Reliability assessment of the OMERACT whole-body magnetic resonance imaging scoring system for juvenile idiopathic arthritis.

Inter-reader reliability of a new scoring system for evaluating joint inflammation and enthesitis in whole body MRI (WBMRI) in juvenile idiopathic arthritis was tested. The scoring system grades 732 item-region combinations of bone marrow and soft tissue changes for commonly involved joints and entheseal sites. Five radiologists rated 17 WBMRI scans through an online rating platform. Item-wise reliability was calculated for 117 items with non-zero scores in >10 % of readings. Interquartile ranges of the five-reader Kappa reliability coefficients were 0.58-0.73 (range: 0.36-0.88) for the joints, 0.65-0.81 (range: 0.39-0.95) for the entheses, and 0.62-0.75 (range: 0.60-0.76) for chronic nonbacterial osteomyelitis-like lesions.

MR Enterography Scores Correlate with Degree of Mucosal Healing in Pediatric Crohn's Disease: A Pilot Study.

Objectives: MR enterography (MRE) Index of Activity (MaRIA) and Clermont are validated scores that correlate with Crohn's disease (CD) activity; however, the Clermont score has not been validated to correlate with the degree of change in mucosal inflammation post induction treatment in children. This pilot study evaluated if MaRIA and Clermont scores can serve as surrogates to ileocolonoscopy for assessing interval change in mucosal inflammation in pediatric CD post-induction treatment. Methods: Children with known or newly diagnosed ileocolonic CD starting or changing therapy underwent ileocolonoscopy, scored with simple endoscopic score for Crohn's disease (SES-CD), and MRE on the same day at two time points (Week 0 and 12). Accuracy of global MaRIA and Clermont indices relative to ileocolonoscopy in detecting degree of post-treatment interval change in mucosal inflammation was assessed through correlational coefficients (r). Inter-reader agreement was calculated for imaging scores through intraclass correlation (ICC). Results: Sixteen children (mean age 11.5 ± 2.8) were evaluated. Global MaRIA/Clermont correlated with SES-CD in detecting the degree of change in mucosal inflammation (r = 0.676 and r = 0.677, P < 0.005, respectively). Correlation for pooled timepoint assessments between SES-CD and global MaRIA/Clermont was moderate (r = 0.546, P < 0.001 and r = 0.582, P < 0.001, respectively). Inter-rater reliability for global MaRIA and Clermont was good (ICC = 0.809 and ICC = 0.768, respectively, P < 0.001). Conclusions: MRE-based global scores correlate with endoscopic indices and may be used to monitor disease changes in children with CD undergoing induction treatment, which can advise the physician if treatment changes should be made.

Are 2 Radiographic Views As Good as 3 Views to Diagnose Ankle Fractures in Children and Adolescents?

OBJECTIVES: Ankle radiographs are among the most commonly obtained trauma images in the pediatric population, with the standard 3 views (AP/mortise [M]/lateral [L]) routinely ordered in the emergency department. The purpose of this study was to compare the diagnostic accuracy, sensitivity, and specificity of sets of 2 views (AP/L or M/L) with the standard 3 views. METHODS: One hundred twenty sets of ankle radiographs of skeletally immature patients obtained in the emergency department of a level 1 pediatric trauma center were used. These included sets with and without fractures. Sets of 3 and 2 views were reviewed by pairs of pediatric-trained orthopedic surgeons, radiologists, and emergency physicians. Each completed 3 randomized viewing sessions where all possible combinations for each set of radiographs were reviewed. Diagnoses for the 3 sets of views were compared for accuracy, sensitivity, and specificity. RESULTS: Overall accuracy, sensitivity, and specificity for all reviewers were as follows: AP/M/L 74%, 94%, and 90%, AP/L 71%, 90%, and 94%, as well as M/L 69%, 90%, and 91%. P values for accuracy, sensitivity, and specificity of AP/L and M/L compared with 3 views were 0.34, 0.04, and 0.52, as well as 0.04, 0.004, and 1.00, respectively. CONCLUSIONS: In skeletally immature patients, statistically significant differences in accuracy were obtained when comparing the standard 3 AP/M/L views with more limited M/L views, suggesting that this set of 2 views is not as accurate. Differences in sensitivity of limited views were also statistically significant. Conversely, differences in accuracy between the standard 3 views and AP/Lateral views were not statistically significant. While more limited AP/L views may be comparable in accuracy and specificity and lead to dramatically decreased radiation and costs, this can be at the expense of less diagnostic sensitivity and increased risk of misdiagnosing or missing certain fractures. LEVEL OF EVIDENCE: Level III.

Predicting Maximal Costal Cartilage Graft Size for Laryngotracheal Reconstruction.

OBJECTIVES/HYPOTHESIS: Current methods of assessing rib cartilage dimensions for laryngotracheal reconstruction (LTR) are inexact, making surgical planning difficult. The purpose of this study was to determine the most appropriate rib for costal cartilage graft LTR to minimize the number of ribs harvested and improve surgical outcomes. STUDY DESIGN: Retrospective review. METHODS: Computed tomography imaging of chest scans in 25 children aged 1 to 18 years was evaluated. The lengths and widths of medial and lateral cartilaginous segments of ribs 4 to 7 were measured bilaterally. Right and left cartilaginous rib dimensions were compared using a two-sample t-test. Linear mixed-effect regression was performed to develop models quantifying the relationship between rib size and patient height, rib side, and rib number. RESULTS: Regression analysis established strong models for medial rib length (R2  = 0.89) and for medial and lateral rib width (R2  = 0.71, 0.77, respectively). There was no difference in rib dimensions across chest sides. Rib length and width increased with child height. Total cartilaginous rib length increased from superiorly to inferiorly, primarily due to an increase in the dimensions of the medial portion of each rib. CONCLUSION: Cartilaginous rib lengths and widths were associated with patient height, with taller children having longer ribs. Inferior ribs were longer than superior ribs, suggesting that inferior ribs may be preferred for LTR. There was no difference in cartilaginous rib length across chest side. Results may help surgeons with preoperative planning. LEVEL OF EVIDENCE: NA Laryngoscope, 132:1682-1686, 2022.

Consensus-driven conceptual development of a standardized whole body-MRI scoring system for assessment of disease activity in juvenile idiopathic arthritis: MRI in JIA OMERACT working group.

OBJECTIVES: Whole body-MRI is helpful in directing diagnostic and treatment approaches, and as a research outcome measure. We describe our initial consensus-driven phase towards developing a whole body-MRI scoring system for juvenile idiopathic arthritis. METHODS: An iterative approach using three rounds of anonymous Delphi surveys followed by a consensus meeting was used to draft the structure of the whole body-MRI scoring system, including the relevant anatomic joints and entheses for assessment, diagnostic item selection, definition and grading, and selection of appropriate MRI planes and sequences. The surveys were completed independently by an international expert group consisting of pediatric radiologists and rheumatologists. RESULTS: Twenty-two experts participated in at least one of three rounds of Delphi surveys and a concluding consensus meeting. A first iteration scoring system was developed which ultimately included the assessment of 100 peripheral, 23 chest, and 76 axial joints, and 64 entheses, with 2-4 diagnostic items graded in each of the items, using binary (presence/absence) and 2-3-level ordinal scores. Recommendations on anatomic MRI planes and sequences were specified as the minimally necessary imaging protocol for the scoring system. CONCLUSION: A novel whole body-MRI scoring system for juvenile idiopathic arthritis was developed by consensus among members of MRI in JIA OMERACT working group. Further iterative refinements, reliability testing, and responsiveness are warranted in upcoming studies.

Toward Developing a Semiquantitative Whole Body-MRI Scoring for Juvenile Idiopathic Arthritis: Critical Appraisal of the State of the Art, Challenges, and Opportunities.

With powerful new therapies available for management of juvenile idiopathic arthritis (JIA), early diagnosis leading to appropriate treatment may prevent long-term structural joint damage. Although magnetic resonance imaging (MRI) is typically used to assess individual body parts, indications for whole body (WB) MRI are increasing. Its utility as a diagnostic and monitoring tool has already been widely investigated in adult rheumatology patients, but less so in pediatric rheumatologic patients. This paper is a comprehensive review of scoring systems and a proposal for the conceptual development of a WB-MRI scoring system for the evaluation of JIA. In this review we identify, summarize, and critically appraise the available literature on the use of WB-MRI in inflammatory arthritis, addressing relevant considerations on components of a classification system that can lead to the development of a future pediatric WB-MRI scoring system for use in children with JIA. We also discuss advantages and challenges of developing such a WB-MRI scoring system for assessment of JIA and outline next steps toward the conceptual development of this scoring system.

Comparison of the diagnostic performance of the 2017 ACR TI-RADS guideline to the Kwak guideline in children with thyroid nodules.

BACKGROUND: The Kwak Thyroid Imaging Reporting and Data System (Kwak-TI-RADS) guideline (2011) and American College of Radiology Thyroid Imaging Reporting and Data System (ACR TI-RADS) guideline (2017) were developed as ultrasound (US) risk stratification tools for detecting thyroid malignancy in adults. OBJECTIVE: The purpose of this study was to investigate the inter-rater reliability and diagnostic performance of the ACR TI-RADS guideline in the pediatric population and compare it to the Kwak guideline. MATERIALS AND METHODS: This retrospective study comprised 75 children who underwent thyroid US at a tertiary-level pediatric hospital. Three pediatric radiologists and one pediatric radiology fellow graded the US findings using the Kwak-TI-RADS and ACR TI-RADS guidelines. We assessed reliability of radiologists' ratings using percentage inter-rater agreement, and intra-class correlation coefficients (ICC2,1). We assessed area-under-the-receiver-operating-characteristic curve (AUROCC) to compare the discriminative diagnostic ability of the Kwak-TI-RADS and ACR TI-RADS scoring systems against histopathology/cytology, or stability on US over a 2-year follow-up period for cases without tissue diagnosis. RESULTS: The inter-rater agreement was significantly better for the ACR TI-RADS level compared to the Kwak-TI-RADS level (P<0.001) using the percentage pairwise agreement. The ROC curves for assessing the diagnostic performance of the two methods showed no significant difference between the methods. The AUROCCs for the Kwak-TI-RADS and ACR TI-RADS levels were 0.74 (95% confidence interval [CI] 0.67-0.82) and 0.72 (95% CI 0.61-0.82), respectively. CONCLUSION: Both the Kwak-TI-RADS and ACR TI-RADS guidelines provide moderate malignancy risk stratification for thyroid nodules in the pediatric population, with better inter-rater agreement for the ACR TI-RADS guideline. Further work to adjust the recommendations for pediatric patients is necessary.

Imaging findings of sterile pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome: differential diagnosis and review of the literature.

Pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome is a rare autosomal-dominant autoinflammatory disease of incomplete penetrance and variable expression. PAPA syndrome is the result of a mutation in the proline serine threonine phosphatase-interacting protein 1 (PSTPIP1/CD2BP1) gene located on chromosome 15, which results in an abnormal overproduction of the pro-inflammatory cytokine interleukin-1ß (IL-1). This syndrome clinically manifests as early onset of recurrent episodes of acute aseptic inflammation of the joints, generally occurring in the first two decades of life, followed by manifestation of characteristic skin lesions in the third decade, after an obvious decline in the joint symptoms. Although uncommon, the potential clinical implications of PAPA syndrome warrant an appropriate diagnosis in a timely fashion.

Infant nephrolithiasis and nephrocalcinosis: Natural history and predictors of surgical intervention.

INTRODUCTION: Renal stone disease diagnosed in the first year of life is relatively uncommon. While risk factors such as low birth weight, furosemide exposure, and metabolic disorders are well established, there exists little information regarding resolution rates and need for surgical intervention. Our study objective was to evaluate urolithiasis and renal calcification resolution rates, time to resolution, and need for surgical intervention in children diagnosed in their first year of life. MATERIAL AND METHODS: REB approved retrospective chart review of children younger than 12 months of age (corrected for prematurity) diagnosed with nephrolithiasis and/or nephrocalcinosis in a tertiary pediatric hospital between April 2000 and August 2015 with a minimum 1-year follow-up period. Exact logistic regression was performed to assess the relationship between size of the largest stone (on either side) and the need for surgical intervention. Kaplan-Meier curves were constructed to examine time to stone resolution among those not requiring surgical intervention. RESULTS: 62 patients (61% male) were diagnosed with stones or nephrocalcinosis by ultrasound at a median age of 2.9 months. Of these, 37% had been admitted to the NICU because of prematurity, low birth weight or comorbidities. A total of 45 patients were found to have stones (Table); 35 of these had a stone at initial ultrasound and 10 initially diagnosed as nephrocalcinosis were later confirmed to have a stone. 67% of all stones were asymptomatic on presentation. Metabolic anomalies were present in 56% (35/62), and 16% (10/62) required medical treatment. Seven patients ultimately required surgical intervention. Stone size was found to predict the eventual need for surgical intervention (OR 3.52, 95% CI 1.47-12.78) for each 0.1 mm increase in diameter). Among patients not requiring surgical intervention (n = 38), the estimated median time to spontaneous resolution of urolithiasis was 1.1 years (95% CI 0.89-1.53, range 2 months-6 years) and 1.2 years for nephrocalcinosis (95% CI 0.59-2.13). CONCLUSIONS: Spontaneous resolution was a common outcome for newborns and infants diagnosed with urolithiasis in the first year of life, but high variability in time-to-resolution was observed. Only a small proportion who had confirmed stones on ultrasound required surgical intervention (15%), and large stone size was a predictive factor for surgery.

Synovial osteochondromatosis in a 14-year-old boy with a history of Legg-Calve-Perthes disease.

We describe a case of a 14-year-old boy with a history of Legg-Calve-Perthes disease diagnosed at the age of 6 years and development of synovial osteochondromatosis of the same hip joint 7 years later. Synovial osteochondromatosis is very rare in children, and to the best of our knowledge, only a single case of Legg-Calve-Perthes disease and secondary synovial osteochondromatosis was described in the literature in a 35-year-old male, making this the first reported case of Legg-Calve-Perthes disease with development of synovial osteochondromatosis in a pediatric patient.