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Regional anatomical structures of the brain are intimately connected to functions corresponding to specific regions and the temporospatial pattern of genetic expression and their functions from the fetal period to old age. Therefore, quantitative brain morphometry has often been employed in neuroscience investigations, while controlling for the scanner effect of the scanner is a critical issue for ensuring accuracy in brain morphometric studies of rare orphan diseases due to the lack of normal reference values available for multicenter studies. This study aimed to provide across-site normal reference values of global and regional brain volumes for each sex and age group in children and adolescents. We collected magnetic resonance imaging (MRI) examinations of 846 neurotypical participants aged 6.0-17.9 years (339 male and 507 female participants) from 5 institutions comprising healthy volunteers or neurotypical patients without neurological disorders, neuropsychological disorders, or epilepsy. Regional-based analysis using the CIVET 2.1.0. pipeline provided regional brain volumes, and the measurements were across-site combined using ComBat-GAM harmonization. The normal reference values of global and regional brain volumes and lateral indices in our study could be helpful for evaluating the characteristics of the brain morphology of each individual in a clinical setting and investigating the brain morphology of ultra-rare diseases.

BACKGROUND: Focal cerebral arteriopathy (FCA) is a common cause of childhood arterial ischemic stroke in previously healthy children. Although its mechanisms are poorly understood, recent studies have suggested inflammatory processes. Magnetic resonance vessel wall imaging (VWI) is a potential imaging biomarker of inflammation. CASE DESCRIPTION: We describe the case of a 7-year-old Japanese girl with right hemiplegia and dysarthria for 3 days. Brain MRI showed acute infarct in the left basal ganglia, and MRA and conventional cerebral angiogram detected vascular stenosis in the left distal internal carotid artery, left M1 and A1 segments. VWI revealed marked vessel wall enhancement and thickening in the left carotid artery, M1, and A2 segments. Based on imaging findings, she was diagnosed with acute ischemic stroke caused by FCA. Because VWI findings were thought to suggest vessel wall inflammation, high-dose steroid therapy was administered in addition to neuroprotective care and antithrombotic therapy. Although her clinical symptoms improved immediately, cerebral arteriopathy worsened on MRA a month after the onset. Subsequently, after 3 months of steroid therapy, vessel wall enhancement on VWI decreased, while arterial stenosis partially improved. At the follow-up 9 months after the onset, she had no recurrent stroke, her arteriopathy had stabilized. DISCUSSION: Definitive evidence of inflammatory mechanisms in FCA is limited, and appropriate management and treatment strategies for FCA are undefined. VWI attempts to demonstrate pathologic processes within the vessel wall, and reversible wall enhancement observed in our patient suggested the presence of inflammation. VWI would help in the evaluation of disease activity in FCA. CONCLUSION: VWI may contribute to the appropriate diagnosis and treatment for FCA to reflect active inflammation. Further work is needed to assess the utility of VWI in pediatric FCA.

BACKGROUND & AIMS: Sarcopenia, resulting from skeletal muscle loss, is a serious concern in children, for whom nutrition and physical activity are essential for growth. In sarcopenia research, the skeletal muscle mass is often obtained from computed tomography (CT) cross-sectional images in both adults and children; however, the method of evaluating skeletal muscle using CT, especially the area range to be measured, has not yet been validated in children. The aim of this study is to establish reference values for three compartments of the abdominal skeletal muscle area (aSMA) obtained from cross-sectional CT images, and to validate the differences among them by assessing correlations with physical development. METHODS: We conducted a single-centre, retrospective, cross-sectional study and included patients aged 1-17 years who underwent abdominal CT at Chiba University Hospital from 2007 to 2020. Patients with chronic diseases such as malignant tumours and inflammatory bowel disease were excluded from the analysis. aSMA was obtained from CT cross-sections at the lumbar L3-L4 intervertebral disc level by dividing it into three compartments: the psoas muscle area (PMA), paraspinal muscle area (PSMA), and total skeletal muscle area (TSMA). Quartile regression curves by sex and muscle compartment were generated using non-crossover and nonparametric regression quantile estimation. The correlation between each compartment of aSMA with height and weight was assessed using Spearman's rank correlation coefficient. RESULTS: We analysed the abdominal CT images of 593 children (male: n = 335, female: n = 258) and generated curves predictive for the 5th, 25th, 50th, 75th, and 95th percentiles for each compartment of aSMA by sex. In patients aged 13 years and older, boys had significantly larger aSMA areas than girls in all three compartments. Among the three compartments, PSMA had the strongest correlation coefficient with height and weight for both boys and girls. CONCLUSIONS: We generated quantile regression curves for three aSMA compartments obtained from cross-sectional CT images and established reference values in children. PSMA values were most strongly correlated with height and weight among the three aSMA compartments.

PURPOSE: Central precocious puberty (CPP) is puberty that occurs at an unusually early age with several negative psychological outcomes. There is a paucity of data on the morphological characteristics of the brain in CPP. This study aimed to determine the structural differences in the brain of patients with CPP. METHODS: We performed voxel- and surface-based morphometric analyses of 1.5 T T1-weighted brain images scanned from 15 girls with CPP and 13 age-matched non-CPP controls (NC). All patients with CPP were diagnosed by gonadotropin-releasing hormone (GnRH) stimulation test. The magnetic resonance imaging (MRI) data were evaluated using Levene's test for equality of variances and a two-tailed unpaired t-test for equality of means. False discovery rate correction for multiple comparisons was applied using the Benjamini-Hochberg procedure. RESULTS: Morphometric analyses of the brain scans identified 33 candidate measurements. Subsequently, increased thickness of the right precuneus was identified in the patients with CPP using general linear models and visualizations of cortical thickness with a t-statistical map and a random field theory map. CONCLUSION: The brain scans of the patients with CPP showed specific morphological differences to those of the control. The features of brain morphology in CPP identified in this study could contribute to further understanding the association between CPP and detrimental psychological outcomes.

BACKGROUND: No previous research papers have reported a comparative survey of local radiologic diagnoses and central review in children with hepatoblastoma. OBJECTIVE: To evaluate the utility of central review of children with hepatoblastoma enrolled in a clinical trial. MATERIALS AND METHODS: The study included 91 children enrolled in a clinical trial conducted by the Japanese Study Group for Pediatric Liver Tumor. We compared the results of the initial pre-treatment extent of tumor (PRETEXT) disease staging performed at local sites with the results obtained on central review to determine the concurrence rates for tumor staging and additional criteria. RESULTS: The concurrence rate for PRETEXT staging was 70%. As the stage increased, the concurrence rate decreased. Using additional criteria, central review identified 143 lesions (157.1%), about 1.8 times higher than the number identified for the local site diagnoses. The additional criterion found most often on central review was "multifocal lesion" (n=19). The concurrence rate for lung metastases was high. However, our central review found many false-positive assertions of hepatic vein lesions, portal vein invasion and extrahepatic lesions among the local site diagnoses. CONCLUSION: In a clinical trial of hepatoblastoma, central review provided a more precise diagnosis than local site diagnoses with respect to severe PRETEXT stages III and IV cases and other cases including hepatic and portal vein invasion. The central review process appears to be effective and essential for improving the quality of clinical trials.

Since leptomeningeal carcinomatosis is rarely observed before diagnosis of the primary cancer, its detection is often delayed. We report the case of a 60-year-old woman who presented with lung adenocarcinoma with leptomeningeal carcinomatosis. Magnetic resonance imaging showed the characteristic abnormal hyperintensity along the ventral surface of the brain stem on fluid-attenuated inversion recovery and diffusion weighted imaging. It had no contrast uptake. Based on these findings, we were able to make an early diagnosis of leptomeningeal carcinomatosis of lung adenocarcinoma. This condition was resolved after treatment with a tyrosine kinase inhibitor.

We report the case of a 2-month-old infant who was found moribund in her crib. Postmortem computed tomography (PMCT) was performed before autopsy. As the baby had a severe subdural hematoma, retinal hemorrhage, and encephalopathy on PMCT, abusive head trauma (AHT) was tentatively diagnosed. At autopsy, no scalp hemorrhages or skull fractures were found; however, the classic triad of AHT was present, mainly on the right side. Additionally, there was dark red discoloration around the heart, and the liver, spleen, and pancreas were enlarged. Peripheral blood was macroscopically cloudy with marked leukocytosis. After careful histological examination, B-cell precursor acute lymphoblastic leukemia (ALL) was diagnosed. All the macroscopic lesions could be attributed to ALL. The manner of death was natural. To the best of our knowledge, this is the first report of infantile ALL mimicking AHT on PMCT images. This case demonstrates the importance of a comprehensive systematic approach to considering differential diagnosis when PMCT shows multiple intracranial hemorrhages suggestive of AHT in an infant.

OBJECTIVE: The impact of clinical information on radiological diagnoses and subsequent clinical management has not been sufficiently investigated. This study aimed to compare diagnostic performance between radiological reports made with and without clinical information and to evaluate differences in the clinical management decisions based on each of these reports. METHODS: We retrospectively reviewed 410 patients who presented with acute abdominal pain and underwent unenhanced (n = 248) or enhanced CT (n = 162). Clinical information including age, sex, current and past history, physical findings, and laboratory tests were collected. Six radiologists independently interpreted CTs that were randomly assigned with or without clinical information, made radiological diagnoses, and scored the diagnostic confidence level. Four general and emergency physicians simulated clinical management (i.e., followed up in the outpatient clinic, hospitalized for conservative therapy, or referred to other departments for invasive therapy) based on reports made with or without the clinical information. Reference standards for the radiological diagnoses and clinical management were defined by an independent expert panel. RESULTS: The radiological diagnoses made with clinical information were more accurate than those made without clinical information (93.7% vs. 87.8%, p = 0.008). Median interpretation time for radiological reporting with clinical information was significantly shorter than that without clinical information (median 122.0 vs. 139.0 s, p < 0.001). Clinical simulation better matched the reference standard for clinical management when radiological diagnoses were made with reference to clinical information (97.3% vs. 87.8%, p < 0.001). CONCLUSION: Access to adequate clinical information enables accurate radiological diagnoses and appropriate subsequent clinical management of patients with acute abdominal pain. KEY POINTS: • Radiological interpretation improved diagnostic accuracy and confidence level when clinical information was provided. • Providing clinical information did not extend the interpretation time required by radiologists. • Radiological interpretation with clinical information led to correct clinical management by physicians.

A man and a woman were found dead in the same car with a burned coal briquette. The cause of death of the woman was assigned to acute carbon monoxide (CO) poisoning without difficulty based on typical findings associated with this condition, including elevation of carboxyhaemoglobin (COHb). However, the man had an unremarkable elevation of COHb and a higher rectal temperature compared to that of the woman. Postmortem computed tomography (PMCT) revealed ambiguous low-density areas in the bilateral globi pallidi. Further analysis by postmortem magnetic resonance (PMMR) imaging showed these lesions more clearly; the lesions appeared as marked high signal intensity areas on both the T2-weighted images and the fluid-attenuated inversion recovery sequences. A subsequent autopsy revealed signs of pneumonia, dehydration, starvation, and hypothermia, suggesting that the man died from prolonged CO poisoning. Both globi pallidi contained grossly ambiguous lesions, and a detailed neuropathologic investigation revealed these lesions to be coagulative necrotic areas; this finding was compatible with a diagnosis of prolonged CO poisoning. This case report shows that postmortem imaging, especially PMMR, is useful for detecting necrotic lesions associated with prolonged CO poisoning. This report further exemplifies the utility of PMMR for detecting brain lesions, which may be difficult to detect by macroscopic analysis.

BACKGROUND: In most surgical textbooks, it has been stated that pain almost always precedes vomiting in patients with appendicitis. However, the usefulness of this classic history item, "pain before vomiting", has been investigated in only one study nearly 50 years ago, in which the cause of abdominal pain could not be identified in more than 40% of patients. Accordingly, our objective was to evaluate the performance of pain before vomiting for the diagnosis of acute appendicitis in patients who presented with both acute abdominal pain and vomiting. METHODS: A retrospective chart review of adult outpatients with abdominal pain and vomiting at three acute care hospitals was performed. The reference standard for appendicitis was a CT scan evaluated by two radiologists. Diagnostic performance of pain before vomiting and the value it added to the Alvarado score were evaluated. RESULTS: Among 310 patients, 24 patients were diagnosed with appendicitis. Diagnostic performance of pain before vomiting was a sensitivity of 95.8% (95% confidence interval [CI] 79.8-99.3) and a specificity of 16.6% (95% CI 12.6-21.4). When combined with the Alvarado score, it ruled out appendicitis in an additional 12% (increased from 32% to 44%) of patients without any false negatives. CONCLUSIONS: "Pain before vomiting" is useful for ruling out appendicitis in patients with abdominal pain and vomiting.

BACKGROUND AND PURPOSE: The purpose of this study was to compare the diagnostic performance between apparent diffusion coefficient (ADC) analysis of one-point measurement and whole-tumor measurement, including radiomics for differentiating pleomorphic adenoma (PA) from carcinoma ex pleomorphic adenoma (CXPA), and to evaluate the impact of inter-operator segmentation variability. MATERIALS AND METHODS: One hundred and fifteen patients with PA and 22 with CXPA were included. Four radiologists with different experience independently placed one-point and whole-tumor ROIs and a radiomics-predictive model was constructed from the extracted imaging features. We calculated the area under the receiver-operator characteristic curve (AUC) for the diagnostic performance of imaging features and the radiomics-predictive model. RESULTS: AUCs of the imaging features from whole-tumor varied between readers (0.50-0.89). The most experienced radiologist (Reader 1) produced significantly high AUCs than less experienced radiologists (Reader 3 and 4; P = 0.01 and 0.009). AUCs were higher for the radiomics-predictive model (0.82-0.87) than for one-point (0.66-0.79) in all readers. CONCLUSION: Some imaging features of whole-tumor and radiomics-predictive model had higher diagnostic performance than one-point. The diagnostic performance of imaging features from whole-tumor alone varied depending on operator experience. Operator experience appears less likely to affect diagnostic performance in the radiomics-predictive model.

A 5-year-old boy with a chromosome-9 abnormality and multiple external and visceral malformations was found in cardiopulmonary arrest during a regular visit to the hospital; he did not respond to cardiopulmonary resuscitation and died. An odontoid process fracture and calcification and fibrosis of the muscles around the superior cervical vertebra were observed during the autopsy. Postmortem computed tomography revealed an anterior dislocation of the atlas; odontoid synchondrosis fracture; and delayed, incomplete bony fusion of the odontoid process relative to his age. The cause of his death was a superior spinal cord injury. The tissue surrounding the upper cervical spine presented with myositis ossificans, suggesting a prior injury. He experienced a minor traffic accident 3 months before his death. It was concluded that the odontoid synchondrosis fracture occurred during the accident based on the incomplete bony fusion and atlantoaxial instability, which were consistent with the findings of myositis ossificans. Delayed fatal dislocation may then have occurred under the influence of a minor external force. Odontoid process abnormalities and atlantoaxial instability are common in patients with trisomy 21 and other congenital diseases; however, the condition's association with chromosome-9 abnormalities has not been reported. In children with various chromosomal abnormalities, periodic assessment of instability and morphology of the cervical spine, and a lowered examination threshold for the children at risk, could prove useful in the prevention injuries leading to fatality, and provide additional information to rule out abuse.

This study was to evaluate the correlation between birth weight and the distance between distal rectal pouch and perineum (P-P distance) and to determine a cutoff value for P-P distance to diagnose low-type imperforate anus in neonates with low-birth weight (LBW).We included 15 neonates with LBW (mean weight, 2012 ± 470 g; range, 906-2452 g) and imperforate anus (surgically confirmed: 11 low type and 3/1 intermediate/high type), who underwent ultrasonography on the day after birth. Type of imperforate anus was defined based on the International Classification of Anorectal Anomalies. The P-P distances on ultrasonograms were measured. Pearson correlation coefficient test and receiver operating characteristic curve were used for statistical analyses.Among all 15 neonates, nonsignificant correlation was observed between the birth weight and P-P distance (r = 0.36; P = 0.18). Mean P-P distance was 9.0 ± 6.6 mm (range, 1.0-24.0 mm) in all neonates, 5.7 ± 2.8 mm (range, 1.0-11.0 mm) in the 11 neonates with low-type imperforate anus, and 18.3 ± 9.1 mm (range, 14.0-24.0 mm) in the 4 neonates with intermediate-/high-type imperforate anus. Using cutoff P-P distance of 12.5 mm, sensitivity and specificity for diagnosis of low-type imperforate anus were 100% (11/11) and 100% (4/4), respectively.In conclusion, nonsignificant correlation was observed between P-P distance and birth weight, and cutoff P-P distance to diagnose low-type imperforate anus was 12.5 mm. Despite the very small sample size in our study, and only 4 neonates with intermediate-/high-type imperforate anus, these findings are important because surgical management whether transperineal anoplasty or diverting colostomy is decided based on the type of imperforate anus, and P-P distance to diagnose the type of imperforate anus was feasible even in neonates with LBW.

Background and Purpose: The superior cervical ganglion and inferior ganglion of the vagus nerve can mimic pathologic retropharyngeal lymph nodes. We studied the cross-sectional anatomy of the superior cervical ganglion and inferior ganglion of the vagus nerve to evaluate how they can be differentiated from the retropharyngeal lymph nodes. MATERIALS AND METHODS: This retrospective study consists of 2 parts. Cohort 1 concerned the signal intensity of routine neck MR imaging with 2D sequences, apparent diffusion coefficient, and contrast enhancement of the superior cervical ganglion compared with lymph nodes with or without metastasis in 30 patients. Cohort 2 used 3D neurography to assess the morphology and spatial relationships of the superior cervical ganglion, inferior ganglion of the vagus nerve, and the retropharyngeal lymph nodes in 50 other patients. RESULTS: All superior cervical ganglions had homogeneously greater enhancement and lower signal on diffusion-weighted imaging than lymph nodes. Apparent diffusion coefficient values of the superior cervical ganglion (1.80 ± 0.28× 10-3mm2/s) were significantly higher than normal and metastatic lymph nodes (0.86 ± 0.10-3 mm2/s, P &lt .001, and 0.73 ± 0.10 × 10-3mm2/s, P &lt .001). Ten and 13 of 60 superior cervical ganglions were hypointense on T2-weighted images and had hyperintense spots on both T1- A nd T2-weighted images, respectively. The latter was considered fat tissue. The largest was the superior cervical ganglion, followed in order by the retropharyngeal lymph node and the inferior ganglion of the vagus nerve (P&lt .001 to P&lt .004). The highest at vertebral level was the retropharyngeal lymph nodes, followed, in order, by the inferior ganglion of the vagus nerve and the superior cervical ganglion (P &lt .001 to = .001). The retropharyngeal lymph node, superior cervical ganglion, and inferior ganglion of the vagus nerve formed a line from anteromedial to posterolateral. CONCLUSIONS: The superior cervical ganglion and the inferior ganglion of the vagus nerve can be almost always differentiated from retropharyngeal lymph nodes on MR imaging by evaluating the signal, size, and position.

OBJECTIVES: To evaluate the diagnostic accuracy of sonography for detection of an internal fistula on the birth day in neonates with an imperforate anus and to compare the diagnostic performance between the suprapubic and perineal approaches. METHODS: We included 46 neonates with an imperforate anus (29 low type and 17 intermediate/high type) who underwent sonography by both the suprapubic and perineal approaches on the birth day. Thirty-nine neonates had internal fistulas, and 12 did not, as surgically proven. Two blinded radiologists evaluated the suprapubic and perineal sonograms for the presence of the internal fistula in consensus. A final diagnosis of the internal fistula was determined on the basis of the findings of both approaches. A receiver operating characteristic analysis was used to compare the diagnostic performance for detection of an internal fistula between the suprapubic and perineal approaches. RESULTS: The sensitivity, specificity, and accuracy of the final diagnosis based on the findings of suprapubic, perineal, and both approaches were 52.9%, 79.4%, and 79.4%; 75.5%, 75.5%, and 75.5%; and 58.7%, 78.3%, and 78.3%, respectively. The diagnostic performance of the perineal approach was significantly better than that of the suprapubic approach (P < .0001). CONCLUSIONS: The diagnostic accuracy of sonography for detection of an internal fistula on the birth day exceeded 75% in neonates with an imperforate anus, and sonography on the birth day is feasible. The perineal approach had superior diagnostic performance over the suprapubic approach. Thus, when evaluating an internal fistula by sonography, we recommend using the perineal approach in addition to the suprapubic approach.

This systematic review outlines the role of sonography in an imperforate anus. The diagnostic performance for type of imperforate anus is superior on the day after birth than that on the day of birth by using the pouch-perineum distance. Three approaches can be used (suprapubic, infracoccygeal, and perineal). The pouch-perineum distance, fistula location, and relationship between the puborectalis muscle and distal rectal pouch are useful for classifying the type of imperforate anus. However, the pouch-perineum distance measured has an overlap between the low and high/intermediate types of imperforate anus. Sonography can be useful for some of the associated anomalies and helpful for surgeons in some cases.

OBJECTIVES: To compare the diagnostic accuracy for the low-type imperforate anus between prone cross-table radiography and sonography. METHODS: We included 20 neonates with imperforate anus: 13 with a surgically proven low type and 7 with an intermediate or high type. The distance between the distal rectal pouch and the perineum (pouch-perineum distance) was measured by both sonography and prone cross-table radiography. A previously established pouch-perineum distance of 10 mm was used as the cutoff for diagnosis of a low-type imperforate anus. The fistula location was also determined with sonography. We then compared the diagnostic accuracy of the imaging methods for a low-type imperforate anus using the cutoff value of the pouch-perineum distance alone and both the cutoff value of the pouch-perineum distance and fistula location. The McNemar test was used for statistical analysis. RESULTS: With the use of only the pouch-perineum distance, the diagnostic accuracy for the low-type imperforate anus based on sonographic measurements was comparable with the accuracy achieved by prone cross-table radiographic measurements (60.0% [12 of 20] versus 45.0% [9 of 20]; P = .625). With the use of the pouch-perineum distance and fistula location, the diagnostic accuracy of sonography was significantly better than the accuracy of prone cross-table radiography (90.0% [18 of 20] versus 45% [9 of 20]; P = .012). CONCLUSIONS: The diagnostic accuracy of sonography for the low-type imperforate anus based on both the pouch-perineum distance and fistula location is better than that of prone cross-table radiography. If the pouch-perineum distance on prone cross-table radiography is greater than 10 mm, a sonographic examination to determine the fistula location could be recommended.

OBJECTIVES: To evaluate how well the tonsils can be viewed, in addition to echogenicity, using ultrasound, and to compare these results between children younger and older than the age of 3. METHODS: We evaluated the tonsils of 99 patients (72.0 ± 59.1 months) by ultrasound. Ultrasound scans of both the left and right side, in both the transverse and longitudinal planes, were obtained. Images were scored with one of four grades according to how well the tonsil border could be distinguished, 0 being the worst and 3 being the best. Grades 2 or 3 (>50% of the tonsil border was detectable) in both tonsils, in either the transverse or longitudinal image, were considered "evaluable." Echogenicity was designated as imperceptible, low echoic, or striated in appearance. Statistical analysis was performed using the Mann-Whitney U test. RESULTS: Bilateral tonsils were evaluable in 96.0% (96/99) of cases. The mean grades were 2.44 ± 0.65/2.03 ± 0.68 in the right transverse/longitudinal images, and 2.40 ± 0.59/2.12 ± 0.73 in the left transverse/longitudinal images. The grades in children older than the age of 3 were significantly higher than those in younger patients (all P < .05). Echogenicity classification on the right and left side revealed a striated appearance in 97.0% (96/99) and 90.0% (89/99) of cases, respectively. CONCLUSIONS: The tonsils of almost all pediatric patients could be evaluated by ultrasound, particularly for patients older than 3 years. Additionally, a striated pattern of the tonsils was observed in most cases.