MRI quantitative muscle characterization in children with X-linked hypophosphatemia.

INTRODUCTION: Children with X Linked Hypophosphatemia (XLH) suffer from carential ricket, bone deformities and lameness. No previous study demonstrated a morphological distinction in muscles in these patients. The aim of this prospective study was to characterize, using Magnetic Resonance Imaging (MRI), the muscle morphology of pelvis, thigh and leg in children with XLH and to compare it with typically developed (TD) children. HYPOTHESIS: We hypothesized that lower limbs muscles in children with XLH are different from TD children and could explain limp walking. MATERIAL AND METHODS: Three-dimensional reconstructions of the muscles were performed in 11 patients with XLH and 15 TD children. Muscle lengths, sections and volumes were calculated and normalized with height and weight. Mean age was 10. RESULTS: Lengths were all smaller in children with XLH except for the Medius/minimus gluteus muscles (p=0.64). The difference seemed higher in muscles with a long tendinous part as semitendinosus (0.139 vs 0,164; p<0.01). All volumes were significantly inferior in children with XLH. This preliminary study showed significant differences in muscle structures between patients with XLH and TD children. DISCUSSION: Medius/minimus gluteus seemed to be particularly developed in children with XLH. Nevertheless it is not possible to conclude if it is related to XLH or a consequence of bone deformities. LEVEL OF PROOF: IV.

Benign enlargement of the subarachnoid spaces and subdural collections-when to evaluate for abuse.

In infants without a history of trauma, subdural haemorrhages should raise the concern for an abusive head injury, particularly when they are associated with bridging vein clotting/rupture or with septations. However, non-haemorrhagic, fluid-appearing subdural collections (also called hygromas) may also be the result of abuse. Subdural collections have also been uncommonly observed in patients with benign enlargement of the subarachnoid spaces (BESS) and a few large-scale studies accurately investigate the incidence and the significance. Currently, there is a wide variation of practices in children with BESS and subdural collections. Due to the social risks associated with abuse evaluation and the perceived risk of radiation exposure, there might be a reluctance to fully evaluate these children in some centres. The diagnosis of physical abuse cannot be substantiated nor safely excluded in infants with BESS and subdural collection(s), without investigation for concomitant traumatic findings. The exact prevalence of occult injuries and abuse in these infants is unknown. In macrocephalic infants with subdural collections and imaging features of BESS, thorough investigations for abuse are warranted and paediatricians should consider performing full skeletal surveys even when fundoscopy, social work consult, and detailed clinical evaluation are unremarkable.

Controversial aspects of imaging in child abuse: a second roundtable discussion from the ESPR child abuse taskforce.

This second roundtable discussion was convened at the 56th European Society of Paediatric Radiology (ESPR) 2022 Annual Meeting in Marseille, France, to discuss controversial aspects of imaging in child abuse. The following topics were discussed: Fracture dating-the published literature is broadly similar with respect to the identification of the radiographic stages of bony healing. The non-expert/general radiologist is encouraged to use broad descriptors of fracture healing (acute, healing or old) within their reports, rather than attempting to date fractures. The more experienced/expert radiologist, who may provide a timeframe/range to assist the courts, should be aware that any published timeframes are not absolute and that recent research indicates that the rate of healing may differ according to the bone affected and the age of the patient. Whole spine imaging in suspected abusive head trauma-this is recommended to enable a complete assessment of the neuraxis when abusive head trauma is suspected or diagnosed, particularly in the presence of intracranial and cervical subdural haemorrhage and cervical ligamentous injury. Cranial imaging in suspected physical abuse-both computed tomography (CT) and magnetic resonance imaging (MRI) remain complimentary depending on the clinical context in which they are used with CT remaining first-line in the assessment of children with (suspected abusive) head trauma prior to an early MRI. MRI is superior in its assessment of parenchymal injury and may be employed as first-line in age appropriate asymptomatic siblings of a child with suspected physical abuse.

Chronic Recurrent Multifocal Osteomyelitis (CRMO) and Juvenile Spondyloarthritis (JSpA): To What Extent Are They Related?

Chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory disease occurring mainly in the pediatric age group (before 16 years) and generally presents as a separate entity. Synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome combines osteoarticular and cutaneous involvement, similar to CRMO, and falls into the spectrum of spondyloarthritis (SpA). The fact that a patient can progress from one disease to another raises the question of whether CRMO, like SAPHO, could fall within the spectrum of SpA, ranging from a predominantly osteoarticular form to an enthesitic form with more or less marked skin involvement. In this review, we set out to discuss this hypothesis by highlighting the differences and similarities between CRMO and juvenile SpA in clinical, radiological and pathophysiological aspects. A common hypothesis could potentially consider intestinal dysbiosis as the origin of these different inflammatory diseases. Interindividual factors such as gender, environment, genetics and/or epigenetic background could act as combined disease modifiers. This is why we suggest that pathophysiology, rather than clinical phenotype, be used to reclassify these diseases.

Analysis of whole-body MRI artifacts in a pediatric population with a special emphasis on the effect of hands position.

PURPOSE: The purpose of this retrospective study was to determine the prevalence of artifacts on whole-body (WB) magnetic resonance imaging (MRI) examination in pediatric patients and identify their causes. MATERIALS AND METHODS: A total of 107 pediatric patients who underwent a total of 107 WB-MRI examinations, including short-tau inversion recovery (STIR) and T1-weighted sequences, were included. There were 62 girls and 45 boys with a mean age of 11 ± 3 (SD) years (age range: 2-16 years). WB-MRI examinations were analyzed for the presence of artifacts on STIR and T1-weighted sequences. Artifacts were further assigned to one of eight categories (motion, partial volume, cross-talk, phase sampling, susceptibility, equipment, noise, and "other") and 19 anatomical sites by a 4-year resident. Prevalence of artifacts were analyzed especially according to hands position during the examination for the upper limbs and patients' age. Age was expressed as a binary variable using median age (10 years) as the cut-off value. All qualitative variables were compared using chi-square test. RESULTS: A total of 3436 artifacts were found. The STIR sequences showed more "noise" artifacts (93/1038; 8.96%) and more "cross-talk" (102/1038; 9.83%) artifacts than T1-weighted sequences (12/1038 [1.16%] and 7/1038 [0.67%], respectively) (P < 0.001 for both). T1-weighted sequences showed more "equipment" (84/1038; 8.09%) and "stair-step" (a subset of "other") (41/1038; 3.95%) artifacts than the STIR sequences (39/1038 [3.76%] and 21/1038 [2.02%], respectively) (P < 0.001 and P = 0.01, respectively). T1-weighted sequences showed fewer artifacts on the wrists when the hands were under the bottom (P = 0.001). T1-weighted sequences showed less "equipment" artifacts when the hands were alongside the body (22/296; 7%) than on the abdomen (48/432; 11%) or under the bottom (14/128; 11%) (P < 0.001). STIR sequences showed more "motion" artifacts when the hands were on the abdomen (54/432; 13%) than alongside the body (30/296; 10%) or under the bottom (15/128; 12%) (P < 0.001). WB-MRI examinations had more "susceptibility" artifacts (38/960; 4%) and more "equipment" artifacts (81/960; 8.4%) in patients older than 10 years than in those under 10 years (23/752 [3.1%] and 42/752 [5.6%]) (P = 0.01 and P < 0.001, respectively). CONCLUSION: Artifacts on WB-MRI do not affect coronal STIR and T1-weighted sequences equally, so the use of both sequence types appears useful. Hands position should be considered with respect to both diagnostic benefit and safety.

Prospective Analysis of Muscle Adiposity in Children With X-linked Hypophosphatemic Rickets vs Control Children.

Context: Children with X-linked hypophosphatemic (XLH) rickets have muscle weakness that severely impairs their function. Intermuscular and intramuscular adipose tissue (IMAT and intraMAT, respectively) may contribute to this muscle weakness. Objective: This work aimed to compare IMAT and intraMAT in XLH children vs typically developing (TD) children. Methods: A prospective, monocentric cohort study was conducted of XLH (n = 11; aged 10.3 years [6-17]) and TD children (n = 22; aged 10.2 years [5-15.5]). All children underwent magnetic resonance imaging of the lower limbs; IMAT and intraMAT percentages were calculated after manual contouring of each muscle of the thigh and the deep fascia at mid-thigh level. Results: XLH children were comparable in age but shorter and heavier than TD children (P = .001 and P = .03, respectively). They had smaller muscle length and volume than TD children (P < .001) but there was no statistically significant difference in muscle cross-sectional area between the groups (P = .833). The total percentage of IMAT was higher in XLH children (8.66% vs 3.60% in TD children; P < .0001). In addition, though the total percentage of intraMAT did not differ significantly (12.58% and 10.85% in XLH and TD children, respectively; P = .143) intraMAT was statistically significantly higher in XLH children than TD children in 4 of the 13 muscles studied. Conclusion: Our results show that IMAT is higher in young children with XLH, independently of obesity and overweight. Further, these results will facilitate both the early prevention of functional and metabolic consequences of the increase in adipose tissue in XLH children.

Imaging findings in abusive head trauma (AHT).

Brain imaging plays a key role in accurately identifying abusive head trauma (AHT). An exact and rapid diagnosis is needed due to the extreme severity of AHT, since there is a risk of neurological sequelae and potentially fatal recurrence. Several medical specialists will work collaboratively to detect and confirm abuse in children: the radiologist has a leading role in this approach. This article describes the most common neuro-imaging patterns of AHT, including extra axial, intra axial, bony, and ligamentous lesions, with a special focus on the dating issue and the differential diagnosis.

Is bridging vein rupture/thrombosis associated with subdural hematoma at birth?

BACKGROUND: The combination of bridging vein rupture/thrombosis and subdural hematoma in infants has recently gained attention as highly suggestive of abusive head trauma. While subdural hematomas are frequently observed at birth, there are no previous studies of bridging vein rupture/thrombosis prevalence in that context. OBJECTIVE: To evaluate the prevalence of bridging vein rupture/thrombosis in newborns with and without subdural hematoma. MATERIALS AND METHODS: This bicentric retrospective study (2012-2019) looked at all brain MRIs performed in neonates. We noted delivery method, demographic data and intracranial injuries and analyzed any clots at the vertex as potential markers of bridging vein rupture/thrombosis. RESULTS: We analyzed 412 MRIs in 412 neonates. Age was (mean ± standard deviation [SD]) 5.4±2.2 days and 312 (76%) infants were full term (38.3±2.9 weeks from last menstrual period). The delivery method was vaginal birth for 42% (n=174), cesarean section for 43% (n=179), and unknown for 14% (n=59). Subdural hematoma was present in 281 MRIs (68.0%, [95% confidence interval = 63.3-72.5]). Six MRIs showed at least one clot at the vertex, assumed to be possible bridging vein rupture/thrombosis (1.5%, [0.5-3.1%]). Only one MRI showed more than two clots at the vertex, in a context of maternal infection. There was no significant difference in terms of gestational age at birth, delivery method or the presence of subdural hematoma or parenchymal injuries between those 6 infants and the 406 others. CONCLUSION: Bridging vein rupture/thrombosis at birth is very rare and unlikely to be related to subdural hematoma.

Imaging patterns in pediatric hypophosphatasia.

Hypophosphatasia is a rare genetic disorder of calcium and phosphate metabolism due to ALPL gene mutations, which leads to abnormal mineralization of the bones and teeth. Hypophosphatasia is characterized by low serum alkaline phosphatase activity and a number of clinical signs, including failure to thrive, bone pain and dental issues. The diagnosis is suspected based on clinical, laboratory and imaging findings and confirmed by genetic testing. Diagnosis in children is often delayed due to a lack of disease awareness, despite specific imaging findings that are a cornerstone of the diagnosis. The recent approval of enzyme replacement therapy (bone-targeted recombinant tissue nonspecific alkaline phosphatase) has given imaging an important role in monitoring treatment efficacy. The aim of this pictorial essay is to review the imaging features of hypophosphatasia at diagnosis and during follow-up, including whole-body magnetic resonance imaging patterns.

Magnetic resonance imaging is a valuable tool to evaluate the therapeutic efficacy of burosumab in children with X-linked hypophosphatemia.

PURPOSE: To examine the MRI diagnostic performance in the assessment of therapeutic response to burosumab in children with X-linked hypophosphatemia (XLH). DESIGN: Prospective longitudinal open cohort. PATIENTS: Seventeen children with XLH, average age of 10.2 ± 2.7 years, had a knee MRI at baseline and after 1 year of burosumab. INTERVENTION: Children received burosumab at an average dose of 1.4 ± 0.5 mg/kg during 1 year for the treatment of severe rickets (the target serum phosphate ≥ 1.2 mmol/L (≥3.7 mg/dL). The primary endpoint was the change from baseline to 12 months in rachitic lesions on knee MRI. Secondary endpoints were changes in biochemical parameters of phosphate and alkaline phosphatase (ALP). RESULTS: One year of treatment with burosumab significantly reduced radiological disease activity on knee MRI (by 44 ± 29% in the transverse extent of widening) which was accompanied by a significant reduction in biochemical activity, namely in serum ALP activity, by 28 ± 17%. Additionally, MRI parameters after 1 year of treatment with burosumab (the maximum width of medial physis at 12 months and the change from baseline in the maximum width of lateral physis) were associated with ALP activity at 12 months. CONCLUSION: We suggest that MRI is a valuable and quantitative tool to evaluate the therapeutic response to burosumab. MRI could be an excellent alternative to standard bone radiographs for evaluation of the rachitic lesions in a clinical setting avoiding repeated exposition to ionizing radiation.

New image quality and dose reduction technique for pediatric digital radiography.

PURPOSE: The purpose of this study was to test a new post-processing and denoising engine for patient dose reduction while maintaining diagnostic image quality (IQ) in pediatric digital radiography (DR). MATERIALS AND METHODS: Pediatric DR images of the thorax, pelvis, abdomen and spine obtained in 174 patients (102 males, 72 females; mean age, 2±1.8 [SD] years; age range: 6 months-9 years) were retrieved. Artificial noise was added to the images to simulate acquisitions at 50%, 32% and 12.5% of the routine dose levels. A total of 696 images corresponding to four dose levels were post-processed using S-Vue™ and further blindly scored by three pediatric radiologists using a scoring grid of 4-6 criteria specifically defined per anatomical area. The mean score was assessed for each area and weight class (5-15 and 15-30kg) and compared across the simulated low dose images. Paired Wilcoxon test was used with a threshold difference of 0.5 (half a criterion) between scores to highlight a significant reduction in image quality. Inter-rater reliability was assessed using intraclass correlation coefficient (ICC). RESULTS: Only the 50% reduced dose images showed non-inferiority when compared to routine images for all of areas and weight classes (P<0.01). Very good inter-rater reliability of the overall scores was observed for the pelvis in the 5-15kg weight class (ICC=0.85) for images at full dose, 50% and 32% reduced dose. For the remaining areas (thorax, abdomen and spine) and weight classes, inter-rater reliability was moderate (ICC: 0.3-0.6). CONCLUSION: S-Vue™ post-processing software allows a two-fold radiation dose reduction while maintaining satisfactory IQ in pediatric DR.

Pediatric idiopathic intervertebral disc calcification (IIDC): a challenging case.

Idiopathic intervertebral disc calcification is a rare condition in children with a very good prognosis. As there are no biological markers, imaging is invaluable for diagnosing this "do not touch" lesion. While the characteristic feature is nucleus pulposus calcification at one or more cervical or thoracic levels, it is important that practitioners be able to recognize atypical patterns so that biopsy can be avoided. Here we report a case of pediatric idiopathic intervertebral disc calcification with contiguous vertebral involvement and anterior longitudinal ligament ossification.

Breath-hold compressed-sensing 3D MR cholangiopancreatography compared to free-breathing 3D MR cholangiopancreatography: prospective study of image quality and diagnostic performance in pancreatic disorders.

PURPOSE: To compare image quality and diagnostic performance of three magnetic resonance cholangiopancreatography (MRCP) protocols in patients with suspected pancreatic abnormalities: free-breathing standard 3D-MRCP (STD), free-breathing compressed sensing 3D-MRCP (CS), and CS 3D-MRCP with acquisition during a single breath-hold > 20 s (BH-CS). METHODS: Informed consent was obtained. We performed 57 MRCPs in 56 prospectively included patients (29 men, median age 59 years). The three protocols were performed in random order. Acquisition time was recorded. Two radiologists blinded to the protocols used 5-point scales to assess image quality parameters (overall image quality, amount of artifacts, background suppression, bile and pancreatic duct visualization) and diagnostic performance (anatomical variants, duct abnormalities, cystic lesions). RESULTS: Acquisition time was 279 s with STD, 176 s with CS (-37%), and 22 s with BH-CS (-93%). STD and BH-CS were not significantly different for overall image quality, artifacts, or background suppression. The BH-CS group had fewer non-diagnostic scans (3% vs. 19% with STD and 21% with CS, p < 0.05), higher-quality scans (78% vs. 66% with STD and 58% with CS, p < 0.05), and milder artifacts (2% vs. 18% with STD and 16% with CS, p < 0.05). The main pancreatic duct was better visualized with BH-CS compared to STD (p = 0.015) and CS (p < 0.001). Diagnostic performance did not differ across the three protocols. There were fewer indeterminate scans in the BH-CS group. CONCLUSION: 3T BH-CS is reliable, saves time, and is not associated with decreases in image quality or diagnostic performance compared to STD and CS.

Subsolid Lung Nodule Classification: A CT Criterion for Improving Interobserver Agreement.

Purpose To evaluate an objective computed tomographic (CT) criterion for distinguishing between part-solid (PS) and nonsolid (NS) lung nodules. Materials and Methods This study received institutional review board approval, and patients gave informed consent. Preoperative CT studies in all patients who underwent surgery for subsolid nodules between 2008 and 2015 were first reviewed by two senior radiologists, who subjectively classified the nodules as PS or NS. A second reading performed 1 month later used predefined classification criteria and involved a third senior radiologist as well as three junior radiologists. Subsolid nodules were classified as PS if a solid portion was detectable in the mediastinal window setting (nonmeasurable, < 50%, or > 50% of the entire nodule) and were otherwise classified as NS (subclassified as pure or heterogeneous). Interreader agreement was assessed with κ statistics and the intraclass correlation coefficient (ICC). Results A total of 99 nodules measuring a median of 20 mm (range, 5-47 mm) in lung window CT images were analyzed. Senior radiologist agreement on the PS/NS distinction increased from moderate (κ = 0.54; 95% confidence interval [CI]: 0.37, 0.71) to excellent (κ = 0.89; 95% CI: 0.80, 0.98) between the first and second readings. At the second readings, agreement among senior and junior radiologists was excellent for PS/NS distinction (ICC = 0.87; 95% CI: 0.83, 0.90) and for subcategorization (ICC = 0.82; 95% CI: 0.77, 0.87). When a solid portion was measurable in the mediastinal window, the specificity for adenocarcinoma invasiveness ranged from 86% to 96%. Conclusion Detection of a solid portion in the mediastinal window setting allows subsolid nodules to be classified as PS with excellent interreader agreement. If the solid portion is measurable, the specificity for adenocarcinoma invasiveness is high. © RSNA, 2017 Online supplemental material is available for this article.