children had a detailed ultrasound (US) examination and most had an MRI examination. We reviewed the medical records of children enrolled in our Pulmonary Hypoplasia Program. Results Of 200 cases of unilateral CDH, 46 (23%) had hernia sacs. Cases of CDH with hernia sac had a higher mean lung-to-head ratio (LHR; 1.61 vs. 1.17; P<0.01), a higher mean observed/expected LHR (0.49 vs. 0.37; P<0.01), and on MRI a higher mean observed/expected total lung volume (0.53 vs. 0.41; P<0.01). Based on a smooth interface between lung and herniated contents, hernia sac or eventration was prospectively questioned by US and MRI in 45.7% and 38.6% of cases, respectively. Postnatally, hernia sac is associated with shorter median periods of admission to the neonatal intensive care unit (45.0 days vs. 61.5 days, P=0.03); mechanical ventilation (15.5 days vs. 23.5 days, P=0.04); extracorporeal membrane oxygenation (251 h vs. 434 h, P=0.04); decreased rates of patch repair (39.0% vs. 69.2%, P<0.01); and pulmonary hypertension (56.1% vs. 75.4%, P=0.03). Conclusion Hernia sac is associated with statistically higher prenatal prognostic measurements and improved postnatal outcomes. Recognition of a sharp interface between lung and herniated contents may allow for improved prenatal diagnosis; however, delivery and management should still occur at experienced quaternary neonatal centers.

Human immunodeficiency virus-related cerebral white matter disease in children Abstract The human immunodeficiency virus (HIV) epidemic seems largely controlled by anti-retroviral treatment with resultant large numbers of children growing up with the disease on long-term treatment, placing them at higher risk to develop HIV-related brain injury, ongoing cognitive impairment and treatment-related neurological complications. Cerebral white matter involvement is a common radiologic finding in HIV infection and the causes of this have overlapping appearances, ranging from diffuse widespread involvement to focal lesions. The varied pathophysiology is broadly grouped into primary effects of HIV, opportunistic infection, vascular disease and neoplasms. White matter changes in children can be different from those in adults. This review provides guidance to radiologists with the diagnostic dilemma of nonspecific cerebral white matter lesions in children with HIV. The authors discuss common causes of HIV-related cerebral white matter disease as well as the role of neuroimaging in the management of these children.