Fibromatosis Colli: A Thorough Description of Its MRI Characteristics and a Review of the Literature.

Introduction: Fibromatosis colli (FC) is a rare pseudotumor of the sternocleidomastoid muscle with an incidence of 0.4%, generally diagnosed using ultrasound between 2 and 4 weeks of age. This is an important entity considering the clinical concerns it causes due to its appearance as a cervical mass with torticollis. Few magnetic resonance imaging (MRI) descriptions of its appearance have been made, with the existing reported cases being sporadic. We aim to provide a thorough description of this paediatric entity. Materials and Methods: We conducted a retrospective study by searching our hospital's database for previous cases of FC where an MRI had been performed. We found six cases of FC where an MRI had been performed. Of these cases, five out of six were contrast-enhanced. We examined the MRIs to be able to discern and describe the MRI characteristics of FC. Results: We found that FC presents a T1 signal isointense to the muscle, a T2 signal hyperintense to the muscle, a variable diffusion signal and a thick enhancing peripheral ring after contrast administration. Discussion: Our results match what has been reported in the literature to date regarding the MRI signal of FC, confirming previous reports. However, we provide new data regarding the characteristic appearance post-enhancement, which was previously unreported. Conclusion: The MRI characteristics of FC have rarely been described, with only a few isolated case reports in the medical literature. We review the current literature, describe the key MRI characteristics of the pathology, and provide the most thorough description to date.

Multiple Cases of Discoid Menisci within a Family: A Case Report and Literature Review.

Discoid menisci are thought to be heritable. However, few documented cases of this occurring within families exist. We present the case of siblings with lateral discoid menisci, documented by knee magnetic resonance imaging (MRI), reinforcing the case for the existence of familial discoid menisci. The children's father also reportedly had a discoid meniscus, but proof was unavailable due to his country of origin's poor record keeping. We put this into the context of other rare, reports of similar cases. Teaching Point: We present further case of discoid menisci occurring within families, a long-held belief with little concrete supporting evidence.

Gaucheroma of Mesenteric Lymph Nodes and Their Ultrasound Appearance: A Case Report.

Gaucher disease represents the largest lysosomal storage disease group worldwide. Possible complications include the development of Gaucheromas, pseudotumors resulting from an accumulation of Gaucher cells. Gaucheromas can affect the liver, spleen, bones, and lymph nodes. Descriptions of the appearance of lymph node gaucheromas exist for computed tomography (CT) and magnetic resonance imaging (MRI) but not, to our knowledge, of their ultrasound characteristics. We present the case of a four-year-old boy with Gaucher disease with lymph node Gaucheromas, discovered during a routine follow-up, and present their ultrasound characteristics. We describe characteristic ultrasound findings of non-B-cell lymphomas and Gaucheroma lymph nodes in comparison. Teaching point: Lymph node Gaucheromas have a characteristic ultrasound appearance and should be searched for in Gaucher's patients.

Case report: Subacute transverse myelitis with gait preservation secondary to Lyme disease and a review of the literature.

Subacute presentation with gait preservation is rare in the initial presentation of transverse myelitis (TM) in children. Lyme TM is poorly described in the literature. Here, we present the case of a 10-year-old boy who presented with neck pain with irradiation in the upper limbs for 13 days, accompanied by a right latero-torticollis. Magnetic resonance imaging (MRI) of the spine showed a hypersignal in the centromedullary T2 weighted image (WI) between C1 and C7, which was suggestive of cervical TM. A lumbar puncture revealed pleocytosis and proteinorachia. The test results of Borrelia IgG in the blood and intrathecal IgG synthesis were positive, confirming the diagnosis of TM secondary to Lyme disease. The patient was treated with high doses of steroids and antibiotics, following which he recovered completely. After a review of the clinical features of the eight previously published pediatric cases, we can conclude that Lyme TM usually has a subacute clinical presentation and is frequently limited to the cervical spine with pure sensory symptoms and gait preservation. Moreover, acute and chronic sphincter dysfunction is rare, and recovery is usually complete.

American College of Radiology (ACR) Appropriateness Criteria and EURO-2000 Guidelines Offer Limited Guidance for MRI Imaging of Pediatric Patients.

PURPOSE: This study aims to evaluate the clinical scenarios addressed by the EURO-2000 guidelines and the ACR appropriateness criteria for referring children to MRI and assessing the referring physician compliance with both guidelines. METHODS: In January 2022, clinical scenarios reported in the last 1000 consecutive MRI requests for children (0-16 years) in one university children's hospital and two general university hospitals in Belgium, Europe, were retrospectively matched to the EURO-2000 guidelines and ACR appropriateness criteria. The number of clinical scenarios addressed and the justification for MRI referral were independently assessed for both guidelines. Pooled data from the three centers were evaluated and then analyzed by center, body area and prescriber using McNemar's test for paired proportions and χ2-tests unpaired proportions. RESULTS: After excluding incomplete or missing MRI requests, 2932 of 3000 requests were included in the analysis. Overall, out of 2932 clinical scenarios, 1229 (37.99%) were addressed by EURO-2000 and 1081 (36.37%) were addressed by the ACR appropriateness criteria (McNemar test, p = 0.12). The proportions of clinical scenarios covered by the two guidelines were statistically similar when comparing centers, but varied across body regions (p < 0.001) and referring physician specialty (p between 0.75 and 0.001). EURO-2000 guidelines provided better coverage for head and spine (p < 0.05), while the ACR appropriateness criteria provided broader coverage for abdomen, pelvis, and musculoskeletal system (p < 0.0001). For addressed clinical scenarios, prescriber compliance for both guidelines was excellent with > 94% of justified MRI examinations in all the centers. CONCLUSIONS: Both the EURO-2000 guidelines and the ACR appropriateness criteria did not address two-thirds of clinical scenarios in children. Head and neck, chest and abdominal-pelvic examinations are the anatomic regions which should receive a specific attention for the future implementation of evidence-based clinical decision support tools for all referring specialists.

Gastroduodenal Intussusception in Peutz-Jeghers Syndrome.

Teaching points: Gastroduodenal intussusception is an infrequent cause of abdominal pain in children, for which a lead-point is nearly ubiquitous, which imposes endoscopic reduction as the first line of treatment.

Topography of Bone Erosions at the Metatarsophalangeal Joints in Rheumatoid Arthritis: Bilateral Mapping by Computed Tomography.

OBJECTIVES: To describe the bilateral anatomical location of bone erosions (BE) at the metatarsophalangeal joints in patients with rheumatoid arthritis using computed tomography. MATERIALS AND METHODS: Eighteen consecutive patients with established rheumatoid arthritis prospectively underwent computed tomography of both forefeet. Each joint surface of the metatarsal heads (MTH) and the proximal phalangeal bases were divided into four quadrants: superior, plantar, tibial, and fibular. The number of BE was cumulatively counted per patient, side, joint, per joint surface, and quadrant. Descriptive statistics, paired and unpaired samples t-tests, Pearson's correlation coefficients, ANOVA 2, and variance component analysis were performed. RESULTS: There were 288 BE at the MTH and 66 at the proximal phalanges. The number of BE in one forefoot was a poor predictor of the absolute number of BE on the contralateral foot "r=0.54" and was unrelated to symptoms. The superior quadrants were less frequently affected than other quadrants for both the MTH "p<0.0001" and proximal phalanges "p<0.001." The tibial quadrant showed a higher number of BE compared to all other quadrants for MTH "p<0.03," proximal phalanges "p<0.01, and for the metatarsophalangeal joint as a whole "p<0.0001." Plantar and fibular quadrants were equally affected "p<0.05." CONCLUSION: BE were found more frequently on the tibial side of the MTH in patients with rheumatoid arthritis.

Preoperative and Postoperative Imaging in Idiopathic Scoliosis: What the Surgeon Wants to Know.

The term idiopathic scoliosis covers a broad spectrum of spinal deformities in the pediatric population without an underlying congenital anomaly of the spine. Depending on the age of presentation, it has both characteristic clinical and imaging features and a different prognosis. The radiologist should provide the surgeon with critical information to assess the degree of deformity and eventually plan surgery. Thoracic deformities and lung volume must also be part of the preoperative assessment. Imaging has a critical role in postsurgical follow-up and in surgical complications. This review highlights the importance of common terminology and measurement methods to avoid incongruences. The different imaging modalities are discussed with their indications and limitations. We pay special attention to imaging modalities that can help the surgeon assess skeletal maturation reliably and thus predict the prognosis of scoliosis. Radiation protection and the risk of cumulative radiation exposure in these patients is emphasized.

Are Automated and Visual Greulich and Pyle-Based Methods Applicable to Caucasian European Children With a Moroccan Ethnic Origin When Assessing Bone Age?

Introduction To test the accuracy of the visual and automated bone age assessment base on the Greulich and Pyle (GP) method in healthy Caucasian European children with a Moroccan ethnic origin. Material and methods Moroccan Caucasian (MC) children were retrospectively and consecutively enrolled along with age- and sex-matched control group (CG) of European Caucasian (EC) children enrolled from the general population. The two groups included 423 children aged from 2 to 15 years with a normal left-hand radiograph performed to rule out a trauma between March 2008 and December 2017. One radiologist, blinded to the BoneXpert® (Visiana, Holte, Denmark) estimates, visually reviewed the radiographs using the GP atlas. The BoneXpert® automatically analysed all 423 radiographs. The intraclass correlation coefficient (ICC), linear regression and Bland-Altman plots were performed to describe the agreement between each method and the chronological age (CA) and the agreement between the two methods. Results Visual bone age assessment was related to the CA in both girls (MC ICC 0.97; EC ICC 0.97) and boys (MC ICC 0.95; EC ICC 0.96). Automated bone age assessment was related to the CA in both girls (MC ICC 0.97; EC ICC 0.96) and boys (MC ICC 0.88; EC ICC 0.96). Bland-Altman plots showed an excellent agreement between the two methods in both sexes and ethnicities before puberty especially in Moroccan boys. Conclusion Visual and automatic bone age assessment based on the GP method, previously validated in the general population of Caucasian European children, can be confidently used in healthy Caucasian European children with a Moroccan ethnic origin.

Chest computed tomography (CT) features in children with reverse transcription-polymerase chain reaction (RT-PCR)-confirmed COVID-19: A systematic review.

To describe the chest CT features reported in children with confirmed COVID-19 infection, published in English literature. A systematic review was completed on PubMed, Embase and Scopus databases on the 1st of June 2020 using the PICO strategy. The NIH Quality Assessment Tool was used to assess the quality of the selected articles. The systematic review was evaluated by Case Series Studies and the Preferred Reporting Items for a Systematic Review and Meta-analysis of Diagnostic Test Accuracy Studies (PRISMA). The extracted data were assessed and compared with those reported in the adult population. Seventy-two articles were retrieved from the database search and screened by the title, abstract and keywords. Eleven articles were deemed eligible for full-text assessment. Nine articles were included for the data extraction and in the final analysis. Chest CT features in children with COVID-19 differ from those in adults. 'Ground-grass opacities' (GGOs) are the most commonly described abnormalities, but closely followed by a combination of GGO and consolidation, not usual in adults. Children tend to have a more variable involvement than the subpleural and posterior and basal topography described in adults. Interlobular thickening and air bronchogram found in adults with COVID-19 are not frequent in children. Pulmonary embolism reported in up to 30% of adults has not been yet reported in children. Original articles describing chest CT features in children with COVID-19 in the English literature are limited to small populations of Chinese children. Chest CT imaging features are very diverse across the selected studies and globally different from those reported in adults. Data from children of different countries would provide a more comprehensive description of chest CT features in children with COVID-19.

Radiotriquetral Ligament in Madelung's Deformity Associated with Leri-Weill's Dyschondrosteosis.

Madelung's deformity (MD) is frequently associated with Leri-Weill's dyschondrosteosis (LWD) even if the primary isolated form (PI-MD) is much more common. Recent studies pointed out how two abnormal ligaments, the Vickers ligament (VL) and the radiotriquetral ligament (RTL), are defining traits of MD. To date, in PI-MD, both VL and RTL have been reported. In MD associated with LWD (LWD-MD), the VL is also present, but the RTL has never been reported. We herein report the first case of MD associated with a genetically confirmed LDW with an RTL, detected on MRI. This report describes the MRI imaging features of MD-LWD, which have not been adequately characterized in previous literature.

Relevance of brain lesion location for cognition in vascular mild cognitive impairment.

BACKGROUND: Vascular mild cognitive impairment (VMCI) is a potentially transitional state between normal aging and vascular dementia. The presence of macroscopic white matter lesions (WML) of moderate or severe extension on brain MRI is the hallmark of the VMCI. OBJECTIVE: To assess the clinical relevance of the frequency of WML in patients with VMCI independently of total lesion volume (LV). METHODS: In this multicenter study, we included 110 patients with VMCI (age: 74.3 ±â€¯6.6 years; sex: 60 women). Cognitive assessment was performed with the VMCI-Tuscany Neuropsychological Battery, which allowed to identify four VMCI groups: amnestic single (n = 9) and multi-domain (n = 76), non-amnestic single- (n = 10) and multi-domain (n = 15). Distribution and frequency of WML on MRI FLAIR images were evaluated with lesion probability map (LPM). Voxelwise statistics was performed with nonparametric permutation tests, controlling for age, sex, slice thickness, center, magnetic field strength, total LV and head size (p < .01, family-wise error-corrected for multiple comparisons across space). RESULTS: LPM of the WML had a fairly symmetric and widespread distribution across brain. A higher frequency of WML along association tracts of the WM such as inferior longitudinal fascicle, inferior fronto-occipital fascicle and superior longitudinal fascicle, was correlated with worst cognitive scores at the Trail Making Test Part A and Copy of the Rey-Osterrieth Complex Figure. The non-amnestic groups showed a higher frequency of WML in the anterior cingulum and superior longitudinal fascicle close to the frontal gyrus. CONCLUSIONS: Our study showed that in patients with VMCI, independently of total LV, the higher frequency of lesions along association tracts of the WM, which mediate intrahemispheric long-range connectivity, is related with psychomotor speed and constructional praxis. Moreover, a prevalence of lesions in the frontal WM seems to characterize VMCI patients with involvement of non-amnestic domains.

Reduced brain atrophy rates are associated with lower risk of disability progression in patients with relapsing multiple sclerosis treated with cladribine tablets.

BACKGROUND: Neuroimaging studies have used magnetic resonance imaging-derived methods to assess brain volume loss in multiple sclerosis (MS) as a reliable measure of diffuse tissue damage. METHODS: In the CLARITY study ( ClinicalTrials.gov NCT00213135), the effect of 2 years' treatment with cladribine tablets on annualized percentage brain volume change (PBVC/y) was evaluated in patients with relapsing MS (RMS). RESULTS: Compared with placebo (-0.70% ± 0.79), PBVC/y was reduced in patients treated with cladribine tablets 3.5 mg/kg (-0.56% ± 0.68, p = 0.010) and 5.25 mg/kg (-0.57% ± 0.72, p = 0.019). After adjusting for treatment group, PBVC/y showed a significant correlation with the cumulative probability of disability progression (HR = 0.67, 95% CI = 0.571, 0.787; p < 0.001), with patients with lower PBVC/y showing the highest probability of remaining free from disability progression at 2 years and vice versa. CONCLUSIONS: Cladribine tablets given annually for 2 years in short-duration courses in patients with RMS in the CLARITY study significantly reduced brain atrophy in comparison with placebo treatment, with residual rates in treated patients being close to the physiological rates.

Early changes of brain connectivity in primary open angle glaucoma.

Our aim was to assess in primary open angle glaucoma (POAG), a major cause of irreversible blindness worldwide, whether diffuse brain changes recently shown in advanced stage can be detected since the early stage. We used multimodal magnetic resonance imaging (MRI) in 57 patients with the three POAG stages and in 29 age-matched normal controls (NC). Voxelwise statistics was performed with nonparametric permutation testing. Compared with NC, disrupted anatomical connectivity (AC) was found in the whole POAG group along the visual pathway and in nonvisual white matter tracts (P < 0.001). Moreover, POAG patients showed decreased functional connectivity (FC) in the visual (P = 0.004) and working memory (P < 0.001) networks whereas an increase occurred in the default mode (P = 0.002) and subcortical (P < 0.001) networks. Altered AC and FC were already present in early POAG (n = 14) in both visual and nonvisual systems (P ≤ 0.01). Only severe POAG (n = 30) showed gray matter atrophy and this mapped on visual cortex (P < 0.001) and hippocampus (P < 0.001). Increasing POAG stage was associated with worsening AC in both visual and nonvisual pathway (P < 0.001), progressive atrophy in the hippocampus and frontal cortex (P < 0.003). Most of the structural and functional alterations within and outside the visual system showed correlation (P < 0.001 to 0.02) with computerized visual field and retinal nerve fiber layer thickness. In conclusion, the complex pathogenesis of POAG includes widespread damage of AC and altered FC within and beyond the visual system since the early disease stage. The association of brain MRI changes with measures of visual severity emphasizes the clinical relevance of our findings. Hum Brain Mapp 37:4581-4596, 2016. © 2016 Wiley Periodicals, Inc.

Appraisal of brain connectivity in radiologically isolated syndrome by modeling imaging measures.

We hypothesized that appraisal of brain connectivity may shed light on the substrate of the radiologically isolated syndrome (RIS), a term applied to asymptomatic subjects with brain MRI abnormalities highly suggestive of multiple sclerosis. We thus used a multimodal MRI approach on the human brain by modeling measures of microstructural integrity of white matter (WM) tracts with those of functional connectivity (FC) at the level of resting state networks in RIS subjects, demographically matched normal controls (NC), and relapsing-remitting (RR) MS patients, also matched with RIS for brain macrostructural damage (i.e., lesions and atrophy). Compared with NC, in both RIS subjects and MS patients altered integrity of WM tracts was present. However, RIS subjects showed, at a less conservative threshold, lower diffusivities than RRMS patients in distinct cerebral associative, commissural, projection, and cerebellar WM tracts, suggesting a relatively better anatomical connectivity. FC was similar in NC and RIS subjects, even in the presence of important risk factors for MS (spinal cord lesions, oligoclonal bands, and dissemination in time on MRI) and increased in RRMS patients in two clinically relevant networks subserving "processing" (sensorimotor) and "control" (working memory) functions. In RIS, the lack of functional reorganization in key brain networks may represent a model of "functional reserve," which may become upregulated, with an adaptive or maladaptive role, only at a later stage in case of occurrence of clinical deficit.

Structural and functional brain changes beyond visual system in patients with advanced glaucoma.

In order to test the hypothesis that in primary open angle glaucoma (POAG), an important cause of irreversible blindness, a spreading of neurodegeneration occurs through the brain, we performed multimodal MRI and subsequent whole-brain explorative voxelwise analyses in 13 advanced POAG patients and 12 age-matched normal controls (NC). Altered integrity (decreased fractional anisotropy or increased diffusivities) of white matter (WM) tracts was found not only along the visual pathway of POAG but also in nonvisual WM tracts (superior longitudinal fascicle, anterior thalamic radiation, corticospinal tract, middle cerebellar peduncle). POAG patients also showed brain atrophy in both visual cortex and other distant grey matter (GM) regions (frontoparietal cortex, hippocampi and cerebellar cortex), decreased functional connectivity (FC) in visual, working memory and dorsal attention networks and increased FC in visual and executive networks. In POAG, abnormalities in structure and FC within and outside visual system correlated with visual field parameters in the poorer performing eyes, thus emphasizing their clinical relevance. Altogether, this represents evidence that a vision disorder such as POAG can be considered a widespread neurodegenerative condition.

Relevance of hypointense brain MRI lesions for long-term worsening of clinical disability in relapsing multiple sclerosis.

BACKGROUND: The accrual of brain focal pathology is considered a good substrate of disability in relapsing-remitting multiple sclerosis (RRMS). However, knowledge on long-term lesion evolution and its relationship with disability progression is poor. OBJECTIVE: The objective of this paper is to evaluate in RRMS the long-term clinical relevance of brain lesion evolution. METHODS: In 58 RRMS patients we acquired, using the same scanner and protocol, brain magnetic resonance imaging (MRI) at baseline and 10±0.5 years later. MRI data were correlated with disability changes as measured by the Expanded Disability Status Scale (EDSS). RESULTS: The annualized 10-year lesion volume (LV) growth was +0.25±0.5 cm(3) (+6.7±8.7%) for T2-weighted (T2-W) lesions and +0.20±0.31 cm(3) (+11.5±12.3%) for T1-weighted (T1-W) lesions. The univariate analysis showed moderate correlations between baseline MRI measures and EDSS at 10 years (p < 0.001). Also, 10-year EDSS worsening correlated with LV growth and the number of new/enlarging lesions measured over the same period (p < 0.005). In the stepwise multiple regression analysis, EDSS worsening over 10 years was best correlated with the combination of baseline T1-W lesion count and increasing T1-W LV (R = 0.61, p < 0.001). CONCLUSION: In RRMS patients, long-term brain lesion accrual is associated with worsening in clinical disability. This is particularly true for hypointense, destructive lesions.

Location of brain lesions predicts conversion of clinically isolated syndromes to multiple sclerosis.

OBJECTIVES: To assess in a large population of patients with clinically isolated syndrome (CIS) the relevance of brain lesion location and frequency in predicting 1-year conversion to multiple sclerosis (MS). METHODS: In this multicenter, retrospective study, clinical and MRI data at onset and clinical follow-up at 1 year were collected for 1,165 patients with CIS. On T2-weighted MRI, we generated lesion probability maps of white matter (WM) lesion location and frequency. Voxelwise analyses were performed with a nonparametric permutation-based approach (p < 0.05, cluster-corrected). RESULTS: In CIS patients with hemispheric, multifocal, and brainstem/cerebellar onset, lesion probability map clusters were seen in clinically eloquent brain regions. Significant lesion clusters were not found in CIS patients with optic nerve and spinal cord onset. At 1 year, clinically definite MS developed in 26% of patients. The converting group, despite a greater baseline lesion load compared with the nonconverting group (7 ± 8.1 cm3 vs. 4.6 ± 6.7 cm3, p < 0.001), showed less widespread lesion distribution (18% vs. 25% of brain voxels occupied by lesions). High lesion frequency was found in the converting group in projection, association, and commissural WM tracts, with larger clusters being in the corpus callosum, corona radiata, and cingulum. CONCLUSIONS: Higher frequency of lesion occurrence in clinically eloquent WM tracts can characterize CIS subjects with different types of onset. The involvement of specific WM tracts, in particular those traversed by fibers involved in motor function and near the corpus callosum, seems to be associated with a higher risk of clinical conversion to MS in the short term.