Radiosynoviorthesis in children with haemophilia.

Recurrent bleeding into joints represents the clinical hallmark of haemophilia and, if not adequately treated, it may cause chronic synovitis and degenerative arthropathy. The first treatment option of recurrent haemarthroses and/or chronic synovitis is represented by synoviorthesis, both chemical and radioisotopic, with a success rate of approximately 80% for both. However, radioisotopic synoviorthesis should be preferred because it makes it possible to obtain complete synovial fibrosis usually in one session, without the need for repeated injections, thus reducing the risk of bleeding complications and concentrate consumption. For all these reasons this procedure should be implemented and supported, particularly in developing countries.

Orthopaedic management of haemophilia arthropathy of the ankle.

Joint bleeding, or haemarthrosis, is the most common type of bleeding episode experienced by individuals with haemophilia A and B. This leads to changes within the joints, including synovial proliferation, which results in further bleeding and chronic synovitis. Blood in the joint can also directly damage the cartilage, and with repeated bleeding, there is progressive destruction of both cartilage and bone. The end result is known as haemophilic arthropathy. The joints most commonly affected are the knees, elbows and ankles, although any synovial joint may be involved. In the ankle, both the tibiotalar and subtalar joints may be affected and joint bleeding and arthropathy can lead to a number of deformities. Haemophilic arthropathy can be prevented through regular factor replacement prophylaxis and implementing physiotherapy. However, when necessary, there are multiple surgical and non-surgical options available. In early ankle arthropathy with absent or minimal joint changes, both radioisotopic and chemical synoviorthesis can be used to reduce the hypertrophied synovium. These procedures can decrease the frequency of bleeding episodes, minimizing the risk of articular cartilage damage. Achilles tendon lengthening can be performed, in isolation or in combination with other surgical measures, to correct Achilles tendon contractures. Both arthroscopic and open synovectomies are available as a means to remove the friable villous layer of the synovium and are often indicated when bleeding episodes cannot be properly controlled by factor replacement therapy or synoviorthesis. In the later stages of ankle arthropathy, other surgical options may be considered. Debridement may be indicated when there are loose pieces of cartilage or anterior osteophytes, and can help to improve the joint function, even in the presence of articular cartilage damage. Supramalleolar tibial osteotomy may be indicated in patients with a valgus deformity of the hindfoot without degenerative radiographic findings. Joint fusion, or arthrodesis, is the treatment of choice in the advanced stages of ankle arthropathy although total ankle replacement is currently available. Early ankle replacement components were associated with a poor outcome, but as implant designs have improved, there have been successful outcomes achieved. As the ankle is a commonly affected joint in many individuals with haemophilia, it is important to add to the knowledge base to validate indications and timing of surgical and non-surgical interventions in ankle arthropathy.

Synoviorthesis in haemophilia patients with inhibitors.

Recurrent bleeding into joints represents the clinical hallmark of haemophilia and, if not adequately treated, it may cause chronic synovitis and degenerative arthropathy. In haemophilia patients with inhibitors, a more severe degree of synovitis is often observed owing to the fact that treatment is more problematic in this setting. The first treatment option of recurrent haemarthroses and/or chronic synovitis is represented by synoviorthesis, both chemical and radioisotopic, with a success rate of approximately 80% for both. However, radioisotopic synoviorthesis should be preferred in inhibitor patients because it makes it possible to obtain complete synovial fibrosis usually in one session, without the need for repeated injections, thus reducing the risk of bleeding complications and concentrate consumption. For all these reasons this procedure should be implemented and supported, particularly in developing countries.

Total joint replacement in patients with inhibitors.

Today, total joint replacement is the treatment of choice for chronic haemophilic arthropathy of the knee and hip in developed and developing countries. After the last World Haemophilia Congress and Musculoskeletal Congress, we cannot say the same for haemophilic patients with inhibitors because elective surgery today is still limited to few centres and extremely few patients. This is because until the first half of the 1990s, performing surgery in haemophilic patients with inhibitors was associated with a high risk of bleeding. With the availability of activated recombinant factor VII, the first surgical procedures were performed, but they still remain limited because of the elevated costs of replacement therapy. Our goal for the future must be to ensure the same possibility of surgical intervention in haemophilic patients both with and without inhibitors. This will be possible, thanks to the experience of some centres with an increased number of patients, where today this kind of surgery is routinely performed.