The role of physiotherapy after total knee arthroplasty in patients with haemophilia

Summary. With the availability of clotting factor concentrates, advances in surgical techniques, better implant design, and improvements in postoperative management, total knee arthroplasty has become the treatment of choice for haemophilia patients suffering from end‐stage haemophilic knee arthropathy. The success of this surgery is also dependent on close collaborations among the orthopaedic surgeon, the haematologist and the physiotherapist. Although haemophilic patients undergoing this surgery would likely benefit from a targeted rehabilitation programme, its specificities, modalities and limitations have thus far not been extensively studied. Employing the published data of rehabilitation after knee prosthesis in patients with osteoarthritis and haemophilic arthropathy along with clinical experience, the authors present a comprehensive and original review of the role of physiotherapy for patients with haemophilia undergoing knee arthroplasty.     

Haemophilic arthropathy of the elbow

Patients affected by haemophilia commonly have recurrent intra-articular bleeding which leads to progressive destruction and instability of joints. Severe arthropathy of the elbow is complicated by pain, stiffness and loss of function which can be debilitating. Conservative measures such as analgesics, physiotherapy and orthotics are commonly used in the management of these patients. Surgery is considered when conservative measures fail to control the symptoms. Total elbow replacement is now increasingly performed with the advances made in factor replacement therapy and evolution of better implants and techniques of total elbow arthroplasty. The pathogenesis, clinical features and radiological changes of haemophilic arthropathy of the elbow are described in this review article.     

Seventy‐two total knee arthroplasties performed in patients with haemophilia using continuous infusion

Background and Objectives Total knee replacement (TKR) is the treatment of choice in case of end‐stage knee arthropathy, the main complication of haemophilia. We report here a retrospective evaluation of 72 total knee replacement in 51 haemophilia A and B patients using continuous infusion of factor concentrates (CIFC). Materials and Methods Patients were evaluated on the basis of the following efficacy and safety criteria: range of motion, surgery‐related blood loss by three different methods, factor consumption and occurrence of short and long term complications. Results Kaplan–Meier analysis showed a removal‐free survival of TKRs of 88.4% 10 years after surgery. Most patients were satisfied with their prosthesis and described pain relief and improved mobility and better quality of life after surgery. The long term follow‐up showed a mean range of motion at 86° with a flexion deformity of 4°. The blood loss differed significantly according to the method used for measurement. No life‐threatening bleeding occurred. Twenty six haematomas (36.1%) and 2 haemarthroses (2.7%) occurred in 38.8% of cases during the first three postoperative weeks, with no significant impact on the orthopaedic outcome. The average factor consumption during hospitalization was 79 IU/kg/day for patients with haemophilia A and 99 IU/kg/day for patients with haemophilia B. Infections occurred in 4.1% of patients. One patient with severe haemophilia A developed an inhibitor. Conclusions The multidisciplinary approach and the homogeneous management of our large cohort allowed the achievement of excellent functional results. Our results confirmed previously reported data on the safety and efficacy of CIFC in situations requiring intensive factor replacement, such as TKR surgery.     

Long-term major joint outcomes in young adults with haemophilia: interim data from the HGDS

A study of major joint outcomes, specifically range of motion and synovitis, was conducted with data from a subset of adolescents enrolled in the prospective Hemophilia Growth and Development Study (HGDS). Clinical observations were carried out over a 7-year period from 1989 to 1996. A secondary aim was to gain insight into factors that might influence decisions regarding maintaining or discontinuing prophylaxis during early adulthood. Twenty-nine participants (median age 17.4 at entry) were included. Median follow-up was 7 years (range: 4.8-7.7). Range of motion (ROM) and synovitis in six major joints (knees, elbows and ankles), were evaluated by physical examination every 6-12 months. At the baseline observation, 73.6% of joints showed no ROM abnormalities or synovitis, and all joints were normal in 11 patients. Of the 11 participants, 54.5% developed abnormalities and 28.1% of normal joints at baseline became abnormal during the follow-up. Ankles were the most severely affected and had persistent progression during late adolescence and adulthood. Elbows and knees did not show progression after the first few years of the follow-up. The progression of haemophilic arthropathy in adolescents and young adults varies from individual to individual and also in the site of affected joints. In view of this, the decision regarding discontinuation of prophylaxis in patients with haemophilia should be individualized.     

Management of fixed flexion contracture of the elbow in haemophilia

The authors stress that prevention of flexion contractures and artropathy by early factor replacement and physical therapy for every haemophiliac is the standard of care. Physical therapy, serial casting, and Quengel cast correction have not proven successful in correction of fixed flexion contractures at the elbow. In the patient who has a flexion contracture that interfered with function, an attempt at physical therapy combined with the use of either the Dynasplint or Flowtron will be tried. If there is no response, a surgical synovectomy combined with a possible radial head resection and anterior capsular relase would be the authors’ procedure of choice. In the face of advanced arthropathy, the authors would consider a distraction arthroplasty.     

Ipsilateral total shoulder and elbow prosthetic replacement in a patient with severe haemophilia B

Since factor replacement concentrates became widely available in the 1970s, total prosthetic replacement of joints in haemophilic patients has become increasingly common, with success particularly in the knee and hip. However, there has been no published series of elbow or shoulder replacements large enough to draw conclusions about the advisability of these procedures in this group of patients.
We report a case of prosthetic replacement of the shoulder in a 58-year-old man with haemophilia B, and replacement of the elbow joint of the same limb 21 months later. The results are initially very encouraging, with no pain in either joint since the early post-operative period. There have been no bleeds into either joint since surgery. Ranges of movement are satisfactory. Follow-up is now 37 months for the shoulder and 16 months for the elbow.     

Successful angiographic embolization of recurrent elbow and knee joint bleeds in seven patients with severe haemophilia

Summary.  In haemophilic joints with high-grade arthropathy, bleeds occur that do not respond to replacement therapy of the deficient coagulation factor. The reason may be pathologically reactive angiogenesis in chronic synovitis. Seven patients with severe haemophilia A or haemophilia B experienced recurrent massive bleeds of one elbow joint or knee joint in the absence of trauma. After initial application of factor VIII or IX (fVIII/fIX; 50 IU kg⁻¹ bodyweight), there was only slow and never complete relief of symptoms. Despite intensive secondary prophylaxis maintaining the plasma level of factor concentrate at minimum 50%, new massive bleeds at the same location occurred. Vascular bleeding was suspected. Angiography of the arteries was performed via the femoral artery. Vessels identified as potential bleeding sources were embolized with embolization fluid (ONYX) in eight joints (six elbow and two knee joints). Under low-dose prophylactic treatment (15 IU fVIII or fIX per kg bodyweight for three times per week), no recurrent severe bleed unresponsive to coagulation factor replacement occurred after a mean observation time of 16 months after embolization. The consumption of factor concentrate decreased to one-third of the amount consumed before embolization. In conclusion, angiographic embolization with a non-adhesive liquid embolic agent might be considered as a promising therapeutic and coagulation factor saving option in joint bleeds not responding to replacement of coagulation factor to normal levels.     

Body structure versus body function in haemophilia: the case of haemophilic ankle arthropathy

Summary. Imaging and clinical scores are the main tools used to evaluate the progression of haemophilic arthropathy (HA). Based on haemophilic ankle arthropathy, this study aimed to explore the concordances between structural and clinical alterations, determined by standard radiological and clinical scores, and functional alterations assessed by three‐dimensional gait analysis (3DGA). In total, 21 adult haemophilia patients underwent extensive ankle evaluation using the physical examination part of the World Federation of Haemophilia joint score, the Arnold–Hilgartner and the Pettersson radiological scores, and self‐reported ankle function assessment using the revised Foot Function Index. Significant associations were found between self‐reported ankle function assessment and the three 3DGA variables representative of joint function (range of motion, peak plantar flexion moment, and peak power generated at the push‐off phase). Radiological and clinical scores were compared with ankle muscle peak power measurement, the most reliable 3DGA gait variable for ankle function. No significant associations were found between both clinical and functional scores and the 3DGA functional assessment. This discordance may be explained by the lack of a direct relationship between functional alterations detected by 3DGA and the structural changes assessed using X‐ray or clinical scoring. Another explanation may be the limitation of clinical and radiological scoring systems in properly determining the severity of HA. Functional assessments such as 3DGA should be used more frequently when monitoring the progression of ankle arthropathy or the effects of therapeutic interventions in adult haemophilia patients.     

Limited extension after linked total elbow arthroplasty in patients with rheumatoid arthritis

Objective: Total elbow arthroplasty (TEA) has become an established procedure to relieve pain and to increase the range of motion of the destructed elbow in patients with rheumatoid arthritis (RA). However, some patients still have limited extension after TEA, and the causes of limited extension after TEA have yet to be elucidated.

Methods: To examine whether widening of the joint space can cause such limited extension, we retrospectively analyzed 55 cases of linked TEA in patients with RA. There were seven male and 40 female with a mean age of 63.8 years (range, 30–80 years) and a mean follow-up of 7.5?±?4.2 years (range, 2.5–15.6 years). The Mayo Elbow Performance Score (MEPS) and radiological measurements were recorded. Widening of the joint space was calculated by subtracting the length measured on postoperative radiograph from preoperative radiograph.

Results: MEPS and range of motion were significantly improved after surgery except for extension. The degree of extension was significantly correlated with radiological widening of the joint space in the limited extension group. Correlation analyses showed that postoperative limited extension was correlated with lower MEPS daily function.

Conclusions: Limited extension after linked TEA is partly derived from perioperative widening of the joint space and potentially limits daily function in patients with RA.