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Biomedical subjects

E C Rodriguez-Merchan

Publications and source records attributed to E C Rodriguez-Merchan.

At least 37 records · Page 2Linked to original sources

Total knee replacement in haemophilia.

We believe that total knee replacement (TKR) is a safe and effective procedure for the management of haemophiliac joint arthropathy; however, the increased risk of infection and non-infective complications remain a cause for concern. TKR in haemophilic patients carries with it an increased risk of post-operative infection in comparison to non-haemophiliac patients. Those patients at particular risk are the HIV-positive haemophiliac patients whose CD4 count is less than 200 cells mm-3. The latest techniques have gone a long way to reducing the complication rate and to achieving results that match those of a similar non-haemophiliac population.

Acquired Immunodeficiency Syndrome↗

Orthopaedic surgery in haemophilic patients with inhibitors: an overview.

Our experience and a review of the literature on inhibitors have shown that, with the availability of recombinant factor VIIa (rFVIIa), haemophilic patients with high inhibitor titres requiring elective orthopaedic surgery can undergo such surgery with a high expectation of success. The advent of rFVIIa has made major elective orthopaedic surgery possible in haemophilic patients with high-titre inhibitors, resulting in an improved quality of life. Recombinant FVIIa appears to be an efficient haemostatic product for surgery in patients suffering from haemophilia A and B with inhibitors. The series of major elective orthopaedic surgical procedures using rFVIIa (n=53) is the largest ever reported in haemophilic patients with inhibitors, despite the long-standing presence of other treatment modalities, such as high-dose human factor VIII (FVIII), porcine FVIII (n=8), and prothrombin complex concentrates/activated prothrombin complex concentrates (n=9). Thorough analysis of each case as part of a multidisciplinary team will allow us to perform elective orthopaedic procedures in patients with inhibitors.

Biological Factors↗

Haemophilic cysts (pseudotumours).

It is very likely that with the advent of widespread maintenance therapy, pseudotumours will be less common in the future. It is important that they are diagnosed early, and prevention of muscular haematomas is the key to reducing their incidence. There are a number of therapeutic alternatives for this dangerous condition: surgical removal, percutaneous management, exeresis and filling of the dead cavity, irradiation, and embolization. The management of a patient with a haemophilic pseudotumour is complex, with a high rate of potential complications. Surgical excision is the treatment of choice but should only be carried out in major haemophilia centres by a multidisciplinary surgical team. The main postoperative complications are death, infection, fistulization, and pathological fractures (even requiring amputations of affected limbs). Pelvic pseudotumours can even become complicated by fistulization to the large bowel and by obstruction of the ureters. Untreated, proximal pseudotumours will ultimately destroy soft tissues, erode bone, and may produce neurovascular complications.

Bone Diseases↗

Total knee arthroplasty in HIV-positive haemophilic patients.

In the era of total joint replacements in orthopaedics, total knee arthroplasty (TKA) should be indicated in haemophilic patients suffering from severe knee pain and disability. However, the expected high risk of infection and other postoperative complications is a concern. Although the message of this article may seem conservative, it should not be inferred that TKA should still be avoided in a haemophilic patient, but rather that the orthopaedic surgeon should weigh the risks and benefits carefully. Clinical and immunological status should be considered before suggesting a total knee replacement to a haemophilic patient. If TKA is contraindicated, knee arthrodesis is the alternative. However, knee joint debridement can relieve pain for several years and delay the need for TKA.

Acquired Immunodeficiency Syndrome↗

Bone fractures in the haemophilic patient.

The goal of modern fracture treatment is to obtain an optimal outcome, with the patient's return to full activity as soon as possible. Nowadays, internal stabilization is indicated in most displaced fractures in adults, whereas external fixation remains the best choice for initial stabilization with severe soft-tissue injuries. If a fracture is correctly treated in a haemophilic patient, it will progress to consolidation in a similar time-frame to fractures occurring in the general population.

Adult↗

Brucellosis as a cause of septic loosening of total hip arthroplasty.

Infection after a total hip arthroplasty is a severe complication and is associated with a high incidence of morbidity. We describe a case of late infection, 5 years after the implantation of a cemented hip prosthesis. The infection was caused by gram-negative Brucella melitensis and occurred in a 63-year-old man who owned cattle. As far as we know, such a complication has never been published before.

Arthroplasty, Replacement, Hip↗

General principles and indications of synoviorthesis (medical synovectomy) in haemophilia.

The indication for a synoviorthesis (medical synovectomy) is chronic haemophilic synovitis causing recurrent haemarthroses, unresponsive to haematological treatment. Synoviorthesis is the intra-articular injection of a certain material to diminish the degree of synovial hypertrophy, decreasing the number and frequency of haemarthroses. There are two basic types of synoviorthesis: chemical synoviorthesis and radiation synoviorthesis. On average, the efficacy of the procedure ranges from 76 to 80%, and can be performed at any age. The procedure slows the cartilaginous damage which intra-articular blood tends to produce in the long term. Synoviorthesis can be repeated up to three times with 3-month intervals if radioactive materials are used (Yttrium-90 and Phosphorus-32), or weekly up to 10-15 times if rifampicin (chemical synovectomy) is used. After 30 years of using radiation synovectomy worldwide, no damage has been reported in relation to the radioactive materials. Radiation synovectomy is currently the preferred procedure when radioactive materials are available, however, rifampicin is an effective alternative method if radioactive materials are not available. Several joints can be injected in a single session, although no more than two joints at the same time is probably the best protocol to follow.

Hemarthrosis↗

The technique of synoviorthesis.

Synoviorthesis (medical synovectomy) is a commonly utilized procedure employed in the management of recurrent intra-articular bleeds and secondary chronic synovitis in haemophilic patients. The procedure involves the intra-articular administration of an agent in order to induce fibrosis in the inflamed synovium so reducing the bleeding tendency. Such agents may either be chemical based (osmic acid, rifampicin) or radioactive (yttrium, gold or phosphorus). Whichever agent is employed care must be taken to avoid extra-articular complications (radiation burn and/or inflammatory reaction) as a result of extravasation or needle track contamination. The most frequently affected joints in haemophilia are the elbows, knees and the ankles, and to a lesser extent the shoulders and hips. This article outlines the techniques of injection of the five aforementioned joints, with details of the anatomical landmarks to perform the injections correctly. Injection of the elbows, knees and ankles may be carried out simply on an out-patient basis under local anaesthesia. However, it is advisable to perform injection of the shoulder or hip under radiographic control in order to ensure accurate placement. Very young children may require either sedation or a general anaesthetic. Strict asepsis is naturally a paramount requirement.

Arthroscopy↗

Yttrium-90 synoviorthesis for chronic haemophilic synovitis: Madrid experience.

Between 1994 and 1999 we performed 66 Yttrium-90 (90Y) synoviortheses on 44 persons with haemophilia (45 knees, 12 elbows, nine ankles). At the time of injection 23 patients were HIV+ and two had inhibitors. The average age was 21.1 years (range: 9-39). Five mCi of 90Y were injected into the knees, and 3 mCi into the elbows and ankles. The average follow-up was 3.5 years (range: 1-6). Of the 45 knees, there were eight excellent, 10 good, 15 fair and 12 poor results. Of the 12 elbows there were three excellent results, five good, three fair and two poor. Of the nine ankles there were no excellent results, four good, three fair and two poor. The elbows had better results than the knees and ankles. The best results were obtained in the youngest patients, and in those with a moderate degree of synovitis, regardless of their HIV status and the presence of inhibitor. 90Y synoviorthesis should be performed in very young patients, when the amount of synovium is still moderate. Once the degree of synovitis has become severe, the expected results of radioactive synoviorthesis are worse.

Adolescent↗

Post-synoviorthesis rehabilitation in haemophilia.

The efficacy of radiosynoviorthesis in the management of chronic haemophilic synovitis has been proven. Like replacement therapy, however, this procedure has no effect on muscle strength or endurance, range of movement, coordination, or the patient's physical condition in general. All the factors just enumerated are essential for the recovery and maintenance of the haemophiliac's quality of life. That is why rehabilitation and physiotherapy are so important, since no medical therapy or surgical procedure are by themselves capable of preventing disabilities.

Hemarthrosis↗

Methods to treat chronic haemophilic synovitis.

The aim of both synoviorthesis and surgical synovectomy is to remove the inflamed and hypervascular synovium as soon as possible in order to prevent the onset of haemophilic arthropathy. Ideally, however, these methods should be performed before the articular cartilage has eroded. Radioactive synoviorthesis is a relatively simple, virtually painless and inexpensive technique for the treatment of chronic haemophilic synovitis, even in patients with inhibitors. Thus, radioactive synoviorthesis is the best choice for patients with persistent synovitis. Personal experience and the general recommendation among orthopaedic surgeons and haematologists is that when three early consecutive synoviorthesis (repeated every 3 months) fail to halt synovitis, a surgical synovectomy (open or by arthroscopy) should be immediately considered.

Chronic Disease↗

Total joint arthroplasty in haemophilia.

In severely affected haemophilic patients arthropathy is a common problem which can lead to considerable pain and functional deficit. Surgical management, including total joint arthroplasty, can be undertaken if conservative management fails. A search of the literature showed that a number of studies describing the use of total knee arthroplasty (TKA) and total hip arthroplasty (THA) in haemophilia have been published, whereas shoulder, elbow and ankle arthroplasties are confined to case reports. This paper reviews the functional outcome of arthroplasty in the different joints, the postoperative and long-term complications, and the impact of HIV. Although complications are commonly described and the surgery is technically demanding, the results suggest that arthroplasty, particularly of the hip and knee, can be a valuable option in the management of severe haemophilic arthropathy.

Arthroplasty, Replacement↗

Intra-articular displaced fractures of the calcaneus. Operative vs non-operative treatment.

Twenty-eight patients with displaced intra-articular fractures of the calcaneus treated by open reduction and fixation were compared with 30 patients with similar fractures treated conservatively. Judged by the clinical and radiographic criteria results were more satisfactory in the surgical group than in the nonoperative group, although high rates of poor results were encountered in both groups.

Calcaneus↗

Joint replacement for a spontaneously ankylosed hip in a haemophilic patient.

A cemented Charnley total hip prosthesis was implanted in a 48-year-old man with mild haemophilia (factor VIII 4 IU dL-1) in his right spontaneously ankylosed hip. At the time of surgery he was anti-HCV positive, anti-HIV negative, and no circulating inhibitors were encountered. The indication for surgery was long-lasting intractable low back and ipsilateral knee pain. At 4-month follow-up, relief of pain was achieved as well as correction of limb-length discrepancy, with a good result according to the Mayo Clinic hip score. Doses of 50 IU kg-1 body weight of recombinant factor VIII (Recombinate; Baxter, Glendale, California, USA) was used during the 2 weeks of admittance to the hospital. The dosage was adjusted according to the recoveries of factor VIII, with an overall factor consumption of 68 000 IU. As far as we know this is the first case reported in the literature of a person with haemophilia in whom a spontaneous hip ankylosis has been satisfactorily converted in a total hip arthroplasty with a short-term follow-up. However, a much longer follow-up is still needed to ascertain the efficacy of this surgical procedure in haemophilia.

Adult↗

Therapeutic options in the management of articular contractures in haemophiliacs.

Haemophilic contracture is seen most commonly as an equinus deformity of the ankle, or at the knee or elbow in the form of a flexion deformity. Treatment options are varied, and decision-making is based on the degree of the contracture, its chronicity, the presence of articular subluxation, the patient's ability to participate in treatment, and the available medical facilities. The treatments available fall into four categories: physiotherapy, orthotics, corrective devices, and surgical procedures. Treatment should be primarily by physiotherapy, splintage, and corrective devices. The late or severe case may require surgical correction in the form of soft-tissue procedures. Soft-tissue correction of muscle shorthening may be performed such as lengthening of the Achilles tendon for equinus deformity of the ankle, or hamstring release of the flexor muscles of the knee. Lower femoral osteotomy has been used for correction of flexion deformity at the knee joint. Mechanical distraction using external fixators for treatment of severe knee flexion contractures has been recently reported with satisfactory results. The main principle underlying the treatment of haemophilic contracture is the restoration of the patient's lifestyle and mobility, rather than anatomic or radiographic normality.

Contracture↗