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Purpose: The purpose of this study was to use shear-wave elastography (SWE) to assess testicular stiffness changes during the Valsalva maneuver in adolescents and young adults with varicocele, to compare these changes according to clinical severity, and to evaluate the role of SWE in the diagnosis of varicocele.Methods: This study included patients undergoing testicular ultrasonography for the diagnosis of varicocele or for post-varicocelectomy follow-up between June 2016 and February 2017. Fifty-four testicles of 27 consecutive patients (mean age, 15.9 years) were classified by clinical grade (grade 0-3). Using SWE, mean testicular stiffness (Emean) was measured at rest and during the Valsalva maneuver. The correlations between multiple ultrasonographic parameters (volume asymmetry, Emean at rest, and absolute and percentage changes in Emean during the Valsalva maneuver) and clinical grade were assessed using the Spearman correlation test.Results: The Emean at rest was similar across clinical grades. During the Valsalva maneuver, increased testicular stiffness was frequently observed in patients with grade 2 or 3 varicocele but rarely observed in those with grade 0 or 1 varicocele. The changes in Emean were positively correlated with the clinical grade (all P0.05).Conclusion: A transient, reversible increase in testicular stiffness during the Valsalva maneuver was observed in adolescents with high-grade varicocele, and the degree of stiffness change was correlated with the clinical grade. Stiffness change identified using SWE during the Valsalva maneuver is a potential surrogate indicator of venous congestion.

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.

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 (Ktrans, Kep, and Ve) at the ileocecal region between the inactive and active groups based on the histopathologic status. The 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 Ktrans value than the inactive group (0.31 ± 0.12 vs. 0.16 ± 0.46 min−1, p = 0.002). Endoscopic finding; PCDAI, CRP, and CDMI scores; and Ktrans value were significant parameters in the identification of the active Crohn’s disease (p = 0.002, p

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.

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...

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.

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

Patients with lysosomal acid lipase (LAL) deficiency and glycogen storage disease (GSD) demonstrated hepatomegaly and dyslipidemia. In our case, a 6-year-old boy presented with hepatosplenomegaly. At 3 years of age, GSD had been diagnosed by liver biopsy at another hospital. He showed elevated serum liver enzymes and dyslipidemia. Liver biopsy revealed diffuse microvesicular fatty changes in hepatocytes, septal fibrosis and foamy macrophages. Ultrastructural examination demonstrated numerous lysosomes that contained lipid material and intracytoplasmic cholesterol clefts. A dried blood spot test revealed markedly decreased activity of LAL. LIPA gene sequencing identified the presence of a novel homozygous mutation (p.Thr177Ile). The patient's elevated liver enzymes and dyslipidemia improved with enzyme replacement therapy. This is the first report of a Korean child with LAL deficiency, and our findings suggest that this condition should be considered in the differential diagnosis of children with hepatosplenomegaly and dyslipidemia.

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.

D-penicillamine has been reported to cause antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis presenting as rapidly progressive glomerulonephritis or pulmonary-renal syndrome mostly in adults. We report a pediatric case of D-penicillamine induced ANCA-associated vasculitis that manifests as a pulmonary-renal syndrome with a mild renal manifestation. A 13-year-old girl who has been taking D-penicillamine for five years under the diagnosis of Wilson disease visited the emergency room because of hemoptysis and dyspnea. She had diffuse pulmonary hemorrhage, microscopic hematuria, and proteinuria. Myeloperoxidase ANCA was positive, and a renal biopsy revealed pauci-immune crescentic glomerulonephritis. Under the diagnosis of D-penicillamine-induced ANCA-associated vasculitis, D-penicillamine was switched to trientine, and the patient was treated with plasmapheresis, glucocorticoid, cyclophosphamide, and mycophenolate mofetil. Pulmonary hemorrhage improved rapidly followed by the disappearance of the hematuria and proteinuria five months later., Graphical Abstract

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.

Purpose: Swyer-James-Macleod syndrome (SJMS) is a rare disorder characterized by the hyperlucency of a unilateral lung due to ip silaterally arrested lung growth. No sufficient information on progressive lung function changes during growth is available in SJMS. Methods: We retrospectively reviewed SJMS cases using a clinical database from January 2000 to August 2018. Results: A total of 40 patients were enrolled in this study. The median age of the patients was 4.8 years (interquartile range [IQR], 2.7-9.0 years), boys (n=21, 52.5%) were more affected, and left lungs (n=27, 67.5%) were more involved. The interval between in fection and diagnosis was 1.8 years (IQR, 0.7-6.4 years). Mycoplasma pneumoniae (n=14) and adenovirus (n=5) were most preva lent among the 21 subjects with presumably identifiable causes. Most of the 16 patients with available pulmonary function data presented moderate obstructive lung function (z-score, median; FEV1: -4.320 [IQR, -5.955 to -3.400] and FVC: -2.600 [IQR, -3.590 to -1.570], respectively) with the more decreased median in forced expiratory flow between 25% and 75% of FVC (FEF25%-75%) (z-score, -4.470; IQR, -5.330 to -3.170). Linear mixed effect models indicated that younger age at first infectious insult was significantly related to decreased FEV1 z-score (P=0.041), and that the FEV1/FVC and FEF25%-75% z-scores were reduced by 0.082 (P=0.005) and 0.069 (P≤0.001) per year. Conclusion: In children with SJMS, the initial infectious insult occurs in the early life with M. pneumoniae being the most common pathogen. Lung function may deteriorate with an obstructive pattern during growth. The impairment can progress slowly not in the FEV1, but in the FEV1/FVC or FEF25%-75%. (Allergy Asthma Respir Dis 2021;9:156-163)

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 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.

Purpose: Pulmonary underdevelopment is one of the pulmonary causes of neonatal mortality, which is categorized into pulmonary agenesis, pulmonary aplasia, and pulmonary hypoplasia. Congenital diaphragmatic hernia is a well-known cause of pulmonary hy poplasia; however, further studies are needed for other etiologies. Patients with pulmonary underdevelopment show varying de grees of clinical severity. Most patients with pulmonary underdevelopment have respiratory distress at birth, while a few have only mild symptoms. Our study investigated the natural course and prognosis of pulmonary underdevelopment by analyzing multiple cases from a single tertiary medical center. Methods: A retrospective review was conducted on patients diagnosed with pulmonary hypoplasia and agenesis from January 2000 to August 2019. Cases were extracted from the clinical database of Seoul National University Children’s Hospital by searching for keywords related to pulmonary underdevelopment. Results: A total of 28 patients were identified. Four patients had pulmonary agenesis, and all were idiopathic. Twenty-four patients had evidence of pulmonary hypoplasia with diverse causes. The median age at diagnosis was 11.5 days (range, 1-240 days) and the mean gestational age was 35 weeks 2 days±3 weeks 6 days. Twenty patients received ventilator care and 11 had various degrees of pulmonary hypertension. The long-term survival rate was 50%. The median age of death among pulmonary hypoplasia patients were 11 days (range, 1-730 days). All patients without ventilator care survived. The incidence of ventilator care, pneumothorax, and pulmonary hypertension were significantly higher among patients who died. Conclusion: The prognosis of pulmonary underdevelopment depends on the severity of respiratory distress and pulmonary hyper tension in earlier life. Patients with severe distress appear to have severe degree of pulmonary hypoplasia. Patients without ventila tor support in the neonatal period survived without significant morbidities. (Allergy Asthma Respir Dis 2020;8:206-212)

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.

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.

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.

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.

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.

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 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.

Xanthogranulomatous cholecystitis (XGC) is a rare form of chronic cholecystitis that is accompanied by xanthomatous histiocytes and chronic inflammation. A 2-month-old boy presented with a right upper abdominal palpable mass. Cholecystectomy with liver wedge resection was done, under the impression that the mass might be a hepatic tumor or liver abscess. Pathologic examination showed XGC with abscess formation. Most cases of XGC were observed in adult and only a few cases were reported in children. We describe a very rare case of XGC in infancy.

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.

Despite many successes of genome-wide association (GWA) studies, known susceptibility variants identified by GWAS have the modest effect sizes and we met noticeable skepticism about the risk prediction model building with large-scale genetic data. However, in contrast with genetic variants, family history of diseases has been largely accepted as an important risk factor in clinical diagnosis and risk prediction though; complicated structures of family history of diseases have limited their application to clinical use. Here, we develop a new method which enables the incorporation of general family history of diseases with the liability threshold model and a new analysis strategy for risk prediction with penalized regression incorporating large-scale genetic variants and clinical risk factors. An application of our model to type 2 diabetes (T2D) patients in Korean population (1846 cases out of 3692 subjects) demonstrates that SNPs accounts for 28.6% of T2D’s variability and incorporation of family history leads to additional improvement of 5.9%. Our result illustrates that family history of diseases can have an invaluable information for disease prediction and may bridge the gap originated from missing heritability.

Gastric adenomyoma in children is a rare benign tumor composed of a mixture of duct-like epithelial structures with smooth muscle bundles. It has been considered as a subtype of ectopic pancreas because the epithelial component resembles that in pancreatic ducts. However, it is now recognized as an independent pathologic lesion. Gastric adenomyoma is usually asymptomatic but could cause obstruction, inflammation, ulceration, and malignant transformation. Hence, to achieve diagnostic certainty and to control symptoms, complete operative resection is recommended. To the best of our knowledge, this is the first case of gastric adenomyoma mimicking a gastric duplication cyst in children.

Background: Meconium obstruction (MO) of prematurity can result in increased morbidity or mortality and prolonged hospitalization if not diagnosed and treated appropriately. Objectives: The aims of our study were to identify the incidence and risk factors associated with MO and to review the treatment outcomes. Methods: A retrospective analysis was undertaken of 101 extremely low birth weight infants (ELBWIs) who were born between January 1, 2007, and April 1, 2009, at Seoul National University Hospital. Prenatal and neonatal factors were compared between the MO and control groups. The treatment outcomes were also reviewed. Results: Twenty-two (22%) patients were diagnosed as having MO. Eighteen of these patients (82%) had prenatal risk factors for MO. Respiratory distress syndrome was more prevalent in the MO group than in the control group (p = 0.001). Overall, 17 of the 22 patients (77%) were relieved with medical treatment and the rest underwent ileostomy. The times to full enteral feeding did not differ between the medically treated group and the control group. However, the surgically treated group required more time to achieve full enteral feeding, and some patients had persistent gastrointestinal problems. Conclusion: MO is not a rare condition in ELBWIs, and the majority of ELBWIs have prenatal risk factors. Medical management was effective, and medically manageable cases had good prognoses for subsequent feeding, whereas some surgically managed cases had persistent gastrointestinal problems.

We report a case of isolated periostitis associated with Takayasu arteritis in a 10-year-old boy presenting with calf pain. Radiographs revealed a localized, thick periosteal reaction with irregular margin in the proximal left fibula. MRI revealed irregular thickening with enhancement of the periosteum in the proximal fibula and heterogeneous enhancement along vascular bundles in the calf. Isolated periostitis is a rare skeletal manifestation of systemic vasculitis that could be misdiagnosed as neoplastic or traumatic periosteal reaction.

To evaluate whether perfusion magnetic resonance (MR) imaging can depict hemodynamic status after revascularization surgery and whether changes at perfusion MR imaging after revascularization surgery correspond with clinical outcome in moyamoya disease.An institutional review board approved this retrospective study; informed consent was waived. Pre- and postoperative perfusion MR imaging data in 67 children with moyamoya disease (mean age, 7.2 years; range, 2-13 years) were included. Regional time to peak (rTTP) and regional cerebral blood volume (rCBV) were calculated by adjusting cerebral time to peak (TTP) and cerebral blood volume (CBV) values by using cerebellar reference values. For quantitative regional analysis, pixel values were divided into five categories (0,2,4,6, and8 seconds), and percentages of pixels in rTTP meeting these time conditions were calculated. Changes in the values after revascularization were calculated. Postoperative clinical outcomes were categorized as follows: 1 indicated excellent; 2, good; 3, fair; and 4, poor. Pre- and postoperative perfusion parameters were compared by using a paired t test; relationships between perfusion parameters and clinical outcomes were investigated by using one-way analysis of variance, with a significance level of .05.rTTP, rCBV, and percentage of pixels of rTTP decreased significantly after revascularization surgery. Pre- and postoperative rTTP were significantly different for the clinical outcome categories. Change in rTTP and change in percentage of pixels of rTTP (0 seconds to6 seconds) were significantly different for the clinical outcome categories.TTP and CBV perfusion maps can depict hemodynamic status after revascularization surgery in moyamoya disease. Furthermore, changes in TTP perfusion maps after revascularization surgery correspond with clinical outcome in patients with moyamoya disease.

To evaluate sonographic findings in ischemic enterocolitis (IEC) and correlate with pathologic findings in an experimental study.Ischemic enterocolitis was induced with ligation of the superior mesenteric artery in 20 rabbits. Plain radiography and ultrasonography (US) were performed. US was done hourly after the ligation using 10 MHz linear probe. US findings were categorized into 2 groups according to the bowel wall echogenicity; the echogenic dots (ED) group and the circumferential granular echogenicity (CGE) group. US findings were compared with the specimen radiography and the histopathology.On US, ED were seen in the bowel of all rabbits after SMA ligation (2.2 +/- 1.3 hours [standard deviation]) and CGE in 16 rabbits (4.1 +/- 0.9 hours). On the specimen radiographs, multiple radiolucent air bubbles were present. Comparing the ED and CGE group, histopathological findings revealed the CGE group had severer injury of the bowel wall than the ED group. On plain radiography, there was progressive bowel distention, but pneumatosis intestinalis (PI) was not evident.ED or CGE are the sonographic findings of ischemic enterocolitis, and bowel wall echogenicity might reflect the degree of ischemic injury.

Widespread use of fetal ultrasonography is accompanied by more frequent detection of antenatal hydronephrosis. Therefore, sonographic evaluation of neonates with a history of antenatal hydronephrosis is becoming more widespread. As an initial postnatal non-invasive imaging modality, ultrasonography is used to screen for persistence of hydronephrosis, determine the level and severity of obstruction, and contribute to appropriate diagnosis and treatment. This review aims to provide a practical overview of the sonographic evaluation of neonatal hydronephrosis and to describe the sonographic findings of conditions associated with hydronephrosis in the newborn.

Crohn's disease is a chronic idiopathic inflammatory disease of the intestines characterized by frequent relapse and remission. It often develops in children and adolescents, who are vulnerable to repeated exposure to ionizing radiations. Magnetic resonance enterography (MRE) is an increasingly important radiation-free imaging modality that is used to evaluate pediatric patients with Crohn's disease. MRE can evaluate extraluminal and extraintestinal abnormalities as well as the status of the bowel wall. In addition, MRE has an advantage in the evaluation of the small bowel involvement. MRE can be used for the initial diagnosis of Crohn's disease, and can aid in the assessment of disease activity and complications such as penetrating and fibrostenotic diseases. The aims of this article are to review the MRE technique for obtaining diagnostic and high-quality images and to discuss interpretations of imaging findings in patients with Crohn's disease.

Objective To assess the diagnostic value of various ultrasound (US) findings and to make a decision-tree model for US diagnosis of biliary atresia (BA). Materials and Methods From March 2008 to January 2014, the following US findings were retrospectively evaluated in 100 infants with cholestatic jaundice (BA, n = 46; non-BA, n = 54): length and morphology of the gallbladder, triangular cord thickness, hepatic artery and portal vein diameters, and visualization of the common bile duct. Logistic regression analyses were performed to determine the features that would be useful in predicting BA. Conditional inference tree analysis was used to generate a decision-making tree for classifying patients into the BA or non-BA groups. Results Multivariate logistic regression analysis showed that abnormal gallbladder morphology and greater triangular cord thickness were significant predictors of BA (p = 0.003 and 0.001; adjusted odds ratio: 345.6 and 65.6, respectively). In the decision-making tree using conditional inference tree analysis, gallbladder morphology and triangular cord thickness (optimal cutoff value of triangular cord thickness, 3.4 mm) were also selected as significant discriminators for differential diagnosis of BA, and gallbladder morphology was the first discriminator. The diagnostic performance of the decision-making tree was excellent, with sensitivity of 100% (46/46), specificity of 94.4% (51/54), and overall accuracy of 97% (97/100). Conclusion Abnormal gallbladder morphology and greater triangular cord thickness (> 3.4 mm) were the most useful predictors of BA on US. We suggest that the gallbladder morphology should be evaluated first and that triangular cord thickness should be evaluated subsequently in cases with normal gallbladder morphology.

OBJECTIVE. As complications of tuberculosis are frequent in infancy, correct diagnosis of tuberculosis in infants is important. The purposes of this study are to summarize radiographic and CT findings of pulmonary tuberculosis in infants and to determine the radiologic features frequently seen in infants with this disease.CONCLUSION. Frequent radiologic findings of pulmonary tuberculosis in infants are mediastinal or hilar lymphadenopathy with central necrosis and air-space consolidations, especially masslike consolidations with low-attenuation areas or cavities within the consolidation. Disseminated pulmonary nodules and airway complications are also frequently detected in this age group. CT is a useful diagnostic technique in infants with tuberculosis because it can show parenchymal lesions and tuberculous lymphadenopathy better than chest radiography. CT scans can also be helpful when chest radiographs are inconclusive or complications of tuberculosis are suspected.

Larsen syndrome is characterized by multiple congenital joint dislocations and a characteristic flattened facial appearance. The aetiology is unknown, but the syndrome is considered a probable connective tissue disorder and the cardiovascular manifestations are similar to those of Marfan syndrome. We describe an 8-year-old girl with the characteristic features of Larsen syndrome who was also affected by a saccular aneurysm of the descending aorta, just distal to the left subclavian artery. We present the radiological findings and review the literature.

Background: Nutcracker syndrome is characterized by gross haematuria caused by left renal vein (LRV) entrapment. Objective: To assess the role of LRV ultrasonography in the diagnosis of the nutcracker syndrome in children. Materials and methods: Twelve children (eight male, four female; mean age 12.8 years) with venographically confirmed nutcracker syndrome (LRV-IVC pressure gradient ≥3 mm Hg) underwent LRV sonography including Doppler spectral analysis (n=7). The diameter and peak velocity (PV) were measured at two sites of the LRV (renal hilum and aortomesenteric portion). The US findings of nutcracker syndrome were compared with those of 20 control subjects using the t-test. We identified the optimal cut-off value of the US parameters for the diagnosis of the nutcracker syndrome using ROC analysis. Results: The PV at the aortomesenteric portion and the ratio of the PV between the two measured points showed significant differences between the two groups (P

Background. Adenoid cystic carcinoma (ACC) of the Bartholin's gland is a rare malignancy of the female genital tract and there have been 62 cases of ACC of the Bartholin's gland in the literature. Cases. Two cases of ACC of the Bartholin's gland are reported. Conclusion. There is no consensus on optimal treatment of ACC of the Bartholin's gland. Most commonly, wide local excision and radical vulvectomy with or without lymph node dissection, are performed. More long-term follow up is recommended to evaluate optimal primary treatment and roles of radiotherapy and chemotherapy because ACC of the Bartholin's gland recurs and metastasizes long after primary treatment.

Background: The diagnosis of squalene aspiration pneumonia in children is often difficult because of minimal non-specific symptoms. Objective: To investigate the radiological findings of squalene aspiration pneumonia in children. Materials and methods: We reviewed the chest radiographs (n = 8) and CT scans (n = 7), including high-resolution CT (n = 3), of eight patients (four boys, four girls; age 3 months to 6 years) with squalene aspiration pneumonia. All patients presented minimal symptoms. Results: Chest radiographs showed right-sided predominantly parahilar infiltrations. The extent and the opacity of the lesions decreased slowly during the follow-up period (mean 5.4 months) after halting the exposure. On CT, affected areas appeared as dense consolidations surrounded by ground-glass opacities showing a crazy-paving pattern in a geographic lobular distribution in all patients. The lesions were predominantly in the right lung and dependent areas in all patients and extensively involved all pulmonary lobes in five patients. Conclusions: These radiological findings, although non-specific, can lead to an appropriate diagnosis, particularly when patients present few symptoms.

Subependymomas are rare, slow-growing, benign intraventricular tumors which occur commonly in the fourth or lateral ventricle. They usually occur in elderly men and often are asymptomatic and discovered incidentally. We report a case of a 6-year-old girl with a symptomatic subependymoma in the third ventricle with MR imaging.