血友病性骨关节病X线诊断(附18例报告)

目的:探讨血友病性骨关节病X线特点,以提高对本病的认识。方法:回顾性分析18例血友病性骨关节病的X线表现,结合文献进行分析。结果:18例共累及36个关节,其中膝关节25个,踝关节10个,肩关节1个。关节囊软组织肿胀、密度增高15例,软组织内斑片状钙化3例,关节囊内血肿3例,关节周围软组织及远端肢体肌肉萎缩3例。关节间隙明显狭窄11例,轻度狭窄5例,关节间隙轻度增宽1例,无明显狭窄1例。关节面硬化、凹凸不平16例,无明显改变2例。股骨髁间凹增宽加深14例,骨骺骨端增粗变方、骨干变细11例,髌骨呈方形5例,骨质囊变9例,骨质疏松15例。并发假性骨肿瘤2例。结论:血友病性骨关节病好发于膝部,骨端骨骺增大变方、骨干变细,股骨髁间凹增宽加深,髌骨呈方形和血友病性假肿瘤表现具有特征性。     

Reproducibility of the pettersson scoring system. An interobserver study

By means of the Pettersson scoring system, the degree of severity of haemophilic arthropathy in 128 knee, elbow and ankle joints was estimated by 5 radiologists. Their scores varied on average by approximately 3 points. Minimal and maximal joint involvement were scored consistently; exclusion of haemophilic arthropathy was possible with the highest degree of certainty. With regard to changes of a moderate degree agreement was seldom reached. With precisely defined characteristics, a high degree of consistency between observers was evident. Interobserver variation in the scoring was more marked in the ankle joint than in the other joints. Scoring was more consistent with the more experienced observers.     

血友病性骨关节病的X线诊断（附15例报告）

本文报告１５例血友病性骨关节病，其中１０例为血友病甲，２例为血友病乙，１例为血友病丙，余２例未分型。均为男性。其Ｘ线表现为关节囊软组织肿胀、密度增高（１５例），骨端或骨骺增大变方（６例），关节间隙变窄、关节面硬化和囊性变（１２例），关节骨端破坏（６例），骨性关节强直（１例），股骨髁间凹和尺骨鹰咀窝增宽变深（１０例），骨膜新生骨（３例）和血友病性假肿瘤（２例）。文中分析本病的Ｘ线表现并讨论其病理基础。     

Association between joint bleeds and Pettersson scores in severe haemophilia

PURPOSE: To assess and quantify the association of haemophilic arthropathy, measured by the radiological Pettersson score, with the number of joint bleeds suffered by the patient. MATERIAL AND METHODS: In order to quantify the effect of the cumulative number of joint bleeds on the Pettersson score, a study was performed using 295 Pettersson scores obtained in 117 patients with severe haemophilia. The patients were born between 1965 and 1994, and had no history of inhibitors. The number of joint bleeds was determined annually from the entry in the clinic and onwards. The localization of bleeds was not assessed. The patients entered the clinic at a median age of 3.6 years, and received intermediate dose prophylaxis, starting at a median age of 5.3 years. Evaluation of 1643 treatment years showed a mean of 6.8 joint bleeds/year. Pettersson scores were obtained at a mean age of 15.9 years (range 1.7-32.9) and had a median value of 4 points (range 0-50). RESULTS AND CONCLUSION: The Pettersson scores began to rise after the age of 5 years and increased with one point for every 13 joint bleeds (95% confidence interval 11-15). No evidence was found of 'autonomous' progression of Pettersson scores, independent of bleeds.     

Musculoskeletal manifestations of hemophilia: imaging features

The purpose of this pictorial assay is to demonstrate the imaging features of the various musculoskeletal manifestations of hemophilia, an X-linked disorder. Depending on the site of recurrent bleeding, musculoskeletal manifestation can be in the form of hemophilic arthropathy and/or soft tissue, intraosseous, or subperiosteal pseudotumors. Radiography, sonography, computed tomography, and especially magnetic resonance imaging help in the evaluation of hemophilic arthropathy and pseudotumors, providing accurate diagnosis, thus avoiding inadvertent procedures and related complications. Treatment includes replacement of clotting factors either continuously or when indicated, radionuclide or open synovectomy in cases of disabling arthropathy.     

Total knee arthroplasty in severe haemophilic patients under continuous infusion of clotting factors

Purpose

Haemophilic arthropathy is painful, invalidating and destructive. Authors report a prospective study of total knee arthroplasties in patients with severe haemophilia under continuous infusion of clotting factors. The purpose is to evaluate the benefits of continuous infusion of clotting factors regarding long-term functional improvement and radio-clinical results.

Methods

From 1998 to 2009, 20 total knee arthroplasties were implanted in 14 patients with a mean age of 36.5?years (24–56). A continuous infusion of anti-haemophilic factors was used and supervised by the physician of the Regional Haemophilia Treatment Centre (CRTH). Evaluation was clinical using the HSS and Oxford scores and radiological.

Results

One patient was lost to follow-up. Median follow-up is 66.5?months (6–134). Oxford score at latest follow-up is 42 (37–46). On revision, HSS score is 91 (84–96). Median flexion gain is 32.5° (?20; 75°). There is a median flexion contracture of 5° (0–15°) and a median extension improvement of 22.5°. We report 2 secondary infectious complications, concerning the same operated knee of a single patient. No post-operative haematoma was reported in our study.

Conclusion

Total knee arthroplasty in haemophilic arthropathy improves both the function and quality of life of this group of patients. Continuous infusion of clotting factors contributes significantly to these results, by allowing early and intensive rehabilitation, and offers security regarding haemorrhagic complications commonly described in the literature and that we have not encountered in our study.

Level of evidence

Therapeutic study, Level IV.     

Haemophilic knee arthropathy: long-term outcome after total knee replacement

Purpose

The objective of this study was to evaluate the long-term outcome and prosthetic survival of primary total knee arthroplasty in haemophilic patients. It was hypothesized that the infection and revision rate are higher and the outcome inferior when compared with patients without haemophilia.

Methods

Between 1985 and 2004, forty-three consecutive primary total knee replacements were performed in thirty haemophilic patients. These patients?? charts were reviewed retrospectively. Twenty-five patients (34 knees) were available for clinical and radiological follow-up. The outcome was assessed using the Knee Society score, WOMAC and Kaplan?CMeier survivorship analysis.

Results

An haematogenous infection occurred in two patients. In three patients, component revision was needed: two because of an infection and one because of a mechanical failure. After a mean follow-up of 9.6?years (2?C20), 94% of the patients rated their result as either excellent or good. At time of follow-up, the Knee Society Score averaged 73.3 points (range, 29?C100) and showed a significant gain (p?<?0.001) compared to preoperative. Flexion contracture could be reduced significantly (p?<?0.001) from 18.1° preoperatively to 8.4° at follow-up, whereas flexion remained unchanged. When infection or any component replacement was set as endpoints, the 10?years prosthetic survival was 90 and 86%, respectively.

Conclusion

Total knee arthroplasty in haemophilic patients is a reliable treatment that results in pain relief and functional improvement with a low risk of postoperative infection. However, neither the postoperative infection rate nor the functional result does reach the same level as in a population not affected by haemophilia.

Level of evidence

IV.     

Imaging features of pubalgia

Pubalgia is a generic term used to describe groin pain due to a multitude of different etiologies such as skeletal (microtraumatic pubic symphysis arthropathy), muscular (adductor or rectus abdominis disorders), or abdominal wall (inguinal hernia) disorders. Diagnosis relies mainly on MRI for musculoskeletal disorders and ultrasound for abdominal wall disorders.     

Radiological features of the visceral and skeletal involvement of hemochromatosis

Hemochromatosis is a multisystem disorder produced by the excessive accumulation of iron in visceral organs and the musculoskeletal system. Clinically the disease may be silent, but characteristic radiological features may point to the diagnosis. The increased iron stores in the organs involved, especially in the liver and pancreas, result in an increased attenuation at unenhanced CT and an decreased signal intensity at MR imaging. Hemochromatosis arthropathy includes degenerative osteoarthritis and chondrocalcinosis. The distribution of the arthropathy is distinctive, but not unique, frequently affecting the second and third metacarpophalangeal joints of the hand. Received 7 October 1996; Revision received 8 January 1997; Accepted 12 February 1997     

Mandibular haemophilic pseudotumour containing a fluid-fluid level.

Osseous haemophilic pseudotumours are uncommon. Mandibular haemophilic pseudotumours are rare, with only approximately 12 cases reported in the literature to date. We present imaging findings of a histopathologically proven mandibular haemophilic pseudotumour. A fluid-fluid level, hitherto not associated with this condition, is also described.     

The destructive wrist arthropathy of pseudogout

The wrist is a frequent site of arthropathy in the pscudogout syndrome (calcium pyrophosphate dihydrate crystal deposition disease). Three cases of severe wrist arthropathy are presented to emphasize the destructive changes which may occur, including necrosis or collapse of the lunate and navicular bones. The roentgenographic findings characteristic of pseudogout in the wrist are discussed.     

Fluid collections and juxta-articular cystic lesions of the shoulder: spectrum of MRI findings

The MR imaging features of fluid collections and juxta-articular cystic lesions of the shoulder are discussed, with special focus on those related to subacromial impingement and rotator cuff tears. Other more unusual fluid collections and cystic lesions are described, including rice-bodies bursitis, idiopathic synovial osteochondromatosis, dialysis-related amyloid arthropathy, hemophilic arthropathy, infectious conditions, non-infectious inflammatory arthritis, and paralabral cysts.     

Long-term results of total knee arthroplasty in haemophilic patients: an 18-year follow-up

Purpose

Total knee arthroplasty (TKA) for patients with end-stage haemophilic arthropathy is considered to be a successful procedure with satisfying mid- to long-term results. It was the purpose of this study to provide clinical and radiological long-term results of TKAs implanted in a consecutive cohort of haemophilic patients.

Methods

Primary TKA was performed in 43 consecutive knees in 30 haemophilic patients. After a mean of 18 (SD ± 4) years, 15 patients (21 knees) with a mean age of 58 (SD ± 8) years were available for follow-up. The outcome was assessed using the Knee Society score, WOMAC, SF-36, Kaplan–Meier survivorship analysis as well as radiographic evaluation of radiolucency.

Results

In 13 (30%) of the 43 consecutive knees, revision surgery was necessary due to infection or aseptic loosening, among which eight (19%) due to aseptic loosening and five (12%) due to haematogenous infection. The calculated 20-year survival rates with revision for any reason or infection as the end points were 59 and 82%, respectively. All patients with the primary TKA in situ observed progressive radiolucent lines around the implants at the final follow-up. The Knee Society clinical and functional score significantly improved from pre- (36 points; SD ± 16 and 62 points; SD ± 19) to post-operatively (73 points; SD ± 15 and 78 points; SD ± 18; p < 0.001). Eighty-six per cent rated their result as either good or excellent. Whereas flexion did not improve, flexion contracture could be reduced significantly from 18° (SD ± 12) to 6° (SD ± 5; p < 0.001) post-operatively.

Conclusion

Total knee arthroplasty in haemophilic patients is associated with high revision, loosening and infection rates after 18 years. However, if revision can be avoided, joint replacement in haemophilic patients helps to relieve pain, achieve higher subjective satisfaction and to restore knee function. Level of evidence IV.

     

Rheumatic diseases presenting as sports-related injuries

Most individuals seeking consultation at sports medicine clinics are young, healthy athletes with injuries related to a specific activity. However, these athletes may have other systemic pathologies, such as rheumatic diseases, that may initially mimic sports-related injuries. As rheumatic diseases often affect the musculoskeletal system, they may masquerade as traumatic or mechanical conditions. A systematic review of the literature found numerous case reports of athletes who presented with apparent mechanical low back pain, sciatica pain, hip pain, meniscal tear, ankle sprain, rotator cuff syndrome and stress fractures and who, on further investigation, were found to have manifestations of rheumatic diseases. Common systemic, inflammatory causes of these musculoskeletal complaints include ankylosing spondylitis (AS), gout, chondrocalcinosis, psoriatic enthesopathy and early rheumatoid arthritis (RA). Low back pain is often mechanical among athletes, but cases have been described where spondyloarthritis, especially AS, has been diagnosed. Neck pain, another common mechanical symptom in athletes, can be an atypical presentation of AS or early RA. Hip or groin pain is frequently related to injuries in the hip joint and its surrounding structures. However, differential diagnosis should be made with AS, RA, gout, psudeogout, and less often with haemochromatosis and synovial chondochromatosis. In athletes presenting with peripheral arthropathy, it is mandatory to investigate autoimmune arthritis (AS, RA, juvenile idiopathic arthritis and systemic lupus erythematosus), crystal-induced arthritis, Lyme disease and pigmented villonodular synovitis. Musculoskeletal soft tissue disorders (bursitis, tendinopathies, enthesitis and carpal tunnel syndrome) are a frequent cause of pain and disability in both competitive and recreational athletes, and are related to acute injuries or overuse. However, these disorders may occasionally be a manifestation of RA, spondyloarthritis, gout and pseudogout. Effective management of athletes presenting with musculoskeletal complaints requires a structured history, physical examination, and definitive diagnosis to distinguish soft tissue problems from joint problems and an inflammatory syndrome from a non-inflammatory syndrome. Clues to a systemic inflammatory aetiology may include constitutional symptoms, morning stiffness, elevated acute-phase reactants and progressive symptoms despite modification of physical activity. The mechanism of injury or lack thereof is also a clue to any underlying disease. In these circumstances, more complete workup is reasonable, including radiographs, magnetic resonance imaging and laboratory testing for autoantibodies.     

Haemophilic pseudotumour presenting with large bowel obstruction

Osseous haemophilic pseudotumours are uncommon. The commonest sites of involvement are the femur and the pelvis. Trauma is the initiating factor in most reported cases and repeated bleeding into the lesion contributes to their growth. Most lesions grow slowly and are often asymptomatic. Complications include massive haemorrhage, infection and pathological fracture. We present an extremely unusual presentation where a large haemophilic pseudotumour of the pelvis extended to impinge the adjacent colon, resulting in large bowel obstruction.     

Joint changes in idiopathic hemochromatosis.

Two patients with idiopathic hemochromatosis and a distinctive arthropathy are presented. The joint findings are characterized by joint narrowing, prominent osteophytes, subcondral sclerosis and multiple lucent subchondral cysts, and chondrocalcinosis. The etiology of this arthropathy and its relationship to chondrocalcinosis are discussed. Occasionally, patients may present with this arthropathy before the onset of the classical signs of idiopathic hemochromatosis.     

Radiologic features of a pyrophosphate-like arthropathy associated with long-term dialysis

In a series of 28 long-term dialysis patients with musculoskeletal complaints, the radiologic findings in six cases resembled those occurring in the arthropathy of idiopathic calcium pyrophosphate dihydrate deposition (CPPD) disease. These findings included osteophytes, subchondral cysts, and cartilage loss in the metacarpophalangeal joints, patellofemoral joints, wrists, and shoulders. Chondrocalcinosis was present in three of the six cases. There were no significant differences in renal function or levels of serum calcium, phosphorus, iron, ferritin, aluminum, or parathormone between these patients and a control group matched for sex and age. Long-term dialysis may be associated with a metabolic arthritis similar to the arthritis which occurs in CPPD deposition disease. The etiology may include deposition of CPPD crystals, hydroxyapatite, or other calcium-containing substances in joints, or it may be related to a number of dialysis-induced metabolic abnor-malities.     

先天发育性（骨）关节病——关节病的新概念

目的：关节病的传统概念其病因是获得性的或由后天继发因素所致，本文提出先天发育性（骨）关节病的概念。材料与方法：本组通过对成骨不全（６例），Ｍａｒｆａｎ综合征（４例），粘多糖病（８例），多发骨骺发育异常（２２例），致密性骨发育不全（２例），干骺软骨发育异常（４例），石骨症（３例），遗传性多发外生骨疣（６例），内生软骨瘤病（４例），Ｍａｆｆｕｃｃｉ病（２例），Ｍａｆｆｕｃｃｉ病（２例），系统性骨纤维发     

Imaging of neuropathic arthropathy

Neuropathic arthropathy is a chronic, progressive joint degeneration with bone fragmentation, ligamentous instability, and dislocation. Diabetes is the leading cause of neuropathic arthropathy. Conventional radiography is the most commonly used imaging modality for diagnosing neuropathic arthropathy. The disease is mostly the hypertrophic type and is manifested by sclerosis of the bone, fragmentation, joint destruction, swelling, large joint effusion, and large osteophyte formation. Computed tomography, magnetic resonance imaging and radionucleide scintigraphy are helpful for diagnosing the disease and may help in distinguishing neuropathic arthropathy from septic arthritis and osteomyelitis.     

Musculoskeletal disorders associated with HIV infection and AIDS. Part I: Infectious musculoskeletal conditions

The musculoskeletal system can be affected by a variety of abnormalities in association with human immunodeficiency virus (HIV) infection. Although not as common as complications involving other organ systems, such as the pulmonary and the central nervous systems, HIV-associated musculoskeletal disorders are sometimes the initial presentation of the viral illness. Knowledge of the existence and the characteristic appearance of the conditions affecting bone, joint, and muscle in HIV-infected patients is valuable to radiologists for diagnosis and to clinicians for detection and appropriate treatment. We reviewed recent literature to provide a comprehensive assessment of the HIV-associated musculoskeletal disorders, and present radiologic examples from our own collection. This article is divided into two parts. In the first part we review the infectious musculoskeletal disorders associated with HIV illness and AIDS, including cellulitis, abscesses, pyomyositis, septic bursitis, septic arthritis, osteomyelitis, and bacillary angiomatosis. We also present a comprehensive spectrum of mycobacterial infections, consisting of tuberculous spondylitis and spondylodiskitis, arthritis, osteomyelitis, and tenosynovitis, as well as infections caused by atypical mycobacteria. Part II of this review will concentrate on non-infectious musculoskeletal conditions, including rheumatic disorders and neoplasms.