Coccidioidomycosis is most commonly a self-limited pulmonary infection. Disseminated disease is rare, occurring in fewer than 1
Comprehensive and systematic radiologic assessment is crucial in differentiating rheumatoid arthritis from other inflammatory arthropathies.
This article reviews evidence for using various imaging studies related to the diagnosis and monitoring of sarcopenia and other clinical conditions associated with muscle depletion. In this setting, DXA is usually appropriate for the assessment of body composition. Thigh ultrasound, performed in conjunction with clinical history and physical examination, may be useful. There is insufficient evidence to support a stand-alone indication for the use of CT of the abdomen (with or without contrast) or chest (with, without and with, without contrast), but a secondary quantitative analysis of muscle may be appropriate using examinations obtained for another clinical indication. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where peer reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation.
Objectives:The diagnostic yield and clinical impact of image-guided core needle biopsy (ICNB) of suspected nonvertebral osteomyelitis in adults is heterogenous in published studies because of small sample size, indicating the need for large cohort studies. Methods:A retrospective analysis of ICNBs was performed from 2010 to 2021 for patients with suspected nonvertebral osteomyelitis. For each biopsy, a series of factors were analyzed, as well as if histopathology was diagnostic of osteomyelitis and if microbiological cultures were positive. Additionally, it was recorded in what way biopsy influenced clinical management regarding antimicrobial treatment. Multivariate statistical analysis was performed to evaluate the factors associated with yield. Results:A total of 883 biopsies performed on 787 patients were included. A histopathologic diagnosis of osteomyelitis was made in 51.6% (381/738) of biopsies, and microbiological cultures were positive in 28.7% (253/883) of biopsies. Antimicrobial exposure before biopsy was negatively associated with positive cultures from bone core samples (odds ratio [OR] = 0.52; 95% confidence interval [CI], .33-.83; P = .0005). Elevated hemoglobin A1c (continuous variable) (OR = 1.38; 95% CI, 1.03-1.86; P = .03), and purulent aspirate (OR = 28.1; 95% CI, 2.67-1.86; P = .03) were positively associated with positive cultures from aspirate samples. Clinical management was affected by ICNB in 26.2% (231/883) of cases. Conclusions:In this large cohort, ICNB yielded approximately 30% positive cultures and changed clinical management in more than one fourth of patients. Summary Statement:In a retrospective study of 883 image-guided biopsies of suspected nonvertebral osteomyelitis, microbiological cultures were positive in 28.7% (253/883) of biopsies, a histopathologic diagnosis of osteomyelitis was made in 51.6% (197/381), and 26.2% (231/883) of biopsies affected clinical management.
To evaluate the diagnostic success of image-guided core needle biopsy (ICNB) of bone and soft tissue lesions in the pediatric population and what factors influence diagnostic success. For 370 biopsies performed on 350 patients 18 years of age and younger, the diagnostic yield (proportion of biopsies histopathologically sufficient for diagnosis) and accuracy (proportion of biopsies in which ICNB specimen concordant with the reference standard histopathology from surgical excision) of the biopsies, as well as a series of patient, lesion-related, and technical factors, were retrospectively analyzed. Multivariate statistical analysis was performed to evaluate what factors predicted diagnostic yield and accuracy. Diagnostic yield was 95.1
Foot infection is a common cause of diabetes-related morbidity, with 20% of patients with diabetic foot ulcers going on to develop osteomyelitis. Radiography of the foot is recommended for the initial imaging evaluation for suspected osteomyelitis of the foot in adult patients with diabetes mellitus. When initial radiographs are negative or indeterminate or when radiographs are positive for osteomyelitis and treatment planning is necessary, the recommended next imaging study is most commonly MRI of the foot. The presence of metal instrumentation in the foot can alter recommendations in select cases. This document discussing evaluation of diabetes-related osteomyelitis of the foot summarizes the literature and makes recommendations for imaging based on the available evidence. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where peer reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation.
Camurati-Engelmann disease, type 1 (CED1, OMIM # 131300) is the rare autosomal dominant skeletal dysplasia caused by select heterozygous loss-of-function defects within the gene TGFB1, which encodes transforming growth factor beta 1 (TGFB1). CED1 mutations are found in TGFB1 exons 1-4 that form the latency-associated peptide (LAP) of pro-TGFB1. Consequently, skeletal action of TGFB1 increases and thereby enhances bone formation manifest clinically as "progressive diaphyseal dysplasia". Beginning 24 years ago negative TGFB1 analysis suggested rare genetic heterogeneity for CED, and Online Mendelian Inheritance In Man designated, of unknown etiology, "CED2" (OMIM % 606631). In 2024, three sporadic occurrences considered CED2 were reported to harbor either of two mutations of TGFB2, which encodes the LAP of transforming growth factor beta 2 (TGFB2). Herein, three adults (father, son, daughter) having the CED2 phenotype in a Peruvian family revealed a novel missense variant (c.108G > T, p.R36S) within the TGFB2 LAP domain. Debilitating painful skeletal disease featuring hyperostosis of entire long bones, worse in the men, presented early in childhood. Aminobisphosphonate therapy seemed helpful. Their TGFB2 variant was within a highly conserved domain across species, absent in the gnomAD database, "possibly damaging" by Polyphen-2, not tolerated by SIFT, homologous with TGFB1 at the same amino acid position (R36) as one reported TGFB2 mutation, co-segregated as autosomal dominant, and "likely pathogenic" per ACMG guidelines.
Bone surface lesions include a variety of benign and malignant tumors and nonneoplastic entities, and knowledge of the different cell types and cortical bone surface anatomy equips radiologists to provide a useful differential diagnosis for these lesions.
Background:Renewed interest in anterior cruciate ligament (ACL) preservation has led to increased focus on tear location within the knee joint in treatment decisions, with primary ACL repair reserved for proximal tears. Retrospective studies have reported varying tear locations in adults when assessed on magnetic resonance imaging (MRI), with few studies comparing tear location on MRI versus intraoperative findings. Purpose:To prospectively determine the distribution of ACL tear location assessed on MRI versus intraoperative evaluation to test the hypothesis that <15% of ACL tears are proximal type avulsions. Study Design:Cohort study (Diagnosis); Level of evidence, 2. Methods:A total of 174 patients with clinically confirmed full-thickness ACL tears between August 2022 and March 2024 were identified and enrolled. Patients with partial tears, chronic injuries, recurrent ACL tears, or multiligamentous injuries were excluded. ACL tear locations were classified on preoperative MRI and intraoperatively using the modified Sherman classification. Patient demographic characteristics, injury mechanism, and surgical details were recorded. Analysis of variance and chi-square and Fisher exact tests were used for analysis, with significance set at P < .05. Results:ACL tear location based on MRI was as follows: 9.8% type I, 22.4% type II, 67.2% type III, and 0.60% type IV, with no type V tears. Arthroscopic tear location distribution was as follows: 26.4% type I (proximal avulsion), 44.8% type II (proximal), 25.8% type III (midsubstance), and 3.4% type IV (distal), with no type V tears. There was 43% agreement between arthroscopic and MRI evaluation overall, with 95.5% agreement when classifying type III tears. Type I tears were more common in older patients, whereas type III tears were more common in younger patients. No significant correlations were appreciated based on patient sex, body mass index, or injury mechanism. Conclusion:Poor agreement was found between imaging and arthroscopic assessment of ACL tear location, as MRI predicted intraoperative ACL tear location in less than half of cases. However, very good agreement was noted when classifying type III tears. Tear pattern was associated with patient age, with more type I tears in older patients and more type III tears in younger patients.
Despite the rarity of primary bone tumors, appropriate imaging evaluation is essential for diagnosis and management. Radiographs are the most appropriate initial imaging study for detection and characterization of the majority of primary bone tumors. Radiographs often provide sufficient information for the diagnosis of primary bone tumors, however, for radiographically occult primary bone tumors, MRI and/or CT can be performed. For indeterminate or aggressive bone tumors on radiographs, MRI or CT are typically the most appropriate next step for the evaluation of anatomic extent, assessment of viability and biopsy or surgical planning. This document focuses on five common variants to guide diagnosis and management of primary bone tumors. In addition to conventional radiographs, appropriate use of MRI, CT, PET/CT, bone scan, image-guided biopsy and ultrasound are discussed. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where peer reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation.
Acral fibrochondromyxoid tumor (AFCMT) is a recently described likely benign mesenchymal neoplasm arising in the distal extremities with distinctive histologic features and a recurrent THBS1::ADGRF5 fusion. We studied an additional 37 cases of AFCMT and expanded on the so-far reported clinicopathologic and molecular findings. Tumors occurred in 21 females and 16 males, ranging in age from 17-78 years (median age: 47), and solely involved the hands (24/37, 65%) or feet (13/37, 35%). Histologic examination revealed well-delineated uni- or multinodular tumors with prominent vasculature-rich septa and bland, chondrocyte-like tumor cells set within abundant chondromyxoid stroma. Immunohistochemical studies showed tumor cells were positive for CD34 (25/27; 93%) and ERG (27/27; 100%), while negative for S100 protein (0/31). Molecular analysis revealed evidence of a THBS1::ADGRF5 fusion in 17 of 19 (89%) successfully tested tumors. Clinical follow-up was available in 8 cases (median: 97 months), with multiple local recurrences in 1 case at 276, 312, and 360 months. We conclude that AFCMT is a distinct entity with reproducible morphologic, immunohistochemical, and molecular genetic features that should be differentiated from other similar appearing acral mesenchymal neoplasms.
Intraosseous schwannoma is a rare benign nerve sheath tumor comprising < 1% of bone tumors. Relatively common locations for this tumor include the skull and mandible, and, to a lesser degree, the spine and sacrum. Intraosseous schwannoma involving the appendicular skeleton is exceedingly rare. The clinical and imaging presentation, as in this case, is nonspecific and includes pain in the setting of a lytic bone lesion. The first step in management is bone biopsy that often produces greater than expected pain. Definitive management is surgical.
To evaluate diagnostic yield and accuracy of image-guided core needle biopsy (ICNB) of suspected malignant osseous lesions in a large cohort of adults, evaluate what factors influence these measures, and offer technical recommendations to optimize yield. A retrospective analysis of 2321 ICNBs performed from 2010 to 2021 was completed. The diagnostic yield and accuracy of the biopsies as well as a series of patient, lesion-related, and technical factors were retrospectively analyzed. Multivariate statistical analysis was performed to evaluate what factors were associated with yield and accuracy. Different cutoff values of total core length and core number were then tested to determine threshold values in relation to increased diagnostic yield. Diagnostic yield was 98.2
Imaging should be performed in patients with a suspected soft tissue mass that cannot be clinically confirmed as benign. Imaging provides essential information necessary for diagnosis, local staging, and biopsy planning. Although the modalities available for imaging of musculoskeletal masses have undergone progressive technological advancements in recent years, their overall purpose in the setting of a soft tissue mass remains unchanged. This document identifies the most common clinical scenarios related to soft tissue masses and the most appropriate imaging for their assessment on the basis of the current literature. It also provides general guidance for those scenarios that are not specifically addressed. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where peer reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation.
HomeRadioGraphicsVol. 43, No. 12 PreviousNext Musculoskeletal ImagingRadioGraphics FundamentalsImaging Evaluation for Calf PainAllison M. Khoo , Kelby B. Napier, Jonathan C. BakerAllison M. Khoo , Kelby B. Napier, Jonathan C. BakerAuthor AffiliationsFrom the Mallinckrodt Institute of Radiology, Washington University School of Medicine, 510 S Kingshighway Blvd, Campus Box 8131, St. Louis, MO 63110.Address correspondence to A.M.K. (email: [email protected]).Allison M. Khoo Kelby B. NapierJonathan C. BakerPublished Online:Nov 16 2023https://doi.org/10.1148/rg.230056MoreSectionsFull textPDF ToolsAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In AbstractThe differential diagnosis in patients with calf and posterior ankle pain can span traumatic, metabolic, infectious and inflammatory, congenital, vascular, and neoplastic causes, and knowledge of anatomic principles and characteristic imaging features contributes to accurate and timely diagnosis.Suggested ReadingsEliahou R, Sosna J, Bloom AI. Between a rock and a hard place: clinical and imaging features of vascular compression syndromes. RadioGraphics 2012;32(1):E33–E49. Link, Google ScholarHarwin JR, Richardson ML. “Tennis leg”: gastrocnemius injury is a far more common cause than plantaris rupture. Radiol Case Rep 2016;12(1):120–123. Crossref, Medline, Google ScholarJamadar DA, Jacobson JA, Theisen SE, et al. Sonography of the painful calf: differential considerations. AJR Am J Roentgenol 2002;179(3):709–716. Crossref, Medline, Google ScholarKane D, Balint PV, Gibney R, Bresnihan B, Sturrock RD. Differential diagnosis of calf pain with musculoskeletal ultrasound imaging. Ann Rheum Dis 2004;63(1):11–14. Crossref, Medline, Google ScholarKarasick D, Schweitzer ME. The os trigonum syndrome: imaging features. AJR Am J Roentgenol 1996;166(1):125–129. Crossref, Medline, Google ScholarKoulouris G, Ting AYI, Jhamb A, Connell D, Kavanagh EC. Magnetic resonance imaging findings of injuries to the calf muscle complex. Skeletal Radiol 2007;36(10):921–927. Crossref, Medline, Google ScholarLy JQ, Bui-Mansfield LT. Anatomy of and abnormalities associated with Kager’s fat Pad. AJR Am J Roentgenol 2004;182(1):147–154. Crossref, Medline, Google ScholarPavlov H, Heneghan MA, Hersh A, Goldman AB, Vigorita V. The Haglund syndrome: initial and differential diagnosis. Radiology 1982;144(1):83–88. Link, Google ScholarArticle HistoryReceived: Mar 20 2023Revision requested: May 24 2023Revision received: June 14 2023Accepted: June 21 2023Published online: Nov 16 2023 FiguresReferencesRelatedDetailsRecommended Articles Posterior Leg Pain: Understanding Soleus Muscle InjuriesRadioGraphics2022Volume: 42Issue: 3pp. 778-788MR Imaging of Muscle Trauma: Anatomy, Biomechanics, Pathophysiology, and Imaging AppearanceRadioGraphics2017Volume: 38Issue: 1pp. 124-148US of the Knee: Scanning Techniques, Pitfalls, and Pathologic ConditionsRadioGraphics2016Volume: 36Issue: 6pp. 1759-1775CT and MR Imaging of the Postoperative Ankle and FootRadioGraphics2016Volume: 36Issue: 6pp. 1828-1848Shear-Wave Elastography: Basic Physics and Musculoskeletal ApplicationsRadioGraphics2017Volume: 37Issue: 3pp. 855-870See More RSNA Education Exhibits Imaging of Calf PainDigital Posters2022Pain in the Calf! - Musculoskeletal Ultrasound in the Diagnosis of Calf PainDigital Posters2022Acute Ankle Tendon Tears, Dislocations And Associated LesionsDigital Posters2021 RSNA Case Collection Near full thickness quadriceps tendon tearRSNA Case Collection2020Peroneus Brevis Split TearRSNA Case Collection2021Complete Anterior Cruciate Ligament TearRSNA Case Collection2021 Vol. 43, No. 12 Slide Presentation Metrics Altmetric Score PDF download
HomeRadioGraphicsVol. 43, No. 5 PreviousNext Musculoskeletal ImagingRadioGraphics FundamentalsMRI Signal Intensity–based Approach to Synovial MassesAllison Khoo , Julie Steinberg, Jonathan Baker, Kelby NapierAllison Khoo , Julie Steinberg, Jonathan Baker, Kelby NapierAuthor AffiliationsFrom the Mallinckrodt Institute of Radiology, Washington University School of Medicine, 1 Barnes Jewish Hospital Plaza, St Louis, MO 63110.Address correspondence to A.K. (email: [email protected]).Allison Khoo Julie SteinbergJonathan BakerKelby NapierPublished Online:Apr 13 2023https://doi.org/10.1148/rg.220081MoreSectionsFull textPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In AbstractBy using an MRI signal intensity–based algorithmic approach to synovial masses, radiologists can narrow the broad differential diagnosis, which includes synovial proliferative pathologic features, inflammatory and infectious arthritis and synovitis, deposition disease, trauma, vascular malformation, and rarely malignancy.Suggested Readings1. Crotty JM, Monu JU, Pope TL Jr. Synovial osteochondromatosis. Radiol Clin North Am 1996;34(2):327–342,xi. Crossref, Medline, Google Scholar2. Dhanda S, Quek ST, Bathla G, Jagmohan P. Intra-articular and peri-articular tumours and tumour mimics: what a clinician and onco-imaging radiologist should know. Malays J Med Sci 2014;21(2):4–19. Medline, Google Scholar3. Goldman AB, DiCarlo EF. Pigmented villonodular synovitis: diagnosis and differential diagnosis. Radiol Clin North Am 1988;26(6):1327–1347. Crossref, Medline, Google Scholar4. Huang GS, Lee CH, Chan WP, Chen CY, Yu JS, Resnick D. Localized Nodular Synovitis of the Knee: MR Imaging Appearance and Clinical Correlates in 21 Patients. AJR Am J Roentgenol 2003;181(2):539–543. Crossref, Medline, Google Scholar5. Kim HK, Zbojniewicz AM, Merrow AC, Cheon JE, Kim IO, Emery KH. MR findings of synovial disease in children and young adults: Part 1. Pediatr Radiol 2011;41(4):495–511; quiz 545–546. Crossref, Medline, Google Scholar6. Llauger J, Palmer J, Rosón N, Bagué S, Camins A, Cremades R. Nonseptic monoarthritis: imaging features with clinical and histopathologic correlation. RadioGraphics 2000;20(Spec No):S263–S278. Link, Google Scholar7. Mattila KA, Aronniemi J, Salminen P, Rintala RJ, Kyrklund K. Intra-articular venous malformation of the knee in children: magnetic resonance imaging findings and significance of synovial involvement. Pediatr Radiol 2020;50(4):509–515. Crossref, Medline, Google Scholar8. Murphey MD, Vidal JA, Fanburg-Smith JC, Gajewski DA. Imaging of synovial chondromatosis with radiologic-pathologic correlation. RadioGraphics 2007;27(5):1465–1488. Link, Google Scholar9. Mutch JAJ, Doyon J, Mottard S. Intra-Articular Metastasis of Lung Carcinoma Presenting as Pigmented Villonodular Synovitis of the Knee: A Case Report. JBJS Case Connect 2013;3(2 Suppl 2):e41. Crossref, Medline, Google Scholar10. Narváez JA, Narváez J, Ortega R, De Lama E, Roca Y, Vidal N. Hypointense synovial lesions on T2-weighted images: differential diagnosis with pathologic correlation. AJR Am J Roentgenol 2003;181(3):761–769. Crossref, Medline, Google Scholar11. Narváez JA, Narváez J, De Lama E, De Albert M. MR imaging of early rheumatoid arthritis. RadioGraphics 2010;30(1):143–163; discussion 163–165. Link, Google Scholar12. Sheldon PJ, Forrester DM, Learch TJ. Imaging of intraarticular masses. RadioGraphics 2005;25(1):105–119. Link, Google Scholar13. Varma DGK, Richli WR, Charnsangavej C, Samuels BI, Kim EE, Wallace S. MR appearance of the distended iliopsoas bursa. AJR Am J Roentgenol 1991;156(5):1025–1028. Crossref, Medline, Google ScholarArticle HistoryReceived: Apr 10 2022Revision requested: May 16 2022Revision received: May 29 2022Accepted: June 8 2022Published online: Apr 13 2023 FiguresReferencesRelatedDetailsAccompanying This ArticleMRI Signal Intensity–based Approach to Synovial MassesApr 13 2023Default Digital Object SeriesRecommended Articles MR Imaging and US of the Wrist TendonsRadioGraphics2016Volume: 36Issue: 6pp. 1688-1700MRI of the Wrist: Algorithmic Approach for Evaluating Wrist PainRadioGraphics2019Volume: 39Issue: 2pp. 447-448Spectrum of Benign Articular and Periarticular Findings at FDG PET/CTRadioGraphics2016Volume: 36Issue: 3pp. 824-839Layered Approach to the Anterior Knee: Normal Anatomy and Disorders Associated with Anterior Knee PainRadioGraphics2018Volume: 38Issue: 7pp. 2069-2101Comprehensive Shoulder US Examination: A Standardized Approach with Multimodality Correlation for Common Shoulder DiseaseRadioGraphics2016Volume: 36Issue: 6pp. 1606-1627See More RSNA Education Exhibits Image-based Approach To Synovial MassesDigital Posters2021Imaging of Synovial Pathologies: Lumps and Bumps of the KneeDigital Posters2019Multimodality Imaging Manifestations of Intra-Articular Tumors and Tumor-Like LesionsDigital Posters2020 RSNA Case Collection Knee Pigmented Villonodular SynovitisRSNA Case Collection2021Dysplasia Epiphysealis HemimelicaRSNA Case Collection2022Gout-KneeRSNA Case Collection2021 Vol. 43, No. 5 Slide PresentationMetrics Altmetric Score PDF download
Abstract Background and Aims Primary hyperoxaluria type 1 (PH1) is a rare genetic disease in which hepatic oxalate overproduction can lead to kidney stones, nephrocalcinosis, kidney failure, and systemic oxalosis, a condition in which calcium oxalate is deposited in various tissues, including bone [1]. Radiological signs of bone oxalosis include findings such as dense metaphyseal bands and coarse trabeculation [2]. No scale exists to grade bone oxalosis severity using X-rays. Method An X-ray grading scale to evaluate systemic oxalosis in specific bones was developed based on expert opinion. Scores on individual items ranged from 0–4, except for ribs and spine, which ranged from 0–2. Higher values represent more advanced oxalosis. To validate the scale, 85 X-ray images from 5 pediatric patients with PH1 who had developed bone oxalosis were collected from charts at Shaare Zedek Medical Center and de-identified [2]. Two blinded, independent raters evaluated each X-ray twice and assigned a grade to each applicable item on the scale. Inter-rater and intra-rater reliability analyses were conducted using the weighted Cohen's kappa with asymptotic 95% confidence intervals (CIs) of the estimate and interpreted as proposed in the literature [3]. Total weighted kappa estimates were generated by pooling all observed ratings across all items. Results Total overall inter-rater (0.83 [95% CI: 0.79, 0.87]) and intra-rater (0.95 [0.93, 0.97]) kappa estimates demonstrated almost perfect agreement. Overall inter-rater kappa estimates were >0.8 to 1.0 (almost perfect agreement) for the left hand/wrist, left hip, left knee (femur), and left humerus; >0.6 to 0.8 (substantial agreement) for the right hip, right knee (tibia), right humerus, spine, and ribs; and >0.4 to 0.6 (moderate agreement) for the right knee (femur) and right knee (fibula). The overall inter-rater kappa estimate for the left knee (fibula) demonstrated poor agreement (−0.08 [−0.27, 0.10]). Overall inter-rater kappa estimates for the right hand/wrist and left knee (tibia) were considered unreliable due to lack of variability in the data, and the standard errors were not estimable. Calculable overall intra-rater kappa estimates were >0.8 to 1.0 (almost perfect agreement) for the spine and >0.6 to 0.8 (substantial agreement) for the right knee (femur) and right knee (fibula). Most other overall intra-rater kappa estimates could not be calculated because the kappas for one or both raters lacked variability. In these instances, intra-rater kappa estimates for the first and second raters demonstrated moderate to almost perfect agreement (>0.4 to 1.0; left hand/wrist, right hip, left hip, left knee [femur], right knee [tibia], left knee [tibia], right humerus, left humerus, and ribs). For the left knee (fibula), intra-rater kappa estimates for the first and second raters were −0.14 (−0.34, 0.05) and 1.00, respectively, and for the right hand/wrist, they were 1.00 and 0.00. Conclusion We developed a novel X-ray–based bone oxalosis grading scale for patients with PH1. Total overall weighted kappa estimates for inter-rater and intra-rater reliability demonstrated almost perfect strength of agreement. Most individual items demonstrated reliable kappa estimates. The right and left knee (fibula) were removed from the scale due to poor reliability.
Vertebral compression fractures (VCFs) can have a variety of etiologies, including trauma, osteoporosis, or neoplastic infiltration. Osteoporosis related fractures are the most common cause of VCFs and have a high prevalence among all postmenopausal women with increasing incidence in similarly aged men. Trauma is the most common etiology in those >50 years of age. However, many cancers, such as breast, prostate, thyroid, and lung, have a propensity to metastasize to bone, which can lead to malignant VCFs. Indeed, the spine is third most common site of metastases after lung and liver. In addition, primary tumors of bone and lymphoproliferative diseases such as lymphoma and multiple myeloma can be the cause of malignant VCFs. Although patient clinical history could help raising suspicion for a particular disorder, the characterization of VCFs is usually referred to diagnostic imaging. The ACR Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances in which evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.