Case studies: orthopaedic surgery in adult patients with haemophilia A with inhibitors

Summary.  Whilst orthopaedic surgery in haemophilia patients without inhibitors is now relatively common in specialized centres, until recently there have been only a few sporadic instances of surgery having been undertaken on patients with inhibitors. The availability of recombinant activated factor VII (rFVIIa) for haemostatic cover during surgery allows procedures to be performed that previously may not have been considered possible. Complications associated with thrombosis are rare in haemophilia patients with inhibitors, but bleeding complications remain a concern. Globally, experience of performing orthopaedic surgery in these patients is increasing and many successful outcomes have been reported. However, more knowledge relating to the incidence and type of bleeding complications liable to be encountered, together with further information about appropriate rescue treatment, would be valuable. Data relating to long-term follow-up after surgery would be useful, as would a comparison of outcomes between haemophilia patients with and without inhibitors. Optimal dosing regimens for rFVIIa as surgical cover are still to be determined and further information is required relating to the cost effectiveness of rFVIIa in surgery. Further study should address these issues.     

核磁共振影像在血友病性关节病中的应用价值探讨

目的：探讨采用核磁共振影像诊断血友病性关节病的价值。方法：采集本院收治血友病患者12例,分别进行x线检查和MRI检查,观察两种检查结果。结果：X线以及核磁共振检查相同病例其对病理改变以及分期结果均有明显差异（P〈0．05）。结论：经x线与核磁共振影像检查结果对比可见,核磁共振对诊断血友病性关节病更为直观、准确。     

Spondylosis in sand rats: a model of intervertebral disc degeneration and hyperostosis

This study defines gross, histopathologic, and radiologic changes associated with intervertebral disc degeneration in a spontaneously occurring form of the disease in aging sand rats (Psammomys obesus). Sand rats (male/female) fed lab chow supplemented with desert salt bush were sacrificed at periods of 3-30 months. Lateral thoracolumbar spine films were obtained. At sacrifice, spines were surgically exposed and gross findings were recorded; after fixation/decalcification, histopathologic studies were carried out using hematoxylin and eosin, and Safranin-O with fast green counterstain. Metabolic studies included correlations of pathologic and radiologic findings with blood glucose and insulin levels. Disc-space narrowing and subchondral endplate sclerosis increased radiologically with age, with more severe lower lumbar disc lesions. Ligamentous calcifications ventral to involved discs and caudal vertebrae were common. Disc thinning and anterior vertebral bony/cartilaginous spurs were more marked with age. Microscopy revealed loss of nucleus pulposus physaliform cells, chondrocyte replication, disc necrosis, and ossification. Hyperglycemia with and without hyperinsulinemia was common. No statistically significant differences in pathologic findings were noted, neither in diabetic versus nondiabetic nor in hyperinsulinemic animals. The sand rat is a model of disc degeneration; similarities with possible overlap with diffuse idiopathic skeletal hyperostosis syndrome were noted.     

多层螺旋CT在强直性脊柱炎骶髂关节病变分期中的应用价值

目的探讨多层螺旋CT（mult-islice spiral CT,MSCT）在强直性脊柱炎（ankylosing spondylitis,AS）骶髂关节病变的诊断及分期中的应用价值。方法回顾性分析82例AS患者骶髂关节的MSCT表现,对其进行分期。结果 82例中,双侧骶髂关节正常9例,单侧受累6例,双侧均受累67例;164个骶髂关节中,0级24个（构成比0.146）,Ⅰ级42个（构成比0.256）,Ⅱ级36个（构成比0.220）,Ⅲ级54个（构成比0.329）,Ⅳ级8个（构成比0.049）。结论 MSCT分期为AS骶髂关节病变提供了半定量分析方法,更客观、准确地反映了患者的病情及骶髂关节受累程度,为长期随访患者的病情和疗效判断提供了客观依据。     

Charcot arthropathy of the knee after unsuccessful spinal stenosis surgery: A case report

IntroductionCharcot arthropathy was first described in 1868 by Jean Martin Charcot as a progressive and destructive joint disease. Diabetes, polyneuropathy, syphilis, syrengomyelia and chronic alcoholism are the main causes of the disease. In this study we present a Charcot arthropathy of the knee seen after unsuccessful spinal stenosis surgery.Presentation of caseWe report here a case of 62 years old patient with Charcot arthropathy at her left knee developed one year after spinal stenosis surgery. The patient’s knee joint was already beyond the fragmentation and coalescence stages at the moment of physical examination. Patient had already been treated for Charcot foot four years before spinal surgery. Because of an unsuccessful spinal surgery, proximal migration of the level of the sensorineural loss negatively affected the polyneuropathy and eventually resulted in Charcot knee joint in a short period of time.DiscussionHowever, the etiology of the neuropathic arthropathy hasn’t been well described yet, it is usually seen at patients with diabetes mellitus as a long-term complication with or without polyneuropathy. In addition to the spinal canal pathologies, it is reported that Charcot arthropathy can be seen even after spinal anesthesia procedures.ConclusionIn conclusion, spinal procedures should be applied with extra caution on the patients with polyneuropathy or any neuropathic arthropathy. It should be remembered that it is possible to encounter unexpected complications such as proximally migration of the level of sensorineural loss and progression of the actual disease after spinal procedures of these patients.     

Total Knee Arthroplasty in a Patient With Hemophilic Arthropathy and High-Titer Inhibitors : A Cost Analysis

A patient with hemophilia A, who developed factor VIII inhibitors and initially had funding for his total knee arthroplasty declined, is discussed. A total of £1 542 000 (US $2 474 603) was spent on medical treatment for recurrent hemarthroses over a 4-year period, while funding was sought. In comparison, the total cost for his knee arthroplasty was £542 858 (US $871 084) including perioperative recombinant clotting factors. Postoperatively, with 1-year follow-up, no further hemoarthroses have occurred; his analgesic requirement is significantly reduced; he has a much improved level of function; and treatment costs are significantly reduced, leading to a substantial overall saving. Our findings suggest that surgical treatment is beneficial and cost-effective for patients with hemophilia with inhibitors. Hospitals should not deny operative treatment based on cost alone.     

Technical and Imaging Report: Fluoroscopic Guidance for Diagnosis and Treatment of Lumbar Synovial Cyst

Lumbar synovial cysts can be a source of low back pain with or without radiculopathy depending upon the size and location of the mass. Options for treatment range from analgesics to surgery, depending upon the degree of nerve root or spinal cord impingement. Attempts at minimally invasive treatment such as computed tomography‐assisted aspiration and cyst rupture are documented in the radiology literature with varying degrees of reported success. This case report is the first to document the fluoroscopically‐guided management of a lumbar synovial cyst in the pain medicine literature and highlights the knowledge and technical skills required when treating such cases.     

Magnetic resonance imaging of dialysis-related amyloidosis of the shoulder and hip

 Objective. The purpose of this study was to evaluate the usefulness of MRI in identifying abnormalities of the periarticular structures in patients with symptoms of dialysis-related amyloid arthropathy. Design and patients. MR images of shoulders and hips in 5 dialysis patients with symptoms of amyloid arthropathy were compared to images of shoulders and hips in 4 asymptomatic dialysis patients, shoulders in 9 nondialysis patients, and hips in 12 nondialysis patients. These were evaluated for the presence of focal periarticular osseous lesions, tendinous and capsular thickening, and periarticular fluid. Results. Increased thickness of the supraspinatus tendon was found in both symptomatic and asymptomatic patients. Capsular thickening at the hip was present in all symptomatic patients, and in 3 of 8 asymptomatic hips. Joint and bursal fluid was common in the symptomatic group, and was present in some asymptomatic individuals. Osseous lesions were detected in the absence of plain film findings, and demonstrated variable signal intensity. Conclusions. MRI is useful in detecting signs of dialysis-related amyloidosis. Findings of a milder degree in asymptomatic dialysis patients suggests that MRI may also be valuable in the early diagnosis of this syndrome.