Factor IX replacement to cover total knee replacement surgery in haemophilia B: a single-centre experience, 2000-2010.

Total knee replacement (TKR) is a well recognized treatment for haemophilic arthropathy. Successful haemostasis can be achieved by bolus doses or continuous infusion (CI) using either recombinant (r) or plasma-derived (pd) factor IX (FIX). We retrospectively analysed our experience of factor replacement to cover TKR in haemophilia B patients and explored factors related to FIX use during surgery. Between 2000 and 2010, 13 primary TKRs were performed in 11 haemophilia B patients. Operations were performed by the same surgeon using standard techniques. Median age was 58 years (42-79). An adjusted CI protocol was used for 5 days followed by bolus doses. FIX:C was maintained at 100 IU dL(-1) in the immediate postoperative period. There was no excess haemorrhage. There was no evidence of thrombosis or infection. All patients received mechanical thromboprophylaxis and only one chemical. CI was used in seven cases. Ten patients received pdFIX. Median hospital stay was 14 days (8-17). Median factor usage was 999 IU kg(-1) (768-1248). During CI, factor consumption was 695 IU kg(-1), 691 IU kg(-1) and 495 IU kg(-1) for BeneFix®, Replenine® and Haemonine, respectively. Clearance of both pdFIX and rFIX reduced during CI. All operations were uncomplicated. The decreased clearance in the CI setting reduced the amount of FIX required to maintain a therapeutic level. This reduction was greater with pdFIX and may be related to pharmacokinetic differences between pdFIX and rFIX. Given the excellent safety profile of the pdFIX products, CI of FIX and particularly pdFIX is safe, efficacious and convenient.

Perioperative clotting factor replacement and infection in total knee arthroplasty.

Total knee arthroplasty, or replacement (TKR), is now the most commonly performed surgical procedure performed in adults with haemophilia. It is indicated when end-stage haemophilic arthropathy results in intractable pain and reduced function. In patients with haemophilia, however, there has always been a concern about the high risk of infection, which carries with it potentially catastrophic consequences. The aims of this study were to review the case series of TKR for haemophilic arthropathy published in the medical literature, comparing the published infection rates and the differing clotting factor replacement regimes employed. Nineteen retrospective case series were identified; representing 556 TKR's in 455 patients with an overall infection rate of 7.9%. Case series which maintained a high level of clotting factor replacement throughout the first two postoperative weeks, however, had an infection rate of 2.15%, significantly lower than that of case series using the clotting factor replacement regime currently recommended in the World Federation of Hemophilia guidelines (9.22% P = 0.00545). We believe this study supports the use of a high level clotting factor replacement regime, replacing clotting factors to maintain them at a higher level for a longer period of time than currently recommended in international guidelines.

Forearm lengthening by distraction osteogenesis in children: a report of 22 cases.

We present our experience of forearm lengthening in children with various conditions performed by a single surgeon between 1995 and 2009. A total of 19 children with a mean age of 9.8 years (2.1 to 15.9) at the time of surgery had 22 forearm lengthenings using either an Ilizarov/spatial and Ilizarov circular frame or a monolateral external fixator. The patients were divided into two groups: group A, in whom the purpose of treatment was to restore the relationship between the radius and the ulna, and group B, in whom the objective was to gain forearm length. The mean follow-up after removal of the frame was 26 months (13 to 53). There were ten patients (11 forearms) in group A with a mean radioulnar discrepancy of 2.4 cm (1.5 to 3.3) and nine patients (11 forearms) in group B. In group A, the mean lengthening achieved was 2.7 cm (1.0 to 5.5), with a lengthening index of 11.1 weeks/cm. Equalisation or overcorrection of the discrepancy was achieved in seven of 11 forearms, but lengthening was only partially successful at preventing subluxation or dislocation of the radial head. In group B, the mean lengthening achieved was 3.8 cm (1.9 to 6.8), with a lengthening index of 7.25 weeks/cm. Common complications in both groups were pin-site infection and poor regenerate formation. Forearm lengthening by distraction osteogenesis is a worthwhile procedure in children that can improve cosmesis and function, particularly in patients with shortening of both radius and ulna.

Total knee replacement in patients with end-stage haemophilic arthropathy: 25-year results.

Haemophilia is an x-linked inherited bleeding disorder which can cause severe arthropathy. We have reviewed the results of 70 primary total knee replacements (TKR) performed in 57 haemophilic patients between 1983 and 2007. The functional results were assessed using the Hospital for Special Surgery (HSS) knee scoring system and Kaplan-Meier survivorship analysis. Six patients died. HSS scores were available for 60 TKRs at a mean follow-up of 9.2 years (2 to 23); 57 (95%) had good or excellent results. Deep infection was recorded in one patient. Kaplan-Meier analysis using infection and aseptic loosening as endpoints showed the survival rate at 20 years to be 94.0%. A reduction in infection, spontaneous haemarthrosis and improvement in the quality of life were noted to justify surgery in our series of patients with a mean age of 43 (25 to 70). We have found that using the latest techniques of continuous infusion of clotting Factor have significantly helped to reduce the complication rates and have achieved results which match those of the non-haemophilic population undergoing TKR.

Treatment of posterior ankle pain by excision of a bipartite talar fragment.

We describe five adolescent patients aged between 13 and 16 years with bipartite ossification of the posteromedial aspect of the talus. All presented without a history of trauma. All the ankles had a similar radiological appearance. Clinically, some restriction of movement was noted in three ankles and two subtalar joints, In addition, pain was noted over the posteromedial aspect of the ankle in three patients. In each patient the bipartite fragment was excised through a posteromedial approach to the ankle. Complete resolution was achieved at six months in three patients, with the remaining two describing exercise-induced symptoms. In one of these this precluded participation in sport. Despite numerous anatomical variations within the tarsus, a case series of a bipartite talus has not previously been reported. This anatomical variation should be recognised to avoid misinterpretation as post-traumatic or other pathological processes. In the presence of recalcitrant symptoms excision is an option, but this is not universally successful in abolishing symptoms.

Current approaches in haemophilic arthropathy of the hip.

The hip is considered to be one of the main load bearing joints of the body. In the haemophilic patient joint bleeds can be catastrophic, leading to long-term joint degeneration and accompanying arthritis. In this review we explore the mechanisms of joint destruction, with particular consideration of the anatomy of the hip and how it may influence disease progression. We also review current strategies for treatment including hip replacement in the haemophilic patient and describe our experiences as a unit. Finally we evaluate future prospects in the management of hip disease in haemophilia.

Ankle arthropathy in the haemophilic patient: a description of a novel ankle arthrodesis technique.

The ankle joint is well known to show early involvement in severe haemophilia. We describe a novel operative technique developed by the senior author. This combines a medial approach to the ankle, medial malleolar osteotomy, bone graft and compression with staples. All patients had excellent pain relief and improvement in function with 100% achieving bony union. There was a significant improvement in Mazur ankle scores following ankle fusion (P < 0.01). This surgical technique gives good results which are reproducible in this patient population.

An outline of the current orthopaedic management of haemophilic disease of the upper limb.

There remains a relative paucity in the literature regarding upper limb manifestations of haemophilic arthropathy. Haemophilia has a wide range of clinical manifestations, often presenting with orthopaedic complications. These arise from multiple haemarthroses which exact a cumulative toll on the fabric of the joints. Although the lower limbs are predominantly affected due to their load-bearing nature, upper limb disease is common. This arises from the mechanical demands on the upper limb as the elbow and shoulder become partially weight bearing on use of walking aids such as elbow crutches.

Primary malignancies mistaken for pseudotumours in haemophilic patients.

Pseudotumours are a rare complication of haemophilia. Surgery undertaken for pseudotumour on a lesion that is subsequently found to be malignant can be expected to adversely affect patient outcome. We present six case reports found from a literature search of patients with haemophilia who underwent surgery on what was a primary malignancy misdiagnosed as a pseudotumour and give a subsequent discussion.

Heterotropic bone formation as a complication of elective joint replacement in haemophilic patients - a case report and literature review.

We report two cases of this rare postoperative condition. Both patients having undergone extensive surgery to their hips, developed increased bone growth in the soft tissue of the hip, accounting for the reduced range of movement, pain and stiffness. Plain x-rays taken confirmed the presence of heterotopic ossification, also known as a variant of myositis ossificans. We review the literature and discuss treatment options of this rare condition in patients with end-stage haemophilic arthropathy.