Applications of Diffusion-Weighted MRI to the Musculoskeletal System.

Diffusion-weighted imaging (DWI) is an established MRI technique that can investigate tissue microstructure at the scale of a few micrometers. Musculoskeletal tissues typically have a highly ordered structure to fulfill their functions and therefore represent an optimal application of DWI. Even more since disruption of tissue organization affects its biomechanical properties and may indicate irreversible damage. The application of DWI to the musculoskeletal system faces application-specific challenges on data acquisition including susceptibility effects, the low T2 relaxation time of most musculoskeletal tissues (2-70 msec) and the need for sub-millimetric resolution. Thus, musculoskeletal applications have been an area of development of new DWI methods. In this review, we provide an overview of the technical aspects of DWI acquisition including diffusion-weighting, MRI pulse sequences and different diffusion regimes to study tissue microstructure. For each tissue type (growth plate, articular cartilage, muscle, bone marrow, intervertebral discs, ligaments, tendons, menisci, and synovium), the rationale for the use of DWI and clinical studies in support of its use as a biomarker are presented. The review describes studies showing that DTI of the growth plate has predictive value for child growth and that DTI of articular cartilage has potential to predict the radiographic progression of joint damage in early stages of osteoarthritis. DTI has been used extensively in skeletal muscle where it has shown potential to detect microstructural and functional changes in a wide range of muscle pathologies. DWI of bone marrow showed to be a valuable tool for the diagnosis of benign and malignant acute vertebral fractures and bone metastases. DTI and diffusion kurtosis have been investigated as markers of early intervertebral disc degeneration and lower back pain. Finally, promising new applications of DTI to anterior cruciate ligament grafts and synovium are presented. The review ends with an overview of the use of DWI in clinical routine. EVIDENCE LEVEL: 5 TECHNICAL EFFICACY: Stage 3.

Borderline Phyllodes Tumor in a Child.

Phyllodes tumor (PT) is considered a rare fibroepithelial tumor. Very few series have been reported in children and adolescents. Based on histopathological features, it can be classified as benign, borderline, or malignant, with the latter having a more aggressive clinical behavior. We report the case of a 10-year-old female who began with an asymptomatic mobile right breast mass. An initial fine needle biopsy (FNB) concluded fibroadenoma (FA). Months later, the mass kept growing, with the appearance of pain and nipple discharge. Benign PT was demonstrated in a new biopsy. A total mastectomy was performed. The post-surgical histopathological examination was compatible with a borderline PT. The patient is now symptom-free and with no signs of relapse. Not all breast masses in the pediatric or adolescent age bracket are FA. Attention is warranted when the clinical behavior does not follow the usual outline. PT has to be considered as a possible diagnosis and treated accordingly.

Diffusion tensor imaging of the physis: the ABC's.

The physis, or growth plate, is the primary structure responsible for longitudinal growth of the long bones. Diffusion tensor imaging (DTI) is a technique that depicts the anisotropic motion of water molecules, or diffusion. When diffusion is limited by cellular membranes, information on tissue microstructure can be acquired. Tractography, the visual display of the direction and magnitude of water diffusion, provides qualitative visualization of complex cellular architecture as well as quantitative diffusion metrics that appear to indirectly reflect physeal activity. In the growing bones, DTI depicts the columns of cartilage and new bone in the physeal-metaphyseal complex. In this "How I do It", we will highlight the value of DTI as a clinical tool by presenting DTI tractography of the physeal-metaphyseal complex of children and adolescents during normal growth, illustrating variation in qualitative and quantitative tractography metrics with age and skeletal location. In addition, we will present tractography from patients with physeal dysfunction caused by growth hormone deficiency and physeal injury due to trauma, chemotherapy, and radiation therapy. Furthermore, we will delineate our process, or "DTI pipeline," from image acquisition to data interpretation.

Fibrous hamartoma of infancy: imaging findings.

BACKGROUND: Fibrous hamartoma of infancy is a benign tumor that typically arises within the first 2 years of life in the subcutaneous and lower dermal layers. Diagnosis can be challenging as it is a rare tumor, and the imaging appearance is not well known. OBJECTIVE: To describe the imaging features in 4 cases of fibrous hamartoma of infancy focusing on ultrasound (US) and magnetic resonance (MR) findings. MATERIALS AND METHODS: In this retrospective IRB-approved study, informed consent was waived. We searched patient charts for histopathology-confirmed fibrous hamartoma of infancy diagnosis between November 2013 and November 2022. We found four cases, three boys and one girl, and the mean age was 1.4 years (5 months-3 years). The lesions were located in the axilla, posterior elbow, posterior neck, and lower back. All four patients underwent ultrasound evaluation of the lesion, and two patients also underwent MRI evaluation. The imaging findings were reviewed by consensus by two pediatric radiologists. RESULTS: US imaging revealed subcutaneous lesions with variably defined hyperechoic regions and intervening hypoechoic bands resulting in a linear "serpentine" pattern or a "multiple semicircle" pattern. MR imaging evidenced heterogeneous soft tissue masses, localized in the subcutaneous fat, and showed hyperintense fat interspersed with hypointense septations on both T1- and T2-weighted images. CONCLUSION: Fibrous hamartoma of infancy has a suggestive appearance on US with heterogeneous, echogenic subcutaneous lesions with intervening hypoechoic portions, in parallel or circumferential arrangement that can be seen as a serpentine or semicircular pattern. On MRI, interspersed macroscopic fatty components show high signal intensity on T1- and T2-weighted images and reduced signal on fat-suppressed inversion recovery images, with irregular peripheral enhancement.

MR Imaging of the Developing Pediatric Marrow: An Overview of Pearls and Pitfalls.

The dynamic and developing pediatric skeleton is a well-elucidated process that occurs in a stepwise faction. Normal development has been reliably tracked and described with Magnetic Resonance (MR) imaging. The recognition of the normal patterns of skeletal development is essential, as normal development may mimic pathology and vice versa. The authors review normal skeleton maturation and the corollary imaging findings while highlighting common marrow imaging pitfalls and pathology.

Soft-Tissue Anaplastic Lymphoma Kinase-Positive Anaplastic Large Cell Lymphoma in a Child Unmasked by COVID-19.

Anaplastic large cell lymphoma (ALCL) is children's most common mature T-cell neoplasm. The majority is positive for anaplastic lymphoma kinase (ALK). Initial presentation as a soft-tissue pelvic mass without nodal involvement is rare and can be easily misdiagnosed. We report a case of a 12-year-old male presenting with pain and movement restriction in the right extremity. Computed tomography (CT) scan revealed a solitary pelvic mass. Initial biopsy examination concluded rhabdomyosarcoma. After developing pediatric multisystemic inflammatory syndrome due to coronavirus disease 2019 (COVID-19), central and peripheral lymph node enlargement appeared. New cervical adenopathy and pelvic mass biopsies were performed. Immunohistochemistry concluded an ALK-positive ALCL with a small-cell pattern. The patient was treated with brentuximab-based chemotherapy and eventually improved. Differential diagnosis of pelvic masses in children and adolescents must include ALCL. An inflammatory trigger may promote the appearance of a typical nodal disease, previously absent. Attention is warranted during histopathological examination to avoid diagnostic errors.

Relapsing Myopathy With Granulomatous Inflammation as Chronic Graft vs Host Disease Feature: A Case Report.

BACKGROUND: Graft-vs-host disease (GVHD) is a common complication of allogeneic hematopoietic stem cell transplant. Myopathy is a rare neuromuscular sign of chronic GVHD, with an incidence of less than 4% in all patients. The data are heterogeneous, and no standard criteria exists for diagnosis or treatment. CASE REPORT: We present the case of an 18-year-old man with acute lymphoblastic leukemia, who developed myopathy associated with GVHD 19 months after allogeneic hematopoietic stem cell transplant from an unrelated donor. The patient had a previous history of acute cutaneous and chronic hepatic GVHD. At the time of symptom onset, the immunosuppressive drugs were tapered. He developed with sudden symmetrical proximal muscle weakness that prevented him from walking. Diagnosis was confirmed using magnetic resonance imaging, electromyography, muscle enzymes, and muscle biopsy results. He initially responded to immunosuppressive therapy but relapsed after quick tapering of prednisone, requiring a prolonged course of steroids and an additional dose of immune globulin intravenous. At the moment of the publication, the patient has 9 months free from GVHD relapse. CONCLUSIONS: GVHD-associated myopathy is a rare complication of hematopoietic stem cell transplant and must be suspected in patients with sudden proximal muscle weakness and moderate pain. Diagnosis is challenging and must include magnetic resonance imaging, electromyography, muscle enzymes, and muscle biopsy results. Usually, all patients respond adequately to immunosuppression.

Isolated Renal Relapse in a Post-Allogenic Transplant Adult Patient With Acute Lymphoblastic Leukemia.

Acute lymphoblastic leukemia (ALL) is an aggressive hematological neoplasm typically more common in children than adults. More prolonged remissions and a potential cure can be achieved if allogeneic hematopoietic stem cell transplantation (allo-HSCT) is performed. Outcomes after allo-HSCT vary significantly among patients, and multiple factors contribute to these outcomes. Isolated extramedullary relapse (iEMR) after allo-HSCT is rare. We present the case of a 43-year-old man who was diagnosed with Philadelphia chromosome-negative (Ph-neg), B-cell ALL and underwent haploidentical allo-HSCT because of high-risk features at diagnosis. One year later, he was admitted to the hospital with facial and peripheral edema, proteinuria, elevated serum creatinine levels, and hypertension. Renal biopsy was performed immediately. Renal infiltration of TdT+ leukemic cells was detected by immunohistochemistry. Bone marrow aspiration, lumbar puncture, and computed tomography (CT) scans were performed to identify other sites of possible relapse. No other sites were identified, and an extramedullary isolated renal relapse was diagnosed. Intensive re-induction with chemotherapy was not possible because of the coronavirus disease 2019 (COVID-19) infection. Six weeks later, a medullary relapse was noted. Medullary infiltration of B-cell ALL after allo-HSCT has a historically poor prognosis; however, iEMR appears to have a better overall prognosis. The optimal treatment for renal iEMR is still a matter of debate.

Three-Dimensional Morphometric Characterization of Femoral Cam Lesions: Quantifying the Radial and Lateral Extents.

OBJECTIVE: To quantify the radial and lateral extents of femoral cam lesions in FAI patients relative to the alpha angle and correlate with clinical data. METHODS: Retrospective study of 81 hips with femoral cam morphology that underwent arthroscopic surgery between 2017 and 2019. At each hour over the clockface, the alpha angle (α) (abnormal defined as > 55°), radial extent, and lateral extent of cam lesions were measured on CT. These measurements were correlated with clinical and arthroscopic data. Statistics included independent samples t-test and chi-squared test with Bonferroni correction and multivariate logistic regression. RESULTS: Larger α at 12:00-4:00 in males vs females (56.6-63.4° vs 44.3-58.5°, P < 0.001) and at 2:00-4:00 with elite sports participation vs without (56.7-70.9° vs 49.6-61.1°, P ≤ 0.004). Independent risk factors for radial extent beyond 12:00-3:00 were: male sex (OR 4.82, 95% CI [1.46, 15.85], P = 0.010), BMI > 25 (OR 4.74, 95% CI [1.61, 14.00], P = 0.005), and elite sports participation (OR 3.28, 95% CI [1.09, 9.82], P = 0.034). Lateral extent increased at 1:00-4:00 in males vs females (7.8-18.6 mm vs 1.6-9.1 mm, P < 0.0001). A 16% prevalence of distal cam lesions was found at locations with normal α, resulting in underestimation of radial extent by an average of 1.7 hours. CONCLUSION: There is a positive correlation between the alpha angle, lateral extent, and radial extent of cam lesions. FAI patients who were male, participated in elite level sports, and had a BMI > 25 had larger cam lesions. A larger alpha angle is a risk factor for cartilage damage. Patients may have distal cam lesions at locations with normal alpha angles, though their significance is unknown.

A biomechanical comparison of matched four-strand and five-strand semitendinosus-gracilis grafts / Estudo biomecânico comparativo entre enxertos quádruplos e quíntuplos pareados de tendões dos músculos semitendíneo e grácil / Estudio biomecânico comparativo entre injertos de tendones semitendinoso e gracilis cuádruple e quíntuple

ABSTRACT Introduction: Recent studies have shown that the likelihood of semitendinosus-gracilis graft rupture is inversely correlated to its diameter. A graft can be prepared in a five-strand or four-strand fashion to increase its diameter. However, the biomechanical superiority of five-strand semitendinosus-gracilis grafts is still under debate. Objective: This study aimed to evaluate the biomechanical characteristics of matched four-strand and five-strand human semitendinosus-gracilis grafts. Methods: We evaluated semitendinosus-gracilis tendons harvested from ten fresh human male and female cadavers, aged 18-60 years. Four-strand or five-strand grafts were prepared with the tendons and fixed to wooden tunnels with interference screws. Each graft was submitted to axial traction at 20 mm/min until rupture; the tests were donor matched. Data were recorded in real time and included the analysis of the area, diameter, force, maximum deformation and stiffness of the grafts. Results: The diameter, area and tunnel size were significantly greater in the five-strand grafts than in the four-strand grafts. There were no significant differences in biomechanical properties. The area and diameter of the graft were positively correlated to stiffness, and inversely correlated to elasticity. There was no significant correlation between graft size and maximum force at failure, maximum deformation or maximum tension. Conclusion: Five-strand hamstring grafts have greater area, diameter and tunnel size than four-strand grafts. There were no significant differences in biomechanical properties. In this model using interference screw fixation, the increases in area and diameter were correlated with an increase in stiffness and a decrease in elasticity. Level of evidence V; biomechanical study.

RESUMEN Introducción: Estudios recientes demostraron que la probabilidad de ruptura de los injertos semitendinoso y gracilis (STG) durante el pos operatorio de reconstrucción de ligamento cruzado anterior (LCA) está inversamente correlacionada a su diámetro. Un injerto puede ser preparado para obtener cuatro o cinco hebras para aumentar su diámetro, pero la superioridad biomecanica de los injertos STG de cinco hebras aún se mantiene en discusión. Objetivo: Evaluar las características biomecánicas de los injertos STG de humanos de cuatro o cinco hebras por pares. Métodos: Fueron evaluados tendones STG de diez cadaveres masculinos y diez cadaveres femeninos frescos, entre los 18 y 60 años. Los injertos de cuatro y cinco hebras fueron fijados en túneles de madera con tornillos de interferencia. Cada injerto fue sometido a una tracción axial de 200mm/min hasta su ruptura; estos tendones fueron separados por pares de acuerdo con sus donadores. Los datos fueron registrados en tiempo real y incluyeron el análisis del área del injerto, diámetro, fuerza, deformación máxima y rigidez. Resultados: Los resultados sobre el diámetro, el área y el tamaño del túnel fueron significativamente mayores en los injertos de cinco hebras que en los de cuatro. No existieron diferencias significativas en las propiedades biomecánicas. El área y el diámetro del injerto fueron correlacionados positivamente con la rigidez e inversamente con la elasticidad. No existió correlación significativa entre el tamaño del injerto y la fuerza máxima al momento de la falla, Máxima deformación o máxima tensión. Conclusión: Los injertos de isquiotibiales de cinco hebras tienen una área, diámetro y tamaño de túnel más grande que los injertos de cuatro hebras. No hubieron diferencias biomecánicas significativas. Los aumentos de área y diámetro en este modelo con la fijación de tornillo de interferencia fueron correlacionados con aumento de en la rigidez y una disminución en la elasticidad. Nivel de evidencia V; estudio biomecánico.

RESUMO Introdução: Estudos recentes demonstraram que a probabilidade de ruptura do enxerto dos tendões do semitendíneo e do grácil (STG) é correlacionada inversamente com seu diâmetro. Um enxerto pode ser preparado de forma quádrupla ou quíntupla para se aumentar o diâmetro. No entanto, a superioridade biomecânica dos enxertos STG quíntuplos ainda está em debate. Objetivo: Este estudo teve como objetivo avaliar as características biomecânicas dos enxertos STG humanos quádruplos ou quíntuplos pareados. Métodos: Foram avaliados tendões STG retirados de dez cadáveres masculinos e femininos frescos, com idades entre 18 e 60 anos. Os enxertos quádruplos ou quíntuplos foram preparados com os tendões e fixados em túneis de madeira com parafusos de interferência. Cada enxerto foi submetido à tração axial a 20 mm/min. até a ruptura; os testes foram pareados de acordo com os doadores. Os dados foram registrados em tempo real e incluíram a análise de área, diâmetro, força, deformação máxima e rigidez dos enxertos. Resultados: O diâmetro, a área e o tamanho do túnel foram significativamente maiores nos enxertos quíntuplos do que nos enxertos quádruplos. Não houve diferenças significativas nas propriedades biomecânicas. A área e o diâmetro do enxerto foram correlacionados positivamente com a rigidez e inversamente com a elasticidade. Não houve correlação significativa entre o tamanho do enxerto e a força máxima na falha, deformação máxima ou tensão máxima. Conclusão: Os enxertos quíntuplos dos músculos isquiotibiais têm maior área, diâmetro e tamanho do túnel do que os enxertos quádruplos. Não houve diferenças significativas nas propriedades biomecânicas. Neste modelo de fixação com parafuso de interferência, aumentos da área e do diâmetro foram correlacionados com o aumento da rigidez e a diminuição na elasticidade. Nível de evidência V; Estudo Biomecânico.

Sarcopenia and preserved bone mineral density in paediatric survivors of high-risk neuroblastoma with growth failure.

BACKGROUND: Survival from paediatric high-risk neuroblastoma (HR-NBL) has increased, but cis-retinoic acid (cis-RA), the cornerstone of HR-NBL therapy, can cause osteoporosis and premature physeal closure and is a potential threat to skeletal structure in HR-NBL survivors. Sarcopenia is associated with increased morbidity in survivors of paediatric malignancies. Low muscle mass may be associated with poor prognosis in HR-NBL patients but has not been studied in these survivors. The study objective was to assess bone density, body composition and muscle strength in HR-NBL survivors compared with controls. METHODS: This prospective cross-sectional study assessed areal bone mineral density (aBMD) of the whole body, lumbar spine, total hip, femoral neck, distal 1/3 and ultradistal radius and body composition (muscle and fat mass) using dual-energy X-ray absorptiometry (DXA) and lower leg muscle strength using a dynamometer. Measures expressed as sex-specific standard deviation scores (Z-scores) included aBMD (adjusted for height Z-score), bone mineral apparent density (BMAD), leg lean mass (adjusted for leg length), whole-body fat mass index (FMI) and ankle dorsiflexion peak torque adjusted for leg length (strength-Z). Muscle-specific force was assessed as strength relative to leg lean mass. Outcomes were compared between HR-NBL survivors and controls using Student's t-test or Mann-Whitney U test. Linear regression models examined correlations between DXA and dynamometer outcomes. RESULTS: We enrolled 20 survivors of HR-NBL treated with cis-RA [13 male; mean age: 12.4 ± 1.6 years; median (range) age at therapy initiation: 2.6 (0.3-9.1) years] and 20 age-, sex- and race-matched controls. Height-Z was significantly lower in HR-NBL survivors compared with controls (-1.73 ± 1.38 vs. 0.34 ± 1.12, P < 0.001). Areal BMD-Z, BMAD-Z, FMI-Z, visceral adipose tissue and subcutaneous adipose tissue were not significantly different in HR-NBL survivors compared with controls. Compared with controls, HR-NBL survivors had lower leg lean mass-Z (-1.46 ± 1.35 vs. - 0.17 ± 0.84, P < 0.001) and strength-Z (-1.13 ± 0.86 vs. - 0.15 ± 0.71, P < 0.001). Muscle-specific force was lower in HR-NBL survivors compared with controls (P < 0.05). CONCLUSIONS: Bone mineral density and adiposity are not severely impacted in HR-NBL survivors with growth failure, but significant sarcopenia persists years after treatment. Future studies are needed to determine if sarcopenia improves with muscle-specific interventions in this population of cancer survivors.

Pre-treatment MRI of leukaemia and lymphoma in children: are there differences in marrow replacement patterns on T1-weighted images?

OBJECTIVES: To investigate the prevalence and distribution of specific marrow patterns on pre-treatment magnetic resonance imaging (MRI) examinations in children with leukaemia and lymphoma and with respect to the anatomic location. MATERIALS AND METHODS: This retrospective IRB-approved and HIPAA-compliant study included children with leukaemia or lymphoma who underwent pre-treatment MRI examinations over 18 years (between 1 January 1995 and 31 August 2013). Two radiologists blinded to the clinical diagnosis reviewed each study to determine the presence or absence of abnormal marrow signal and, when present, sub-categorised the pattern into diffuse, patchy, or focal abnormal marrow. Chi-square and Fisher's exact tests were used to compare marrow patterns between leukaemia and lymphoma. RESULTS: The study included 50 children (32 males and 18 females; mean age 9.5 ± 5.3 years) with 54 MRI examinations (27 leukaemia and 27 lymphoma) that included 26 spine and 28 non-spine studies. Marrow replacement was present on 43 (80%) studies, significantly more common with leukaemia than with lymphoma (p = 0.039). The diffuse replacement pattern was significantly more common with leukaemia when compared to lymphoma (p < 0.001) and the focal pattern was only observed with lymphoma. In the spine, the diffuse pattern was observed with lymphoma (3/14, 21%). All patients with leukaemia and MRI outside of the spine showed marrow involvement. CONCLUSION: Marrow replacement is common on MRI from children with leukaemia and lymphoma. A diffuse pattern was significantly associated with leukaemia on studies outside of the spine and a focal pattern was only observed with lymphoma, independently of the anatomic location. KEY POINTS: • Bone marrow replacement on pre-treatment MRI examinations in children with leukaemia and lymphoma was observed in 93% (25/27) and 67% (18/27), respectively. • Diffuse pattern of marrow replacement was significantly more common in leukaemia even though this pattern was also observed with lymphoma on the spine MRI studies. • Focal pattern of marrow replacement was present only with lymphoma and not with leukaemia regardless of the anatomic location.

Quantitative imaging of the spine in adolescent idiopathic scoliosis: shifting the paradigm from diagnostic to comprehensive prognostic evaluation.

PURPOSE: We aimed to provide a perspective review of the available quantitative imaging modalities of the spine for prognostic evaluation of the adolescent idiopathic scoliosis (AIS). METHODS: A technical description of the current imaging technologies for quantitative assessment of the pediatric spine with scoliosis was provided, and the pros and cons of each method were discussed. Imaging modalities that quantify the overall 3D alignment of the spine as well as the structural specification of the spinal bone, intervertebral disc, endplates, and ligaments as it pertains to development and progression of the idiopathic spinal deformities in adolescents were discussed. RESULTS: Low-dose and microdose stereoradiography, ultrasound, and rasterstereography provide quantitative imaging of the 3D spinal alignment with low or no radiation in standing posture which allows repetitive imaging for early detection of the curve development. Quantitative magnetic resonance imaging, including ultrashort dual-echo time and T1-rho can provide quantitative assessment of the spinal tissues relevant to development of idiopathic spinal deformity in pediatric population. New computed tomography scans that uses dual-energy can provides high-resolution measure of the current-state of the bone quality and morphology as well as the osteogenic properties of the bone by quantitative evaluation of the bone marrow. CONCLUSION: The presented imaging modalities can provide a wide spectrum of quantifiable information relevant to development and progression of the spinal deformity. Clinical application of these technologies can change the paradigm in clinical assessment of the pediatric scoliosis by improving our understanding of the pathogenesis of the idiopathic scoliosis.

Magnetic resonance imaging evaluation of osteoid osteoma: utility of the dark rim sign.

BACKGROUND: While typical patterns of osteoid osteoma have been described on CT, MRI findings can overlap among different diseases, and atypical patterns exist. In this study, we assessed the presence of a novel dark rim sign and its utility in the MRI diagnosis of osteoid osteoma. OBJECTIVE: The purpose of this retrospective study was to assess the utility of the dark rim sign seen on MRI in children with osteoid osteoma. MATERIALS AND METHODS: MRI studies from 36 pediatric patients with osteoid osteoma and a control group of patients with either osteomyelitis or stress fracture were analyzed and then compared for the presence of the dark rim sign. Patients from the osteoid osteoma group were further divided based on nidus location and evaluated for the presence of the dark rim sign. RESULTS: The relationship between the dark rim sign and osteoid osteoma was statistically significant (P<0.001). A dark rim sign was identified in 25 of the 36 patients with osteoid osteoma. None of the control patients had a dark rim sign. The dark rim sign had 69.4% sensitivity, 100% specificity, 100% positive predictive value and 72.5% negative predictive value for detecting osteoid osteoma. The relationship between dark rim sign and nidus location was statistically significant (P<0.001) such that endosteal and medullary osteoid osteomas were more likely to have a dark rim sign than intracortical osteoid osteomas. CONCLUSION: When the nidus of an osteoid osteoma is in an endosteal or medullary location, the dark rim sign may aid in the diagnosis.

Gaucher disease status and treatment assessment: pilot study using magnetic resonance spectroscopy bone marrow fat fractions in pediatric patients.

OBJECTIVE: To assess magnetic resonance spectroscopy (MRS) bone marrow fat fractions' ability to discern between untreated Gaucher disease patients and healthy controls based on assessment of bone marrow infiltration and evaluate response to enzyme replacement therapy (ERT) on serial imaging. METHODS: This retrospective case-controlled study compared conventional MRI and bone marrow MRS findings in six pediatric and young adult Gaucher disease patients with age- and sex-matched controls, examining femoral neck and lumbar spine bone marrow fat fractions and bone marrow burden (BMB) scores. Separate analysis of six patients with serial imaging on ERT was performed with analysis of fat fractions, BMB scores, organ volumes, and serum chitotriosidase. RESULTS: Untreated patients had significantly lower femoral and lumbar spine fat fractions than controls (0.32 versus 0.67, p = 0.041 and 0.17 versus 0.34, p = 0.041, respectively). Total BMB scores were significantly higher in patients (8.0 versus 3.5, p = 0.015). In patients on ERT with average follow-up of 3.5 years, femoral neck fat fraction was the sole significant predictor of treatment duration (R square: 0.804, p < 0.001) when adjusted for age. Femoral neck fat fraction also correlated with lumbar spine fat fraction, liver volume and chitotriosidase (p < 0.05). MRS test-retest reliability was excellent (Pearson correlations: 0.96, 0.99; p-values <0.001). BMB inter-rater reliability was good overall with an intra-class correlation coefficient of 0.79 for total score, although lumbar spine score reliability was poor at 0.45. CONCLUSION: MRS-derived bone marrow fat fractions appear capable of detecting Gaucher disease severity and monitoring treatment-related changes as a predictor of ERT duration in pediatric and young adult patients.

Imaging Biomarkers of the Physis: Cartilage Volume on MRI vs. Tract Volume and Length on Diffusion Tensor Imaging.

BACKGROUND: Current methods to predict height and growth failure are imprecise. MRI measures of physeal cartilage are promising biomarkers for growth. PURPOSE: In the physis, to assess how 3D MRI volume measurements, and diffusion tensor imaging (DTI) measurements (tract volume and length) correlate with growth parameters and detect differences in growth. We compared patients exposed to cis-retinoic acid, which causes physeal damage and growth failure, with normal subjects. STUDY TYPE: Case-control. POPULATION: Twenty pediatric neuroblastoma survivors treated with cis-retinoic acid and 20 age- and sex-matched controls. FIELD STRENGTH/SEQUENCE: 3T; DTI and 3D double-echo steady-state (DESS) sequences. ASSESSMENT: On distal femoral MR studies, physeal 3D volume and DTI tract measurements were calculated and compared to height. STATISTICAL TESTS: We used partial Spearman correlation, analysis of covariance, logistic regression, Wald test, and the intraclass correlation coefficient (ICC). RESULTS: The height percentile correlated most strongly with DTI tract volumes (r = 0.74), followed by mean tract length (r = 0.53) and 3D volume (r = 0.40) (all P < 0.02). Only tract volumes and lengths correlated with annualized growth velocity. Relative to controls, patients showed smaller tract volumes (8.00 cc vs. 13.71 cc, P < 0.01), shorter tract lengths (5.92 mm vs 6.99 mm, P = 0.03), and smaller ratios of 3D cartilage volume to tract length; but no difference (4.51 cc vs 4.85 cc) in 3D MRI volumes. The 10 patients with the lowest height percentiles had smaller tract volumes (5.07 cc vs. 10.93 cc, P < 0.01), but not significantly different 3D MRI volumes. Tract volume is associated with abnormal growth, with an accuracy of 75%. DATA CONCLUSION: DTI tract volume of the physis/metaphysis predicts abnormal growth better than physeal cartilage volumetric measurement and correlates best with height percentile and growth velocity. EVIDENCE LEVEL: 2 TECHNICAL EFFICACY: Stage 2 J. Magn. Reson. Imaging 2020;52:544-551.

It's time to recognize the perichondrium.

The perichondrium is a complex structure centered at the chondro-osseous junction of growing bones. It plays an important role in both normal skeletal development and in pathological conditions. This review illustrates the normal anatomy, function and imaging appearance of the perichondrium from fetal development to older childhood. The radiologic appearance of the perichondrium in skeletal trauma, infection and tumors in which it plays a role also are reviewed.

Lesión multinodular y vacuolar de fosa posterior. Presentación de caso en Colombia / Multinodular and Vacuolating Posterior Fossa Lesions of Unknown Significance. Case Report in Colombia

Existen múltiples lesiones quísticas que se pueden encontrar en el encéfalo y en la fosa posterior. Entre estas, una nueva entidad denominada MV PLUS (por su sigla en inglés de multinodular and vacuolating posterior fossa lesions of unknown significance) tiene características imagenológicas similares al tumor multinodular vacuolante, pero como lo indican su sigla en inglés, está localizado en la fosa posterior. Estos tumores se definen como un conjunto de lesiones subcorticales nodulares pequeñas, de alta señal en secuencias con informaciónT2-FLAIR, con o sin realce al administrar medio de contraste. Pueden diferenciarse de otras entidades quísticas porque son lesiones agrupadas, sin cambios inflamatorios reactivos, presentes en una corteza de apariencia normal y sin evolución en el seguimiento. Esto último hace que se consideren lesiones benignas, no agresivas. Se presenta el caso de un paciente de 21 años de edad, a quien de manera incidental se le encontró en la región central del vermis una lesión de aspecto multiquístico, sin cambios durante el seguimiento en los últimos 3 años, con diagnóstico probable de MV PLUS.

There are multiple types of cystic lesions that can be found in the brain and in the posterior fossa. Among these, a new entity called MV PLUS (Multinodular and Vacuolating Posterior Fossa Lesions of Unknown Significance) has similar imaging characteristics to the vacuolating multinodular tumor, but as indicated by its acronym in English, it is located in the posterior fossa. These tumors are defined as a group of small, high intensity nodular subcortical images in the T2-FLAIR, with or without post-contrast enhancement. They can be differentiated from other cystic entities, because they are clustered lesions, without reactive inflammatory changes, in a cortex of normal appearance and without changes at follow-up. The latter, causes them to be considered benign, non-aggressive lesions. We present the case of a 21-year-old patient, who presented an incidentally found lesion in the central region of the vermis. MRI showed a multicystic-looking mass, that had no changes at follow-up in the last 3 years and without obvious differential diagnosis, suggestive of MV PLUS.

When Local Bone Pain Is Just the Tip of the Iceberg-A Case Report of Three Patients With Chronic Multifocal Recurrent Osteomyelitis and Some Red Flags to Help Make the Diagnosis.

Chronic recurrent multifocal osteomyelitis (CRMO) is an uncommon cause of chronic inflammatory bone pain in children that can be disabling. Often, this diagnosis is considered only after a prolonged workup, leading to frustration for families and unnecessary interventions for patients. Here we describe three cases of CRMO to increase awareness of how it may present. The first patient had a typical presentation of focal bone pain (knee), for which she underwent bone scan (hint of >1 lesion), had a bone biopsy to rule out malignancy, received empiric antibiotics for presumed infection, and finally had whole-body imaging confirming CRMO when symptoms persisted. The second patient had a similar workup, but initially presented with clavicular pain. This location should raise suspicion for CRMO, as it is an uncommon location for infectious osteomyelitis. The third patient presented with delayed growth and right hip pain, and simultaneously developed palmoplantar pustulosis. These secondary findings can also serve as red flags for CRMO, as it has been linked to this skin condition and inflammatory bowel disease. All patients improved on non-steroidal anti-inflammatory (NSAID) medications, methotrexate, and/or tumor necrosis factor (TNF)-α antagonists. By raising awareness of clinical findings suggestive of CRMO, this report may help expedite diagnosis, so patients can be started on anti-inflammatory therapy.

Evaluating growth failure with diffusion tensor imaging in pediatric survivors of high-risk neuroblastoma treated with high-dose cis-retinoic acid.

BACKGROUND: The survival of patients with high-risk neuroblastoma has increased with multimodal therapy, but most survivors demonstrate growth failure. OBJECTIVE: To assess physeal abnormalities in children with high-risk neuroblastoma in comparison to normal controls by using diffusion tensor imaging (DTI) of the distal femoral physis and adjacent metaphysis. MATERIALS AND METHODS: We prospectively obtained physeal DTI at 3.0 T in 20 subjects (mean age: 12.4 years, 7 females) with high-risk neuroblastoma treated with high-dose cis-retinoic acid, and 20 age- and gender-matched controls. We compared fractional anisotropy (FA), normalized tract volume (cm3/cm2) and tract concentration (tracts/cm2) between the groups, in relation to height Z-score and response to growth hormone therapy. Tractography images were evaluated qualitatively. RESULTS: DTI parameters were significantly lower in high-risk neuroblastoma survivors compared to controls (P<0.01), particularly if the patients were exposed to both cis-retinoic acid and total body irradiation (P<0.05). For survivors and controls, DTI values were respectively [mean ± standard deviation]: tract concentration (tracts/cm2), 23.2±14.7 and 36.7±10.5; normalized tract volume (cm3/cm2), 0.44±0.27 and 0.70±0.21, and FA, 0.22±0.05 and 0.26±0.02. High-risk neuroblastoma survivors responding to growth hormone compared to non-responders had higher FA (0.25±0.04 and 0.18±0.03, respectively, P=0.02), and tract concentration (tracts/cm2) (31.4±13.7 and 14.8±7.9, respectively, P<0.05). FA, normalized tract volume and tract concentration were linearly related to height Z-score (R2>0.31; P<0.001). Qualitatively, tracts were nearly absent in all non-responders to growth hormone and abundant in all responders (P=0.02). CONCLUSION: DTI shows physeal abnormalities that correlate with short stature in high-risk neuroblastoma survivors and demonstrates response to growth hormone treatment.