Reliability and construct validity of the compatible MRI scoring system for evaluation of elbows in haemophilic children.

We assessed the reliability and construct validity of the Compatible MRI scale for evaluation of elbows, and compared the diagnostic performance of MRI and radiographs for assessment of these joints. Twenty-nine MR examinations of elbows from 27 boys with haemophilia A and B [age range, 5-17 years (mean, 11.5)] were independently read by four blinded radiologists on two occasions. Three centres participated in the study: (Toronto, n = 24 examinations; Atlanta, n = 3; Cuiaba, n = 2). The number of previous joint bleeds and severity of haemophilia were reference standard measures. The inter-reader reliability of MRI scores was substantial (ICC = 0.73) for the additive (A)-scale and excellent (ICC = 0.83) for the progressive (P)-scale. The intrareader reliability was excellent for both P-scores (ICC = 0.91) and A-scores (ICC = 0.93). The total P- and A-scores correlated poorly (r = 0.36) or moderately (r = 0.54), but positively, with clinical-laboratory measurements. The total MRI scores demonstrated high accuracy for discrimination of presence or absence of arthropathy [P-scale, area-under-the-curve (AUC) = 0.94 +/- 0.05; A-scale, AUC = 0.89 +/- 0.06], as did the soft tissue scores of both scales (P-scale, AUC = 0.90 +/- 0.06; A-scale, AUC = 0.86 +/- 0.06). Areas-under-the-curve used to discriminate severe disease demonstrated high accuracy for both P-MRI scores (AUC = 0.83 +/- 0.09) and A-MRI scores (AUC = 0.87 +/- 0.09), but non-diagnostic ability to discriminate mild disease. Similar results were noted for radiographic scales. In conclusion, both MRI scales demonstrated substantial to excellent reliability and accuracy for discrimination of presence/absence of arthropathy, and severe/non-severe disease, but poor to moderate convergent validity for total scores and non-diagnostic discriminant validity for mild/non-mild disease. Compared with radiographic scores, MRI scales did not perform better for discrimination of severity of arthropathy.

Reliability and construct validity of the compatible MRI scoring system for evaluation of haemophilic knees and ankles of haemophilic children. Expert MRI working group of the international prophylaxis study group.

We tested the reliability and construct validity of the Compatible magnetic resonance imaging (MRI) scale for the evaluation of haemophilic knees and ankles and compared the diagnostic performance of MRI and plain film radiographs. Sagittal and coronal gradient-echo 1.5-T MR images of knees (n=22) and ankles (n=23) were obtained from boys (age range 4-16 years; mean 11 years) in two centres (Toronto, n=26; Europe, n=19). The MR images were independently read by four blinded radiologists on two occasions. Number of previous joint bleedings and laboratory level of severity of haemophilia were the reference standards for imaging assessment. Both components of the MRI scale demonstrated high inter- and intrareader intraclass correlation coefficients (progressive (P) scale, 0.91 and 0.94; additive (A) scale, 0.81 and 0.92 respectively). The correlation between the osteochondral domain of the MRI scale and patient's age was moderate. Otherwise, correlations between A- and P-scales and clinical laboratory measurements were weak. The areas under the curve (AUCs) used for discrimination of disease severity were similar for the A- and P-scales (AUCs used for mild disease, A-scale, 0.72+/-0.07; P-scale, 0.69+/-0.08; P=0.23; AUCs for severe disease, A-scale, 0.93+/-0.05; P-scale, 0.87+/-0.08; P=0.05). No differences were noted between the AUCs of the MRI and radiographic scales used for discrimination of late osteoarticular changes; MRI scales performed better for discrimination of early changes. In conclusion, both MRI scales demonstrated excellent reliability, poor convergent validity, and moderate and excellent validity for discrimination of mild and severe diseases respectively. Compared with radiographic scores, the MRI scales performed better for discrimination of early osteoarticular changes.

Evolution of the imaging tests in hemophilia with emphasis on radiography and magnetic resonance imaging.

Even before the discovery of X-rays in 1895 attempts were being made to classify the joint destruction that occurs in hemophilic arthropathy. The advent of radiography added impetus to the search for the optimum classification system. Subsequent attempts have included advanced imaging methods, especially magnetic resonance imaging (MRI). Because of its high spatial resolution and ability to visualize soft tissue abnormalities, MRI is ideally suited as the best way to classify arthropathy. A literature search was carried out to document and list all the imaging methods for hemophilic arthropathy that have been published up to the present. All published classification systems are discussed and listed in tables. MRI has superior imaging capability and will probably become the most important modality for radiological classification of hemophilic arthropathy in the future.

Reliability of progressive and additive MRI scoring systems for evaluation of haemophilic arthropathy in children: expert MRI Working Group of the International Prophylaxis Study Group.

Effective treatment of haemophilic arthropathy requires a detailed evaluation of joint integrity. Methodological assessment of magnetic resonance imaging (MRI) scores are needed to assure reproducibility of measurements when comparing results of clinical trials conducted in different centres. We compared the reliability of two MRI scoring systems for assessment of haemophilic arthropathy: one progressive system that displays the most severe change and one additive system that depicts osteochondral and soft tissue-related changes. A total of 47 1.5 T MRI examinations of knees (n = 21) and ankles (n = 26) of 42 haemophilic boys, age range, 22 months to 18 years, performed at different centres (Toronto, n = 20, Europe, n = 12 and Denver, n = 15) were independently reviewed by four radiologists at two occasions. Twenty-two examinations were from children <9 years and 25 from children >/=9. Sagittal and coronal gradient-echo (MPGR, 3D FLASH with fat saturation, GRASS) images were obtained. The MRI examinations of the ankle and knee studies presented with osteochondral abnormalities in 38.5% and 23.8% of the cases respectively. The two scoring systems demonstrated an excellent inter-reader [progressive, 0.88; additive (A, e, s and h components), 0.86] and intra-reader [progressive, 0.92; additive (A, e, s and h components), 0.93] reliability using intraclass correlation coefficients (ICCs). Although ICCs were slightly higher for knees when compared with ankles, and for older children when compared with younger children, all values fell within excellent inter- and intra-reader reliability categories. The two MRI scoring systems demonstrated a comparable reliability. This result constitutes the basis for further development of a combined MRI scoring system for assessment of haemophilic arthropathy, which incorporates progressive and additive components.

Compatible scales for progressive and additive MRI assessments of haemophilic arthropathy.

The international MRI expert subgroup of the International Prophylaxis Study Group (IPSG) has developed a consensus for magnetic resonance imaging (MRI) scales for assessment of haemophilic arthropathy. A MRI scoring scheme including a 10 step progressive scale and a 20 step additive scale with identical definitions of mutual steps is presented. Using the progressive scale, effusion/haemarthrosis can correspond to progressive scores of 1, 2, or 3, and synovial hypertrophy and/or haemosiderin deposition to 4, 5, or 6. The progressive score can be 7 or 8 if there are subchondral cysts and/or surface erosions, and it is 9 or 10 if there is loss of cartilage. Using the additive scale, synovial hypertrophy contributes 1-3 points to the additive score and haemosiderin deposition contributes 1 point. For osteochondral changes, 16 statements are evaluated as to whether they are true or false, and each true statement contributes 1 point to the additive score. The use of these two compatible scales for progressive and additive MRI assessments can facilitate international comparison of data and enhance the accumulation of experience on MRI scoring of haemophilic arthropathy.

Physical therapy and imaging outcome measures in a haemophilia population treated with factor prophylaxis: current status and future directions.

Routine infusions of factor VIII to prevent bleeding, known as prophylaxis, and other intensive therapies are being more broadly applied to patients with haemophilia. These therapies differ widely in replacement product usage, cost, frequency of venous access and parental effort. In order to address residual issues relating to recommendations, implementation, and evaluations of prophylaxis therapy in persons with haemophila, a multinational working group was formed and called the International Prophylaxis Study Group (IPSG). The group was comprised of haemophilia treaters actively involved in studies of prophylaxis from North America and Europe. Two expert committees, the Physical Therapy (PT) Working Group and the Magnetic Resonance Imaging (MRI) Working Group were organized to critically assess existing tools for assessment of joint outcome. These two committees independently concluded that the WFH Physical Examination Scale (WFH PE Scale) and the WFH X-ray Scale (WFH XR Scale) were inadequately sensitive to detect early changes in joints. New scales were developed based on suggested modifications of the existing scales and called the Haemophilia Joint Health Score (HJHS) and the International MRI Scales. The new scales were piloted. Concordance was measured by the intra-class correlation coefficient of variation. Reliability of the HJHS was excellent with an inter-observer co-efficient of 0.83 and a test-retest value of 0.89. The MRI study was conducted using both Denver and European scoring approaches; inter-reader reliability using the two approaches was 0.88 and 0.87; test-retest reliability was 0.92 and 0.93. These new PT and MRI scales promise to improve outcome assessment in children on early preventive treatment regimens.

Consensus perspectives on prophylactic therapy for haemophilia: summary statement.

Participants in an international conference on prophylactic therapy for severe haemophilia developed a consensus summary of the findings and conclusions of the conference. In the consensus, participants agreed upon revised definitions for primary and secondary prophylaxis and also made recommendations concerning the need for an international system of pharmacovigilance. Considerations on starting prophylaxis, monitoring outcomes, and individualizing treatment regimens were discussed. Several research questions were identified as needing further investigation, including when to start and when to stop prophylaxis, optimal dosing and dose interval, and methods for assessment of long-term treatment effects. Such studies should include carefully defined cohorts, validated orthopaedic and quality-of-life assessment instruments, and cost-benefit analyses.

Radiological assessment of haemophilic arthropathy with emphasis on MRI findings.

Medical imaging of haemophilic joints is important for detecting abnormalities, grading their severity and selecting the appropriate therapy. The plain-film scoring systems for staging joint disease that were developed prior to the availability of magnetic resonance imaging (MRI) are inadequate for planning modern prevention and treatment. MRI is capable of delineating all of the soft tissue findings long before they are evident on plain radiographs. In this paper, an MRI scoring system is presented along with examples of joint effusion, haemarthrosis, synovial hypertrophy, haemosiderin deposition, erosions, cysts and cartilage loss. MRI is a powerful tool in the diagnosis, staging and treatment of patients with haemophilic joint disease.

Traumatic injuries: imaging of peripheral musculoskeletal injuries.

The current dominant role of conventional radiography must be reassessed at increasingly shorter intervals in view of the continuing emergence of new imaging modalities that are available to diagnose peripheral musculoskeletal injuries. In comparison with conventional radiography, digital radiographic techniques offer advantages for optimization of image quality and dose, such as a wider dynamic range and post-processing of images. Currently, digital luminescence radiography (storage phosphor radiography) is the most commonly used digital method for obtaining radiographs, using the established positioning projections and routines of the film-screen technique. A new process, radiography with flat-panel amorphous silicon detectors, is still under development. Computed tomography is a valuable tool for diagnosing injuries of the peripheral musculoskeletal system, especially when three-dimensional data sets are acquired; these allow reformating images in all planes desired (2D technique) or in a volumetric format (3D technique). Established indications for CT in the peripheral skeleton are hip fractures, wrist injuries and calcaneal fractures; however, CT may be used as a supplement to radiography in every region of the body. Sonography is beginning to play an increasingly important role in trauma. Muscle and tendon injuries are the most common indications, but worthwhile information can be gained of the shoulder, elbow, hip, and knee joints, supplementing conventional or digital radiography. Magnetic resonance imaging effectively visualizes traumatic changes of the skeleton and the peripheral soft tissues. It is the method of choice to detect occult fractures. It can be used to diagnose muscle and tendon injuries. Joint injuries, especially in the knee and the shoulder joint, are common indications for MRI in the posttraumatic setting.

Late clinical, plain X-ray and magnetic resonance imaging findings in haemophilic joints treated with radiosynoviorthesis.

The clinical, plain X-ray and magnetic resonance imaging (MRI) findings were studied in 13 haemophilic joints previously treated with radiosynoviorthesis. (32)P had been injected into the joints at a median of 16 years earlier in an attempt to halt recurrent haemorrhage. Prior to (32)P injection, the majority of joints demonstrated bone damage evident on plain X-ray, secondary to recurrent haemorrhage. At the follow-up evaluation we found plain X-rays were adequate to identify cysts, erosions and cartilage loss in these very damaged joints. MRI was superior to clinical examination and plain X-ray in identifying synovial hyperplasia and effusions.

MRI findings in haemophilic joints treated with radiosynoviorthesis with development of an MRI scale of joint damage.

We hypothesized that magnetic resonance imaging (MRI) scans taken prior to radiosynoviorthesis may be predictive of response to the procedure in persons with haemophilia. Specifically, response would be inversely related to the severity of synovial hyperplasia. Radiosynoviorthesis was administered to 21 joints with recurrent haemorrhage (target joints). A detailed self-report of haemorrhage history, joint evaluation with scoring according to the World Federation of Haemophilia orthopaedic joint and pain scales, plain radiographs, and MRI studies of the joints were performed pre- and post-radiosynoviorthesis. To augment comparison of the MRI findings to those assessed using the Arnold-Hilgartner and Pettersson scales, a provisional MRI scale for evaluation of haemophilic arthropathy was designed. We found the MRI findings prior to the procedure were not predictive of clinical response; independent of the severity of synovial hyperplasia, most joints bled less and showed improvement by the WFH orthopaedic score. There was generally no change in the severity of synovial hyperplasia after the procedure. We conclude that MRI evaluation is not routinely indicated prior to radiosynoviorthesis.

Accuracy of diagnosis of subtle chest disease and subtle fractures with a teleradiology system.

OBJECTIVE: This study compared conventional thoracic and skeletal radiographs with 12-bit digitized images of the same radiographs in terms of subjective image quality and accuracy of diagnosis of subtle disease. MATERIALS AND METHODS: Thirty-six chest radiographs with normal findings, 48 chest radiographs with abnormal findings, 29 skeletal radiographs with normal findings, and 26 skeletal radiographs with abnormal findings were included in this study. The images were chosen for the subtlety of their findings (nodules, pneumothoraces, interstitial lung disease, fractures). Each set of images was laser-digitized and viewed independently by three radiologists at a teleradiology workstation with a 1280 x 1024 pixel matrix monitor equipped with an eight-bit-per-pixel gray-scale display. All images were viewed at maximum resolution. After review of the digitized images for image quality and for the presence of abnormalities, each radiologist analyzed the analog images in a similar way. RESULTS: Sensitivities for detecting nodules, pneumothoraces, and interstitial lung disease on digitized chest radiographs were 58%, 75%, and 90%, respectively, compared with 62%, 79%, and 92%, respectively, on the original radiographs. Sensitivity for fracture detection on digitized bone radiographs was 87% compared with 88% on analog radiographs. None of these differences was statistically significant. CONCLUSION: Digitization of radiographs for primary diagnosis by teleradiology results in a slight decrease in sensitivity for detection of subtle abnormalities, provided that the images are viewed at maximum resolution.

Premature osteoarthrosis in professional dancers.

OBJECTIVE: The purpose of this study was to determine whether arthrosis begins at an unusually early age in professional dancers; if there is an association between hypermobility and osteoarthrosis in dancers; and if osteoarthrosis is a contributing factor to retirement from a professional career in this population. DESIGN: Dance and injury history; physical examinations of back, lower extremities, and ligamentous laxity; and radiographs of lower extremity joints were performed in retired dancers. The dancers' radiographs were compared to those of age-matched nondancers. The radiologist was blinded to the origin of the radiographs. PARTICIPANTS: Fourteen retired dancers aged 27 to 46 years who had performed professionally for a minimum of 10 years were included in the study. Thirty-six age-matched nondancers with injuries or pain in various lower extremity joints were used as controls. MAIN OUTCOME MEASURES: This study measured radiographic findings of osteoarthrosis, including sclerosis, joint space narrowing, osteophytes, and subchondral cysts; hypermobility (after Klemp) as manifested by > 3 of 5 tests being positive; and the dancers' reasons for retirement. RESULTS: Changes of arthrosis were found in 34 of 56 joints in 14 dancers and in 3 of 36 joints in 36 nondancers. Hip calcifications were found in 10 hips in 7 dancers and in 1 nondancer's hip. None of the participants in this study was hypermobile or had retired because of arthrosis. CONCLUSIONS: The prevalence of arthrosis in knees, ankles, and first metatarsophalangeal joints in young dancers was increased when compared to that of nondancers in the same age group. Arthrosis does not necessarily cause retirement from a performance career.

Knee version associated with anterior knee pain.

Version of the knee in the presence and absence of anterior knee pain was evaluated by computed tomography in this study. Version of the knee is defined as the static rotation of the tibia with respect to the femur in full knee extension. Fourteen patients in whom conservative management for anterior knee pain failed were compared with 14 volunteers with no symptoms. Computed tomography images of the femoral condyles and tibial plateau were obtained with the knee extended. The angle between the bicondylar and posterior tibial axes was measured. This angle, representing external rotation of the tibia relative to the femur, was increased significantly in patients with symptoms (7 degrees) compared with volunteers with no symptoms (1 degree). This increased knee version identifies a unique morphologic characteristic of the knee with anterior pain.