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Background Pediatric patients with liver disease require noninvasive monitoring to evaluate the risk of fibrosis progression. This study aimed to identify the significant factors affecting liver stiffness values using two-dimensional shear-wave elastography (2D-SWE), and determine whether liver stiffness can predict the fibrosis stage of various childhood liver diseases. Methods This study included 30 children (22 boys and 8 girls; mean age, 5.1 ± 6.1 years; range, 7 days–17.9 years) who had undergone biochemical evaluation, 2D-SWE examination, histopathologic analysis of fibrosis grade (F0 to F3), assessment of necroinflammatory activity, and steatosis grading between August 2016 and March 2020. The liver stiffness from 2D-SWE was compared between fibrosis stages using Kruskal–Wallis analysis. Factors that significantly affected liver stiffness were evaluated using univariate and multivariate linear regression analyses. The diagnostic performance was determined from the area under the receiver operating curve (AUC) values of 2D-SWE liver stiffness. Results Liver stiffness at the F0-1, F2, and F3 stages were 7.9, 13.2, and 21.7 kPa, respectively (P P P = 0.021, respectively). However, in patients with alanine aminotransferase (ALT) levels below 200 IU/L, the only factor affecting liver stiffness was fibrosis stage (P = 0.030). The liver stiffness value could distinguish significant fibrosis (≥ F2) with an AUC of 0.950 (cutoff value, 11.3 kPa) and severe fibrosis (F3 stage) with an AUC of 0.924 (cutoff value, 18.1 kPa). The 2D-SWE values for differentiating significant fibrosis were 10.5 kPa (≥ F2) and 18.1 kPa (F3) in patients with ALT levels below 200 IU/L. Conclusion The liver stiffness values on 2D-SWE can be affected by both fibrosis and necroinflammatory grade and can provide excellent diagnostic performance in evaluating the fibrosis stage in various pediatric liver diseases. However, clinicians should be mindful of potential confounders, such as necroinflammatory activity or transaminase level, when performing 2D-SWE measurements for liver fibrosis staging.

Background While ETV6- NTRK3 fusion is common in infantile fibrosarcoma, NTRK1/3 fusion in pediatric tumors is scarce and, consequently, not well known. Herein, we evaluated for the presence of NTRK1/3 fusion in pediatric mesenchymal tumors, clinicopathologically and immunophenotypically. Methods We reviewed nine NTRK fusion-positive pediatric sarcomas confirmed by fluorescence in situ hybridization and/or next-generation sequencing from Seoul National University Hospital between 2002 and 2020. Results One case of TPR-NTRK1 fusion-positive intracranial, extra-axial, high-grade undifferentiated sarcoma (12-year-old boy), one case of LMNA-NTRK1 fusion-positive low-grade infantile fibrosarcoma of the forehead (3-year-old boy), one case of ETV6-NTRK3 fusion-positive inflammatory myofibroblastic tumor (IMT) (3-months-old girl), and six cases of ETV6-NTRK3 fusion-positive infantile fibrosarcoma (median age: 2.6 months, range: 1.6–5.6 months, M: F = 5:1) were reviewed. The Trk immunopositivity patterns were distinct, depending on what fusion genes were present. We observed nuclear positivity in TPR-NTRK1 fusion-positive sarcoma, nuclear membrane positivity in LMNA-NTRK1 fusion-positive sarcoma, and both cytoplasmic and nuclear positivity in ETV6-NTRK3 fusion-positive IMT and infantile fibrosarcomas. Also, the TPR-NTRK1 fusion-positive sarcoma showed robust positivity for CD34/nestin, and also showed high mitotic rate. The LMNA-NTRK1 fusion-positive sarcoma revealed CD34/S100 protein/nestin/CD10 coexpression, and a low mitotic rate. The IMT with ETV6-NTRK3 fusion expressed SMA. Six infantile fibrosarcomas with ETV6-NTRK3 fusion showed variable coexpression of nestin (6/6)/CD10 (4/5)/ S100 protein (3/6). Conclusions All cases of NTRK1 and NTRK3 fusion-positive pediatric tumors robustly expressed the Trk protein. A Trk immunopositive pattern and CD34/S100/nestin/CD10/SMA immunohistochemical expression may suggest the presence of NTRK fusion partner genes. LMNA-NTRK1 fusion sarcoma might be a low-grade subtype of infantile fibrosarcoma. Interestingly, more than half of the infantile fibrosarcoma cases were positive for S100 protein and CD10. The follow-up period of TPR-NTRK1 and LMNA-NTRK1 fusion-positive tumors are not enough to predict prognosis. However, ETV6-NTRK3 fusion-positive infantile fibrosarcomas showed an excellent prognosis with no evidence of disease for an average of 11.7 years, after gross total resection of the tumor.

This study aimed to evaluate a deep learning model for generating synthetic contrast-enhanced CT (sCECT) from non-contrast chest CT (NCCT). A deep learning model was applied to generate sCECT from NCCT. We collected three separate data sets, the development set (n = 25) for model training and tuning, test set 1 (n = 25) for technical evaluation, and test set 2 (n = 12) for clinical utility evaluation. In test set 1, image similarity metrics were calculated. In test set 2, the lesion contrast-to-noise ratio of the mediastinal lymph nodes was measured, and an observer study was conducted to compare lesion conspicuity. Comparisons were performed using the paired t-test or Wilcoxon signed-rank test. In test set 1, sCECT showed a lower mean absolute error (41.72 vs 48.74; P P P P P P ≤ .001). Synthetic CECT generated from NCCT improves the depiction of mediastinal lymph nodes.

Objectives The diagnostic role of an ultrasound (US) and plasma renin activity (PRA) combination is unknown, despite the usefulness of Doppler US as a screening tool for renovascular hypertension (RVHT). The purpose of this study was to evaluate the diagnostic usefulness of US for RVHT in children stratified according to PRA. Methods We identified 336 children who underwent renal Doppler US examinations for hypertension and divided them into a high-renin group (n = 177) and a normal-renin group (n = 159) based on PRA and stratified them by age. The Doppler US findings were retrospectively reviewed, and computed tomographic angiography (CTA) for the same children was used as the reference standard. Results In the high-renin group, 36 patients had positive Doppler US findings that were confirmed by CTA in 32 cases. The sensitivity and specificity values for Doppler US in the high-renin group were 84.2% and 93.6%, respectively. In the normal-renin group, 10 patients had positive Doppler US findings; these positive findings were confirmed by CTA in 9 cases. The sensitivity and specificity values for US in the normal-renin group were 100.0% and 100.0%. There were anatomic variations (n = 3) and segmental artery stenosis (n = 2) among the cases with false-negative US findings, which were confirmed by CTA. Conclusions If patients have high PRA, a Doppler US examination should be performed with caution to avoid false-negative detection. If patients have normal PRA, renal Doppler US might be adequate for diagnosis of RVHT to avoid unnecessary CTA.

Background: The guideline for managing pediatric thyroid nodules has not been established. We investigated the appropriateness of the Korean Society of Thyroid Radiology (KSThR) guidelines and the ...

Objective To describe the anatomic locations and imaging features of posterior lung herniation in unilateral pulmonary agenesis and aplasia, focusing on radiograph-CT/MRI correlation. Materials and methods A total of 10 patients (seven with pulmonary agenesis and three with pulmonary aplasia, male: female = 1:9, mean age 7.3 years, age range from 1 month to 20 years) were included. Chest radiographs (n = 9), CT (n = 9), and MRI (n = 1) were reviewed to assess the type of lung underdevelopment, presence of anterior and posterior lung herniation, bronchus origin, supplying artery, and draining vein of the herniated lung. Results Pulmonary agenesis/aplasia more commonly affected the left lung (n = 7) than the right lung (n = 3). Anterior lung herniation was observed in nine of the 10 patients. Posterior lung herniation was observed in seven patients with left pulmonary agenesis/aplasia. Two patients showed posterior lung herniation crossing the midline but not beyond the aorta, and five patients showed the posteriorly herniated right lower lobe crossing the midline to extend into the left hemithorax farther beyond the descending thoracic aorta through the space between the esophagus and the aorta. This anatomical configuration resulted in a characteristic radiographic finding of a radiolucent area with a convex lateral border and a vertical medial border in the left lower lung zone, revealing a tongue-like projection on CT and MRI. Conclusion Posterior lung herniation occurs in unilateral left lung agenesis/aplasia. Approximately 70% of the cases of posterior lung herniation reveal a unique radiolucent tongue-like projection in the left lower lung zone on imaging studies, which is caused by the extension of the posteriorly herniated right lung farther beyond the descending aorta.

Purpose The purpose of this study was to identify imaging risk factors on contrast-enhanced hip MRI after closed reduction of developmental dysplasia of the hip (DDH) that could predict future development of avascular necrosis (AVN) of the femoral head. Materials and methods Fifty-eight infants (F: M = 53: 5, aged 3–18 months) who underwent immediate postoperative contrast-enhanced hip MRI after closed reduction of DDH were included in this study. Quality of reduction (concentric vs eccentric reduction with or without obstacles), abduction angle of the hip, presence of ossific nucleus, and pattern of contrast enhancement of the femoral head were retrospectively evaluated on MRI. Interobserver agreement of contrast enhancement pattern on MRI were evaluated by two radiologists. Development of AVN was determined through radiographic findings at 1 year after reduction. Results AVN of the femoral head developed in 13 (22%) of 58 patients. Excessive abduction of the hip joint (OR 4.65, [95% CI 1.20, 18.06] and global decreased enhancement of the femoral head (OR 71.66, [95% CI 10.54, 487.31]) exhibited statistically significant differences between the AVN and non-AVN groups (P < 0.05). Eccentric reduction (P = 0.320) did not show statistically significant difference between two groups and invisible ossific nucleus (P = 0.05) showed borderline significance. Multi-variable logistic regression indicated that global decreased enhancement of the femoral head was a significant risk factor of AVN (OR 27.92, 95% CI [4.17, 350.18]) (P = 0.0031). Interobserver agreement of contrast enhancement pattern analysis and diagnosis of AVN were good (0.66, 95% CI [0.52, 0.80]). Conclusion Contrast-enhanced hip MRI provides accurate anatomical assessment of the hip after closed reduction of DDH. Global decreased enhancement of the femoral head could be used as a good predictor for future development of AVN after closed reduction of DDH.

We investigated the feasibility of free-breathing modified Look-Locker inversion recovery (MOLLI) sequence for measuring hepatic T1 values in children and young adults. To investigate the accuracy and the reproducibility of the T1 maps, a phantom study was performed with 12 different gadoterate meglumine concentrations and the T1 relaxation times of phantoms measured with the MOLLI sequence were compared against those measured with three different sequences: spin-echo inversion recovery, variable flip angle (VFA), and VFA with B1 correction. To evaluate the feasibility of free-breathing MOLLI sequence, hepatic T1 relaxation times obtained by free-breathing and breath-hold technique in twenty patients were compared. The phantom study revealed the excellent accuracy and reproducibility of MOLLI. In twenty patients, the mean value of hepatic T1 values obtained by free-breathing (606.7 ± 64.5 ms) and breath-hold (609.8 ± 64.0 ms) techniques showed no significant difference (p > 0.05). The Bland–Altman plot between the free-breathing and breath-hold revealed that the mean difference of T1 values was − 3.0 ms (− 0.5%). Therefore, T1 relaxation times obtained by MOLLI were comparable to the values obtained using the standard inversion recovery method. The hepatic T1 relaxation times measured by MOLLI technique with free-breathing were comparable to those obtained with breath-hold in children and young adults.

BACKGROUND. Hippocampal sclerosis (HS) is a leading cause of medically refractory temporal lobe epilepsy in children. The diagnosis is clinically important because most patients with HS have good p...

To evaluate the image quality of low iodine concentration, dual-energy CT (DECT) combined with a deep learning-based noise reduction technique for pediatric abdominal CT, compared with standard iodine concentration single-energy polychromatic CT (SECT).From December 2016 to May 2017, DECT with 300 mg•I/mL contrast medium was performed in 29 pediatric patients (17 boys, 12 girls; age, 2-19 years). The DECT images were reconstructed using a noise-optimized virtual monoenergetic reconstruction image (VMI) with and without a deep learning method. SECT images with 350 mg•I/mL contrast medium, performed within the last 3 months before the DECT, served as reference images. The quantitative and qualitative parameters were compared using paired t tests and Wilcoxon signed-rank tests, and the differences in radiation dose and total iodine administration were assessed.The linearly blended DECT showed lower attenuation and higher noise than SECT. The 60-keV VMI showed an increase in attenuation and higher noise than SECT. The combined 60-keV VMI plus deep learning images showed low noise, no difference in contrast-to-noise ratios, and overall image quality or diagnostic image quality, but showed a higher signal-to-noise ratio in the liver and lower enhancement of lesions than SECT. The overall image and diagnostic quality of lesions were maintained on the combined noise reduction approach. The CT dose index volume and total iodine administration in DECT were respectively 19.6% and 14.3% lower than those in SECT.Low iodine concentration DECT, combined with deep learning in pediatric abdominal CT, can maintain image quality while reducing the radiation dose and iodine load, compared with standard SECT.• An image noise reduction approach combining deep learning and noise-optimized virtual monoenergetic image reconstruction can maintain image quality while reducing radiation dose and iodine load. • The 60-keV virtual monoenergetic image reconstruction plus deep learning images showed low noise, no difference in contrast-to-noise ratio, and overall image quality, but showed a higher signal-to-noise ratio in the liver and a lower enhancement of lesion than single-energy polychromatic CT. • This combination could offer a 19.6% reduction in radiation dose and a 14.3% reduction in iodine load, in comparison with a control group that underwent single-energy polychromatic CT with the standard protocol.

Background The image quality of abdominal magnetic resonance imaging (MRI) in children who cannot hold their breath has been severely impaired by motion artifacts. Purpose To evaluate the usefulness of T1-weighted (T1W) BLADE MRI for axial abdominal imaging in children who cannot hold their breath. Material and Methods Two different BLADE sequences, with and without an inversion recovery (IR-BLADE), were compared to conventional turbo-spin echo (TSE) with a high number of excitations in 18 consecutive patients who cannot hold their breath. Overall image quality, motion artifact, radial artifact, hepatic vessel sharpness, renal corticomedullary differentiation, and lesion conspicuity were retrospectively assessed by two radiologists, using 4- or 5-point scoring systems. Signal variations of each sequence were measured for a quantitative comparison. The acquisition times of the three sequences were compared. Results IR-BLADE and BLADE showed significantly improved overall image quality and reduced motion artifact compared with TSE. IR-BLADE showed significantly better hepatic vessel sharpness and corticomedullary differentiation compared to both BLADE and TSE. Radial artifacts were only observed on IR-BLADE and BLADE. In nine patients with lesions, there were no significant differences in lesion conspicuity among three sequences. Compared to TSE, both IR-BLADE and BLADE showed decreased signal variations in the liver and muscle, and an increased signal variation through air. The mean acquisition times for IR-BLADE, BLADE, and TSE were comparable. Conclusion Compared to the TSE sequence, T1W IR-BLADE for pediatric abdominal MRI resulted in improved image quality, tissue contrast with a diminished respiratory motion artifact, and a comparable acquisition time.

To develop and evaluate a deep learning-based artificial intelligence (AI) model for detecting skull fractures on plain radiographs in children.This retrospective multi-center study consisted of a development dataset acquired from two hospitals (n = 149 and 264) and an external test set (n = 95) from a third hospital. Datasets included children with head trauma who underwent both skull radiography and cranial computed tomography (CT). The development dataset was split into training, tuning, and internal test sets in a ratio of 7:1:2. The reference standard for skull fracture was cranial CT. Two radiology residents, a pediatric radiologist, and two emergency physicians participated in a two-session observer study on an external test set with and without AI assistance. We obtained the area under the receiver operating characteristic curve (AUROC), sensitivity, and specificity along with their 95% confidence intervals (CIs).The AI model showed an AUROC of 0.922 (95% CI, 0.842-0.969) in the internal test set and 0.870 (95% CI, 0.785-0.930) in the external test set. The model had a sensitivity of 81.1% (95% CI, 64.8%-92.0%) and specificity of 91.3% (95% CI, 79.2%-97.6%) for the internal test set and 78.9% (95% CI, 54.4%-93.9%) and 88.2% (95% CI, 78.7%-94.4%), respectively, for the external test set. With the model's assistance, significant AUROC improvement was observed in radiology residents (pooled results) and emergency physicians (pooled results) with the difference from reading without AI assistance of 0.094 (95% CI, 0.020-0.168;A deep learning-based AI model improved the performance of inexperienced radiologists and emergency physicians in diagnosing pediatric skull fractures on plain radiographs.

Toxicity of carmustine and cyclophosphamide can cause pulmonary injury after hematopoietic stem cell transplantation. To evaluate the radiologic findings of pulmonary injuries following carmustine- and cyclophosphamide-based preparative regimens in children. From 2010 to 2014, 35 children received carmustine- and cyclophosphamide-based preparative regimens. Fourteen of 35 children presented with symptoms and radiologic abnormalities. Eight of 14 children had no evidence of infection, cardiogenic edema, or other explainable causes. We retrospectively analyzed their chest radiographs and CT scans for ground-glass opacity, consolidation, septal thickening and pleural effusion. Major chest radiographic findings were bilateral diffuse ground-glass opacity (n=8) and septal thickening (n=7). CT findings were multifocal patchy (n=4) or inhomogeneously diffuse (n=4) ground-glass opacity, multifocal consolidations (n=7) and septal thickening (n=7). All of these lesions at CT were bilateral, but showed lower lobe predominance in 88, 100, and 63%, respectively. There was no central/peripheral or anterior/posterior predilection. Six children had small pleural effusions, which were bilateral in five children. Bilateral ground-glass opacity with or without consolidation, septal thickening and pleural effusion were common radiologic findings in pulmonary injury following carmustine- and cyclophosphamide-based preparative regimens.

The aim of this study was to identify the signal intensity (SI) changes in the dentate nucleus (DN) and the globus pallidus (GP) on unenhanced T1-weighted magnetic resonance (MR) images after multiple administration of gadolinium-based contrast agents (GBCAs) in children and compare those changes between linear and macrocyclic GBCAs.This retrospective study was approved by the institutional review board and the requirement for informed consent was waived. We identified 92 children who underwent at least 4 consecutive MR examinations exclusively using either linear GBCA (gadodiamide or gadopentetate dimeglumine, n = 41) or macrocyclic GBCA (gadoterate meglumine, n = 51). Signal intensity ratio changes in the DN to pons and GP to thalamus between the first and last MR examinations were calculated.The SI ratios in the linear group increased significantly between the first and last MR examinations (mean difference: DN to pons, 0.0461 ± 0.0480, P0.001; GP to thalamus, 0.0332 ± 0.0385, P0.001), but not in the macrocyclic group (mean difference: DN to pons, -0.0010 ± 0.0371, P = 0.855; GP to thalamus, 0.0007 ± 0.0294, P = 0.867). In the linear regression analysis, the numbers of administrations of gadodiamide and gadopentetate dimeglumine were highly associated with the differences in SI ratios (DN to pons, P0.001 and P = 0.003; GP to thalamus, P0.001 and P = 0.002, respectively).The SIs of the DN and GP on unenhanced T1-weighted images increased after serial administrations of linear GBCA, but not macrocyclic GBCA, in children. The number of linear GBCA administration had a linear association with the SI changes in the DN and GP.

To evaluate the predictive value of ultrasonography in children with clinically suspicious hepatic veno-occlusive disease (VOD) after hematopoietic stem cell transplantation (HSCT). Among 216 children who underwent HSCT, 70 also underwent colour Doppler ultrasonography. Of these, 59 had only one sign/symptom, which did not fulfil the diagnostic criteria (clinical suspicion of VOD) at that time. VOD was confirmed in 20 patients (VOD group), while 39 had other conditions (non-VOD group). The following findings were reviewed and compared between groups: left portal vein (peak velocity, direction), left hepatic artery (peak-systolic/end-diastolic velocities, resistive index), middle hepatic vein (peak velocity, phasicity), hepatomegaly, splenomegaly, gallbladder wall thickness, and ascites. The VOD group showed significantly higher reversed flow in portal vein (P = 0.011), peak systolic velocity of left hepatic artery (P = 0.028), monophasicity of middle hepatic vein (P = 0.015), hepatomegaly (P = 0.001), gallbladder wall thickness (P

This study aimed to evaluate the feasibility and added value of transcatheter dynamic contrast-enhanced magnetic resonance (MR) lymphangiography for nontraumatic lymphatic disorders. Five patients (2 males and 3 females; median age, 16.0 years; range, 3-74 years) who underwent both intranodal and transcatheter dynamic contrast-enhanced MR lymphangiography for suspected nontraumatic lymphatic leakages from June 2017 to January 2020 were included in this retrospective study. The imaging findings of both dynamic contrast-enhanced MR lymphangiography techniques were assessed for the presence of chylolymphatic reflux or direct sign of leakage. Intranodal dynamic contrast-enhanced MR lymphangiography demonstrated chylolymphatic reflux into the thoracic area in 2 patients (40%) but no direct evidence of leakage in any of the 5 patients. Transcatheter dynamic contrast-enhanced MR lymphangiography revealed chylolymphatic reflux and extravasation of the contrast agent in all 5 patients (100%). In conclusion, transcatheter dynamic contrast-enhanced MR lymphangiography may reveal additional signs of reflux and extravasation even when the findings of intranodal dynamic contrast-enhanced MR lymphangiography are negative.

Computed tomography (CT) has generated public concern associated with radiation exposure, especially for children. Lowering the tube voltage is one strategy to reduce radiation dose. To assess the image quality and radiation dose of non-enhanced brain CT scans acquired at 80 kilo-voltage peak (kVp) compared to those at 120 kVp in children. Thirty children who had undergone both 80- and 120-kVp non-enhanced brain CT were enrolled. For quantitative analysis, the mean attenuation of white and gray matter, attenuation difference, noise, signal-to-noise ratio, contrast-to-noise ratio and posterior fossa artifact index were measured. For qualitative analysis, noise, gray-white matter differentiation, artifact and overall image quality were scored. Radiation doses were evaluated by CT dose index, dose-length product and effective dose. The mean attenuations of gray and white matter and contrast-to-noise ratio were significantly increased at 80 kVp, while parameters related to image noise, i.e. noise, signal-to-noise ratio and posterior fossa artifact index were higher at 80 kVp than at 120 kVp. In qualitative analysis, 80-kVp images showed improved gray-white differentiation but more artifacts compared to 120-kVp images. Subjective image noise and overall image quality scores were similar between the two scans. Radiation dose parameters were significantly lower at 80 kVp than at 120 kVp. In pediatric non-enhanced brain CT scans, a decrease in tube voltage from 120 kVp to 80 kVp resulted in improved gray-white matter contrast, comparable image quality and decreased radiation dose.

Purpose Kaposiform hemangioendothelioma (KHE) is a rare, aggressive vascular tumor that typically occurs during infancy or early childhood. Though several case reports have discussed the imaging findings of KHE, larger comprehensive studies are lacking. The purpose of this study was to evaluate the imaging findings of KHE in children. Materials and methods A total of twelve cases of pathologically proven KHE were collected by searching our institution’s pathology database for children diagnosed between January 2004 and April 2016 (6 male, 6 female; median age: 3 months; age range 7 days − 18 years). CT (n = 7) and MRI (n = 9) findings were retrospectively evaluated. The location, morphology, enhancement, and growth pattern were analyzed. Results KHEs involved various locations: superficial or deep soft tissue of the extremities (n = 4); abdomen (n = 3; 2 cases, pancreas; 1 case, small bowel), neck and mediastinum (n = 1); chest wall, diaphragm, and pericardium (n = 1); abdominal wall (n = 1); and head (IAC, CP angle) (n = 2). Eight of 10 cases (80%) with CT/MR findings were locally invasive and involved two or more adjacent organs. Three cases presented as well-defined solid masses, and seven were associated with infiltrative lesions of the surrounding areas with (n = 4) or without definite solid regions (n = 3). In nine patients with MRI, all KHEs were heterogeneous and hyperintense to muscles on T2-weighted images (T2WI), while four KHEs consisted of some regions that were nearly isointense to muscle. Eight of the 10 cases with imaging exhibited heterogeneous intense enhancement, while only one demonstrated mild enhancement. Signal voids (n = 2), engorged vessels (n = 1), calcification (n = 3), hemorrhage (n = 1), or bone changes (n = 4) were infrequently observed. Four patients (33%) had Kasabach-Merritt phenomenon, and recurrence was observed in two cases. Conclusion KHEs occurred in various locations, affected mostly infants, and generally exhibited intense heterogeneous enhancement. In more than half of the included cases, KHEs were highly infiltrative and locally invasive with ill-defined margins. Awareness of these features should prompt radiologists to include KHE in the differential diagnosis for pediatric masses.

To evaluate the clinical usefulness of dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) in children and young adults with Crohn's disease.From August 2017 to October 2018, 30 patients with Crohn's disease (21 males and 9 females; mean age 15.1 ± 2.5 years) underwent DCE-MRI with MRI enterography. We assessed the endoscopic finding, pediatric Crohn's disease activity index (PCDAI), C-reactive protein (CRP) level (mg/dL), Crohn's disease MR index (CDMI) score, and the perfusion parameters of DCE-MRI (KThe active Crohn's disease group showed higher PCDAI, CRP, and CDMI scores than the inactive group (22.2 ± 18.8 vs. 6.3 ± 6.4, p = 0.027; 1.32 ± 1.79 vs. 0.10 ± 0.13, p = 0.005; 7.4 ± 3.9 vs. 4.5 ± 3.0, p = 0.047, respectively). The active Crohn's disease group showed a higher KK• With dynamic contrast-enhanced MRI, we can quantitatively monitor the Crohn's disease status in pediatric patients and provide proper management plans to clinicians. • The K

To evaluate whether a frequency-selective non-linear blending (BC) technique can improve tissue contrast and infarct detection on non-enhanced brain CT (NECT) in postoperative Moyamoya (MMD) patients.From January 2010 to December 2017, 33 children (13boys and 20girls; mean age 9.1±3.4 years) with MMD postoperatively underwent NECT followed by diffusion MRI. We compared the contrast-to-noise ratio (CNR) between gray matter (GM) and white matter (WM) in NECT and BC images and the CNR between the infarct lesion and adjacent normal-appearing brain in NECT and BC images using a paired t-test. We assessed image noise, GM-WM differentiation, artifacts, and overall quality using a Wilcoxon signed rank test. A McNemar two-tailed test was conducted to compare the diagnostic accuracy of infarct detection.The CNR between GM and WM and the CNR of the infarct was better in BC images than in NECT images (3.9±1.0 vs. 1.8±0.6, P0.001 and 3.6±0.3 vs. 1.9±0.2, P0.001), with no difference in overall image quality observed. The sensitivity and specificity of infarct detection were 55.0% and 76.9% using NECT, and 70.0% and 69.2% using BC technique. The diagnostic accuracy of NECT and BC technique was 63.6% (21/33) and 69.7% (23/33), respectively.This study showed that the BC technique improved CNR and maintained image quality. This technique may also be used to identify ischemic brain changes in postoperative MMD patients by improving the CNR of the infarct lesion.

To evaluate the image quality of a double-low protocol (low tube voltage and low iodine concentration) for abdominal CT in children. The double-low protocol was compared to the conventional protocol in pediatric patients weighing less than 40 kg from May 2016 to December 2016. Double-low protocol (Group A, n = 18): tube voltage, 70 kVp; and iodine concentration,: 250 mgI/mL versus Conventional protocol (Group B, n = 13): tube voltage, 80–100 kVp; and iodine concentration, 350 mgI/mL. Mean attenuation, noise, signal-to-noise ratio (SNR), and contrast-to-noise ratio (CNR) were compared between the two groups. Image contrast, noise, beam-hardening artifacts, and overall image quality were subjectively scored. Reader performance for correctly differentiating two groups by visual assessment was evaluated. Radiation dose and total iodine load were recorded. The mean attenuations of the portal vein and liver and the mean image noise in Group A were higher than in Group B (p = 0.04, 0.03, 0.004, respectively). The mean SNR and CNR of the main portal vein and liver were lower in Group A without any statistically significant difference. There were no statistically significant differences between the two groups in qualitative analysis (image contrast, image noise, and overall image quality) with substantial agreement between the reviewers (weighted kappa values; 0.59–0.76). Significantly diminished radiation dose and iodine load were observed in Group A compared with Group B (25.0%, 36.8% reduction; p = 0.007, 0.006, respectively). The double-low protocol was feasible for pediatric abdominal CT and reduced both radiation dose and iodine load, while maintaining image quality.

Objective To evaluate the diagnostic performance of a deep learning algorithm for the automated detection of developmental dysplasia of the hip (DDH) on anteroposterior (AP) radiographs. Materials and Methods Of 2601 hip AP radiographs, 5076 cropped unilateral hip joint images were used to construct a dataset that was further divided into training (80%), validation (10%), or test sets (10%). Three radiologists were asked to label the hip images as normal or DDH. To investigate the diagnostic performance of the deep learning algorithm, we calculated the receiver operating characteristics (ROC), precision-recall curve (PRC) plots, sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) and compared them with the performance of radiologists with different levels of experience. Results The area under the ROC plot generated by the deep learning algorithm and radiologists was 0.988 and 0.988–0.919, respectively. The area under the PRC plot generated by the deep learning algorithm and radiologists was 0.973 and 0.618–0.958, respectively. The sensitivity, specificity, PPV, and NPV of the proposed deep learning algorithm were 98.0, 98.1, 84.5, and 99.8%, respectively. There was no significant difference in the diagnosis of DDH by the algorithm and the radiologist with experience in pediatric radiology (p = 0.180). However, the proposed model showed higher sensitivity, specificity, and PPV, compared to the radiologist without experience in pediatric radiology (p < 0.001). Conclusion The proposed deep learning algorithm provided an accurate diagnosis of DDH on hip radiographs, which was comparable to the diagnosis by an experienced radiologist.

OBJECTIVE: To assess the role of arterial spin-labeling (ASL) perfusion MRI in identifying cerebral perfusion changes after indirect revascularization in children with moyamoya disease. MATERIALS AND METHODS: We included pre- and postoperative perfusion MRI data of 30 children with moyamoya disease (13 boys and 17 girls; mean age ± standard deviation, 6.3 ± 3.0 years) who underwent indirect revascularization between June 2016 and August 2017. Relative cerebral blood flow (rCBF) and qualitative perfusion scores for arterial transit time (ATT) effects were evaluated in the middle cerebral artery (MCA) territory on ASL perfusion MRI. The rCBF and relative time-to-peak (rTTP) values were also measured using dynamic susceptibility contrast (DSC) perfusion MRI. Each perfusion change on ASL and DSC perfusion MRI was analyzed using the paired t test. We analyzed the correlation between perfusion changes on ASL and DSC images using Spearman's correlation coefficient. RESULTS: The ASL rCBF values improved at both the ganglionic and supraganglionic levels of the MCA territory after surgery (p = 0.040 and p = 0.003, respectively). The ATT perfusion scores also improved at both levels (p < 0.001 and p < 0.001, respectively). The rCBF and rTTP values on DSC MRI showed significant improvement at both levels of the MCA territory of the operated side (all p < 0.05). There was no significant correlation between the improvements in rCBF values on the two perfusion images (r = 0.195, p = 0.303); however, there was a correlation between the change in perfusion scores on ASL and rTTP on DSC MRI (r = 0.701, p < 0.001). CONCLUSION: Recognizing the effects of ATT on ASL perfusion MRI may help monitor cerebral perfusion changes and complement quantitative rCBF assessment using ASL perfusion MRI in patients with moyamoya disease after indirect revascularization.

Background Previous classification systems of pectus excavatum have been based on subjective morphologic characteristics. We sought to suggest a new classification system derived from objective variables. Methods Patients who underwent surgical repair of pectus excavatum without a history of previous chest operations were included. Objective morphologic variables were measured from chest computed tomography scan images, and classification was performed by hierarchical clustering of measured indexes. Clinical relevance of the suggested classification was also verified. Results Included were 230 patients who underwent operation for pectus excavatum from January 2001 to August 2013. These patients were classified into two major groups: typical (group I; 197 [85.7%]) and atypical (group II; 33 [14.3%]). Group I was further classified into three subgroups according to flatness and symmetry of the chest wall. Group II was further classified into four subgroups according to the severity of sternal torsion and sternal angulation. Two unique types of deformity were identified in group II: the double distortion subgroup (group IIa; 8 [3.5%]) and the reverse torsion subgroup (group IIc; 16 [7.0%]). Scoliosis was more frequently associated with group IIa ( p = 0.008). Conclusions Morphologic classification obtained from computed tomography indexes hierarchical clustering identified seven distinct subtypes of pectus excavatum.

Purpose To assess the usefulness of the relative position of the superior mesenteric artery (SMA) and superior mesenteric vein (SMV) in diagnosing intestinal malrotation in situs anomaly. Materials and methods From January 2004 to April 2015, 33 patients with situs anomalies were enrolled in this study who underwent abdominal USG, CT or MRI as well as upper gastrointestinal series (UGIS) or surgery: situs inversus (n = 16), left isomerism (n = 10), and right isomerism (n = 7); age 21.2 ± 23.2 years (mean ± standard deviation), range 0–72 years. The intestinal malrotation was confirmed with UGIS and/or operation in 16 patients. Relative positions of the SMV to the SMA were classified into four groups by reviewing abdominal USG, CT, or MRI: right sided, left sided, ventral sided, and dorsal sided. The incidence of malrotation was analyzed for each group. Results In 16 patients with situs inversus, there was reversed SMA-SMV relationship: left sided (n = 11) or ventral sided (n = 5). One situs inversus patient with ventral sided SMV had intestinal malrotation (6.25%). 17 patients with situs ambiguus showed various SMA-SMV relationships (ventral sided, n = 7; left sided, n = 5; right sided, n = 4; dorsal sided, n = 1). Among them, 15 patients (88.2%) had intestinal malrotation. Two patients with normal rotation had either right sided or dorsal sided SMV. Conclusion Situs ambiguus was commonly associated with intestinal malrotation with a variable SMA-SMV relationship. Reversal of the mesenteric vascular relationship was observed in situs inversus with normal rotation, not excluding the possibility of intestinal malrotation.

To evaluate the changes in using patterns of brain magnetic resonance imaging (MRI) in preterm infants after introduction of a MR-compatible incubator coil system.Brain MRIs for preterm infants with the MR-compatible incubator coil from March 2010 to July 2014 (n=154, group A) were compared with MRIs prior to the introduction of the incubator coil, from March 2005 to February 2010 (n=65, group B). Clinical data, MRI findings, acquisition time, and incidence of adverse events during the study were retrospectively reviewed. For the qualitative analysis of the examinations, the presence of motion artefact, spatial resolution, and overall image quality were assessed. Signal uniformity of each sequence was evaluated for a quantitative comparison.Comparing with group B, Group A was significantly younger (36+3 vs. 38+3 weeks, p0.001), had a significantly lower body weight (2006.6 and 2390.3g respectively; p0.001) at the time of MRI, and had shorter time interval (54.3±2.6 vs. 70.5±4.4days, p=0.002) between birth and examination. Abnormal findings were noted more frequently in group A (n=100, 65%) than in B (n=24, 37%. p=0.001) with a significantly higher incidence of diffusion restriction (n=21, 13.6% vs. n=4, 6.2%, p=0.034). Mean image acquisition time was significantly shorter in group A (21.4±4.5 vs. 25.4±5.5min, p0.001) with significant lower adverse events during MRI (n=26, 40 vs. n=6, 3.9%, p0.001). Group A exhibited significantly less motion artefact, better spatial resolution, and better overall image quality with decreased signal variation than group B (all p0.001).Application of the MR-compatible incubator for preterm brain MRI evaluation is safer and provides more timely evaluation of preterm infants with better image quality.

To evaluate the ultrasonography (US) features of Alagille syndrome (ALGS), as compared with biliary atresia (BA) or neonatal hepatitis (NH).Our study included 23 ALGS, 75 BA and 70 NH patients. The initial US images were retrospectively reviewed for gallbladder (GB) morphology with systemic classification, GB length and luminal area, presence of triangular-cord (TC) sign and hypertrophied hepatic-artery. The presence of anomalies associated with ALGS was evaluated. The diagnostic values of each finding and their combinations were evaluated.Both ALGS (57%) and BA (79%) were more frequently associated with abnormal GB shapes than NH (19%, all P0.001). The short and small GBs were more frequently observed in ALGS and BA than in NH (all P0.001). None in the ALGS and NH showed TC sign, while 41% in the BA did (all P0.001). Hypertrophied hepatic-artery was noted less frequently in both ALGS (13%) and NH (14%) than in BA (83%, all P0.001). The combination of US criteria with associated anomalies increased the positive-predictive-value for ALGS.Abnormal shaped GB with absence of the TC sign and hypertrophied hepatic-artery and presence of associated anomalies can be a differential point of ALGS.

It is difficult to diagnosis early stage germ cell tumors originating in the basal ganglia, but early recognition is important for better outcome. To evaluate serial MR images of basal ganglia germ cell tumors, with emphasis on the features of early stage tumors. We retrospectively reviewed serial MR images of 15 tumors in 14 children and young adults. We categorized MR images of the tumors as follows: type I, ill-defined patchy lesions (

Objective To evaluate the incidence and risk factors of emetic complications associated with the intravenous administration of low-osmolality iodinated contrast media (ICM) in children undergoing computed tomography (CT). Materials and methods All children who underwent contrast-enhanced CT between April 2017 and July 2019 were included. Pediatric patients were instructed on the preparative dietary protocol at our institution. Experienced nurses in the radiology department monitored the children during the CT scans and recorded any emetic complications in their electronic medical records. These data were used to calculate the incidence of emetic complications. Various patient factors and technical factors, including fasting duration, the type and volume of ICM, and ongoing chemotherapy, were evaluated to identify risk factors for emetic complications using univariate and multivariate logistic regression analyses. Results Among the 864 children (mean age, 8.4 ± 5.7 years) evaluated, 18 (2.1%) experienced emetic complications (6 experienced nausea only and 12 experienced nausea and vomiting). None of the children developed aspiration pneumonia. The mean fasting duration of patients with emesis was 7.9 ± 5.7 hours (range, 3-21 hours), whereas that of patients without nausea was 8.7 ± 5.7 hours (range, 0-24 hours). Fasting duration was not associated with the development of nausea and vomiting (p = 0.634). Multivariate logistic regression analysis revealed that ongoing chemotherapy (odds ratio [OR] = 4.323; 95% confidence interval [CI] = 1.430-13.064; p = 0.009), iomeprol use (OR = 7.219; 95% CI = 1.442-36.146; p = 0.016), and iohexol use (OR = 5.241; 95% CI = 1.350-20.346; p = 0.017) were independent risk factors for emetic complications. Conclusion Only a small proportion (2.1%) of children experienced nausea or vomiting after exposure to low-osmolality ICM. Many children underwent excessive fasting; however, fasting duration was not associated with nausea and vomiting. Moreover, ongoing chemotherapy and the use of iomeprol or iohexol were identified as potential risk factors for emetic complications in children.

The purpose of this study was to evaluate the usefulness of radially sampled 3D fat-suppressed T1-weighted gradient-echo sequences (radial volumetric interpolated breath-hold examination [radial VIBE]) for contrast-enhanced brain MRI of children through comparison with a magnetization-prepared rapid-acquisition gradient-echo (MP-RAGE) sequence.Sixty-five consecutive contrast-enhanced brain MRI examinations performed with axial MP-RAGE and radial VIBE sequences were included. For quantitative analysis, coefficients of variation of gray matter and white matter and CSF and relative contrast between tissue types (gray matter and white matter, gray matter and CSF, and white matter and CSF) for each sequence were calculated. For qualitative assessment, motion, pulsation artifacts, overall image quality, and lesion conspicuity were retrospectively scored on a 5-point scale. Quantitative and qualitative subgroup analyses were performed for patients with serious motion artifacts.Images obtained with the radial VIBE sequence had fewer motion and pulsation artifacts than those obtained with the MP-RAGE sequence (MP-RAGE vs radial VIBE motion score, 3.57 ± 1.00 vs 4.52 ± 0.51; pulsation score, 3.57 ± 0.60 vs 4.91 ± 0.21; all p0.001). Among 25 images with serious motion artifacts, radial VIBE images had significantly higher scores for all qualitative parameters, including overall image quality, than did MP-RAGE images (overall image quality for MP-RAGE vs radial VIBE, 2.63 ± 0.82 vs 3.42 ± 0.55, p0.001).The study showed that a radial fat-suppressed T1-weighted gradient-echo sequence is a viable alternative to conventional cartesian acquisition for contrast-enhanced brain imaging of restless children.

BACKGROUND AND PURPOSE: For the postoperative follow-up in pediatric patients with Moyamoya disease, it is essential to evaluate the degree of neovascularization status. Our aim was to quantitatively assess the neovascularization status after bypass surgery in pediatric Moyamoya disease by using color-coded digital subtraction angiography. MATERIALS AND METHODS: Time-attenuation intensity curves were generated at ROIs corresponding to surgical flap sites from color-coded DSA images of the common carotid artery, internal carotid artery, and external carotid artery angiograms obtained pre- and postoperatively in 32 children with Moyamoya disease. Time-to-peak and area under the curve values were obtained. Postoperative changes in adjusted time-to-peak (ΔTTP) and ratios of adjusted area under the curve changes (ΔAUC ratio) of common carotid artery, ICA, and external carotid artery angiograms were compared across clinical and angiographic outcome groups. To analyze diagnostic performance, we categorized clinical outcomes into favorable and unfavorable groups. RESULTS: The ΔTTP at the common carotid artery increased among clinical and angiographic outcomes, in that order, with significant differences ( P = .003 and .005, respectively). The ΔAUC ratio at the common carotid artery and external carotid artery also increased, in that order, among clinical and angiographic outcomes with a significant difference (all, P = .000). The ΔAUC ratio of ICA showed no significant difference among clinical and angiographic outcomes ( P = .418 and .424, respectively). The ΔTTP for the common carotid artery of >1.27 seconds and the ΔAUC ratio of >33.5% for the common carotid artery and 504% for the external carotid artery are revealed as optimal cutoff values between favorable and unfavorable groups. CONCLUSIONS: Postoperative changes in quantitative values obtained with color-coded DSA software showed a significant correlation with outcome scores and can be used as objective parameters for predicting the outcome in pediatric Moyamoya disease, with an additional cutoff value calculated through the receiver operating characteristic curve.

The purpose of this study was to compare wide-volume and helical pediatric 320-MDCT of the chest with respect to radiation dose and image quality.From November 2012 to September 2013, 59 wide-volume and 47 helical pediatric chest 320-MDCT images were obtained. The same tube potential and effective tube current-time product were applied in the two groups according to patient weight (group A,10 kg, n = 18; group B, 10-19.9 kg, n = 60; group C, 20-39.9 kg, n = 28). To compensate for overranging, adjusted CT dose index (CTDI) was calculated by dividing dose-length product (DLP) by the scan ranges imaged. Adjusted CTDI, DLP, overall image quality, motion artifact, noise, and scan ranges were compared by Mann-Whitney U test or t test.The adjusted CTDI was significantly lower in the group who underwent wide-volume CT than in the group who underwent helical CT (weight group A, p0.001; group B, p0.001; group C, p = 0.003). The DLP was lower in the wide-volume group than in the helical CT group in weight groups A (p0.001) and B (p0.001) but not in group C (p = 0.162). All CT scans were of diagnostic quality, and there was no significant difference between the wide-volume and helical CT groups (p = 0.318). The motion artifact score was significantly higher in the wide-volume group than in the helical CT group in groups B (p0.001) and C (p = 0.010) but not in group A (p = 0.931). The noise was significantly lower in the wide-volume group than in the helical CT group (p0.001).In pediatric chest CT, use of wide-volume CT can decrease radiation exposure while preserving image quality. It is associated with less noise than helical CT but may be subject to more motion artifact.

This study aimed to assess the therapeutic results of ultrasound (US)-guided water-soluble contrast enema in very low birth weight (VLBW) preterm infants (1,500 g) with meconium obstruction and to study factors that affect therapeutic results.This study included a total of 33 consecutive VLBW infants with clinically diagnosed meconium obstruction underwent US-guided water-soluble contrast enema, from April 2007 to March 2014. Patients were classified into two groups based on to procedure outcome: the success group (evacuation of the meconium plug resolution followed by improved bowel distention within 2 days of the procedure, without additional interventions), and the failure group (the contrast enema failed to relieve the obstruction, or other procedure-related complications occurred). Patient- and mother-related clinical factors and procedure-related factors were compared between both groups.Overall success rate was 54.5%, with 18 successful (M:F=10:8), and 15 failure (M:F=7:8) cases. When compared with the failure group, the success group patients showed statistically significant older gestational age (29(+1) vs. 27 weeks; p=0.028), larger birth weight (1023.1g vs. 790.3g; p=0.048), and higher body weight on the day of the procedure (1036.2g vs. 801.6g, p=0.049). However, no statistically significant differences were seen between other patient and maternal factors. Among the procedure-related factors, retrial of contrast injection during the procedure was associated with significantly higher success than the single trial (p=0.027). The presence of refluxed contrast into the distal ileum was the statistically significant predictor for success of the procedure (p=0.038). There were three cases of bowel perforation (9.1% per person).US-guided water-soluble contrast enema in VLBW infants with meconium obstruction showed a 54.5% success rate and a 9.1% perforation rate per person. Among the procedure-related factors, retrial of contrast injection during the procedure and the presence of refluxed contrast into the distal ileum were related to the success of the procedure.

Objectives To analyze the texture features on cranial sonography in preterm neonates with white matter injury quantitatively and to correlate these features with magnetic resonance imaging (MRI). Methods The study included 33 preterm neonates treated in our neonatal intensive care unit who underwent serial cranial sonography and brain MRI near term. Patients were subdivided into 3 groups according to the presence and severity of white matter injury as revealed by MRI: normal (group 1; n = 20), mild (group 2; n = 5), and severe (group 3; n = 8). The periventricular echogenicity on sonography was evaluated quantitatively with second-order gray-level statistics (gray-level co-occurrence matrix [GLCM] method). Four GLCM texture features representing homogeneity were extracted in 12 directions: (1) angular second moment (ASM), (2) inverse differential moment (IDM), (3) contrast, and (4) entropy. Results Thirty of 48 features showed a statistically significant difference between groups 1 and 3 (ASM in 9 directions, IDM in 6 directions, contrast in 3 directions, and entropy in all 12 directions). There were no significant differences observed between groups 1 and 2 or groups 2 and 3. The mean contrast and entropy values were generally lower in group 1 than group 3, whereas the mean ASM and IDM values were higher in group 1. Conclusions Severe white matter injury could be identified by using GLCM texture analysis, whereas mild white matter injury observed on MRI could not be evaluated by GLCM analysis. Quantitative texture analysis using the GLCM may serve as a complementary tool for quantitative assessment of periventricular echogenicity.

Knowledge of the optimal lengths for central venous catheterization prior to the procedure may lessen the need for repositioning and prevent vascular complications. To establish the optimal lengths for non-tunneled central venous catheter insertion through the right and left internal jugular veins. We included 92 children who received US-guided central venous catheterization via right or left internal jugular veins in intensive care units. The calculated distance between the skin and carina was considered the optimal length for right and left internal jugular venous catheterization. Univariate and multivariate linear regression analyses was used to identify predictors. Age, height and weight showed significant correlations with optimal insertion lengths for right and left internal jugular vein approaches on univariate analysis, while height was the only significant independent predictor of optimal insertion length. The optimal insertion lengths (cm) suggested by our data are, for the right internal jugular vein 0.034 × height (cm) + 3.173, and for the left 0.072 × height (cm) + 2.113.

Radial k-space sampling techniques have been shown to reduce motion artifacts in adult abdominal MRI. To compare a T2-weighted radial k-space sampling MRI pulse sequence (BLADE) with standard respiratory-triggered T2-weighted turbo spin echo (TSE) in pediatric abdominal imaging. Axial BLADE and respiratory-triggered turbo spin echo sequences were performed without fat suppression in 32 abdominal MR examinations in children. We retrospectively assessed overall image quality, the presence of respiratory, peristaltic and radial artifact, and lesion conspicuity. We evaluated signal uniformity of each sequence. BLADE showed improved overall image quality (3.35 ± 0.85 vs. 2.59 ± 0.59, P

Mutations of the surfactant protein (SP)-C gene (SFTPC) have been associated with neonatal respiratory distress syndrome (RDS) and childhood interstitial lung disease (ILD). If accurate diagnosis and proper management are delayed, irreversible respiratory failure demanding lung transplantation may ensue. A girl was born at term but was intubated and given exogenous surfactant due to RDS. Cough and tachypnea persisted, and symptoms rapidly progressed at 16 months of age despite treatment with antibiotics, oral prednisolone, methylprednisolone pulse therapy, and intravenous immunoglobulin. At 20 months, she visited our hospital for a second opinion. A computed tomography scan showed a diffuse mosaic pattern with ground-glass opacity and subpleural cysts compatible with ILD. A video-assisted thoracoscopic lung biopsy revealed ILD with eosinophilic proteinaceous material and macrophages in the alveolar space. Bilateral lung transplant from a 30-month-old child was done, and she was discharged in room air without acute complications. Genetic analysis revealed a novel c.203T>A, p.Val68Asp mutation of SP-C, based on the same exon as a known pathogenic mutation, p.Glu66Lys., Graphical Abstract

BACKGROUND In free flap reconstruction for head and neck cancer, achieving a haemodynamic target using excessive fluid infusion is associated with decreased flap survival rates and extended hospital stays. We hypothesized that goal-directed haemodynamic therapy would improve flap survival rates and shorten hospitalization periods. METHODS Patients scheduled for free flap reconstruction were randomly assigned to a goal-directed haemodynamic therapy group (n = 31) or a conventional haemodynamic therapy control group (n = 31). The control group received extra bolus fluid and ephedrine or norepinephrine to maintain a mean arterial pressure ≥ 65 mmHg. The goal-directed haemodynamic therapy group received a colloid solution as the extra bolus fluid to maintain a stroke volume variation < 12%; dobutamine, ephedrine, or norepinephrine was administered to maintain a cardiac index ≥ 2.5 l/min/m2 and mean arterial pressure ≥ 65 mmHg. Enhanced recovery after surgery protocols were not used except for fluid therapy. An otolaryngologist blinded to group assignments assessed flap outcomes and classified them as 'survival,' 'at risk' or 'failure.' RESULTS The hospitalization period was not significantly different between the groups. The goal-directed haemodynamic therapy group had significantly shorter intensive care unit stays and a higher flap survival rate. The crystalloid volume was significantly lower in goal-directed haemodynamic therapy group. Reoperation rates, post-operative complications, and laboratory data including inflammatory markers were similar between the groups. CONCLUSION Compared to conventional haemodynamic therapy, goal-directed haemodynamic therapy does not reduce hospitalization periods; it may, however, reduce the length of intensive care unit stays and increase flap survival rates. Further studies including multi-centre trials with larger sample sizes are warranted.

Purpose To evaluate the patients’ morphologic factors affecting radiation dose in pediatric chest CT. Materials and methods From November 2013 to May 2015, 315 pediatric chest CT scans were obtained using a CT scanner, and classified into 5 groups according to the patients’ age. For each age group, the chest CT scans were divided into two subgroups. A cut-off value used was the 75th percentile of size-specific dose estimates (SSDE), age-specific diagnostic reference level (DRL): less than the 75th percentile of SSDE (Group A, n = 238) and greater than the 75th percentile of SSDE (Group B, n = 77). All CT scans were performed with the same protocol using automatic tube voltage selection and current modulation techniques. The morphologic factors of the patients including body mass index (BMI), arm angles, presence of medical devices in the scan field, and degree of off-centering within the CT gantry were compared between groups A and B. Results Group B showed narrower arm angles on scout and coronal reformatted images, higher frequency of the presence of devices and higher BMI than group A (P < 0.001, P < 0.001; P = 0.018, and P < 0.001, respectively). In multivariate analysis, narrower arm angles, the presence of devices on the scout images and higher BMI were independently associated with higher SSDE (P = 0.001, P = 0.037, and P < 0.001, respectively). Conclusions During acquisition of the scout images, arms-down position and the presence of medical devices were associated with a high radiation dose above age-specific DRLs in pediatric chest CT, regardless of repositioning before the actual scanning. In addition, off-centering had no clinical impact on radiation dose in the routine practice.

The 320-row multidetector computed tomography (CT) scanner has multiple scan modes, including volumetric modes. To compare the image quality and radiation dose of 320-row CT in three acquisition modes — helical, one-shot volume, and wide-volume scan — at pediatric brain imaging. Fifty-seven children underwent unenhanced brain CT using one of three scan modes (helical scan, n=21; one-shot volume scan, n=17; wide-volume scan, n=19). For qualitative analysis, two reviewers evaluated overall image quality and image noise using a 5-point grading system. For quantitative analysis, signal-to-noise ratio, image noise and posterior fossa artifact index were calculated. To measure the radiation dose, adjusted CT dose index per unit volume (CTDIadj) and dose length product (DLP) were compared. Qualitatively, the wide-volume scan showed significantly less image noise than the helical scan (P=0.009), and less streak artifact than the one-shot volume scan (P=0.001). The helical mode showed significantly lower signal-to-noise ratio, with a higher image noise level compared with the one-shot volume and wide-volume modes (all P

Background Neurofibromatosis type 1 (NF1) is an autosomal dominant disease with prominent neurocutaneous manifestations. The most common intracranial imaging finding of NF1 on brain magnetic resonance imaging (MRI) is the high-signal intensity foci without a mass effect or growth in size. Patient Description We describe two children with NF1 in whom brain MRI showed growing cystic lesions and adjacent white matter signal abnormalities, which were confirmed as non-neoplastic cystic degeneration and reactive gliosis. Conclusion Growing cyst-like white matter lesions can be seen on serial brain MRI in children with NF1. Reactive gliosis with cystic degeneration could be a pathogenic basis of these cystic lesions.

Background With the emergence of macrolide resistance, concerns about the efficacy of macrolides for the treatment of Mycoplasma pneumoniae (MP) pneumonia in children have been raised. This study aimed to determine the effect of macrolide resistance on the outcome of children who were hospitalized with MP pneumonia. Methods Between 2010 and 2015, we performed culture of MP from nasopharyngeal samples obtained from children who were hospitalized with pneumonia at five hospitals in Korea. Macrolide resistance was determined by the analysis of 23S rRNA gene transition and the minimal inhibitory concentrations of four macrolides. Medical records were reviewed to analyze the clinical response to treatment with macrolides. Results MP was detected in 116 (4.8%) of the 2436 children with pneumonia. MP pneumonia was prevalent in 2011 and 2015. Of the 116 patients with MP pneumonia, 82 (70.7%) were macrolide-resistant. There were no differences in the age distribution, total duration of fever, and chest x-ray patterns between the macrolide-susceptible and macrolide-resistant groups. After macrolide initiation, mean days to defervescence were longer in the macrolide-resistant group than in macrolide-susceptible group (5.7 days vs. 4.1 days, P = 0.021). However, logistic regression analysis revealed that the presence of extrapulmonary signs (P = 0.039), homogeneous lobar consolidation (P = 0.004), or parapneumonic effusion (P

To decrease the negative appendectomy rate in children, knowledge of the misleading imaging findings on US and CT in negative appendicitis cases is important. To evaluate the negative appendectomy rate and describe the imaging findings of US and CT that lead radiologists to misdiagnose acute appendicitis in children. From 2007 to 2013, 374 children operated for suspected appendicitis were proved to either have acute appendicitis (n = 348) or to be negative for appendicitis (n = 26) on pathological reports. Negative appendectomy rates were compared among imaging modalities, age groups and genders. We retrospectively reviewed US and CT findings from negative appendectomy cases. The overall negative appendectomy rate was 7.0% (26/374). There were no statistically significant differences among the subgroups. The most common misleading presentations on US were sonographic tenderness (9/16, 56%) and non-compressibility (9/16, 56%). The most common misleading finding on CT were the presence of an appendicolith or hyperdense feces (5/12, 42%). Periappendiceal fat inflammation was observed in only one case of negative appendicitis on US and on CT. Radiologists can misdiagnose children with equivocal diameters of appendices as having acute appendicitis when sonographic tenderness or non-compressibility is present on US and when an appendicolith or hyperdense feces is noted on CT. The possibility of negative appendicitis should be borne in mind when periappendiceal fat inflammation is absent or minimal in indeterminate cases.

A 4-month-old boy presented with persistent gross hematuria. At ultrasonography, a 3.5-cm echogenic mass with posterior shadowing and tumor vascularity was detected within the right renal pelvis. Precontrast CT showed a slightly hyperattenuating mass in the renal pelvis. At MRI the mass was heterogeneously hypointense on T2-weighted images and isointense on T1-weighted images. Contrast-enhanced CT and MRI both revealed peripheral enhancement of the mass. A histological diagnosis of ossifying renal tumor of infancy was made after open pyelostomy and tumor enucleation. We suggest that ossifying renal tumor of infancy should be considered when a mass with posterior acoustic shadowing and tumor vascularity on US, hyperattenuation on precontrast CT and hypointensity on T2-weighted MRI is seen within the renal pelvis of an infant with hematuria.

BackgroundRadiologic evaluation of children with Mycoplasma pneumoniae is important for diagnosis and management.ObjectiveTo investigate the correlation between chest radiographic findings and the clinical features in children with Mycoplasma pneumoniae pneumonia.Materials and methodsThis study included 393 hospitalized children diagnosed with M. pneumoniae pneumonia between January 2000 and August 2016. Their clinical features and chest radiographs were reviewed. Radiographic findings were categorized and grouped as consolidation group (lobar or segmental consolidation) and non-consolidation group (patchy infiltration, localized reticulonodular infiltration, or parahilar peribronchial infiltration).ResultsLobar or segmental consolidation (37%) was the most common finding, followed by parahilar or peribronchial infiltration (27%), localized reticulonodular infiltration (21%) and patchy infiltration (15%). The consolidation group was more frequently accompanied by pleural effusions (63%), compared to the non-consolidation group (16%). Compared with patients in the non-consolidation group, those in the consolidation group were associated with a significantly higher rate of hypoxia, tachypnea, tachycardia, extrapulmonary manifestations, prolonged fever, and longer periods of anti-mycoplasma therapy and hospitalization. Lobar or segmental consolidation was significantly more frequent in children ≥5 years old (44%) compared with children 2-5 years old (34%) and ConclusionThe chest radiographic findings of children with M. pneumoniae pneumonia correlate well with the clinical features. Consolidative lesions were frequently observed in older children and were associated with more severe clinical features.

Objective To retrospectively evaluate the value of computerized 3D texture analysis for differentiating pulmonary metastases from non-metastatic lesions in pediatric patients with osteosarcoma. Materials and methods This retrospective study was approved by the institutional review board. The study comprised 42 pathologically confirmed pulmonary nodules in 16 children with osteosarcoma who had undergone preoperative computed tomography between January 2009 and December 2014. Texture analysis was performed using an in-house program. Multivariate logistic regression analysis was performed to identify factors for differentiating metastatic nodules from non-metastases. A subgroup analysis was performed to identify differentiating parameters in small non-calcified pulmonary nodules. The receiver operator characteristic curve was created to evaluate the discriminating performance of the established model. Results There were 24 metastatic and 18 non-metastatic lesions. Multivariate analysis revealed that higher mean attenuation (adjusted odds ratio [OR], 1.014, P = 0.003) and larger effective diameter (OR, 1.745, P = 0.012) were significant differentiators. The analysis with small non-calcified pulmonary nodules (7 metastases and 18 non-metastases) revealed significant inter-group differences in various parameters. Logistic regression analysis revealed that higher mean attenuation (OR, 1.007, P = 0.008) was a significant predictor of non-calcified pulmonary metastases. The established logistic regression model of subgroups showed excellent discriminating performance in the ROC analysis (area under the curve, 0.865). Conclusion Pulmonary metastases from osteosarcoma could be differentiated from non-metastases by using computerized texture analysis. Higher mean attenuation and larger diameter were significant predictors for pulmonary metastases, while higher mean attenuation was a significant predictor for small non-calcified pulmonary metastases.

Objective To compare quantitative and qualitative image quality parameters in pediatric abdominopelvic dual-energy CT (DECT) using noise-optimized virtual monoenergetic image (VMI) and conventional VMI at different kiloelectron volt (keV) levels. Materials and Methods Thirty-six consecutive abdominopelvic DECT scans were retrospectively included. Noise-optimized VMI and conventional VMI were reconstructed at seven energy levels, from 40 keV to 100 keV at 10 keV intervals. The contrast-to-noise ratio (CNR) and signal-to-noise ratio (SNR) of the liver, pancreas, and aorta were objectively measured and compared. Image quality was evaluated subjectively regarding image noise, image blurring of solid organ, bowel image quality and severity of beam-hardening artifacts. Optimal monoenergetic levels in keV for both algorithms were determined based on overall image quality score. Results The maximal CNR and SNR values for all investigated organs were observed at 40 keV in noise-optimized VMI (CNR and SNR of liver, pancreas, aorta in order [CNR; 20.93, 17.34, 46.75: SNR; 37.39, 33.80, 63.21]), at 60–70 keV and at 70 keV in conventional VMI (CNR; 8.12, 5.67, 15.97: SNR; 19.57, 16.66, 26.65). In qualitative image analysis, noise-optimized VMI and conventional VMI showed the best overall image quality scores at 60 keV and at 70 keV, respectively. Noise-optimized VMI at 60 keV showed superior CNRs, SNRs, and overall image quality scores compared to conventional VMI at 70 keV (p < 0.001). Conclusion Optimal energy levels for noise-optimized VMI and conventional VMI were 60 keV and at 70 keV, respectively. Noise-optimized VMI shows superior CNRs, SNRs and subjective image quality over conventional VMI, at the optimal energy level.

Objective To determine the correlation between cerebral blood flow (CBF) on arterial spin labeling (ASL) MRI and the degree of postoperative revascularization assessed on digital subtraction angiography in children with moyamoya disease (MMD). Materials and methods Twenty-one children (9 boys and 12 girls; mean age, 8.4 ± 3.6 years; age range, 3-16 years) with MMD who underwent both pseudocontinuous ASL MRI at 1.5T and catheter angiography before and after superficial temporal artery encephaloduroarteriosynangiosis were included in this retrospective study. The degree of revascularization in the middle cerebral artery (MCA) territory was evaluated on external carotid angiography and was graded on a 3-point scale. On ASL CBF maps, regions of interest were manually drawn over the MCA territory of the operated side at the level of the centrum semi-ovale and over the cerebellum. The normalized CBF (nCBF) was calculated by dividing the CBF of the MCA territory by the CBF of the cerebellum. Changes in nCBFs were calculated by subtracting the preoperative nCBF values from the postoperative nCBF values. The correlation between nCBF changes measured with ASL and the revascularization grade from direct angiography was evaluated. Results The nCBF value on the operated side increased after the operation (p = 0.001). The higher the degree of revascularization, the greater the nCBF change was: poor revascularization (grade 1), -0.043 ± 0.212; fair revascularization (grade 2), 0.345 ± 0.176; good revascularization (grade 3), 0.453 ± 0.182 (p = 0.005, Jockheere-Terpstra test). The interobserver agreement was excellent for the measured CBF values of the three readers (0.91-0.97). Conclusion The nCBF values of the MCA territory obtained from ASL MRI increased after the revascularization procedure in children with MMD, and the degree of nCBF change showed a significant correlation with the degree of collateral formation evaluated via catheter angiography.

Objective To compare image qualities between vendor-neutral and vendor-specific hybrid iterative reconstruction (IR) techniques for abdominopelvic computed tomography (CT) in young patients. Materials and Methods In phantom study, we used an anthropomorphic pediatric phantom, age-equivalent to 5-year-old, and reconstructed CT data using traditional filtered back projection (FBP), vendor-specific and vendor-neutral IR techniques (ClariCT; ClariPI) in various radiation doses. Noise, low-contrast detectability and subjective spatial resolution were compared between FBP, vendor-specific (i.e., iDose1 to 5; Philips Healthcare), and vendor-neutral (i.e., ClariCT1 to 5) IR techniques in phantom. In 43 patients (median, 14 years; age range 1–19 years), noise, contrast-to-noise ratio (CNR), and qualitative image quality scores of abdominopelvic CT were compared between FBP, iDose level 4 (iDose4), and ClariCT level 2 (ClariCT2), which showed most similar image quality to clinically used vendor-specific IR images (i.e., iDose4) in phantom study. Noise, CNR, and qualitative imaging scores were compared using one-way repeated measure analysis of variance. Results In phantom study, ClariCT2 showed noise level similar to iDose4 (14.68–7.66 Hounsfield unit [HU] vs. 14.78–6.99 HU at CT dose index volume range of 0.8–3.8 mGy). Subjective low-contrast detectability and spatial resolution were similar between ClariCT2 and iDose4. In clinical study, ClariCT2 was equivalent to iDose4 for noise (14.26–17.33 vs. 16.01–18.90) and CNR (3.55–5.24 vs. 3.20–4.60) (p > 0.05). For qualitative imaging scores, the overall image quality ([reader 1, reader 2]; 2.74 vs. 2.07, 3.02 vs. 2.28) and noise (2.88 vs. 2.23, 2.93 vs. 2.33) of ClariCT2 were superior to those of FBP (p < 0.05), and not different from those of iDose4 (2.74 vs. 2.72, 3.02 vs. 2.98; 2.88 vs. 2.77, 2.93 vs. 2.86) (p > 0.05). Conclusion Vendor-neutral IR technique shows image quality similar to that of clinically used vendor-specific hybrid IR technique for abdominopelvic CT in young patients.

Contains fulltext : 118083.pdf (Publisher’s version ) (Open Access) OBJECTIVE: To develop an high resolution computed tomography (HRCT) scoring system for the assessment of bronchopulmonary dysplasia (BPD) and determine its usefulness as compared with the chest radiographic score. MATERIALS AND METHODS: Forty-two very low-birth-weight preterm infants with BPD (25 male, 17 female) were prospectively evaluated with HRCT performed at the mean age of 39.1-week postmenstrual age. Clinical severity of BPD was categorized as mild, moderate or severe. The HRCT score (0-36) of each patient was the sum of the number of bronchopulmonary segments with 1) hyperaeration and 2) parenchymal lesions (linear lesions, segmental atelectasis, consolidation and architectural distortion), respectively. We compared the HRCT scores with the chest radiographic scores (the Toce system) in terms of correlation with clinical severity. RESULTS: The HRCT score had good interobserver (r = 0.969, p < 0.001) and intraobserver (r = 0.986, p < 0.001) reproducibility. The HRCT score showed better correlation (r = 0.646, p < 0.001) with the clinical severity of BPD than the chest radiographic score (r = 0.410, p = 0.007). The hyperaeration score showed better correlation (r = 0.738, p < 0.001) with the clinical severity of BPD than the parenchymal score (r = 0.523, p < 0.001). CONCLUSION: We have developed a new HRCT scoring system for BPD based on the quantitative evaluation of pulmonary abnormalities of BPD consisting of the hyperaeration score and the parenchymal score. The HRCT score shows better correlation with the clinical severity of BPD than the radiographic score.