Neonatal neurosonography practices: a survey of active Society for Pediatric Radiology members.

BACKGROUND: While neonatal brain US is emerging as an imaging modality with greater portability, widespread availability and relative lower cost compared to MRI, it is unknown whether US is being maximized in infants to increase sensitivity in detecting intracranial pathology related to common indications such as hemorrhage, ischemia and ventriculomegaly. OBJECTIVE: To survey active members of the Society for Pediatric Radiology (SPR) regarding their utilization of various cranial US techniques and reporting practices in neonates. MATERIALS AND METHODS: We distributed an online 10-question survey to SPR members to assess practice patterns of neonatal cranial US including protocol details, use of additional sonographic views, perceived utility of spectral Doppler evaluation, and germinal matrix hemorrhage and ventricular size reporting preferences. RESULTS: Of the 107 institutions represented, 90% of respondents were split evenly between free-standing children's hospitals and pediatric departments attached to a general hospital. We found that most used template reporting (72/107, 67%). The anterior fontanelle approach was standard practice (107/107, 100%). We found that posterior fontanelle views (72% sometimes, rarely or never) and high-frequency linear probes to evaluate far-field structures (52% sometimes, rarely or never) were seldom used. Results revealed a range of ways to report germinal matrix hemorrhage and measure ventricular indices to assess ventricular dilatation. There was substantial intra-institutional protocol and reporting variability as well. CONCLUSION: Our results demonstrate high variability in neurosonography practice and reporting among active SPR members, aside from the anterior fontanelle views, template reporting and linear high-resolution near-field evaluation. Standardization of reporting germinal matrix hemorrhage and ventricular size would help ensure a more consistent application of neonatal US in research and clinical practice.

Neonatal diagnosis of biliary atresia: a practical review and update.

Biliary atresia is challenging to diagnose because many of the clinical and imaging features of this condition overlap with those of other causes of cholestasis in newborns. When jaundice persists beyond 2 weeks of age, the neonate should be evaluated for cholestasis, and biliary atresia - the most common cause of neonatal cholestasis - should be considered. It is critical to diagnose biliary atresia early because failure to treat can result in hepatic fibrosis and death in less than 1 year. In this paper, we review the current diagnostic imaging methods, differential considerations and treatment options for biliary atresia.

The use of a pediatric appendicitis pathway in a large integrated health system reduced computed tomography imaging in the ED.

BACKGROUND: Appendicitis is the most common cause of an acute surgical abdomen in children. Diagnosis is often challenging as few pediatric patients present with classic symptoms. Clinicians are thus dependent on imaging to reach an accurate diagnosis. Although computerized tomography (CT) has high sensitivity and specificity, it has the disadvantage of imparting ionizing radiation. Ultrasound (US) is readily available and has comparable accuracy to CT when performed by experienced sonographers. We sought to examine the impact of a system-wide process improvement plan on CT use and other metrics in pediatric patients who presented to the Emergency Department (ED) with suspected appendicitis. METHODS: This is a retrospective study of the impact of a Pediatric Appendicitis Pathway (PAP) within a large integrated hospital system with 12 EDs including 3 designated hub EDs. Patients were placed in an initial risk category utilizing the Pediatric Appendicitis Score (PAS), and received US of the appendix at a hub ED if indicated by the PAS. Patients presenting to community EDs who required US appendix were transferred to hub EDs for imaging. Patients presenting in the 6-month pre-implementation period were compared to patients presenting in a 14-month post-implementation period on CT and US utilization, negative and missed appendectomy rates, and ED length of stay (LOS). RESULTS: 1874 patients (401 pre-PAP and 1473 post-PAP) were included in the study. At the hub EDs the rate of CT imaging for suspected appendicitis was reduced from 31% to 17% with a resultant increase in US utilization from 83% (333/401) to 90% (1331/1473) (p < 0.001). At community general EDs (404 pre-PAP and 449 post-PAP), the rate of CT was decreased from 45% (181/404) to 32%(144/449) (p < 0.001)) There was no significant change in the negative appendectomy rate pre-PAP (1/59 = 1.7%) and post-PAP (4/168 = 2.4%) (p = 0.99) at the hub EDs. There were no missed appendicitis cases after PAP implementation compared to 1 case in the pre-PAP period. Overall LOS was similar pre and post-PAP, however LOS was longer in patients that required transfer from community general EDs to hub EDs (median 264 vs 342 min, p < 0.001). CONCLUSIONS: A PAP that stratified patients into risk groups using the PAS and encouraged the use of US as a first line imaging modality, reduced the number of CT performed in a large integrated health system without significant changes to clinical outcomes. Furthermore, transferring select patients for an US as opposed to obtaining an initial CT in community general EDs was feasible and reduced CT use in the pediatric population.

Changes in factors influencing fellowship choices among radiology residents from 2008 to 2018 and methods that may increase interest in the pediatric radiology subspecialty.

BACKGROUND: Fewer residents are choosing a career in pediatric radiology, which is contributing to an ongoing shortage of pediatric radiologists. OBJECTIVE: To identify potential causes of reduced interest in pediatric radiology as a career given a projected worsening of a nationwide shortage of pediatric radiologists. MATERIALS AND METHODS: An online questionnaire using previously published questions was approved and distributed by the Program Directors in Diagnostic Radiology to diagnostic radiology residents on behalf of the Society for Pediatric Radiology (SPR). Descriptive statistics including means with standard error and independent t-tests were used to compare mean scores between survey years. RESULTS: Nearly all of the 353 respondents (90.9%) planned on pursuing a fellowship. The majority (57.7%) identified their fellowship subspecialty before the 3rd year of residency with only 5.7% selecting pediatric radiology. Overall, 18.2% of survey respondents favored academic practice compared to 40% in the pediatric radiology subgroup. Fellowship choices were most strongly based on area of strong personal interest, marketability and area of strong personal knowledge, while the pediatric radiology subgroup emphasized area of strong personal interest, increased interaction with other physicians and enjoyable residency rotations. The pediatric radiology subgroup believed their impact on patient care was more significant than other subspecialties. Pediatric radiology job opportunities were thought to be more limited, geographically confining, and to have lower salaries than other subspecialties. More flexible job opportunities and higher demand were identified as factors needing to change before a resident would consider a pediatric radiology career. CONCLUSION: The influence on fellowship selection is multifactorial. By emphasizing the favorable job market and marketability of pediatric radiology in all practice types/geographic locations, correcting perceived salary gaps and stressing the impact on patient care as early as medical school, the number of residents choosing a career in pediatric radiology may grow.

Is Intussusception a Middle-of-the-Night Emergency?

OBJECTIVES: Intussusception is the most common abdominal emergency in pediatric patients aged 6 months to 3 years. There is often a delay in diagnosis, as the presentation can be confused for viral gastroenteritis. Given this scenario, we questioned the practice of performing emergency reductions in children during the night when minimal support staff are available. Pneumatic reduction is not a benign procedure, with the most significant risk being bowel perforation. We performed this analysis to determine whether it would be safe to delay reduction in these patients until normal working hours when more support staff are available. METHODS: We performed a retrospective review of intussusceptions occurring between January 2010 and May 2015 at 2 tertiary care institutions. The medical record for each patient was evaluated for age at presentation, sex, time of presentation to clinician or the emergency department, and time to reduction. The outcomes of attempted reduction were documented, as well as time to surgery and surgical findings in applicable cases. A Wilcoxon rank test was used to compare the median time with nonsurgical intervention among those who did not undergo surgery to the median time to nonsurgical intervention among those who ultimately underwent surgery for reduction. Multivariable logistic regression was used to test the association between surgical intervention and time to nonsurgical reduction, adjusting for the age of patients. RESULTS: The median time to nonsurgical intervention was higher among patients who ultimately underwent surgery than among those who did not require surgery (17.9 vs 7.0 hours; P < 0.0001). The time to nonsurgical intervention was positively associated with a higher probability of surgical intervention (P = 0.002). CONCLUSIONS: Intussusception should continue to be considered an emergency, with nonsurgical reduction attempted promptly as standard of care.

Value of gadolinium-enhanced MRI in detection of acute appendicitis in children and adolescents.

OBJECTIVE: The aim of this study was to determine both the value of gadolinium-enhanced MRI in children with suspected acute appendicitis and the best sequences for detecting acute appendicitis, to thereby decrease imaging time. MATERIALS AND METHODS: This was a retrospective review of pediatric patients with suspected appendicitis who had undergone MRI at our institution between 2010 and 2011 after an indeterminate ultrasound examination. MRI examinations included T1-weighted unenhanced and contrast-enhanced, T2-weighted, and balanced steady-state free precession (SSFP) sequences in axial and coronal planes. Sequences were reviewed together and individually by five radiologists who were blinded to the final diagnosis. Radiologists were asked to score their confidence of appendicitis diagnosis using a 5-point scale. The diagnostic performance of each MR sequence was obtained by comparing the mean area under the curve (AUC) using receiver operating characteristic (ROC) analysis. RESULTS: A total of 49 patients with clinically suspected appendicitis were included, of whom 16 received a diagnosis of appendicitis. The mean AUCs for reviewing all sequences together, contrast-enhanced sequences alone, T2-weighted sequences alone, and balanced SSFP alone were 0.984, 0.979, 0.944, and 0.910, respectively. No significant difference was observed between reviewing all sequences together versus contrast-enhanced sequences alone (p = 0.90) and T2-weighted sequences alone (p = 0.23). A significant difference was observed between contrast-enhanced sequences and balanced SSFP (p < 0.03). CONCLUSION: Gadolinium-enhanced images and T2-weighted images are most helpful in the assessment of acute appendicitis in the pediatric population. These findings have led to protocol modifications that have reduced imaging time.

Clinical and radiological distinction between spondylothoracic dysostosis (Lavy-Moseley syndrome) and spondylocostal dysostosis (Jarcho-Levin syndrome).

In 1938, Saul Jarcho and Paul Levin from Johns Hopkins Hospital reported cases of thoracic insufficiency due to vertebral and rib anomalies. Nearly 30 years later, in 1966, Norman Lavy and associates from Indiana University reported a similar syndrome in a family from Puerto Rico. Lavy's description was followed by a report by John E. Moseley from New York City, where the name spondylothoracic dysplasia (dysostosis) was first used. For more than half a century, there has been confusion regarding the distinction between these two phenotypically similar syndromes that cause thoracic insufficiency. Spondylocostal dysostosis (SCD), or Jarcho-Levin syndrome, causes mild to moderate respiratory insufficiency, is panethnic and has been linked to genes such as DLL3, which is known to be associated with the Notch pathway. In contrast, spondylothoracic dysostosis (STD), or Lavy-Moseley syndrome, results in more severe respiratory compromise, is largely linked to Puerto Rican cohorts and is thought to be associated to the MESP2 gene, also a Notch pathway gene. Long-term studies of Puerto Rican cohorts with STD contradicts the previously held belief that individuals affected with STD have markedly diminished life expectancy with as many as 25% surviving into later childhood and adult life.

Monitoring early tumor response to drug therapy with diffuse optical tomography.

Although anti-angiogenic agents have shown promise as cancer therapeutics, their efficacy varies between tumor types and individual patients. Providing patient-specific metrics through rapid noninvasive imaging can help tailor drug treatment by optimizing dosages, timing of drug cycles, and duration of therapy-thereby reducing toxicity and cost and improving patient outcome. Diffuse optical tomography (DOT) is a noninvasive three-dimensional imaging modality that has been shown to capture physiologic changes in tumors through visualization of oxygenated, deoxygenated, and total hemoglobin concentrations, using non-ionizing radiation with near-infrared light. We employed a small animal model to ascertain if tumor response to bevacizumab (BV), an anti-angiogenic agent that targets vascular endothelial growth factor (VEGF), could be detected at early time points using DOT. We detected a significant decrease in total hemoglobin levels as soon as one day after BV treatment in responder xenograft tumors (SK-NEP-1), but not in SK-NEP-1 control tumors or in non-responder control or BV-treated NGP tumors. These results are confirmed by magnetic resonance imaging T2 relaxometry and lectin perfusion studies. Noninvasive DOT imaging may allow for earlier and more effective control of anti-angiogenic therapy.

Contrast ultrasound imaging for identification of early responder tumor models to anti-angiogenic therapy.

Agents targeting vascular endothelial growth factor (VEGF) have been validated as cancer therapeutics, yet efficacy can differ widely between tumor types and individual patients. In addition, such agents are costly and can have significant toxicities. Rapid noninvasive determination of response could provide significant benefits. We tested if response to the anti-VEGF antibody bevacizumab (BV) could be detected using contrast-enhanced ultrasound imaging (CEUS). We used two xenograft model systems with previously well-characterized responses to VEGF inhibition, a responder (SK-NEP-1) and a non-responder (NGP), and examined perfusion-related parameters. CEUS demonstrated that BV treatment arrested the increase in blood volume in the SK-NEP-1 tumor group only. Molecular imaging of α(V)ß(3) with targeted microbubbles was a more sensitive prognostic indicator of BV efficacy. CEUS using RGD-labeled microbubbles showed a robust decrease in α(V)ß(3) vasculature following BV treatment in SK-NEP-1 tumors. Paralleling these findings, lectin perfusion assays detected a disproportionate pruning of smaller, branch vessels. Therefore, we conclude that the response to BV can be identified soon after initiation of treatment, often within 3 days, by use of CEUS molecular imaging techniques. The use of a noninvasive ultrasound approach may allow for earlier and more effective determination of efficacy of antiangiogenic therapy.

Hepatic pulmonary fusion in an infant with a right-sided congenital diaphragmatic hernia and contralateral mediastinal shift.

Hepatic pulmonary fusion is extremely rare with only 9 previous cases reported in the literature. In typical cases, the clinician should be alerted to the possibility of hepatic pulmonary fusion if the chest radiograph shows a large opacity on the right side without a contralateral mediastinal shift. The authors present a case of right-sided diaphragmatic hernia and hepatic pulmonary fusion with associated contralateral mediastinal shift discovered beyond the neonatal period. The 9 previous cases were retrospectively reviewed with special attention to mediastinal shift on preoperative chest radiograph, operative procedure, and mortality. Only one previous case demonstrated a contralateral mediastinal shift. The most common procedure performed was partial separation of the hepatic pulmonary fusion and approximation of the diaphragmatic defect. Four of the previous 9 patients died. In our case, reduction of bowel and approximation of the diaphragmatic defect around the fused liver and lung have been successful.