Treatment practices and response in kaposiform hemangioendothelioma: A multicenter cohort study.

BACKGROUND AND OBJECTIVES: Kaposiform hemangioendothelioma (KHE) and tufted angioma (TA) are rare vascular tumors in children historically associated with significant morbidity and mortality. This study was conducted to determine first-line therapy in the absence of available prospective clinical trials. METHODS: Patients from 17 institutions diagnosed with KHE/TA between 2005 and 2020 with more than 6 months of follow-up were included. Response rates to sirolimus and vincristine were compared at 3 and 6 months. Durability of response and response to other treatment modalities were also evaluated. RESULTS: Of 159 unique KHE/TA subjects, Kasabach-Merritt phenomenon (KMP) was present in 64 (40.3%), and only two patients were deceased (1.3%). Over 60% (n = 96) demonstrated treatment response at 3 months, and more than 70% (n = 114) by 6 months (no significant difference across groups). The vincristine group had higher radiologic response at 3 months compared to sirolimus (72.7% vs. 20%, p = .03), but there were no differences between these groups at 6 months. There were no differences in rates of recurrent or progressive disease between vincristine and sirolimus. CONCLUSIONS: In this large, multicenter cohort of 159 patients with KHE/TA, rates of KMP were consistent with historical literature, but the mortality rate (1.3%) was much lower. Overall treatment response rates were high (>70%), and there was no significant difference in treatment response or durability of disease comparing sirolimus to vincristine. Our results support individualized treatment decision plans depending on clinical scenario and patient/physician preferences. Response criteria and response rates reported here will be useful for guiding future treatment protocols for vascular tumors.

RADIAL: leveraging a learning management system to support radiology education.

Increasing volume and complexity of cases in academic radiology and the drive toward pediatric sub-specialization have threatened knowledge assimilation for radiologists. There is a clear need for a system that retrieves vetted information from the excess available on the internet. Partnered with an interdisciplinary team from the Graduate School of Education, the authors created the first comprehensive learning management system (LMS) for radiology, implemented in the reading room to augment image interpretation and point-of-care education. The LMS supports quantitative analysis using a robust analytics platform to evaluate user statistics, facilitating improved quality of patient care by revolutionizing the way radiologists assimilate knowledge. This integration promises to enhance workflow and point-of-care teaching and to support the highest quality of care.

Pediatric magnetic resonance imaging training versus job-readiness: using education research tools to re-align.

BACKGROUND: Fellows begin MRI training with variable experience and expertise. To better serve patients, pediatric radiology fellows should gain competence in MRI that enables seamless transition to independent practice. OBJECTIVE: We implemented a needs assessment survey and conducted a focus group discussion to identify knowledge gaps and inform creation of a curriculum for pediatric body MRI. MATERIALS AND METHODS: We electronically distributed a comprehensive anonymous needs assessment survey in October 2016 to current fellows and recent (<5 years) graduates from Accreditation Council for Graduate Medical Education (ACGME)-accredited pediatric radiology fellowships, with follow-up in January 2017. We conducted a focus group discussion among current fellows at our institution in October 2017 to inform a better understanding of the results. RESULTS: Eighty-one pediatric radiologists (8 fellows/73 attendings) completed the survey (24%); 5 current fellows participated in the focus group. The technical issues most commonly identified with limited or no instruction during training included setting up an MR service, accessory equipment (coil) selection and MRI field inhomogeneity correction. Areas needing increased attention and inclusion within the curriculum included coil choice/patient positioning (n=42, 52%), contrast agents (n=40, 49%), field strength (n=33, 41%) and strategies for motion correction (n=33, 41%). Most fellows were uncomfortable with setting up an MR service (n=57, 70%), correcting field inhomogeneity (n=56, 69%) and improving image quality (n=50, 62%). The focus group showed consensus that there was insufficient MR training in residency to prepare them for fellowship. The group also preferred shorter lectures and streaming via video education/tutorials. CONCLUSION: While traditional instruction emphasizes image interpretation, trainees in pediatric radiology need a curriculum that also emphasizes technical and non-interpretive aspects of MRI.

Diagnostic performance and role of the contrast enema for low intestinal obstruction in neonates.

PURPOSE: We aim to evaluate the diagnostic performance and relationship between clinical characteristics, imaging findings, and final diagnosis for the neonatal contrast enema (CE). METHODS: Retrospective 10-year review of all neonatal CEs including imaging findings, clinical information, indication, and final diagnosis from discharge summaries, surgical reports, and pathology (reference standard). Two blinded pediatric radiologists reinterpreted 366 CEs for obstruction, microcolon, rectosigmoid index (RSI), serrations, meconium, ileal cut-off, transition zone, diagnosis, and level of confidence. CE diagnostic performance was calculated versus reference standard. RESULTS: Diagnoses included Hirschsprung disease (HD) (15.8%), small left colon syndrome (14.8%), small intestinal atresia/colonic atresia (SIA/CA) (12.6%), meconium ileus (MI) (4.4%), and normal (48.9%). CE had a moderate specificity (87.7%) and low sensitivity (65.5%) for HD; abnormal RSI and serrations showed high specificities (90.3%, 97.4%) but low sensitivities (46.6%, 17.2%). CE showed high specificity (97.4%) and low sensitivity (56.3%) for MI blinded to cystic fibrosis status. Microcolon was specific (96.6%) but not sensitive (68.8%) for MI. CE showed highest PPV (73.1%) (specificity 95.6%, sensitivity 82.6%) for SIA/CA. Microcolon with an abrupt cut-off was specific (99.1%) but not sensitive (41.3%) for atresias. CONCLUSION: Neonatal CE demonstrates high specificities and low to moderate sensitivities across all diagnoses, with lowest performance in HD. CLINICAL TRIAL REGISTRATION: None.