Hemophilia and Hemosiderotic Synovitis

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Hemosiderotic synovitis is a condition that results from chronic, repetitive intra-articular hemorrhage. Hemophilia is the bleeding disorder most commonly associated with intra-articular bleeding, but recurrent hemorrhage can also occur secondary to pigmented villonodular synovitis, synovial hemangioma, and repetitive trauma. The consequences of chronic hemarthrosis are direct damage to the articular cartilage and persistent hemosiderotic synovitis that leads to synovial hypertrophy and capsular thickening.85 The changed he-mosiderotic synovial tissue also produces catabolic cytokines and enzymes that further damage the articular cartilage.86,87 As the synovium becomes increasingly scarred, there is gradual conversion from friable hyperemic tissue to fibrotic scar tissue.88 The extensive fibrotic scarring in the periarticular tissues is responsible for the joint contractures so characteristically seen in hemophilia patients. In the limited confines of the hip, hypertrophic synovium may impinge during motion and weightbearing; this may not only cause painful mechanical symptoms and articular cartilage wear, but may induce recurrent bleeding. It is felt that direct damage to the chon-dral surface and chronic synovitis occur in parallel and set up a vicious cycle ultimately culminating in articular surface de struction and joint contracture similar to the process that occurs in osteoarthritis.85

Acute hemarthrosis is probably the most common type of bleeding in hemophilia patients, with 80% of the cases reported in the knees, elbows, and ankles.88,89 Hemarthrosis affecting the hip joint occurs, but by report not as commonly as it does in the other, more peripheral joints. This can be explained by the hip joint's deep location, and the protection from mild to moderate trauma provided by the thick overlying muscular envelope. This protective anatomy may also make realization of bleeding into the hip joint less likely and more difficult to diagnose. Swelling around the hip and pelvic girdle are clinically difficult to detect, and if mild intracap-sular bleeding has occurred or is occurring, it may be contained and clinically impossible to identify. It has been stated that the chronic synovitis reported in other joints is rarely a problem in the hip for patients with hemophilia.90 In this patient population, which endures and functions with chronic pain, the symptoms in the hip region may be just better tolerated than the same process in a peripheral joint. Mild and intermittent hip symptoms therefore may be more significant in the hemophilia patient, and detailed examination and treatment in these situations may justified earlier. Delayed and/or inadequate treatment of unrecognized hemarthrosis and syn-ovitis can trigger the pathologic changes within the joint that will eventually lead to a painful and disabling arthropathy.89,91 The mainstay for treatment of hemosiderotic synovitis, he-marthrosis, and arthropathy in the patient with hemophilia is conservative management with clotting factor replacement. Physical therapy,92 intermittent steroid injections, immobi lization, and activity restriction are also useful adjuvants to factor replacement after an acute bleed. Chemical and radioactive synovectomy have been shown to reduce bleeding in patients who have not responded to conservative therapy, but the procedure does not provide pain relief when mechanical symptoms or arthritic changes are present within a joint.93 Radioactive synovectomy is indicated in patients with inhibitors to the clotting factor, patients with advanced human immunodeficiency virus or advanced hepatitis, and in those patients with multiple joint involvement.94

Orthopedic treatment regimes are dependent on the classification of the synovitis and the stage of the arthropathy. Synovitis can be classified into acute and chronic forms. When the synovitis has already become chronic, early arthroscopic synovectomy is recommended in the knee joint as an attempt to slow the otherwise inexorable progress of the arthropathy. Failure to treat in the early phase of the pathology causes problems in the correction of further stages.95

Orthopedic surgical management of the hip joint in patients with hemophilia has essentially been limited to total hip arthroplasty when painful and debilitating end-stage arthropa-thy is present. In reports with medium-term follow-up, the loosening rate for cemented prostheses has been high, and the complication and infection rate significant.96,97 Some patients, however, become so disabled and confined by the severe hip joint destruction and pain, combined with increasingly frequent need for clotting factor replacement, that reconstructive surgery becomes the optimal treatment modality, despite the risk.

Synovectomy has been effectively used for the treatment of recurrent bleeds and early degenerative changes in other joints, but this has only been mentioned in the literature for the hip.98 The reluctance regarding surgical intervention in the hip may be due to the presence of fibrosis or the potential morbidity of an open hip synovectomy in this population. Arthroscopy may offer a less invasive approach to the hip, but to date this procedure and its potential utility have not been investigated. Joint and periarticular contracture are relative contraindications to hip arthroscopy, and the procedure at this stage of the disease process would likely be of much benefit. Arthroscopic synovectomy of the hip in the hemophilia patient may be worthwhile in the early stages of recurrent hemarthrosis when synovial irritation, thickening, and fibrosis are acute. At this stage articular degeneration is minimal, and may be curtailed by debulking the synovium, reducing the volume of bleeding surface area, and decreasing the amount of scar forming within the hip joint capsule.

Synovectomy for hemophilic arthropathy is safe and efficacious in reducing recurrent haemarthroses and joint pain. Synovectomy should not be performed to improve joint mobility. The progression of the arthropathy is not arrested, and subsequently many patients will be candidates for arthroplasty or arthrodesis.

Arthroscopic synovectomy is the preferred procedure for the knee and ankle joints, although open synovectomy offers an excellent alternative. The greatest risk of these procedures is decreased range of motion, and this is most problematic in the young child who cannot cooperate with a program of physical therapy.94

Arthroscopic surgery, as a relatively low-risk technique, combined with early functional rehabilitation, can be used to achieve satisfactory results in patients with hemophilic arthropathy.99

In a study, nine knees in eight patients with severe hemophilia A, and 1 patient with hemophilia B who underwent arthroscopic synovectomy during the period of 1980-1985 were observed prospectively for 10-15-year follow-up. One complication occurred immediately postoperatively in an 8-year-old boy, in whom a severe hemarthrosis developed that required arthroscopic evacuation. His postoperative recovery was compromised, leading to significant loss of motion. Recurrent hemarthroses developed in only one patient after an injury to the knee. A second arthroscopic synovectomy was performed 45 months after the initial procedure. Other than the patient who lost motion after the postoperative complication, all patients initially regained or improved their range of motion. The latest follow-up, however, showed several patients losing motion, which correlated with clinical and radiographic evidence of progressive changes of the hemophilic arthropathy. The one patient with Factor IX deficiency required a total knee replacement 8 years after synovectomy. Arthroscopic synovectomy was effective in reducing recurrent hemarthrosis and maintaining range of motion; however, joint deterioration continued to occur, though probably at a slower rate.100,101

Both techniques reduce hemarthrosis. Synovectomy may be a useful adjunct to treatment in carefully selected patients with rheumatoid arthritis and hemophilia.102 There is usually a net loss of range of motion with the open versus a net gain with the arthroscopic procedure, and roentgenographic progression of hemophilic arthropathy is slowed but not halted after synovectomy.103

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Responses

  • marcel
    Is hemophilic synovitis painful?
    6 months ago

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