Preoperative imaging findings to predict 2-year native liver survival after the Kasai procedure in patients with biliary atresia.

OBJECTIVES: To investigate the feasibility of using preoperative imaging indices to predict 2-year native liver survival after the Kasai procedure in patients with biliary atresia (BA). MATERIALS AND METHODS: The retrospective review included 190 BA patients who underwent the Kasai procedure between 2000 and 2020, with preoperative US and/or MRI, excluding cases with less than 2-year follow-up period. Multivariable logistic regression analysis was performed to identify imaging indices to predict 2-year native liver survival. Kasai failure was defined as the need for liver transplantation or death within 2 years of the Kasai procedure. RESULTS: Of the 90 patients included, all had preoperative US, and 61 also had MRI. Kasai failure occurred in 52% (47/90). Preoperative US identified gallbladder length (OR 0.40, 95% CI 0.17-0.95, p = 0.039; cutoff 1.6 cm, AUC 67.66) and biliary cysts (OR 24.64, 95% CI 1.97-308.08, p = 0.013) as significant Kasai failure predictors, with a combined accuracy of 73% (60/82). For patients having both preoperative US and MRI, significant predictors were hepatic artery diameter (OR 6.75, 95% CI 1.31-34.88, p = 0.023; cutoff 2 mm, AUC 73.83) and biliary cysts (OR 23.89, 95% CI 1.43-398.82, p = 0.027) on US, and gallbladder length (OR 0.25, 95% CI 0.08-0.76, p = 0.014; cutoff 1.2 cm, AUC 74.72) and spleen size (OR 2.53, 95% CI 1.02-6.29, p = 0.045; cutoff 6.9 cm, AUC 73.72) on MRI, with a combined accuracy of 85% (52/61). CONCLUSION: Preoperative US and/or MRI enhance the 2-year native liver survival prediction in BA patients after the Kasai procedure. CLINICAL RELEVANCE STATEMENT: BA patients with hepatic artery diameter > 2 mm (US), gallbladder length < 1.6 cm (US) or < 1.2 cm (MRI), spleen size > 6.9 cm (MRI), and absence of biliary cysts (US/MRI) have a decreased likelihood of 2-year native liver survival. KEY POINTS: • Preoperative US and/or MRI can predict the probability of achieving 2-year native liver survival following the Kasai procedure. • Combining US and MRI improved the accuracy to 85% for predicting 2-year native liver survival in BA patients. • The hepatic artery diameter > 2 mm (US), gallbladder length < 1.6 cm (US) or < 1.2 cm (MRI), spleen size > 6.9 cm (MRI), and no biliary cysts (US/MRI) are significant predictors of Kasai failure in patients with biliary atresia.

Superb Microvascular Imaging in Pediatric Focal Nodular Hyperplasia.

OBJECTIVE: To investigate superb microvascular imaging (SMI), a novel Doppler ultrasound technique that can visualize low-velocity microvascular flow, for assessing pediatric focal nodular hyperplasia (FNH). PATIENTS AND METHODS: Nine FNH lesions in 6 patients were enrolled. On SMI and color Doppler imaging (CDI), intralesional vascularity was assessed visually and categorized as typical spoke-wheel pattern (central vessel radiating from the center to the periphery), multifocal spoke-wheel pattern, and nonspecific pattern. We compared the vascular features of the lesions between SMI and CDI and evaluated vascular patterns according to lesion size. RESULTS: In terms of vascularity pattern, the typical spoke-wheel pattern of FNH was noted more frequently on SMI (67%) than on CDI (11%; P < 0.05). In addition, a multifocal spoke-wheel pattern was noted in all remaining lesions (33%) on SMI. On the contrary, a nonspecific vascular pattern was detected in the majority (78%) of CDI. Regarding the lesion size and vascularity on SMI, the typical spoke-wheel pattern was seen more frequently in the small FNH group than in the large FNH group. The intralesional vascular signal was detected more frequently on SMI (100%) than on CDI (89%). CONCLUSION: SMI is feasible in evaluating FNH in children and has a greater ability to demonstrate the spoke-wheel pattern than CDI.

Differentiation of autoimmune pancreatitis from pancreatic adenocarcinoma using CT characteristics: a systematic review and meta-analysis.

OBJECTIVES: To determine informational CT findings for distinguishing autoimmune pancreatitis (AIP) from pancreatic ductal adenocarcinoma (PDAC) and to review their diagnostic accuracy. METHODS: A systematic and detailed literature review was performed through PubMed, EMBASE, and the Cochrane library. Similar descriptors to embody the identical image finding were labeled as a single CT characteristic. We calculated the pooled diagnostic odds ratios (DORs) of each CT characteristic using a bivariate random-effects model. RESULTS: A total of 145 various descriptors from 15 studies (including 562 AIP and 869 PDAC patients) were categorized into 16 CT characteristics. According to the pooled DOR, 16 CT characteristics were classified into three groups (suggesting AIP, suggesting PDAC, and not informational). Seven characteristics suggesting AIP were diffuse pancreatic enlargement (DOR, 48), delayed homogeneous enhancement (DOR, 46), capsule-like rim (DOR, 34), multiple pancreatic masses (DOR, 16), renal involvement (DOR, 15), retroperitoneal fibrosis (DOR, 13), and bile duct involvement (DOR, 8). Delayed homogeneous enhancement showed a pooled sensitivity of 83% and specificity of 85%. The other six characteristics showed relatively low sensitivity (12-63%) but high specificity (93-99%). Four characteristics suggesting PDAC were discrete pancreatic mass (DOR, 23), pancreatic duct cutoff (DOR, 16), upstream main pancreatic duct dilatation (DOR, 8), and upstream parenchymal atrophy (DOR, 7). CONCLUSION: Eleven CT characteristics were informational to distinguish AIP from PDAC. Diffuse pancreatic enlargement, delayed homogeneous enhancement, and capsule-like rim suggested AIP with the highest DORs, whereas discrete pancreatic mass suggested PDAC. However, pooled sensitivities of informational CT characteristics were moderate. CLINICAL RELEVANCE STATEMENT: This meta-analysis underscores eleven distinctive CT characteristics that aid in differentiating autoimmune pancreatitis from pancreatic adenocarcinoma, potentially preventing misdiagnoses in patients presenting with focal/diffuse pancreatic enlargement. KEY POINTS: • Diffuse pancreatic enlargement (pooled diagnostic odds ratio [DOR], 48), delayed homogeneous enhancement (46), and capsule-like rim (34) were CT characteristics suggesting autoimmune pancreatitis. • The CT characteristics suggesting autoimmune pancreatitis, except delayed homogeneous enhancement, had a general tendency to show relatively low sensitivity (12-63%) but high specificity (93-99%). • Discrete pancreatic mass (pooled diagnostic odds ratio, 23) was the CT characteristic suggesting pancreatic ductal adenocarcinoma with the highest pooled DORs.

Pleuroparenchymal fibroelastosis after hematopoietic stem cell transplantation in children: a propensity score-matched analysis.

OBJECTIVES: To investigate the incidence, risk factors, and clinical outcomes of pleuroparenchymal fibroelastosis (PPFE) in pediatric hematopoietic stem cell transplantation (HSCT) recipients. METHODS: This single-center, retrospective, case-control study included 738 consecutive patients who underwent chest CT more than 3 months after HSCT. We identified patients who fulfilled the diagnostic criteria for PPFE and assessed their clinical characteristics and radiologic findings. Propensity score-matched analysis was performed using four covariates (age, sex, HSCT type, and primary disease). The risk factors and clinical outcomes of PPFE were analyzed using the Fine and Gray regression model and stratified log-rank test in the matched groups. RESULTS: PPFE was identified in 4% (31/738, 8.3 ± 3.1 years, 15 males) of the pediatric HSCT recipients with a median time of 2.7 years after HSCT, and it occurred following allogeneic (5%, 15/317), autologous (4%, 15/379), or both (2%, 1/42). Matching yielded 30 and 130 cases in the PPFE and control groups, respectively. The PPFE group showed more frequent late-onset noninfectious pulmonary complications (LONIPCs) and pneumonia more than 3 months after HSCT (p < 0.05). Multivariable analysis showed a significantly higher risk of PPFE in HSCT recipients who had pneumonia more than 3 months after HSCT (hazard ratio = 10.78 [95% confidence interval: 4.29, 27.13], p < 0.001). The PPFE group showed higher mortality (73%, 22/30) and poorer median overall survival (6.8 years [95% confidence interval: 4.1, 9.5]) than the control group (p < 0.001). CONCLUSIONS: PPFE represents a severe type of LONIPC after HSCT. HSCT recipients with pneumonia after HSCT may have an increased risk of PPFE. KEY POINTS: • The incidence of pleuroparenchymal fibroelastosis is not negligible (4%), and it can occur after either allogeneic or autologous hematopoietic stem cell transplantation. • Pleuroparenchymal fibroelastosis after hematopoietic stem cell transplantation showed poor outcome with a high mortality rate of 73% and median overall survival of 6.8 years. • After hematopoietic stem cell transplantation, pneumonia may increase the risk of pleuroparenchymal fibroelastosis development in children. • Lung biopsy should not be indicated in patients with pleuroparenchymal fibroelastosis findings on chest CT as it can cause refractory pneumothorax without helping the diagnosis.

Effectiveness and complication rates of percutaneous transhepatic fluoroscopy-guided management of common bile duct stones: a single-arm meta-analysis.

OBJECTIVES: To perform a systematic review and meta-analysis to determine the success and complication rate of percutaneous transhepatic fluoroscopy-guided management (PTFM) for the removal of common bile duct stones (CBDS). METHODS: A comprehensive literature search of multiple databases was conducted to identify original articles published between January 2010 and June 2022, reporting the success rate of PTFM for the removal of CBDS. A random-effect model was used to summarize the pooled rates of success and complications with 95% confidence intervals (CIs). RESULTS: Eighteen studies involving 2554 patients met the inclusion criteria and were included in the meta-analysis. Failed or infeasible endoscopic management was the most common indication of PTFM. The meta-analytic summary estimates of PTFM for the removal of CBDS were as follows: rate of overall stone clearance 97.1% (95% CI, 95.7-98.5%); stone clearance at first attempt 80.5% (95% CI, 72.3-88.6%); overall complications 13.8% (95% CI, 9.7-18.0%); major complications 2.8% (95% CI, 1.4-4.2%); and minor complications 9.3% (95% CI, 5.7-12.8%). Egger's tests showed the presence of publication bias with respect to the overall complications (p = 0.049). Transcholecystic management of CBDS had an 88.5% pooled rate for overall stone clearance (95% CI, 81.2-95.7%), with a 23.0% rate for complications (95% CI, 5.7-40.4%). CONCLUSION: The systematic review and meta-analysis answer the questions of the overall stone clearance, clearance at first attempt, and complication rate of PTFM by summarizing the available literature. Percutaneous management could be considered in cases with failed or infeasible endoscopic management of CBDS. CLINICAL RELEVANCE STATEMENT: This meta-analysis highlights the excellent stone clearance rate achieved through percutaneous transhepatic fluoroscopy-guided removal of common bile duct stones, potentially influencing clinical decision-making when endoscopic treatment is not feasible. KEY POINTS: • Percutaneous transhepatic fluoroscopy-guided management of common bile duct stones had a pooled rate of 97.1% for overall stone clearance and 80.5% for clearance at the first attempt. • Percutaneous transhepatic management of common bile duct stones had an overall complication rate of 13.8%, including a major complication rate of 2.8%. • Percutaneous transcholecystic management of common bile duct stones had an overall stone clearance rate of 88.5% and a complication rate of 23.0%.

Systematic review and meta-analysis of MRI features for differentiating autoimmune pancreatitis from pancreatic adenocarcinoma.

OBJECTIVES: To identify reliable MRI features for differentiating autoimmune pancreatitis (AIP) from pancreatic ductal adenocarcinoma (PDAC) and to summarize their diagnostic accuracy. METHODS: We conducted a systematic literature review and meta-analysis using PubMed, EMBASE, and the Cochrane Library to identify original articles published between January 2006 and July 2021. The pooled diagnostic accuracy, including the diagnostic odds ratios (DORs) with 95% confidence intervals (CIs) of the identified features, was calculated using a bivariate random effects model. RESULTS: Twelve studies were included, and 92 overlapping descriptors were subsumed under 16 MRI features. Ten features favoring AIP were diffuse enlargement (DOR, 75; 95% CI, 9-594), capsule-like rim (DOR, 52; 95% CI, 20-131), multiple main pancreatic duct (MPD) strictures (DOR, 47; 95% CI, 17-129), homogeneous delayed enhancement (DOR, 46; 95% CI, 21-104), low apparent diffusion coefficient value (DOR, 30), speckled enhancement (DOR, 30), multiple pancreatic masses (DOR, 29), tapered narrowing of MPD (DOR, 15), penetrating duct sign (DOR, 14), and delayed enhancement (DOR, 13). Six features favoring PDAC were target type enhancement (DOR, 41; 95% CI, 11-158), discrete pancreatic mass (DOR, 35; 95% CI, 15-80), upstream MPD dilatation (DOR, 13), peripancreatic fat infiltration (DOR, 10), upstream parenchymal atrophy (DOR, 5), and vascular involvement (DOR, 3). CONCLUSION: This study identified 16 informative MRI features to differentiate AIP from PDAC. Among them, diffuse enlargement, capsule-like rim, multiple MPD strictures, and homogeneous delayed enhancement favored AIP with the highest DORs, whereas discrete mass and target type enhancement favored PDAC. KEY POINTS: • The MRI features with the highest pooled diagnostic odds ratios (DORs) for autoimmune pancreatitis were diffuse enlargement of the pancreas (75), capsule-like rim (52), multiple strictures of the main pancreatic duct (47), and homogeneous delayed enhancement (46). • The MRI features with the highest pooled DORs for pancreatic ductal adenocarcinoma were target type enhancement (41) and discrete pancreatic mass (35).

Systematic review and meta-analysis of percutaneous transluminal forceps biopsy for diagnosing malignant biliary strictures.

OBJECTIVES: To perform a systematic review and meta-analysis to determine the diagnostic performance of percutaneous transluminal forceps biopsy (PTFB) for differentiating malignant from benign biliary stricture. METHODS: A comprehensive literature search of the PubMed, EMBASE, and Ovid MEDLINE databases was conducted to identify original articles published between January 2001 and January 2021 reporting the diagnostic accuracy of PTFB. A random-effects model was used to summarize the diagnostic odds ratio and other measures of accuracy. RESULTS: Fourteen studies involving 1762 patients met the inclusion criteria and were included in the meta-analysis. The meta-analysis summary estimates of PTFB for diagnosis of malignant biliary strictures were as follows: sensitivity 81% (95% confidence interval [CI], 78-81%); specificity 100% (95% CI, 98-100%); diagnostic odds ratio 85.34 (95% CI, 38.37-189.81). The area under the curve of PTFB was 0.948 in the diagnosis of malignant biliary strictures. The diagnostic sensitivity was higher in intrinsic (85%) than in extrinsic (73%) biliary strictures. The pooled rate of all complications was 10.3% (95% CI, 7.0-14.2%), including a major complication rate of 3.1%. CONCLUSION: These data demonstrate that PTFB is sensitive and highly specific for diagnosing malignancy in biliary strictures. PTFB should be incorporated into future guidelines for tissue sampling in biliary cancer, especially in cases with failed endoscopic management. KEY POINTS: • PTFB had a good overall diagnostic performance for differentiating malignant from benign biliary strictures, with a meta-analysis summary estimate of 81% for sensitivity and 100% for specificity. • PTFB had higher sensitivity for cholangiocarcinoma (85%) than for other cancers (73%). • PTFB had a 100% technical success rate and a 10.3% rate for complications, including a 3.1% rate for major complications.

Gd-EOB-DTPA MRI for focal nodular hyperplasia-like lesions in pediatric cancer survivors.

OBJECTIVES: To investigate the hepatobiliary enhancement patterns of gadoxetic acid (Gd-EOB-DTPA) MRI and the temporal evolution of focal nodular hyperplasia (FNH)-like lesions in pediatric cancer survivors. METHODS: We retrospectively included pediatric cancer survivors who had new liver lesions detected during surveillance imaging and who were diagnosed with FNH-like lesions by Gd-EOB-DTPA MRI without the aid of a hepatobiliary phase. The hepatobiliary enhancement patterns of FNH-like lesions were categorized as homogeneous hyperintense/isointense, heterogeneous hyperintense, and ring-like enhancement. Temporal changes in the FNH-like lesions were evaluated by follow-up Gd-EOB-DTPA MRI. Statistical analyses included one-way analysis of variance and Spearman's rank correlation test. RESULTS: A total of 132 radiologically diagnosed FNH-like lesions in 18 patients showed the three different hepatobiliary enhancement patterns: homogeneous hyperintense/isointense (n = 65, 49%), heterogeneous hyperintense (n = 24, 18%), and ring-like enhancement (n = 43, 33%). A weak positive correlation was found between the lesion size and the hepatobiliary enhancement pattern (p = 0.015). Follow-up MRI showed alterations in the size and number of 55 FNHs in 8 patients, including stable size (n = 15, 27%), increased size (n = 17, 31%), decreased size (n = 11, 20%), disappearance (n = 12, 22%), and 74 new lesions (5 patients, 63%). CONCLUSIONS: FNH-like lesions in pediatric cancer survivors showed three different hepatobiliary enhancement patterns and various temporal changes. Recognition of the radiological characteristics of FNH-like lesions may avoid unnecessary invasive procedures and reduce patients/parents' anxiety. KEY POINTS: • FNH-like lesions in pediatric cancer survivors showed three different hepatobiliary enhancement patterns. The most common was homogeneous hyperintense/isointense, followed by heterogeneous hyperintense, and ring-like enhancement. • FNH-like lesions in pediatric cancer survivors can show various temporal changes during follow-up.

Evaluation of the image quality and dose reduction in digital radiography with an advanced spatial noise reduction algorithm in pediatric patients.

OBJECTIVES: To evaluate whether the advanced spatial noise reduction (ASNR) algorithm installed in a digital radiography system generates acceptable images at a lower dose than a conventional denoising algorithm in pediatric patients. METHODS: Nine sets of 30 images of pediatric patients, classified under three protocols and three age groups, were retrospectively selected. Different levels of low-dose image sets of these 270 images were generated by a noise simulation tool after validation testing using phantoms. Each image set was obtained with both the ASNR and conventional algorithm, and grouped randomly and blinded. Three experienced pediatric radiologists were asked to pick the "image with optimum dose" among images of different dose levels with an ALARA (as low as reasonably achievable) perspective. Dose reduction rates for each protocol and age group were calculated, and entrance skin exposure (ESE) was calculated using the values of kVp and mAs, assuming a standard body depth for each age group. RESULTS: With the ASNR algorithm, estimated dose reduction rates were highest for abdominal radiographs (45.0%, 27.3%, and 24.3% in infants, children, and adolescents, respectively, p < 0.001). The mean dose reductions for all age groups in the abdominal, chest, and skull radiographs were 32.8%, 12.9%, and 23.2%, respectively (p < 0.001). Average of the calculated ESE was lower with the ASNR algorithm than with the conventional algorithm group (p < 0.001). CONCLUSIONS: The ASNR algorithm facilitated optimization of image quality with a higher reduction in radiation dose than the conventional algorithm, making it more acceptable for use in pediatric patients. KEY POINTS: •ASNR algorithm in DR system improves image quality via enhanced contrast and noise removal by estimating actual noise distribution based on a multi-scale noise covariance and frequency processing. •Noise simulation tool (NST) generating images of different dose levels can be used for evaluation of the optimum dose without unnecessary additional radiation exposure to pediatric patients. •Retrospective clinical study using NST showed that the ASNR algorithm enabled a higher reduction in radiation dose than the conventional algorithm in pediatric patients.

Structural MRI and tract-based spatial statistical analysis of diffusion tensor imaging in children with hemimegalencephaly.

PURPOSE: To investigate the gross white matter abnormalities in the structural brain MR imaging as well as white matter microstructural alterations using tract-based spatial statistics (TBSS) analysis of diffusion tensor imaging (DTI) in both affected and contralateral cerebral hemispheres of children with hemimegalencephaly (HMEG). METHODS: From 2003 to 2019, we retrospectively reviewed brain MR images in 20 children (11 boys, 2 days-16.5 years) with HMEG, focusing on gross white matter abnormalities. DTI was evaluated in 12 patients (8 boys, 3 months-16.5 years) with HMEG and 12 age-, sex-, and magnetic field strength-matched control subjects. TBSS analysis was performed to analyze main white matter tracts. Regions of significant differences in fractional anisotropy (FA) were determined between HMEG and control subjects and between affected and contralateral hemispheres of HMEG. RESULTS: Gross white matter abnormalities were noted in both affected (n = 20, 100%) and contralateral hemisphere (n = 4, 20%) of HMEG. FA values were significantly decreased in both hemispheres of HMEG, compared with control subjects (P < 0.05). Contralateral hemispheres of HMEG showed regions with significantly decreased FA values compared with affected hemispheres (P < 0.05). CONCLUSIONS: In addition to gross white matter abnormalities particularly evident in affected hemispheres, DTI analysis detected widespread microstructural alterations in both affected and contralateral hemispheres in HMEG suggesting HMEG may involve broader abnormalities in neuronal networks.

Outcome and risk factors associated with perirenal subcapsular fluid collections in extremely preterm infants with acute kidney injury.

OBJECTIVES: To investigate the incidence of, clinical outcome of, and risk factors for perirenal subcapsular fluid collections in extremely preterm infants with acute kidney injury (AKI). METHODS: Extremely preterm infants with AKI who underwent renal ultrasonography (US) during neonatal intensive care unit stay were classified into two groups according to the presence of a perirenal subcapsular fluid collection at US. Clinical outcome was compared, and relevant data were analysed, including demographics and comorbidities of the infants, as well as maternal demographics. The risk factor of perirenal subcapsular fluid in infants with AKI was tested with univariate and multivariate logistic regression analysis. RESULTS: A perirenal subcapsular fluid collection was detected in 7 of 56 (13%) extremely preterm infants with AKI (male to female ratio, 5:2; mean gestational age, 23.6 ± 1.4 weeks) and it appeared bilaterally in most cases (86%, 6/7). The mortality rate was higher in infants with perirenal subcapsular fluid collections and AKI (86%, 6/7) than with AKI alone (35%, 17/49) (p = 0.015). Infants with perirenal subcapsular fluid collections and AKI were of a lower gestational age, and more frequently showed episodes of intestinal perforation, use of medication having potential to impair renal function, and a history of maternal chorioamnionitis (p < 0.05). Multivariate analysis revealed a significantly higher risk for perirenal subcapsular fluid collections in extremely preterm infants who were treated with anti-fungal agents (OR, 13.2 (95% CI: 1.5, 119.4); p = 0.022). CONCLUSIONS: Although a perirenal subcapsular fluid collection occurred in a small proportion of extremely preterm infants with AKI, its presence was associated with high mortality. The use of anti-fungal agents was an independent risk factor for a perirenal subcapsular fluid collection. KEY POINTS: • A perirenal subcapsular fluid collection may occur in association with acute kidney injury. • A perirenal subcapsular fluid collection has a grave prognostic implication in extremely preterm infants. • The use of anti-fungal agent might be associated with perirenal subscapular fluid collections in critically ill extremely preterm infants with AKI.

Early US findings of biliary atresia in infants younger than 30 days.

PURPOSE: To investigate and compare ultrasound (US) findings for the diagnosis of biliary atresia (BA) in infants younger than 30 days with those of infants older than 30 days. MATERIALS AND METHODS: From 2000 to 2015, we reviewed hepatobiliary US images in 12 BA infants younger than 30 days (younger BA group) and 62 BA infants older than 30 days (older BA group) before Kasai procedure. Eight (67%) of younger BA group underwent follow-up US examinations before Kasai procedure. Our review of the images focused on triangular cord sign, gallbladder (GB) abnormalities, vascular changes, and signs of portal hypertension. RESULTS: The triangular cord sign was present in 17% of younger BA group and in 56% of older BA group (P=.024). GB abnormalities were commonly identified in both groups. The hepatic artery diameter was significantly smaller in younger BA group than in older BA group (P<.001). Signs of portal hypertension were less common in younger BA group (17%) than in older BA group (84%) (P<.001). Follow-up US of two infants in younger BA group showed a new appearance of the triangular cord sign. CONCLUSION: BA infants younger than 30 days showed atypical US findings compared with those older than 30 days. KEY POINTS: • BA infants younger than 30 days show atypical US findings. • GB abnormalities were common in both younger and older BA group. • Subsequent US examination may be helpful to diagnose BA in young infants.

Baseline Wall Thickness Is Lower in Mucosa-Healed Segments 1 Year After Infliximab in Pediatric Crohn Disease Patients.

OBJECTIVES: We aimed to quantitatively investigate the therapeutic response to combined immunosuppression treatment by magnetic resonance enterography (MRE) in active luminal Crohn disease (CD) in the pediatric population. METHODS: Pediatric patients with moderate-to-severe luminal CD, who received scheduled infliximab and azathioprine, were included in this preliminary study. Ileocolonoscopy and MRE were performed at baseline and at 1 year, and Simple Endoscopic Score for Crohn's Disease (SES-CD) and Magnetic Resonance Index of Activity (MaRIA) scores were calculated. The correlation between SES-CD and MaRIA scores were investigated with analysis per person and per segment. RESULTS: A total of 167 segments from 17 patients were evaluated by both Ileocolonoscopy and MRE. SES-CD and MaRIA scores showed significant correlations on both per-person analysis (ρ = 0.699, P < 0.001) and per-segment analysis (ρ = 0.596, P < 0.001). Analysis according to ileocolonic location of each segment revealed that the correlation strength was strongest in the right colon (ρ = 0.653, P < 0.001), whereas the correlation in the rectum was statistically insignificant (ρ = 0.29, P = 0.096). A comparative analysis of MaRIA components revealed a significantly thinner bowel wall thickness at baseline in endoscopically healed segments (50/65) compared with unhealed segments (15/65) (median 4.3 vs 7.2 mm, P = 0.036). CONCLUSIONS: Therapeutic response to combined immunosuppression at 1 year assessed by MRE correlates with ileocolonoscopy in pediatric patients with CD. Bowel wall thickness of the involved segments at baseline may affect treatment response to combined immunosuppression.

Neuroplasticity for spontaneous functional recovery after neonatal hypoxic ischemic brain injury in rats observed by functional MRI and diffusion tensor imaging.

For infants and children, an incredible resilience from injury is often observed. There is growing evidence that functional recovery after brain injury might well be a consequence of the reorganization of the neural network as a process of neuroplasticity. We demonstrate the presence of neuroplasticity at work in spontaneous recovery after neonatal hypoxic ischemic (HI) injury, by elucidating a precise picture in which such reorganization takes place using functional MRI techniques. For all 12 siblings, 6 rats were subjected to severe HI brain injury and 6 rats underwent sham operation only. Severe HI brain injury was induced to postnatal day 7 (p7) Sprague-Dawley rats according to the Rice-Vannucci model (right carotid artery occlusion followed by 150min of hypoxia with 8% O2 and 92% of N2). Brain activation maps along with anatomical and functional connectivity maps related to the sensory motor function were obtained at adult (p63) using blood oxygen level dependent (BOLD)-functional MRI (fMRI), resting state-functional MRI (rs-fMRI) and diffusion tensor imaging (DTI); each of these MRI data was related to sensory motor functional outcome. In-depth investigation of the functional MRI data revealed: 1) intra-hemispheric expansion of BOLD signal activation in the contralesional undamaged hemisphere for ipsilesional forepaw stimuli to include the M2 and Cg1 in addition to the S1 and M1 wide spreading in the anterior and posterior directions, 2) inter-hemispheric transfer of BOLD signal activation for contralesional forepaw stimuli, normally routed to the injured hemisphere, to analogous sites in the contralesional undamaged hemisphere, localized newly to the M1 and M2 with a reduced portion of the S1, 3) inter-hemispheric axonal disconnection and axonal rewiring within the undamaged hemisphere as shown through DTI, and 4) increased functional interactions within the cingulate gyrus in the HI injured rats as shown through rs-fMRI. The BOLD signal amplitudes as well as DTI and rs-fMRI data well correlate with behavioral tests (tape to remove). We found that function normally utilizing what would be the injured hemisphere is transferred to the uninjured hemisphere, and functionality of the uninjured hemisphere remains not untouched but is also rewired in an expansion corresponding to the newly formed sensorimotor function from both the contralesional and the ipsilesional sides. The conclusion drawn from the data in our current study is that enhanced motor function in the contralesional hemisphere governs both the normal and damaged sides, indicating that active plasticity with brain laterality was spontaneously generated to overcome functional loss and established autonomously through normal experience via modification of neural circuitry for neonatal HI injured brain.

Congenital Adrenal Neuroblastoma With and Without Cystic Change: Differentiating Features With an Emphasis on the of Value of Ultrasound.

OBJECTIVE: The purpose of this study was to compare the features of congenital adrenal neuroblastomas with and without cystic changes and to emphasize the value of ultrasound in the diagnostic evaluation of cystic congenital adrenal neuroblastoma. MATERIALS AND METHODS: A total of 41 patients with surgically confirmed congenital adrenal neuroblastoma were enrolled. We divided the patients into two groups according to presence or absence of cystic change in the tumor, as determined from the initial ultrasound findings. Clinical and laboratory findings, disease stage, and patient outcome were investigated with a statistical comparison between the two groups. The imaging findings for cystic congenital adrenal neuroblastoma were reviewed to compare the additional diagnostic value of CT and MRI when paired with ultrasound. RESULTS: There were 22 patients (54%) in the group without cystic changes and 19 patients (46%) in the group with cystic changes. Prenatal detection and absence of metastasis were significantly more common in the cystic group than in the noncystic group (p < 0.05). Sensitivities of tumor marker levels were also significantly lower in the cystic group. Patient outcome was excellent, and there was no significant difference between the groups. With regard to imaging of cystic congenital adrenal neuroblastoma, in the 15 cases in which CT or MRI was paired with ultrasound, no additional diagnostic information was discerned with CT or MRI. CONCLUSION: Nearly one-half of congenital adrenal neuroblastomas are cystic, and these tumors have clinical and laboratory features that distinguish them from noncystic congenital adrenal neuroblastoma. Diagnostic tests, including CT, MRI, and assessment of tumor markers, have low diagnostic value in the evaluation of cystic congenital adrenal neuroblastoma.

Diagnosis of neonatal ovarian torsion: Emphasis on prenatal and postnatal sonographic findings.

PURPOSE: Our aim was to retrospectively review the imaging findings of patients with neonatal ovarian torsion, emphasizing prenatal and postnatal sonographic findings. METHODS: Eleven patients who had had neonatal ovarian torsion diagnosed surgically (n = 9) or clinicoradiologically (n = 2) were enrolled. Prenatal and postnatal sonographic features, including sequential postnatal change, were reviewed. Clinical and pathologic features were also investigated. RESULTS: All patients except one had a fetal ovarian cyst (mean, 5.3 cm) detected on third-trimester sonography, either simple (n = 6) or complex (n = 4). In all 11 patients, initial postnatal sonography had revealed a complex cyst (mean, 4.7 cm) with intracystic clot or debris, the double-wall sign, a fluid-fluid level, and multiple septation. None of the patients had had symptoms or signs related to the ovarian torsion. Follow-up sonography in seven patients had revealed increased echogenicity of the cyst wall with frequent calcification and a decrease in size of the cyst. In two patients, the interval of the change in cyst position was noted, and autoamputation of the torsed ovary had been surgically confirmed. Serous cystadenoma had been identified in one patient. CONCLUSIONS: Neonatal ovarian torsion most commonly manifests as an asymptomatic complex cyst on sonography due to torsion of a fetal ovarian cyst. Serial monitoring of a fetal ovarian cyst for its resolution or changes in its appearance is mandatory for making an early diagnosis of torsion. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 44:290-297, 2016.

Clinical and imaging features of focal nodular hyperplasia in children.

OBJECTIVE: The purpose of this article is to review the clinical and imaging features of focal nodular hyperplasia (FNH) developed in children. MATERIALS AND METHODS: At a single institution, pediatric patients who underwent imaging studies and who had pathologically proven FNH were studied. Clinical characteristics, including presenting symptoms and signs and the presence of underlying disease, were reviewed from the medical records. Imaging features of FNHs, including the number, size, ultrasound echogenicity and vascularity, CT attenuation, MRI signal intensity and enhancement pattern, and the presence of a central scar, were evaluated. RESULTS: Twenty-five patients (11 boys and 14 girls; median age, 8.6 years) were found to have a solitary (n = 23) or multiple (n = 2) FNH lesions with a mean size of 4.9 cm (range, 1-10 cm). Multiple lesions were associated with small size of the lesions and history of malignancy treated by chemotherapy. Most patients were asymptomatic (n = 22). Biliary atresia was the most common underlying disease (n = 5). On ultrasound, FNHs most commonly appeared to be isoechoic and hypervascular. On dynamic CT and MRI, strong enhancement on the arterial phase and becoming isoattenuated or of isointense signal intensity on the portal or delayed phase was common. A central scar was usually noted in large lesions in about half the cases. CONCLUSION: Pediatric FNH is uncommon and usually is found incidentally in otherwise healthy children. However, it may occur in children who have underlying diseases, including biliary atresia. In addition, it can be encountered during surveillance of childhood cancer survivors with less common imaging features, including lack of a central scar and multiplicity.

Devastating metabolic brain disorders of newborns and young infants.

Metabolic disorders of the brain that manifest in the neonatal or early infantile period are usually associated with acute and severe illness and are thus referred to as devastating metabolic disorders. Most of these disorders may be classified as organic acid disorders, amino acid metabolism disorders, primary lactic acidosis, or fatty acid oxidation disorders. Each disorder has distinctive clinical, biochemical, and radiologic features. Early diagnosis is important both for prompt treatment to prevent death or serious sequelae and for genetic counseling. However, diagnosis is often challenging because many findings overlap and may mimic those of more common neonatal conditions, such as hypoxic-ischemic encephalopathy and infection. Ultrasonography (US) may be an initial screening method for the neonatal brain, and magnetic resonance (MR) imaging is the modality of choice for evaluating metabolic brain disorders. Although nonspecific imaging findings are common in early-onset metabolic disorders, characteristic patterns of brain involvement have been described for several disorders. In addition, diffusion-weighted images may be used to characterize edema during an acute episode of encephalopathy, and MR spectroscopy depicts changes in metabolites that may help diagnose metabolic disorders and assess response to treatment. Imaging findings, including those of advanced MR imaging techniques, must be closely reviewed. If one of these rare disorders is suspected, the appropriate biochemical test or analysis of the specific gene should be performed to confirm the diagnosis.

Value of CT for ERCP Endoscopists to Identify the Type of Gastroenteric Anastomosis in Patients with Previous Subtotal Gastrectomy.

UNLABELLED: Background/Aims: The aim of this study was to evaluate whether endoscopic retrograde cholangiopancreatography (ERCP) endoscopists can distinguish the type of gastroenteric anastomosis in patients with previous subtotal gastrectomy based on CT findings, particularly in biliary emergencies. METHODOLOGY: A total of 70 abdominal CT scans from patients who had undergone gastrectomy (n = 36, Billroth I; n = 34, Billroth II) were enrolled. The shuffled images were reviewed by 3 ERCP endoscopists blinded to clinical data. The endoscopists were asked to provide the most probable type of anastomosis. The sensitivity, specificity and interobserver agreement were analyzed for identifying Billroth II gastrectomy. RESULTS: The ERCP endoscopists were able to identify the type of anastomosis based on CT findings with a sensitivity, specificity and interobserver agreement of 100%, 97.2%, and 0.98, respectively. The key CT features for distinguishing Billroth II gastrectomy from Billroth I gastrectomy were: i) loss of continuity between the remnant stomach and duodenum; ii) less distended duodenal bulb; iii) the presence of a closed duodenal stump with surgical staples and iv) the presence of continuity between the remnant stomach and the jejunum. CONCLUSIONS: ERCP endoscopists were able to use CT findings to distinguish the type of gastroenteric anastomosis in patients with previous gastrectomy.