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The identification of children with traumatic brain injury (TBI) who are at risk of death or poor global neurological functional outcome remains a challenge. Magnetic resonance imaging (MRI) can detect several brain pathologies that are a result of TBI; however, the types and locations of pathology that are the most predictive remain to be determined. Forty-two critically ill children with TBI were recruited prospectively from pediatric intensive care units at five Canadian children's hospitals. Pathologies detected on subacute phase MRIs included cerebral hematoma, herniation, cerebral laceration, cerebral edema, midline shift, and the presence and location of cerebral contusion or diffuse axonal injury (DAI) in 28 regions of interest were assessed. Global functional outcome or death more than 12 months post-injury was assessed using the Pediatric Cerebral Performance Category score. Linear modeling was employed to evaluate the utility of an MRI composite score for predicting long-term global neurological function or death after injury, and nonlinear Random Forest modeling was used to identify which MRI features have the most predictive utility. A linear predictive model of favorable versus unfavorable long-term outcomes was significantly improved when an MRI composite score was added to clinical variables. Nonlinear Random Forest modeling identified five MRI variables as stable predictors of poor outcomes: presence of herniation, DAI in the parietal lobe, DAI in the subcortical white matter, DAI in the posterior corpus callosum, and cerebral contusion in the anterior temporal lobe. Clinical MRI has prognostic value to identify children with TBI at risk of long-term unfavorable outcomes.

Chronic recurrent and multifocal osteomyelitis (CRMO) is a nonsporadic autoinflammatory disorder. Currently, it is diagnosed based on clinical, radiologic, pathological, and longitudinal data. Numerous aspects should be highlighted due to increased knowledge in imaging and immunology. We emphasize the use of whole-body MRI, which is a non-invasive diagnostic strategy. A literature review was carried out on longitudinal studies. Commonly, the mean age at diagnosis is 11 years, ranging between 3 and 17. The most common sites are the long bone metaphysis, particularly femoral and tibial metaphysis. In addition, the pelvis, spine, clavicle, and mandible may be involved. In long bones, the radiologic appearance can show typical structure, mixed lytic and sclerotic, sclerotic or lytic. It is frequently metaphyseal or juxta-physeal, with hyperostosis or periosteal thickening. The involvement of the vertebral skeleton is often multifocal. Therefore, whole-body MRI is essential in identifying subclinical lesions. CRMO is a polymorphic disorder in which whole-body MRI is beneficial to demonstrate subclinical edema. Vertebral collapse requires long-term monitoring.

Objectives To assess the concordance of whole-body MRI (WB-MRI) and an FDG-PET/CT-based reference standard for the initial staging in children with Hodgkin lymphoma (HL) Methods Children with newly diagnosed HL were included in this prospective, multicentre, international study and underwent WB-MRI and FDG-PET/CT at staging. Two radiologists and a nuclear medicine physician independently evaluated all images. Discrepancies between WB-MRI and FDG-PET/CT were assessed by an expert panel. All FDG-PET/CT errors were corrected to derive the FDG-PET/CT-based reference standard. The expert panel corrected all reader errors in the WB-MRI DWI dataset to form the intrinsic MRI data. Inter-observer agreement for WB-MRI DWI was calculated using overall agreement, specific agreements and kappa statistics. Concordance for correct classification of all disease sites and disease stage between WB-MRI (without DWI, with DWI and intrinsic WB-MRI DWI) and the reference standard was calculated as primary outcome. Secondary outcomes included positive predictive value, negative predictive value and kappa statistics. Clustering within patients was accounted for using a mixed-effect logistic regression model with random intercepts and a multilevel kappa analysis. Results Sixty-eight children were included. Inter-observer agreement between WB-MRI DWI readers was good for disease stage (κ = 0.74). WB-MRI DWI agreed with the FDG-PET/CT-based reference standard for determining disease stage in 96% of the patients versus 88% for WB-MRI without DWI. Agreement between WB-MRI DWI and the reference standard was excellent for both nodal (98%) and extra-nodal (100%) staging. Conclusions WB-MRI DWI showed excellent agreement with the FDG-PET/CT-based reference standard. The addition of DWI to the WB-MRI protocol improved the staging agreement. Key Points • This study showed excellent agreement between WB-MRI DWI and an FDG-PET/CT-based reference standard for staging paediatric HL. • Diffusion-weighted imaging is a useful addition to WB-MRI in staging paediatric HL. • Inter-observer agreement for WB-MRI DWI was good for both nodal and extra-nodal staging and determining disease stage.

The petrous apex (PA) is involved in a myriad of pathological conditions, some of which are exclusive in children. Diagnosis may be difficult due to vague clinical presentation, and local examination is challenging owing to its inaccessible location. This is further complicated by multiple unfused sutures and ongoing PA pneumatization in children. Cross-sectional imaging is vital for the evaluation of the PA lesions, due to their precarious location and proximity to the major neurovascular structures. Several classification systems have been proposed for these lesions based on their site of origin, solid or cystic appearance, surgical or non-surgical (no touch lesions) management, and benign or malignant nature. In this article, we emphasize the distinctive role of different cross-sectional imaging modalities in the diagnosis of pediatric PA lesions, with special attention to normal variants that should not be mistaken for pathology. We also propose a radiological classification and algorithmic approach to aid in the precise diagnosis and facilitate appropriate management of the various PA lesions in children.

Purpose: It has been shown that oral contrast does not improve the diagnostic accuracy of Computed Tomography (CT) for appendicitis in pediatric patients; however, the cohorts in these studies were not stratified by weight or body mass index. The purpose of this study is to assess the benefit of oral contrast administration for identifying the appendix in younger children in the lower weight quartile. Materials and Methods: This retrospective study comprised 100 patients (2–10 years) in lower weight quartile who had intravenous contrast-enhanced CT of the abdomen and pelvis, 37 of which with oral contrast, and 63 without. A pediatric radiologist and a pediatric radiology fellow independently assessed whether the appendix was visualized or not. In case of discrepancy, an additional pediatric radiologist was the “tie-breaker.” Chi-squared test was used to compare the proportion of visualized appendix between the groups (with and without oral contrast). Inter-rater reliability was determined using Cohen’s Kappa coefficient. Results: There was no significant difference in the visualization of the appendix between the group with oral contrast and without ( P = 1). The Cohen Kappa coefficients were .33 (.05, .62) and .91 (.73, 1.00) for the “no oral” and “oral” groups, respectively, yielding evidence of a difference ( P = .007). Conclusions: There was no significant difference in the visualization of the appendix using CT with or without oral contrast in low-weight pediatric patients. The inter-rater reliability, however, was significantly higher in the group given oral contrast. Additional studies assessing the value of oral contrast for the sole indication of appendicitis may provide clearer results.

OBJECTIVE: To determine the relative importance weights of items and grades of a newly developed additive outcome measure called the juvenile idiopathic arthritis (JIA) magnetic resonance imaging (MRI) scoring system for the temporomandibular joint (TMJ) (JAMRIS-TMJ). METHODS: An adaptive partial-profile, discrete choice experiment (DCE) survey using the 1000Minds platform was independently completed by members of an expert group consisting of radiologists and non-radiologist clinicians to determine the group-averaged relative weights for the JAMRIS-TMJ. Subsequently, an image-based vignette ranking exercise was done, during which experts individually rank ordered 14 patient vignettes for disease severity while blinded to the weights and unrestricted to JAMRIS-TMJ assessment criteria. Validity of the weighted JAMRIS-TMJ was tested by comparing the consensus-graded, DCE-weighted JAMRIS-TMJ score of the vignettes with their unrestricted image-based ranks provided by the experts. RESULTS: Nineteen experts completed the DCE survey, and 21 completed the vignette ranking exercise. Synovial thickening and joint enhancement showed higher weights per raw score compared to bone marrow items and effusion in the inflammatory domain, while erosions and condylar flattening showed nonlinear and higher weights compared to disk abnormalities in the damage domain. The weighted JAMRIS-TMJ score of the vignettes correlated highly with the ranks from the unrestricted comparison method, with median Spearman's ? of 0.92 (interquartile range [IQR] 0.87-0.95) for the inflammation and 0.93 (IQR 0.90-0.94) for the damage domain. CONCLUSION: A DCE survey was used to quantify the importance weights of the items and grades of the JAMRIS-TMJ. The weighted score showed high convergent validity with an unrestricted, holistic vignette ranking method.

The pediatric reproductive organs are optimally imaged with a full bladder. The filling of the bladder, however, often leads to significant delay in diagnosis and can subject the patient to invasive bladder catheterization. As the key imaging feature in ovarian torsion is unilateral ovarian enlargement, we suspected that a torsed ovary is large enough to be visualized even if the bladder is not well distended. The purpose of this study was to retrospectively investigate if clinically suspected adnexal torsion can be excluded based on non-visualization of the ovaries on transabdominal ultrasound (US) with a non-distended bladder in pediatric patients. This retrospective study comprised 349 girls (1–19 years old) between Jan. 1, 2013, and July 30, 2018. Three hundred and forty-one of the girls were referred to transabdominal US to assess for adnexal torsion and/or appendicitis, and the ovaries were initially not visualized on US. Their bladders were subsequently filled and rescanned with a distended bladder showing the ovaries. Ovarian volumes and time between US scans were documented. The ratio of the volume of the larger ovary to the smaller one was calculated. Nine girls had surgically proven adnexal torsion and a preoperative transabdominal US with a non-distended bladder. There was an overlap of one girl between the two groups. The negative predictive value (NPV), positive predictive value (PPV), and sensitivity and specificity for exclusion of adnexal torsion based on non-visualization of the ovaries on US with a non-distended bladder were calculated. One of the girls (1/341) who had a US study done with a non-distended bladder in which the ovaries were not visualized had a positive diagnosis of adnexal torsion. In eight of the nine girls who had surgically proven adnexal torsion, the torted ovary was identified with a non-distended bladder. The NPV and PPV for exclusion of adnexal torsion with a non-distended bladder was 1.0 and 0.8, respectively. The specificity and sensitivity were 99.4% and 88.9%, respectively. The mean and median time difference between the initial scan and the scan after bladder filling was 105.1 min (standard deviation [SD] -65.8) and 89.0 min (interquartile range [IQR]- 59.0, 130.5), respectively. Non-visualization of the ovaries with a non-distended bladder on transabdominal US study can help exclude clinically suspected adnexal torsion, alleviating the need for bladder filling and prolonging the wait time in the emergency department. Inclusion of non-visualization of the ovaries as one of the features in a predictive score for adnexal torsion should be considered.

Autosomal dominant Dandy-Walker malformation and occipital cephalocele (ADDWOC) is a rare, congenital, and incompletely penetrant malformation that is considered to be part of the Dandy-Walker spectrum of disorders. Affected individuals often present with an occipital cephalocele with a bony skull defect, but typically have normal neurological development. Here, we report on a three-generation family in which individuals have variable phenotypes that are consistent with the ADDWOC spectrum: arachnoid cysts in the proband and his maternal grandfather, an occipital cephalocele in the proband and his brother, and a small bony defect in the proband's mother. Whole exome sequencing identified a rare heterozygous variant in NID1 (NM_002508.2:c.1162C>T, (p.Gln388Ter)) in the proband, his brother, and his mother. Sanger sequencing confirmed the presence of this variant in the maternal grandfather. The identical c.1162C>T variant was previously identified in variably affected members of a three-generation family with ADDWOC. This case report provides further evidence that variants in NID1 may be clinically relevant for the development of a phenotype that is consistent with ADDWOC, and extends the phenotype of NID1-associated ADDWOC to include arachnoid cysts. Given that the Dandy-Walker malformation itself is not a pre-requisite to this spectrum of phenotypes, we also suggest a novel term for the NID1-associated disorder in order to give emphasis to this phenotypic variability: "Autosomal Dominant Posterior Fossa Anomalies with Occipital Cephaloceles."

Timely, accurate and reliable assessment of fetal brain development is essential to reduce short and long-term risks to fetus and mother. Fetal MRI is increasingly used for fetal brain assessment. Three key biometric linear measurements important for fetal brain evaluation are Cerebral Biparietal Diameter (CBD), Bone Biparietal Diameter (BBD), and Trans-Cerebellum Diameter (TCD), obtained manually by expert radiologists on reference slices, which is time consuming and prone to human error. The aim of this study was to develop a fully automatic method computing the CBD, BBD and TCD measurements from fetal brain MRI. The input is fetal brain MRI volumes which may include the fetal body and the mother's abdomen. The outputs are the measurement values and reference slices on which the measurements were computed. The method, which follows the manual measurements principle, consists of five stages: 1) computation of a Region Of Interest that includes the fetal brain with an anisotropic 3D U-Net classifier; 2) reference slice selection with a Convolutional Neural Network; 3) slice-wise fetal brain structures segmentation with a multiclass U-Net classifier; 4) computation of the fetal brain midsagittal line and fetal brain orientation, and; 5) computation of the measurements. Experimental results on 214 volumes for CBD, BBD and TCD measurements yielded a mean $L_1$ difference of 1.55mm, 1.45mm and 1.23mm respectively, and a Bland-Altman 95% confidence interval ($CI_{95}$) of 3.92mm, 3.98mm and 2.25mm respectively. These results are similar to the manual inter-observer variability. The proposed automatic method for computing biometric linear measurements of the fetal brain from MR imaging achieves human level performance. It has the potential of being a useful method for the assessment of fetal brain biometry in normal and pathological cases, and of improving routine clinical practice., 15 pages, 8 figures, presented in CARS 2020, submitted to IJCARS

Prenatal ultrasound (US) is the first prenatal imaging tool for screening and evaluation of posterior fossa malformations since it is noninvasive, widely available, and safe for both mother and child. Fetal MRI is a widely used secondary technique to confirm, correct, or complement questionable US findings and plays an essential role in evaluating fetuses with suspected US findings and /or positive family history. The main sequences of fetal MRI consist of T2-weighted (T2w) ultrafast, single-shot sequences. Axial, coronal, and sagittal images are typically acquired allowing for a detailed evaluation of the posterior fossa contents. Also, various complimentary sequences, such as T1w, T2*w gradient sequences, or advanced techniques, including diffusion-weighted imaging, diffusion tensor imaging, and magnetic resonance spectroscopy, may provide additional information based on the studied malformation. Inclusion of these techniques should be done with careful risk-benefit analysis. The use of fetal MRI also aims to evaluate for associated anomalies. In addition, prenatal diagnosis of posterior fossa malformations is still a challenge but advances in knowledge in human developmental anatomy, genetic, and imaging recognition patterns have enabled us to shed some light on prognostic information that will help with the counseling of families. Finally, high-resolution late third trimester fetal MRI offers a safe alternative to early postnatal MR imaging, basically taking advantage of the uterine environment as a kind of "maternal incubator." Our goal is to discuss the spectrum of prenatal posterior fossa pathologies that can be studied by fetal MRI and their key neuroimaging features.

Objectives To compare WB-MRI with an [18F]FDG-PET/CT-based reference for early response assessment and restaging in children with Hodgkin’s lymphoma (HL). Methods Fifty-one children (ages 10–17) with HL were included in this prospective, multicentre study. All participants underwent WB-MRI and [18F]FDG-PET/CT at early response assessment. Thirteen of the 51 patients also underwent both WB-MRI and [18F]FDG-PET/CT at restaging. Two radiologists independently evaluated all WB-MR images in two separate readings: without and with DWI. The [18F]FDG-PET/CT examinations were evaluated by a nuclear medicine physician. An expert panel assessed all discrepancies between WB-MRI and [18F]FDG-PET/CT to derive the [18F]FDG-PET/CT-based reference standard. Inter-observer agreement for WB-MRI was calculated using kappa statistics. Concordance, PPV, NPV, sensitivity and specificity for a correct assessment of the response between WB-MRI and the reference standard were calculated for both nodal and extra-nodal disease presence and total response evaluation. Results Inter-observer agreement of WB-MRI including DWI between both readers was moderate (κ 0.46–0.60). For early response assessment, WB-MRI DWI agreed with the reference standard in 33/51 patients (65%, 95% CI 51–77%) versus 15/51 (29%, 95% CI 19–43%) for WB-MRI without DWI. For restaging, WB-MRI including DWI agreed with the reference standard in 9/13 patients (69%, 95% CI 42–87%) versus 5/13 patients (38%, 95% CI 18–64%) for WB-MRI without DWI. Conclusions The addition of DWI to the WB-MRI protocol in early response assessment and restaging of paediatric HL improved agreement with the [18F]FDG-PET/CT-based reference standard. However, WB-MRI remained discordant in 30% of the patients compared to standard imaging for assessing residual disease presence. Key Points • Inter-observer agreement of WB-MRI including DWI between both readers was moderate for (early) response assessment of paediatric Hodgkin’s lymphoma. • The addition of DWI to the WB-MRI protocol in early response assessment and restaging of paediatric Hodgkin’s lymphoma improved agreement with the [18F]FDG-PET/CT-based reference standard. • WB-MRI including DWI agreed with the reference standard in respectively 65% and 69% of the patients for early response assessment and restaging. Supplementary Information The online version contains supplementary material available at 10.1007/s00330-021-08026-1.

BACKGROUND AND PURPOSE: The superior diagnostic accuracy of fetal MR imaging in detecting fetal brain abnormalities has been previously demonstrated; however, the ability of fetal MR imaging to prognosticate postnatal outcome is not well-studied. We performed a retrospective analysis to determine the prognostic accuracy of fetal MR imaging in predicting postnatal neurodevelopmental outcome. MATERIALS AND METHODS: We identified all fetal MR imaging performed at the Children9s Hospital of Eastern Ontario during a 10-year period and assessed agreement between prenatal prognosis and postnatal outcome. Prenatal prognosis was determined by a pediatric neurologist who reviewed the fetal MR imaging report and categorized each pregnancy as having a favorable, indeterminate, or poor prognosis. Assessment of postnatal neurodevelopmental outcome was made solely on the basis of the child’s Gross Motor Function Classification System score and whether the child developed epilepsy. Postnatal outcome was categorized as favorable, intermediate, or poor. We also assessed the diagnostic accuracy of fetal MR imaging by comparing prenatal and postnatal imaging diagnoses. RESULTS: We reviewed 145 fetal MR images: 114 were included in the assessment of diagnostic accuracy, and 104 were included in the assessment of prognostic accuracy. There was 93.0% agreement between prenatal and postnatal imaging diagnoses. Prognosis was favorable in 44.2%, indeterminate in 50.0%, and poor in 5.8% of pregnancies. There was 93.5% agreement between a favorable prenatal prognosis and a favorable postnatal outcome. CONCLUSIONS: A favorable prenatal prognosis is highly predictive of a favorable postnatal outcome. Further studies are required to better understand the role of fetal MR imaging in prognosticating postnatal development, particularly in pregnancies with indeterminate and poor prognoses.

The aim of our study was to compare whole-body diffusion-weighted MRI (WB-DWI-MRI) to fluoro-2-deoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) in the assessment of initial staging and treatment response in pediatric patients with Hodgkin lymphoma.This prospective study comprised 11 children with Hodgkin lymphoma. Whole-body DWI-MRI and FDG-PET/CT were obtained at baseline and after 2 cycles of chemotherapy. Two radiologists measured the apparent diffusion coefficient (ADC) values of the sites of involvement agreed upon in consensus and 1 nuclear medicine physician assessed the PET/CT. Reliability of radiologists' ratings was assessed by intraclass correlation coefficients (ICCThere was good agreement between the raters for nodal and extranodal ADC measurements. The sensitivity and PPV of DW-MRI relative to PET/CT of nodal disease was 0.651 and 1.0, respectively, at baseline, and 0.697 and 0.885 at follow-up. The sensitivity and PPV of extranodal disease were 0.545 and 0.6 at baseline, and 0.167 and 0.333 at follow-up. Diffusion-weighted MRI determined correct tumor stage in 8 of 11 examinations. There was poor correlation between the ADC ratios and the absolute change in SUV between baseline and follow-up (0.348).Our experience showed that WB-DWI-MRI is inferior to PET/CT for initial staging and assessment of treatment response of Hodgkin lymphoma in pediatric patients.

Cranial US allows for the evaluation of premature closure (synostosis) or abnormal widening of the cranial sutures. An understanding of the normal anatomy is required to help define the presence or absence of abnormality. To provide reference for normal ultrasound measurements of cranial sutures during the child’s first year. We included children ages 0 to 12 months who were referred to the hospital during 2011–2013 for radiographic evaluation of cranial sutures. Cranial US study was focused on evaluating the sagittal, coronal, lambdoid and metopic sutures. We measured the hypoechoic gap between the bones (patent suture). Two readers performed the measurements, blinded to clinical indications and previous reports. Estimates of the 10th, 25th, 50th, 75th and 90th percentiles were achieved for ages 1–12 months. Of 129 children whose families consented to cranial US, 11 were excluded because of craniosynostosis and 3 for suboptimal quality of cranial US images. In 115 patients measurements of normal cranial sutures were obtained (75 boys [65%], ages 0.26–11.27 months). For each suture, the suture size decreased significantly with age (P

Occipital cortical malformation is a rare neurodevelopmental disorder characterized by pachygyria and polymicrogyria of the occipital lobes as well as global developmental delays and seizures. This condition is due to biallelic, loss-of-function mutations in LAMC3 and has been reported in four unrelated families to date. We report an individual with global delays, seizures, and polymicrogyria that extends beyond the occipital lobes and includes the frontal, parietal, temporal, and occipital lobes. Next-generation sequencing identified a homozygous nonsense mutation in LAMC3: c.3190C>T (p.Gln1064*). This finding extends the cortical phenotype associated with LAMC3 mutations.

Background: Accurate fetal brain volume estimation is of paramount importance in evaluating fetal development. The aim of this study was to develop an automatic method for fetal brain segmentation from magnetic resonance imaging (MRI) data, and to create for the first time a normal volumetric growth chart based on a large cohort. Subjects and Methods: A semi-automatic segmentation method based on Seeded Region Growing algorithm was developed and applied to MRI data of 199 typically developed fetuses between 18 and 37 weeks' gestation. The accuracy of the algorithm was tested against a sub-cohort of ground truth manual segmentations. A quadratic regression analysis was used to create normal growth charts. The sensitivity of the method to identify developmental disorders was demonstrated on 9 fetuses with intrauterine growth restriction (IUGR). Results: The developed method showed high correlation with manual segmentation (r2 = 0.9183, p < 0.001) as well as mean volume and volume overlap differences of 4.77 and 18.13%, respectively. New reference data on 199 normal fetuses were created, and all 9 IUGR fetuses were at or below the third percentile of the normal growth chart. Discussion: The proposed method is fast, accurate, reproducible, user independent, applicable with retrospective data, and is suggested for use in routine clinical practice.

Aims: To compare changes in gross motor skills and functional mobility between ambulatory children with cerebral palsy who underwent a 1-week clinic-based virtual reality intervention (VR) followed by a 6-week, therapist-monitored home active video gaming (AVG) program and children who completed only the 6-week home AVG program. Methods: Pilot non-randomized controlled trial. Five children received 1 hour of VR training for 5 days followed by a 6-week home AVG program, supervised online by a physical therapist. Six children completed only the 6-week home AVG program. The Gross Motor Function Measure Challenge Module (GMFM-CM) and Six Minute Walk Test (6MWT) evaluated change. Results: There were no significant differences between groups. The home AVG-only group demonstrated a statistically and clinically significant improvement in GMFM-CM scores following the 6-week AVG intervention (median difference 4.5 points, interquartile range [IQR] 4.75, p = 0.042). The VR + AVG group demonstrated a statistically and clinically significant decrease in 6MWT distance following the intervention (median decrease 68.2 m, IQR 39.7 m, p = 0.043). All 6MWT scores returned to baseline at 2 months post-intervention. Conclusion: Neither intervention improved outcomes in this small sample. Online mechanisms to support therapist-child communication for exercise progression were insufficient to individualize exercise challenge.

Postmortem CT for investigating childhood deaths is increasingly utilised as a noninvasive adjunct or alternative to standard autopsy; however there are no standardised published imaging protocols. This article describes a standardised imaging protocol that has been developed based on current practices of international postmortem imaging practitioners and experts. This recommendation is expected to be useful for postmortem imaging centres wishing to update their existing practices and for those starting paediatric postmortem CT as a new service.

The Kwak Thyroid Imaging Reporting and Data System (Kwak-TI-RADS) guideline (2011) and American College of Radiology Thyroid Imaging Reporting and Data System (ACR TI-RADS) guideline (2017) were developed as ultrasound (US) risk stratification tools for detecting thyroid malignancy in adults. The purpose of this study was to investigate the inter-rater reliability and diagnostic performance of the ACR TI-RADS guideline in the pediatric population and compare it to the Kwak guideline. This retrospective study comprised 75 children who underwent thyroid US at a tertiary-level pediatric hospital. Three pediatric radiologists and one pediatric radiology fellow graded the US findings using the Kwak-TI-RADS and ACR TI-RADS guidelines. We assessed reliability of radiologists’ ratings using percentage inter-rater agreement, and intra-class correlation coefficients (ICC2,1). We assessed area-under-the-receiver-operating-characteristic curve (AUROCC) to compare the discriminative diagnostic ability of the Kwak-TI-RADS and ACR TI-RADS scoring systems against histopathology/cytology, or stability on US over a 2-year follow-up period for cases without tissue diagnosis. The inter-rater agreement was significantly better for the ACR TI-RADS level compared to the Kwak-TI-RADS level (P

Serine deficiency disorders can result from deficiency in one of three enzymes. Deficiency of the second enzyme, 3-phosphoserine aminotransferase (PSAT), has been reported in two siblings; the eldest investigated for acquired microcephaly, spasticity and epilepsy. Our patient had neurological symptoms at birth. Fetal magnetic resonance imaging (MRI) at 35-week gestation demonstrated microencephaly and gyral simplification (anterior posterior) which was confirmed upon postnatal MRI. Congenital microcephaly was apparent at birth. PSAT deficiency was confirmed when exome sequencing identified biallelic mutations in

Inflammation and damage in the temporomandibular joint (TMJ) often develop without clinical symptoms but can lead to severe facial growth abnormalities and impaired health-related quality of life, making early diagnosis of TMJ changes crucial to identify. Inflammatory and osteochondral changes detectable through magnetic resonance imaging (MRI) occur in TMJs of approximately 40% of children with juvenile idiopathic arthritis (JIA), and no other imaging modality or physical method of examination can reliably detect these changes. Therefore contrast-enhanced MRI is the diagnostic standard for diagnosis and interval monitoring of JIA. However the specific usage of MRI for TMJ arthritis is not standardized at present. There is a recognized need for a consensus effort toward standardization of an imaging protocol with required and optional sequences to improve detection of pathological changes and shorten study time. Such a consensus imaging protocol is important for providing maximum information with minimally necessary sequences in a way that allows inter-site comparison of results of clinical trials and improved clinical management. In this paper we describe the challenges of TMJ imaging and present expert-panel consensus suggestions for a standardized TMJ MRI protocol.

Objective: The temporomandibular joints (TMJs) are frequently affected in children with juvenile idiopathic arthritis (JIA). Early detection is challenging, as major variation is present in scoring TMJ pathology on magnetic resonance imaging (MRI). Consensus-driven development and validation of an MRI scoring system for TMJs has important clinical utility in timely improvement of diagnosis and serving as an outcome measure. We report on a multi-institutional collaboration toward developing a TMJ MRI scoring system for JIA. Methods: Seven readers independently assessed MRI scans from 21 patients (42 TMJs, from patients ages 6–16 years) using 3 existing MRI scoring systems from American, German, and Swiss institutions. Reliability scores, scoring system definitions, and items were discussed among 10 JIA experts through 2 rounds of Delphi surveys, nominal group voting, and subsequent consensus meetings to create a novel TMJ MRI scoring system. Results: Average-measure absolute agreement intraclass correlation coefficients (avICCs) for the total scores of all 3 scoring systems were highly reliable at 0.96 each. Osteochondral items showed higher reliability than inflammatory items. An additive system was deemed preferable for assessing minor joint changes over time. Eight items were considered sufficiently reliable and/or important for integration into the consensus scoring system: bone marrow edema and enhancement (avICC 0.57–0.61, smallest detectable difference [SDD] ± 45–63% prior to redefining), condylar flattening (avICC 0.95–0.96, SDD ± 23–28%), effusions (avICC 0.85–0.88, SDD ± 25–26%), erosions (avICC 0.94, SDD ± 20%), synovial enhancement and thickening (previously combined, avICC 0.90–0.91, SDD ± 33%), and disk abnormalities (avICC 0.90, SDD ± 19%). Conclusion: A novel TMJ MRI scoring system was developed by consensus. Further iterative refinements and reliability testing are warranted in upcoming studies.

Aim To investigate if magnetic resonance imaging (MRI) features of the placenta are different in fetuses with and without central nervous system (CNS) abnormalities. Material and methods Institutional research ethics board approval was obtained. Fetal MRI of 97 singleton pregnancies were analysed retrospectively (19–25 weeks gestation), 65 with CNS morphological abnormalities and 32 controls. Placental T2 signal intensity, placental and fetal volumes, placental-to-fetal volume ratio, and placental apparent diffusion coefficient (ADC) values were assessed. Measurements were compared with the presence or absence of CNS fetal abnormalities using the Mann–Whitney test. Separate slopes models and intercept models were used to check for significant differences in the slopes and intercepts, respectively, among the groups. Results Placental ADC values were significantly lower in placentas of fetuses with CNS abnormalities compared to controls (p=0.04). Placental T2 signal intensity, fetal and placental volumes did not differ between the two groups. The rate of increase in fetal-to-placental volume ratio with gestational age (GA) was greater among the controls. Conclusion The presence of fetal CNS abnormalities is associated with reduced ADC values of the placenta. Moreover, placentas of fetuses with CNS abnormalities show a less rapid increase in fetal to placental volume ratio with GA. Therefore, ADC mapping, as well as different growth kinetics of the placenta relative to the fetus, may potentially serve as early markers of pathological neurodevelopment.

BACKGROUND AND PURPOSE: Stronger magnetic fields have the potential to improve fetal image resolution. Our objective was to detect whether there was better anatomic resolution of brain structures in fetuses imaged with a 3T magnet compared with a 1.5T magnet. MATERIALS AND METHODS: Multiple cerebral and facial anatomic structures were retrospectively assessed in 28 fetal MR imaging scans with normal findings (12 at 3T and 16 at 1.5T) with a 0–3 grading score. Fetuses were assessed during the second trimesters (gestational age, 20–24 weeks). The association between the quality ratings and magnetic field strengths (1.5T versus 3T) was evaluated by a linear mixed-effects model. A quantitative assessment of the signal intensity was also performed in the different layers of the developing brain. Comparative log-ratios were calculated across the different layers of the fetal brain. RESULTS: There was a statistically significant interaction between location and magnetic field strength (P < .001). The cerebral structures of the cerebellum, pons, venous system, semicircular canal, and cochlea showed statistically significant higher values on the 3T magnet. Similarly, statistical significance was also obtained on the quantitative assessment of the multilayer appearance of the brain; the 3T magnet had a median factor of 8.38 higher than the 1.5T magnet (95% CI, 4.73–14.82). Other anatomic structures assessed in the supratentorial compartment of the brain showed higher values on the 3T magnet with no statistical significance. CONCLUSIONS: Both magnets depict cerebral and facial normal anatomic structures; however, our data indicates better anatomic detail on the 3T than on the 1.5T magnet.

OBJECTIVE. Our objective was to evaluate the diagnostic accuracy and reliability of MRI and its ability to depict responsiveness to treatment for the evaluation of the axial joints (temporomandibular joint [TMJ], spinal joints, and sacroiliac joints) in juvenile idiopathic arthritis (JIA). CONCLUSION. There is fair (grade B) evidence that MRI is an accurate diagnostic method for evaluating early and intermediate changes in the TMJ in JIA and insufficient evidence to indicate MRI is an accurate diagnostic method for detecting JIA in the spinal (grade I) and sacroiliac (grade I) joints.

Objective To describe a case of pediatric central nervous system (CNS) venulitis. Background Primary angiitis of the CNS is a rare but increasingly well-recognized cause of morbidity in children. It primarily involves the arteries and arterioles of the CNS, with only 1 published case of a pediatric patient found to have isolated CNS venulitis on brain biopsy. Case Report A 17-year-old female with a 4-year history of migraines presented with increasingly frequent migraines and right-sided hemiplegia. Infectious, hematologic, and rheumatologic work-ups were negative. Brain magnetic resonance imaging showed multiple rim-enhancing lesions consistent with calcifications affecting the deep left white matter. On brain biopsy, there was evidence of an inflammatory process involving small veins and venules. The patient displayed clinical improvement with a course of high-dose steroids and 6 monthly cyclophosphamide infusions followed by maintenance therapy with mycophenolate mofetil. Discussion We describe a case of pediatric CNS venulitis presenting with migraine.

Dysembryoplastic neuroepithelial tumors (DNET) are classically considered as benign, cortically based tumors that are stable. However, there were case reports that suggested DNET may not be as benign as previously thought. The purpose of our study was to identify atypical location, characteristics, and behavior of DNET in children.The MRI images of 51 patients with pathologically proven DNET were retrospectively reviewed. The following were assessed: tumor location, neuroimaging appearances including hemorrhage, calcification and edema, tumor growth preoperatively and after subtotal resection, tumor recurrence, malignant transformation, and metastatic seeding.Two (3.9 %) patients had intraventricular lesions, three (5.9 %) had associated edema, three (5.9 %) had calcifications, and one (2 %) had hemorrhage on preoperative CT and MRI. Sixteen of 51 (31.4 %) lesions exhibited enhancement post gadolinium administration. Six of 29 (20.7 %) preoperative lesions that had follow-up imaging were enlarging prior to surgery. In 6 of 18 (33.3 %) with subtotal resection, there was an increase in size of the residual tumor. Tumor recurrence at the surgical bed occurred in 3 of 30 (10 %) patients who had gross total resection. Two of 51 (3.9 %) cases developed secondary lesions distant to the primary tumor; the secondary lesions were within the lateral ventricles.In children, DNET may have atypical location, characteristics and behavior, including growth of primary or residual lesions and multifocal tumor. These findings emphasize the need for follow-up of patients with DNET post-resection.

Distal deletion of the long arm of chromosome 10 is associated with a dysmorphic craniofacial appearance, microcephaly, behavioral issues, developmental delay, intellectual disability, and ocular, urogenital, and limb abnormalities. Herein, we present clinical, molecular, and cytogenetic investigations of four patients, including two siblings, with nearly identical terminal deletions of 10q26.3, all of whom have an atypical presentation of this syndrome. Their prominent features include ataxia, mild-to-moderate intellectual disability, and hyperemia of the hands and feet, and they do not display many of the other features commonly associated with deletions of this region. These results point to a novel gene locus associated with ataxia and highlight the variability of the clinical presentation of patients with deletions of this region.

BACKGROUND: Radiography, typically the first-line imaging study for diagnosis of craniosynostosis, exposes infants to ionizing radiation. We aimed to compare the accuracy of cranial ultrasound (CUS) with radiography for the diagnosis or exclusion of craniosynostosis. METHODS: Children aged 0 to 12 months who were assessed for craniosynostosis during 2011–2013 by using 4-view skull radiography and CUS of the sagittal, coronal, lambdoid, and metopic sutures were included in this prospective study. Institutional review board approval and parental informed consent were obtained. CUS and radiography were interpreted independently and blindly by 2 pediatric radiologists; conflicts were resolved in consensus. Sutures were characterized as closed, normal, or indeterminate. Correlation between CUS and radiography and interreader agreement were examined for each suture. RESULTS: A total of 126 children (82 boys, 64.5%) ages 8 to 343 days were included. All sutures were normal on CUS and radiography in 115 patients (93.7%); craniosynostosis of 1 suture was detected in 8 (6.3%, 5 sagittal, 2 metopic, 1 coronal). In 3 cases the metopic suture was closed (n = 2) or indeterminate on CUS (n = 1) but normally closed on radiography. CUS sensitivity was 100%, specificity 98% (95% confidence interval 94%–100%). Reader agreement was 100% for sagittal, coronal, and lambdoid sutures (κ = 0.80); after consensus, disagreement remained on 3 metopic sutures. CONCLUSIONS: In this series, CUS could be safely used as a first-line imaging tool in the investigation of craniosynostosis, reducing the need for radiographs in young children. Additional assessment may be required for accurate assessment of the metopic suture.

Occipital bone compression of the superior sagittal sinus occurs in supine neonates. The authors previously showed that this compression is associated with neonatal cerebral sinovenous thrombosis, an important neonatal neurological condition, presumably via increased venous stasis. They hypothesized that a pillow alleviating occipital compression could improve cerebral venous flow. Neonates without cerebral sinovenous thrombosis requiring brain magnetic resonance imaging/venography were prospectively enrolled. Demographics, imaging indications, head position, and superior sagittal sinus compression were recorded. Flow velocities in the anterior and posterior superior sagittal sinus and sigmoid sinus before and after pillow placement were quantified using Doppler ultrasound. A total of 10 neonates (6 female, median postconceptional age 40.6 weeks) were enrolled. Flow velocities increased beyond the superior sagittal sinus compression point following pillow placement: 0.09 ± 0.04 m/sec to 0.15 ± 0.12 m/sec ( P = .047) for posterior superior sagittal sinus; 0.10 ± 0.05 m/sec to 0.19 ± 0.15 m/sec ( P = .005) for sigmoid sinus. Pillow decompression can improve neonatal cerebral venous flow, representing a potential noninvasive intervention for the prevention and treatment of neonatal cerebral sinovenous thrombosis.

Color Doppler US (CDUS) has been used for evaluation of cerebral venous sinuses in neonates. However, there is very limited information available regarding the appearance of superficial and deep normal cerebral venous sinuses using CDUS and the specificity of the technique to rule out disease.To determine the specificity, inter-modality and inter-reader agreement of color Doppler US (CDUS). To evaluate normal cerebral venous sinuses in neonates in comparison to MR venography (MRV).Newborns undergoing a clinically indicated brain MRI were prospectively evaluated. All underwent a dedicated CDUS of the cerebral venous sinuses within 10 h (mean, 3.5 h, range, and 2-7.6 h) of the MRI study using a standard protocol.Fifty consecutive neonates participated in the study (30 males [60%]; 25-41 weeks old; mean, 37 weeks). The mean time interval between the date of birth and the CDUS study was 19.1 days. No cases showed evidence of thrombosis. Overall agreement for US reading was 97% (range, 82-100%), for MRV reading, 99% (range, 96-100%) and for intermodality, 100% (range, 96-100%). Excellent US-MRI agreement was noted for superior sagittal sinus, cerebral veins, straight sinus, torcular Herophili, sigmoid sinus, superior jugular veins (94-98%) and transverse sinuses (82-86%). In 10 cases (20%), MRV showed flow gaps whereas normal flow was demonstrated with US. Visualization of the inferior sagittal sinus was limited with both imaging techniques.Excellent reading agreement was noted for US, MRV and intermodality. CDUS is highly specific to rule out cerebral venous thrombosis in neonates and holds potential for clinical application as part of clinical-laboratory-imaging algorithms of pre/post-test probabilities of disease.

Most of the common skeletal dysplasias have some manifestation in the hand. Many have characteristic findings in the hand that lead to the diagnosis. Hand bones are also affected in many systemic hematologic and metabolic conditions. The diagnosis can be clinched on a single hand radiograph if characteristic findings are present. This pictorial essay illustrates characteristic findings of 20 common conditions including bone dysplasias and metabolic and hematologic abnormalities on a single hand radiograph. It also includes some common hand abnormalities without systemic skeletal abnormalities.

The authors reviewed the clinical, laboratory, and imaging data from cases of diarrhea-associated hemolytic uremic syndrome (HUS D+), diagnosed at our institution, from 2001 to 2008. The timing and utility of ultrasonographic features of HUS D+ were analyzed. The aim of the study was to determine factors that could aid in the early diagnosis of this disease. A total of 13 children with HUS D+ were identified out of 23 patients with HUS diagnosed during this time period. Evidence of Escherichia coli 0157:H7 was found in 9 cases (70%). Ultrasound studies were ordered in 10 patients (71%), all of which showed renal sonographic findings compatible with HUS. Ultrasound was performed at a mean of 13 days after onset of the diarrhea. Of note, 2 patients whose ultrasounds were performed at the beginning of their diarrheal illness manifested ultrasonographic features suggestive of HUS when there was only a mild increase in serum creatinine and no decrease in hemoglobin or platelets, suggesting that ultrasonography can identify renal involvement early in the course of the disease before other systemic signs appear. Early renal ultrasound may be a useful adjunct in the initial evaluation in children with bloody diarrhea. Evidence of increased renal echogenicity in a patient with bloody diarrhea could aid in early recognition of HUS when other diagnoses such as intussusceptions are being entertained, potentially allowing early intervention.

Objective To reassess the utility and validity of ultrasound (US) screening in infants with lumbar midline skin stigmata (MSS) that may be associated with tethering of the spinal cord. Study design We conducted a prospective observational study of 254 infants under age 6 months with suspicious dorsal MSS between 2005 and 2007. All infants were examined by US and neurosurgical clinical evaluation, and 50 infants also underwent magnetic resonance imaging (MRI). The US and MRI findings were analyzed for correlation. Associations between the imaging findings and the presence of the low-risk skin lesions simple dimple (113 cases) and deviated gluteal fold (DGF; 44 cases) also were evaluated. Results Analysis of US and MRI results for the cohort of 50 neonates in whom both examinations were performed showed high concordance. The low-risk group of infants with simple dimple and DGF constituted 157 US procedures, 96% of which were of high quality, providing clear visualization of spinal components. None demonstrated any clinically significant pathological findings. Conclusions Our data reaffirm the reliability of US as a screening tool for tethered cord syndrome. Infants with low-risk lesions, such as simple dimple and DGF, may be absolved from US screening, because these findings alone do not indicate underlying pathological lesions. We propose a simplified diagnostic classification system for MSS.

The pituitary gland can demonstrate a variety of pathologies with different clinical presentations. Amongst them, pituitary abscess is a rare infectious disease for which contrast-enhanced MRI aids the diagnostic pathway. We present a 16-year-old girl with imaging and surgical findings consistent with primary pituitary abscess.

OBJECTIVE. Our objective was to semiquantitatively evaluate the diagnostic accuracy of MRI for evaluation of synovium and cartilage of peripheral joints in juvenile idiopathic arthritis (JIA) according to the levels of evidence and recommendations of the Canadian Task Force on Periodic Health Examination guidelines.CONCLUSION. Articles were screened using MEDLINE, EMBASE, DARE, and the Cochrane Library. Two independent reviewers assessed whether the currently available MRI techniques are accurate for diagnosis of synovial hypertrophy and cartilage degeneration in children with JIA. Overall, there is fair (grade B) strength of evidence that MRI is an accurate diagnostic method for evaluating synovium and cartilage and for assessing clinical responsiveness to treatment in peripheral joints in JIA.

OBJECTIVE. The purpose of this article is to present the imaging findings of skeletal and brain abnormalities in thanatophoric dwarfism, a lethal form of dysplastic dwarfism.CONCLUSION. The bony abnormalities associated with thanatophoric dwarfism include marked shortening of the tubular bones and ribs. Abnormal temporal lobe development is a common associated feature and can be visualized as early as the second trimester. It is important to assess the brains of fetuses with suspected thanatophoric dwarfism because the presence of associated brain malformations can assist in the antenatal diagnosis of thanatophoric dwarfism.

The diastrophic dysplasia family of osteochondrodysplasias comprises a spectrum of skeletal diseases characterized by abnormal growth and remodelling of cartilage and bone. They are caused by mutations in the diastrophic dysplasia sulfate transporter (DTDST) gene. Different defects in this gene product give rise to the variety of phenotypes based on the level of residual transport capacity. We reported a case of a fetus with this spectrum, evaluated and diagnosed with fetal MRI.

Importance Lateral ankle injuries without radiographic evidence of a fracture are a common pediatric injury. These children are often presumed to have a Salter-Harris type I fracture of the distal fibula (SH1DF) and managed with immobilization and orthopedic follow-up. However, previous small studies suggest that these injuries may represent ankle sprains rather than growth plate fractures. Objectives To determine the frequency of SH1DF using magnetic resonance imaging (MRI) and compare the functional recovery of children with fractures identified by MRI vs those with isolated ligament injuries. Design, Setting, and Participants A prospective cohort study was conducted between September 2012 and August 2014 at 2 tertiary care pediatric emergency departments. We screened 271 skeletally immature children aged 5 to 12 years with a clinically suspected SH1DF; 170 were eligible and 140 consented to participate. Interventions Children underwent MRI of both ankles within 1 week of injury. Children were managed with a removable brace and allowed to return to activities as tolerated. Main Outcomes and Measures The proportion with MRI-confirmed SH1DF. A secondary outcome included the Activity Scale for Kids score at 1 month. Results Of the 135 children who underwent ankle MRI, 4 (3.0%; 95% CI, 0.1%-5.9%) demonstrated MRI-confirmed SH1DF, and 2 of these were partial growth plate injuries. Also, 108 children (80.0%) had ligament injuries and 27 (22.0%) had isolated bone contusions. Of the 108 ligament injuries, 73 (67.6%) were intermediate to high-grade injuries, 38 of which were associated with radiographically occult fibular avulsion fractures. At 1 month, the mean (SD) Activity Scale for Kids score of children with MRI-detected fibular fractures (82.0% [17.2%]) was not significantly different from those without fractures (85.8% [12.5%]) (mean difference, −3.8%; 95% CI, −1.7% to 9.2%). Conclusions and Relevance Salter-Harris I fractures of the distal fibula are rare in children with radiograph fracture–negative lateral ankle injuries. These children most commonly have ligament injuries (sprains), sometimes associated with radiographically occult avulsion fractures. Children with fractures detectable only by MRI had a comparable recovery with those with sprains when treated with a removable ankle brace and self-regulated return to activities. This work has the potential to simplify the care of these common injuries, safely minimizing the inconveniences and costs of overtreatment.

The brain has a remarkable capacity for reorganization following injury, especially during the first years of life. Knowledge of structural reorganization and its consequences following perinatal injury is sparse. Here we studied changes in brain tissue volume, morphology, perfusion, and integrity in children with hemiplegia compared to typically developing children, using MRI. Children with hemiplegia demonstrated reduced total cerebral volume, with increased cerebrospinal fluid (CSF) and reduced total white matter volumes, with no differences in total gray matter volume, compared to typically developing children. An increase in cortical thickness at the hemisphere contralateral to the lesion (CLH) was detected in motor and language areas, which may reflect compensation for the gray matter loss in the lesion area or retention of ipsilateral pathways. In addition, reduced cortical thickness, perfusion, and surface area were detected in limbic areas. Increased CSF volume and precentral cortical thickness and reduced white matter volume were correlated with worse motor performance. Brain reorganization of the gray matter within the CLH, while not necessarily indicating better outcome, is suggested as a response to neuronal deficits following injury early in life. Guy’s and St Thomas’ Charity and the Marnie Kimelman Trust

A new task force on postmortem imaging was established at the annual meeting of the European Society of Paediatric Radiology (ESPR) in Graz, Austria, in 2015. The postmortem task force is separate from the child abuse task force as it covers all aspects of fetal, neonatal and non-forensic postmortem imaging. The main focus of the task force is the guidance and standardization of non-radiographic postmortem imaging, particularly postmortem CT and postmortem MRI. This manuscript outlines the starting point of the task force, with a mission statement, outline of current experience, and short- and long-term goals.

The objective of our study was to characterize benign lesions showing increased 18F-FDG uptake and to determine their incidence on whole-body FDG PET/CT performed in oncologic patients. In addition, the performance of PET alone and PET/CT in characterizing lesions as benign was compared.A retrospective review of 1,134 consecutive reports of PET/CT studies performed in patients with proven or suspected malignancy over a 6-month period yielded 289 patients with 313 lesions that showed increased FDG uptake but were suspected to be benign (nonphysiologic) or indeterminate. Lesions were subjectively categorized on the basis of the intensity of FDG uptake (mild, moderate, or marked) as compared with background activity. For each lesion, a decision was made as to whether a benign diagnosis could be obtained by the CT part of the study, the PET pattern, or clinical correlation, or whether histologic sampling was necessary. The performance of PET alone and PET/CT for characterizing lesions as benign was compared. Two hundred twenty-nine of the lesions were assessed further: 210 were benign and 19, malignant. The final diagnosis was determined by pathology (n = 67), PET/CT follow-up (n = 58), correlative imaging (n = 59), clinical correlation (n = 32), or typical benign pattern on PET/CT (n = 13).The causes for benign uptake of FDG were inflammatory processes (n = 154, 73.3%), benign tumors (n = 23, 11%), hematoma or seroma (n = 17, 8.1%), fracture (n = 7, 3.3%), fat necrosis (n = 3, 1.4%), and others (n = 6, 2.9%). For lesions with moderate or marked uptake of FDG (n = 117, 55.7%), a benign diagnosis could have been suggested on either PET or CT (e.g., a "hot" osteophyte) in 33 lesions (28.2%), on CT alone (e.g., peritoneal fat necrosis) in 38 lesions (32.5%), on PET alone (e.g., sialadenitis) in 10 lesions (8.5%), or by clinical correlation (e.g., dental abscess) in four lesions (3.4%). A benign diagnosis could not be established without histology (e.g., colonic polyp) in 32 lesions (27.4%). The performance of PET/CT was superior to that of PET alone in characterizing lesions as benign (p0.001).Benign lesions with increased FDG uptake are found in more than 25% of the PET/CT studies performed in patients with proven or suspected malignancy, with inflammation being the most common cause. Lesion characterization on the CT portion of the PET/CT study increases the specificity of PET/CT reporting, especially for lesions with moderate or marked FDG uptake.

Segmental spinal dysgenesis (SSD) is a rare congenital spinal abnormality characterized by segmental dysgenesis or agenesis of the thoracolumbar or lumbar spine, congenital kyphosis, and abnormal configuration of the underlying spinal cord. A unique feature of SSD is that the vertebrae are present above and below the defect, and there is often a lower cord segment in the caudal spinal canal. We report a fetal MRI case of SSD with postmortem and neuropathological correlations. Our report confirms already published findings including the presence of a neurenteric cyst but is the first to document anterior spinal artery segmental agenesis in SSD.

F18-fluorodeoxyglucose (FDG)-positron emission tomography (PET) studies have clinical value in suspected recurrent or metastatic colorectal cancer cases. Because this modality is not accessible for many patients, a camera-based FDG (CB-FDG) coincidence imaging was suggested as an alternative. Although inferior in resolution to a dedicated PET system, it can make FDG studies available to more patients. We assessed the clinical value of CB-FDG in patients with recurrent colorectal cancer.The disease stage and treatment approach in 83 patients were twice determined by an oncologist and a surgeon, first based on the patient's records and blind to CB-FDG findings and then with the inclusion of FDG results in the decision-making analysis.On a lesion-based analysis, the sensitivity of CB-FDG was 95% and the specificity was 81% compared with 88% and 64%, respectively, for computed tomography. Adding FDG findings led to disease-stage alteration in 47 patients (57%), upstaging in 35 (42%), and downstaging in 12 (15%). FDG localized the tumor sites in 21 of 26 patients (81%) with suspected clinical recurrence and a negative conventional imaging workup. In 8 patients, FDG ruled out viable tumor tissue suggested by other modalities. The oncologist's suggested treatment approach was altered in 54% of the patients and the surgeon altered the decision on operability in 28%.CB-FDG assessment has clinical value for both staging and selecting treatment in patients with recurrent colorectal cancer and can be considered an alternative to an nonaccessible dedicated PET system.

Wandering spleen is an extremely rare condition in which the spleen is lacking its normal ligamentous attachments and therefore can move to an ectopic position in the abdomen or pelvis. Wandering spleen predisposes the patient to life-threatening complications due to torsion of the spleen's vascular pedicle, with resulting splenic infarction, portal hypertension, and bleeding. Because of the nonspecific symptoms, imaging plays an important role. To our knowledge, only a few case reports describing wandering spleen in the pediatric population have been published, including 2 cases in infancy. We report a case of splenic torsion in an infant with a preoperative diagnosis made on the basis of color and power Doppler sonography. The diagnosis was confirmed by contrast-enhanced helical computed tomography (CT) and proved at surgery. On sonography, a diffusely hypoechoic spleen and a mass at the splenic hilum representing the torsed splenic pedicle were shown. The splenic hilar mass correlates with the CT "whirl" sign indicative of torsion.

Aim To review the long-term clinical and imaging follow-up of eight children with histopathologically proven childhood primary central nervous system angiitis (cPACNS). Materials and methods Eight children (5–17 years, five female and three male patients) with biopsy-proven cPACNS vasculitis were reviewed retrospectively. All children were followed at the Children's Hospital of Eastern Ontario. Magnetic resonance imaging (MRI) images of the brain parenchyma and vessel pattern of the arteries of the circle of Willis were reviewed at baseline and follow-up(s). All MRI images were correlated with clinical and histopathological findings. Results Seven (87.5%) children demonstrated parenchymal abnormalities: five of the seven (71.4%) presented with non-haemorrhagic lesions, one (14.3%) presented with a parenchymal bleed, and one (14.3%) showed diffuse oedema. One child remained negative for brain parenchymal abnormality at baseline and follow-up. Of the seven children with a parenchymal abnormality, parenchymal enhancement was seen in two (28.5%) and leptomeningeal enhancement was seen in one child. Only two children had positive diffusion-weighted imaging (DWI) restriction at baseline. Five of the seven children (71.4%) showed no magnetic resonance angiography (MRA) abnormalities at baseline. In one, MRA detected complete absence of flow. Follow-up MRA (24–74 months) demonstrated that five of the seven (71.4%) children had progressive paucity of the peripheral vascularity. Two children (28.5%) with shorter-term follow-up (7 and 13 months) did not demonstrate MRA changes. Conclusion Long-term follow-up MRA demonstrated progressive paucity of peripheral vessels, which could be secondary to the inflammation affecting the peripheral vasculature causing reduction in vessel lumen size beyond the resolution of the maximum intensity projection (MIP) reconstruction.

Infants with phosphomannomutase 2 – congenital disorder of glycosylation (PMM2-CDG), formerly known as CDG1a, present with failure to thrive, visceral dysfunction, thromboembolic events and developmental delays noted before 6 months of age. Diagnosis is often delayed due to the considerable variability in phenotype. Characteristic, but not universal, features include inverted nipples and abnormal subcutaneous fat pads. Neuroimaging performed in the first 4 months of life may be normal, although cerebellar and brainstem atrophy is usual after 3 months of age. Cerebellar and brainstem atrophy have been noted as early as 11 days of life. We present an infant whose typical subcutaneous and retroperitoneal fat deposits were clinically occult, but identified on body MRI.