Free-breathing accelerated whole-body MRI using an automated workflow: Comparison with conventional breath-hold sequences.

Whole-body magnetic resonance imaging (MRI) has become increasingly popular in oncology. However, the long acquisition time might hamper its widespread application. We sought to assess and compare free-breathing sequences with conventional breath-hold examinations in whole-body MRI using an automated workflow process. This prospective study consisted of 20 volunteers and six patients with a variety of pathologies who had undergone whole-body 1.5-T MRI that included T1-weighted radial and Dixon volumetric interpolated breath-hold examination sequences. Free-breathing sequences were operated by using an automated user interface. Image quality, diagnostic confidence, and image noise were evaluated by two experienced radiologists. Additionally, signal-to-noise ratio was measured. Diagnostic performance for the overall detection of pathologies was assessed using the area under the receiver operating characteristics curve (AUC). Study participants were asked to rate their examination experiences in a satisfaction survey. MR free-breathing scans were rated as at least equivalent to conventional MR scans in more than 92% of cases, showing high overall diagnostic accuracy (95% [95% CI 92-100]) and performance (AUC 0.971, 95% CI 0.942-0.988; p < 0.0001) for the assessment of pathologies at simultaneously reduced examination times (25 ± 2 vs. 32 ± 3 min; p < 0.0001). Interrater agreement was excellent for both free-breathing (Ï° = 0.96 [95% CI 0.88-1.00]) and conventional scans (Ï° = 0.93 [95% CI 0.84-1.00]). Qualitative and quantitative assessment for image quality, image noise, and diagnostic confidence did not differ between the two types of MR image acquisition (all p > 0.05). Scores for patient satisfaction were significantly better for free-breathing compared with breath-hold examinations (p = 0.0145), including significant correlations for the grade of noise (r = 0.79, p < 0.0001), tightness (r = 0.71, p < 0.0001), and physical fatigue (r = 0.52, p = 0.0065). In summary, free-breathing whole-body MRI in tandem with an automated user interface yielded similar diagnostic performance at equivalent image quality and shorter acquisition times compared to conventional breath-hold sequences.

Diffusion-weighted whole-body magnetic resonance imaging with background body signal suppression was useful in a patient with isolated myocardial abscess confined to the right atrial wall: a case report.

BACKGROUND: Myocardial abscess is often associated with infective endocarditis (IE), and isolated myocardial abscess without IE is rare. Echocardiography and computed tomography (CT) are often used to diagnose myocardial abscess; however, to the best of our knowledge, diffusion-weighted whole-body magnetic resonance imaging with background body signal suppression (DWIBS) has not been used. Here, we present a case of myocardial abscess without IE that was diagnosed using DWIBS. CASE PRESENTATION: A 72-year-old Japanese man with a history of hypertension, dyslipidemia, and retinitis pigmentosa presented to our hospital with malaise and a fever lasting 10 days. Blood test results showed elevated inflammatory marker levels (white blood cell count 18,700/µL and C-reactive protein level 23.0 mg/dL). Infection was suspected; however, the source of the infection could not be identified. DWIBS, which was performed on day 7 of admission to determine the source of infection, showed a high signal surrounding the right wall, suggesting inflammation. Contrast-enhanced CT performed on day 1 of hospitalization revealed a low-density area in the same region; however, the pathological implications of this finding could not be determined. Based on DWIBS findings, we concluded that the condition presented as a myocardial abscess that was confined specifically to the right atrial wall. Three sets of blood cultures revealed negative findings, and echocardiography showed no vegetation or valve regurgitation. Therefore, the patient was diagnosed with an isolated myocardial abscess uncomplicated with IE. An electrocardiogram on admission showed no P waves, and the patient had a junctional rhythm. However, on day 20 of hospitalization, he developed a complete atrioventricular block. After complete myocardial abscess healing following antibiotic treatment was confirmed, the patient underwent pacemaker implantation. Ten months after surgery, the patient had no signs of infection recurrence. CONCLUSIONS: Based on history and physical examination alone, diagnosis of an isolated myocardial abscess can be challenging. In addition to CT and echocardiography, DWIBS might be helpful for the diagnosis of myocardial abscesses.

Whole body MRI with Diffusion Weighted Imaging versus 18F-fluorodeoxyglucose-PET/CT in the staging of lymphomas.

PURPOSE: To assess the diagnostic performance of Whole Body (WB)-MRI in comparison with 18F-Fluorodeoxyglucose-PET/CT (18F-FDG-PET/CT) in lymphoma staging and to assess whether quantitative metabolic parameters from 18F-FDG-PET/CT and Apparent Diffusion Coefficient (ADC) values are related. MATERIALS AND METHODS: We prospectively enrolled patients with a histologically proven primary nodal lymphoma to  undergo 18F-FDG-PET/CT and WB-MRI, both performed within 15 days one from the other, either before starting treatment (baseline) or during treatment (interim). Positive and negative predictive values of WB-MRI for the identification of nodal and extra-nodal disease were measured. The agreement between WB-MRI and 18F-FDG-PET/CT for the identification of lesions and staging was assessed through Cohen's coefficient k and observed agreement. Quantitative parameters of nodal lesions derived from 18F-FDG-PET/CT and WB-MRI (ADC) were measured and the Pearson or Spearman correlation coefficient was used to assess the correlation between them. The specified level of significance was p ≤ 0.05. RESULTS: Among the 91 identified patients, 8 refused to participate and 22 met exclusion criteria, thus images from 61 patients (37 men, mean age 30.7 years) were evaluated. The agreement between 18F-FDG-PET/CT and WB-MRI for the identification of nodal and extra-nodal lesions was 0.95 (95% CI 0.92 to 0.98) and 1.00 (95% CI NA), respectively; for staging it was 1.00 (95% CI NA). A strong negative correlation was found between ADCmean and SUVmean of nodal lesions in patients evaluated at baseline (Spearman coefficient rs = - 0.61, p = 0.001). CONCLUSION: WB-MRI has a good diagnostic performance for staging of patients with lymphoma in comparison with 18F-FDG-PET/CT and is a promising technique for the quantitative assessment of disease burden in these patients.

Whole-body magnetic resonance imaging for prostate cancer assessment: Current status and future directions.

Over the past decade, updated definitions for the different stages of prostate cancer and risk for distant disease, along with the advent of new therapies, have remarkably changed the management of patients. The two expectations from imaging are accurate staging and appropriate assessment of disease response to therapies. Modern, next-generation imaging (NGI) modalities, including whole-body magnetic resonance imaging (WB-MRI) and nuclear medicine (most often prostate-specific membrane antigen [PSMA] positron emission tomography [PET]/computed tomography [CT]) bring added value to these imaging tasks. WB-MRI has proven its superiority over bone scintigraphy (BS) and CT for the detection of distant metastasis, also providing reliable evaluations of disease response to treatment. Comparison of the effectiveness of WB-MRI and molecular nuclear imaging techniques with regard to indications and the definition of their respective/complementary roles in clinical practice is ongoing. This paper illustrates the evolution of WB-MRI imaging protocols, defines the current state-of-the art, and highlights the latest developments and future challenges. The paper presents and discusses WB-MRI indications in the care pathway of men with prostate cancer in specific key situations: response assessment of metastatic disease, "all in one" cancer staging, and oligometastatic disease.

MRI in the Diagnosis and Treatment Response Assessment of Chronic Nonbacterial Osteomyelitis in Children and Adolescents.

PURPOSE OF REVIEW: To explain the central role of magnetic resonance imaging (MRI) in the diagnosis and follow-up of chronic nonbacterial osteomyelitis (CNO) in children and adolescents, centering on practical technical aspects and salient diagnostic features. RECENT FINDINGS: In the absence of conclusive clinical features and widely accepted laboratory tests, including validated disease biomarkers, MRI (whether targeted or covering the entire body) currently plays an indispensable role in the diagnosis and therapy response assessment of CNO. Whole-body MRI, which is the reference imaging standard for CNO, can be limited to a short tau inversion recovery (STIR) coronal image set covering the entire body and a STIR sagittal set covering the spine, an approximately 30-min examination with no need for intravenous contrast or diffusion-weighted imaging. The hallmark of CNO is periphyseal (metaphyseal and/or epi-/apophyseal) osteitis, identified as bright foci on STIR, with or without inflammation of the adjacent periosteum and surrounding soft tissue. Response to bisphosphonate treatment for CNO has some unique MRI findings that should not be mistaken for residual or relapsing disease. Diagnostic features and treatment response characteristics of MRI in pediatric CNO are discussed, also describing the techniques used, pitfalls encountered, and differential diagnostic possibilities considered during daily practice.

Clinical utility of whole body diffusion-weighted imaging in an immunocompetent adult with atypical cat scratch disease.

BACKGROUND: Cat scratch disease (CSD) is an infectious disease caused by Bartonella henselae. CSD follows a typical course, characterized by regional lymphadenopathy. In atypical CSD, the lesions spread to systemic organs and can cause fever of unknown origin (FUO). A previous study showed the usefulness of whole-body magnetic resonance imaging (WB-MRI) with diffusion-weighted imaging (DWI) for limited areas in the diagnosis of FUO, but there are no studies on the clinical utility of whole-body DWI (WB-DWI). We herein report the case of an immunocompetent young man in whom contrast-enhanced CT-unidentifiable multiple liver abscess and osteomyelitis were successfully detected by WB-DWI. Follow-up with a liver biopsy helped confirm an atypical CSD diagnosis. CASE PRESENTATION: A 23-year-old previously healthy man was admitted for a 19-day history of high fever despite 3-day treatment by azithromycin. His physical examination was unremarkable and contrast-enhanced CT showed only a low attenuated area in the right lobe of the liver, indicating a cyst. WB-DWI revealed multiple nodular lesions of hypo-diffusion in the liver, spine, and pelvic region. The biopsy specimens of the liver abscess showed no evidence of tuberculosis/malignancy and the polymerase chain reaction (PCR) test of liver abscess aspirate showed positive findings for Bartonellahenselae, confirming the diagnosis of CSD. He completed minocycline monotherapy for a total of 60 days without any deterioration. CONCLUSIONS: WB-DWI can be useful for the diagnosis of atypical CSD with hepatic and bone involvement, which can cause FUO in young immunocompetent adults.

Automatic segmentation of whole-body adipose tissue from magnetic resonance fat fraction images based on machine learning.

OBJECTIVE: To propose a fully automated algorithm, which is implemented to segment subcutaneous adipose tissue (SAT) and internal adipose tissue (IAT) from the total adipose tissue for whole-body fat distribution analysis using proton density fat fraction (PDFF) magnetic resonance images. MATERIALS AND METHODS: Adipose tissue segmentation was implemented using the U-Net deep neural network model. All datasets were collected using a 3.0 T magnetic resonance imaging (MRI) scanner for whole-body scan of 20 volunteers covering from neck to knee with about 160 images for each volunteer. PDFF images were reconstructed based on chemical-shift-encoded fat-water imaging. After selecting the representative PDFF images (total 906 images), the manual labeling of the SAT area was used for model training (504 images), validation (168 images), and testing (234 images). RESULTS: The automatic segmentation model was validated through three indices using the validation and test sets. The dice similarity coefficient, precision rate, and recall rate were 0.976 ± 0.048, 0.978 ± 0.048, and 0.978 ± 0.050, respectively, in both validation and test sets. CONCLUSION: The proposed algorithm can reliably and automatically segment SAT and IAT from whole-body MRI PDFF images. The proposed method provides a simple and automatic tool for whole-body fat distribution analysis to explore the relationship between fat deposition and metabolic-related chronic diseases.

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.

Diagnostic accuracy of imaging approaches for early tumor detection in children with Li-Fraumeni syndrome.

BACKGROUND: The Toronto protocol for cancer surveillance in children with Li-Fraumeni syndrome has been adopted worldwide. OBJECTIVE: To assess the diagnostic accuracy of the imaging used in this protocol. MATERIALS AND METHODS: We conducted a blinded retrospective review of imaging modalities in 31 pediatric patients. We compared imaging findings with the reference standards, which consisted of (1) histopathological diagnosis, (2) corresponding dedicated imaging or subsequent surveillance imaging or (3) clinical outcomes. We individually analyzed each modality's diagnostic performance for cancer detection and assessed it on a per-study basis for chest and abdominal regional whole-body MRI (n=115 each), brain MRI (n=101) and abdominal/pelvic US (n=292), and on a per-lesion basis for skeleton/soft tissues on whole-body MRI (n=140). RESULTS: Of 763 studies/lesions, approximately 80% had reference standards that identified 4 (0.7%) true-positive, 523 (85.3%) true-negative, 5 (0.8%) false-positive, 3 (0.5%) false-negative and 78 (12.7%) indeterminate results. There were 3 true-positives on whole-body MRI and 1 true-positive on brain MRI as well as 3 false-negatives on whole-body MRI. Sensitivities and specificities of tumor diagnosis using a worst-case scenario analysis were, respectively, 40.0% (95% confidence interval [CI]: 7.3%, 83.0%) and 38.2% (95% CI: 29.2%, 48.0%) for skeleton/soft tissues on whole-body MRI; sensitivity non-available and 97.8% (95% CI: 91.4%, 99.6%) for chest regional whole-body MRI; 100.0% (95% CI: 5.5%, 100.0%) and 96.8% (95% CI: 90.2%, 99.2%) for abdominal regional whole-body MRI; sensitivity non-available and 98.3% (95% CI: 95.3, 99.4) for abdominal/pelvic US; and 50.0% (95% CI: 2.7%, 97.3%) and 93.8% (95% CI: 85.6%, 97.7%) for brain MRI. CONCLUSION: Considerations for optimizing imaging protocol, defining criteria for abnormalities, developing a structured reporting system, and practicing consensus double-reading may enhance the diagnostic accuracy for tumor surveillance.

Screening of cancer predisposition syndromes.

Pediatric patients with cancer predisposition syndromes are at increased risk of developing malignancies compared with their age-matched peers, necessitating regular surveillance. Screening protocols differ among syndromes and are composed of a number of elements, imaging being one. Surveillance can be initiated in infants, children and adolescents with a tumor known or suspected of being related to a cancer predisposition syndrome or where genetic testing identifies a germline pathogenic gene variant in an asymptomatic child. Pre-symptomatic detection of malignant neoplasms offers potential to improve treatment options and survival outcomes, but the benefits and risks of screening need to be weighed, particularly with variable penetrance in many cancer predisposition syndromes. In this review we discuss the benefits and risks of surveillance imaging and the importance of integrating imaging and non-imaging screening elements. We explore the principles of surveillance imaging with particular reference to whole-body MRI, considering the strategies to minimize false-negative and manage false-positive whole-body MRI results, the value of standardized nomenclature when reporting risk stratification to better guide patient management, and the need for timely communication of results to allay anxiety. Cancer predisposition syndrome screening is a multimodality, multidisciplinary and longitudinal process, so developing formalized frameworks for surveillance imaging programs should enhance diagnostic performance while improving the patient experience.

Whole-body MRI at initial presentation of pediatric chronic recurrent multifocal osteomyelitis and correlation with clinical assessment.

BACKGROUND: Chronic recurrent multifocal osteomyelitis (CRMO) is a diagnosis of exclusion, relying heavily on whole-body magnetic resonance imaging (WB-MRI) for diagnosing and evaluating response to therapy. Information with respect to disease distribution and imaging correlation with clinical disease severity at initial presentation is lacking. OBJECTIVE: To retrospectively characterize distribution of disease on WB-MRI and to correlate imaging findings with disease severity at initial rheumatology presentation. MATERIALS AND METHODS: Using a modified version of a recently devised imaging-based scoring system, we evaluated disease distribution and correlation between findings on WB-MRI and clinical disease severity in 54 patients presenting for initial evaluation of CRMO. Symptomatic lesion sites were extracted from chart review and physician global assessment was determined by the consensus of two rheumatologists. RESULTS: Sites of CRMO involvement evident on imaging at initial presentation had a strong predilection for the pelvis and lower extremities. There was significant correlation between the number of lesions detected on WB-MRI and total clinical severity score at initial rheumatology presentation (P<0.01). However, no other imaging parameter correlated with disease severity. CONCLUSION: While the overall number of lesions identified on MRI correlates with clinical severity scores at initial imaging, other MR parameters of CRMO lesions may not be reliable indicators of disease severity at initial presentation. Further research is needed to assess whether these parameters are implicated in longitudinal disease severity or overall response to therapy.

Chronic non-bacterial osteomyelitis in children: Outcomes, quality of life.

BACKGROUND: Chronic non-bacterial osteomyelitis is a chronic sterile inflammatory bone condition. We aimed to describe patients' clinical and radiographic findings and to evaluate their response to therapy and their quality of life. METHODS: This cross-sectional study included 18 patients from a single center in Turkey whose clinical, radiological features, and outcomes were reviewed retrospectively. The quality of the patients' lives after treatment was compared with healthy controls using the Pediatric Quality of Life Inventory 4.0. RESULTS: The median age of disease onset was 12 years (IQR 10-14 years) and 11 (61.1%) patients were male. The median follow-up duration was 15 months (IQR 12-22 months). The persistent form of chronic non-bacterial osteomyelitis was the most common pattern in 15 (83.3%) patients and a recurrent pattern was defined in three (16.7%) patients. The lesions were multifocal in all patients and 15 (83.3%) patients had symmetric distribution in whole-body magnetic resonance imaging. The most common sites of arthritis were the knee and sacroiliac joints. Methotrexate was used in 16 (88.9%) patients as first-line therapy. However, some patients were unresponsive to the first-line therapy and needed tumor necrosis factor-α inhibitors (55.6%) and bisphosphonates (16.7%). We observed remission in only four (22.2%) patients, and three (16.7%) patients were unresponsive. The patients had a significantly poorer quality of life than controls (P = 0.005). CONCLUSIONS: Chronic non-bacterial osteomyelitis is an insidious disease that requires detailed analysis for diagnosis and whole-body magnetic resonance imaging is an effective tool for its diagnosis. Despite the advanced treatment, patients with chronic non-bacterial osteomyelitis have a poor quality of life.

Physical therapy assessment and whole-body magnetic resonance imaging findings in children with glycogen storage disease type IIIa: A clinical study and review of the literature.

INTRODUCTION: Early recognized manifestations of GSD III include hypoglycemia, hepatomegaly, and elevated liver enzymes. Motor symptoms such as fatigue, muscle weakness, functional impairments, and muscle wasting are typically reported in the 3rd to 4th decade of life. OBJECTIVE: In this study, we investigated the early musculoskeletal findings in children with GSD IIIa, compared to a cohort of adults with GSD IIIa. METHODS: We utilized a comprehensive number of physical therapy outcome measures to cross-sectionally assess strength and gross motor function including the modified Medical Research Council (mMRC) scale, grip and lateral/key pinch, Gross Motor Function Measure (GMFM), Gait, Stairs, Gowers, Chair (GSGC) test, 6 Minute Walk Test (6MWT), and Bruininks-Oseretsky Test of Motor Proficiency Ed. 2 (BOT-2). We also assessed laboratory biomarkers (AST, ALT, CK and urine Glc4) and conducted whole-body magnetic resonance imaging (WBMRI) to evaluate for proton density fat fraction (PDFF) in children with GSD IIIa. Nerve Conduction Studies and Electromyography results were analyzed where available and a thorough literature review was conducted. RESULTS: There were a total of 22 individuals with GSD IIIa evaluated in our study, 17 pediatric patients and 5 adult patients. These pediatric patients demonstrated weakness on manual muscle testing, decreased grip and lateral/key pinch strength, and decreased functional ability compared to non-disease peers on the GMFM, 6MWT, BOT-2, and GSGC. Additionally, all laboratory biomarkers analyzed and PDFF obtained from WBMRI were increased in comparison to non-diseased peers. In comparison to the pediatric cohort, adults demonstrated worse overall performance on functional assessments demonstrating the expected progression of disease phenotype with age. CONCLUSION: These results demonstrate the presence of early musculoskeletal involvement in children with GSD IIIa, most evident on physical therapy assessments, in addition to the more commonly reported hepatic symptoms. Muscular weakness in both children and adults was most significant in proximal and trunk musculature, and intrinsic musculature of the hands. These findings indicate the importance of early assessment of patients with GSD IIIa for detection of muscular weakness and development of treatment approaches that target both the liver and muscle.

Comparison of the diagnostic performance and impact on management of 18F-FDG PET/CT and whole-body MRI in multiple myeloma.

PURPOSE: Comparative data on the impact of imaging on management is lacking for multiple myeloma. This study compared the diagnostic performance and impact on management of 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) and whole-body magnetic resonance imaging (WBMRI) in treatment-naive myeloma. METHODS: Forty-six patients undergoing 18F-FDG PET/CT and WBMRI were reviewed by a nuclear medicine physician and radiologist, respectively, for the presence of myeloma bone disease. Blinded clinical and imaging data were reviewed by two haematologists in consensus and management recorded following clinical data ± 18F-FDG PET/CT or WBMRI. Bone disease was defined using International Myeloma Working Group (IMWG) criteria and a clinical reference standard. Per-patient sensitivity for lesion detection was established. McNemar test compared management based on clinical assessment ± 18F-FDG PET/CT or WBMRI. RESULTS: Sensitivity for bone lesions was 69.6% (32/46) for 18F-FDG PET/CT (54.3% (25/46) for PET component alone) and 91.3% (42/46) for WBMRI. 27/46 (58.7%) of cases were concordant. In 19/46 patients (41.3%) WBMRI detected more focal bone lesions than 18F-FDG PET/CT. Based on clinical data alone, 32/46 (69.6%) patients would have been treated. Addition of 18F-FDG PET/CT to clinical data increased this to 40/46 (87.0%) patients (p = 0.02); and WBMRI to clinical data to 43/46 (93.5%) patients (p = 0.002). The difference in treatment decisions was not statistically significant between 18F-FDG PET/CT and WBMRI (p = 0.08). CONCLUSION: Compared to 18F-FDG PET/CT, WBMRI had a higher per patient sensitivity for bone disease. However, treatment decisions were not statistically different and either modality would be appropriate in initial staging, depending on local availability and expertise.

Whole-body magnetic resonance imaging in children - how and why? A systematic review.

Whole-body magnetic resonance imaging (MRI) is increasingly being used for a number of indications. Our aim was to review and describe indications and scan protocols for diagnostic value of whole-body MRI for multifocal disease in children and adolescents, we conducted a systematic search in Medline, Embase and Cochrane for all published papers until November 2018. Relevant subject headings and free text words were used for the following concepts: 1) whole-body, 2) magnetic resonance imaging and 3) child and/or adolescent. Included were papers in English with a relevant study design that reported on the use and/or findings from whole-body MRI examinations in children and adolescents. This review includes 54 of 1,609 papers identified from literature searches. Chronic nonbacterial osteomyelitis, lymphoma and metastasis were the most frequent indications for performing a whole-body MRI. The typical protocol included a coronal STIR (short tau inversion recovery) sequence with or without a coronal T1-weighted sequence. Numerous studies lacked sufficient data for calculating images resolution and only a few studies reported the acquired voxel volume, making it impossible for others to reproduce the protocol/images. Only a minority of the included papers assessed reliability tests and none of the studies documented whether the use of whole-body MRI affected mortality and/or morbidity. Our systematic review confirms significant variability of technique and the lack of proven validity of MRI findings. The information could potentially be used to boost attempts towards standardization of technique, reporting and guidelines development.

Whole-body magnetic resonance imaging in the evaluation of children with fever without a focus.

BACKGROUND: Fever without a focus is defined as a temperature of 38° C or higher as the single presenting symptom. After extensive investigation, a large percentage (12-67%) of cases remain undiagnosed. OBJECTIVE: To assess the diagnostic value of whole-body magnetic resonance imaging (WB-MRI) in children with fever without a focus. MATERIALS AND METHODS: A retrospective study was performed to identify children who underwent WB-MRI for fever without a focus. Ninety-two children, 50 boys, with a mean age of 6.1 years were included. A multidisciplinary team of physicians completed in consensus a medical record review that included: 1) immune status, 2) underlying chronic conditions, 3) hospitalization status at onset of fever, and 4) results of tissue, body fluid cultures and biopsies. Original MRI reports were evaluated. WB-MRI studies were categorized into helpful WB-MRI and not helpful WB-MRI. RESULTS: A final diagnosis for the cause of the fever was available for 68/92 cases (73.9%), which were determined to be infectious in 33/68 (48.5%), oncological in 3/68 (4.4%), rheumatological etiologies in 23/68 (33.8%) and miscellaneous in 9/68 (13.2%) cases. WB-MRI was found to be helpful in 62/92 cases (67.4%) and not helpful in 30/92 cases (32.6%). WB-MRI was 10.2 times less likely to be helpful in immunosuppressed children and almost 5.7 times less likely to be helpful in cases of prolonged fever (>3 weeks) at the time of MRI (P≤0.01). CONCLUSION: WB-MRI provides helpful information in approximately 2/3 of children with fever without a focus. In most cases, it was helpful to exclude the need of further investigation.

123Iodine-metaiodobenzylguanidine scintigraphy versus whole-body magnetic resonance imaging with diffusion-weighted imaging in children with high-risk neuroblastoma - pilot study.

BACKGROUND: The prognostic value of the International Society of Paediatric Oncology European Neuroblastoma Research Network (SIOPEN) skeletal score using 123iodine-metaiodobenzylguanidine (MIBG) has been confirmed for people with high-risk neuroblastoma. Whole-body MRI with diffusion-weighted imaging is used increasingly. OBJECTIVE: To compare the original SIOPEN score and its adaption by diffusion-weighted imaging in high-risk stage 4 neuroblastoma and to evaluate any consequences of score differences on overall survival. MATERIALS AND METHODS: This retrospective observational study included pediatric patients who underwent MIBG scintigraphy and whole-body MRI, including diffusion-weighted imaging, between 2010 and 2015. Semi-quantitative skeletal scores for each exam were calculated independently. A difference of two or more points was defined as clinically relevant and counted as M+ (more in diffusion-weighted imaging) or S+ (more in MIBG). In cases of a negative result in one of the studies, residual disease of 1 point was also rated as relevant. We tested correlation and differences on an exam basis and also grouped by different therapeutic conditions. Overall survival was used to evaluate prognostic relevance. RESULTS: Seventeen children with 25 paired examinations were evaluated. Median MIBG scintigraphy score was 0 (interquartile range [IQR] 0-4, range 0-25) vs. a median whole-body MRI score of 1 (IQR 0-5.5, range 0-35) (P=0.018). A relevant difference between whole-body MRI and MIBG scintigraphy was noted in 14 of the 25 paired examinations (M+: n=9; S+: n=5). After treatment, the median survival of cases with M+ was 14 months (IQR 4-59, range 1-74 months), while S+ cases showed a median survival of 49 months (IQR 36-52, range 36-52 months) (P=0.413). CONCLUSION: The SIOPEN scoring system is feasible for whole-body MRI but might result in slightly higher scores, probably because of MRI's superior spatial resolution. Further studies are necessary to validate any impact on prognosis.

Whole-body MRI in pediatric undefined inflammatory conditions.

BACKGROUND: Whole-body magnetic resonance imaging (WBMRI) is a multiregional imaging technique suitable for investigating the extent of multisystemic diseases without exposure to radiation, with a high sensitivity to bone alterations. The aim of our study was to evaluate the role of WBMRI in the workup of children with non-specific musculoskeletal features and non-indicative laboratory and instrumental data, who were suspected to have a rheumatologic disease. METHODS: We retrospectively analyzed medical records, including laboratory tests and radiological data of 34 children who had been evaluated due to non-specific musculoskeletal manifestations, for which a WBMRI was prescribed. RESULTS: We included 34 children, 19 females and 15 males, mean age 10 years (range 2-16 years), with the following clinical features: diffuse arthralgia (12 children), persistent fever (2 children), persistent fever and diffuse arthralgia (20 children). Serologic inflammatory markers were increased in 29/34 patients. Twenty-five children had already received X-ray and / or ultrasound before WBMRI, with a negative / uninformative result. WBMRI was performed 3-6 weeks (median, 3.5 weeks) after the initial presentation of symptoms. In 22/34 (65%) children, WBMRI revealed some abnormalities that supported the final diagnosis. Twelve out of 34 children (35%) were be affected by chronic recurrent multifocal osteomyelitis. CONCLUSIONS: WBMRI is helpful in pediatric rheumatology for the differential diagnosis of undefined inflammatory conditions. It appears to be a promising tool, especially in the detection of multifocal bone lesions. The diagnosis that mainly benefits from WBMRI in our series is chronic recurrent multifocal osteomyelitis. WBMRI can also help in excluding neoplastic diseases.

[Imaging of tumor predisposition syndromes]. / Bildgebung bei Tumorprädispositionssyndromen.

CLINICAL ISSUE: Tumor predisposition syndromes (TPS) are a heterogeneous group of genetic cancers. About 10% of the approximately 2200 malignancies in the childhood in Germany develop due to an inherited disposition, whereby TPS may be underdiagnosed. The focus of this review is set on imaging of Li-Fraumeni syndrome, neurofibromatoses, tuberous sclerosis, overgrowth, and neuroendocrine syndromes. STANDARD RADIOLOGICAL METHODS: In order to detect tumors at an early stage, screening at specific time intervals for each TPS are required. Ultrasonography and magnetic resonance imaging (MRI), especially whole-body MRI, are particularly important imaging modalities. METHODOLOGICAL INNOVATIONS: Innovative MRI techniques can increase image quality and patient comfort. MRI acquisition time can be significantly reduced through optimized acceleration factors, motion robust radial sequences and joint acquisition and readout of multiple slices during excitation. Thus, shorter MRI examinations can be performed in younger children without anesthesia. PRACTICAL RECOMMENDATION: Regular screening with ultrasound and MRI can reduce the morbidity and mortality of the patients affected with TPS.

Whole-body MRI in the diagnosis of paediatric CNO/CRMO.

Chronic recurrent multifocal osteomyelitis (CRMO) is an auto-inflammatory disorder affecting the skeleton of children and adolescents. Whole-body MRI (WBMRI) is key in the diagnosis and follow-up of CRMO. Imaging protocols should include sagittal short Tau inversion recovery of the spine, imaging of the hands and feet, and T1 images for distinguishing normal bone marrow. CRMO lesions can be metaphyseal, epiphyseal and physeal-potentially causing growth disturbance and deformity. Spinal lesions are common, important and can cause vertebral collapse. Lesion patterns include multifocal tibial and pauci-focal patterns that follow a predictable presentation and course of disease. Common pitfalls of WBMRI include haematopoietic marrow signal, metaphyseal signal early on in bisphosphonate therapy and normal high T2 signal in the hands and feet. Pictorial reporting assists in recording lesions and follow-up over time. The purpose of this paper is to review the different WBMRI protocols, imaging findings, lesion patterns and common pitfalls in children with CRMO.