Excerpts from upcoming book, Orthopaedic Principles A Review 2nd edition
- Hemophilia, both classic/hemophilia A (factor VIII def) and hemophilia B (factor IX def) are characterised by recurrent spontaneous haemarthroses.
- Starts manifesting after child is ambulant and must be included in the DD of acute painful swelling of a single joint in young males as inadvertent needle aspiration is hazardous.
- In patients with severe hemophilia, failure to clot after circumcision, immunizations, or lip lacerations sustained in falls often brings the diagnosis to light
- Muscle bleeding is noted because of swelling and nerve compression, most commonly affecting the psoas muscle and the femoral nerve
- Recurrent haemarthrosis lead to development of chronic destructive arthritis.
- Commonly involves knee, elbow, ankle and shoulder. Note that the presence of an effusion in an ankle is heralded initially by obliteration of the “hollow” around the malleoli.
- Once a joint has sustained a bleeding episode, it is much more likely to be affected again; this is called a “target joint”.
Characterised as Mild disease when 5-25% factor is present, Moderate 1-5% factor present, Severe < 1% factor present
- Pathology: The inflammatory changes in hemophilic synovitis are similarin some respects to those of rheumatoid arthritis and pigmentedvillonodularsynovitis
- Subsurface vascularity becomes markedly hyperplastic, with dilated venous sinusoids and an aneurysmal appearance.
- The joint surface loses its luster and, with advanced disease, becomes eroded in rivet-like tracts.
- In end-stage disease, the densely pigment-stained hyperplastic membrane undergoes metaplasia to fibrous tissue,and contractures develop, eventually leading to fibrous ankylosis
Modified Arnold and Hilgartner Radiological Classification of Haemophilic Arthritis
Stage I-soft tissue swelling
Stage II- slight narrowing of the articular space and squaring of the bone ends
Stage III-marked narrowing of articular space
Stage IV- joint disintegration
1. Joint effusions (haemarthrosis)
2. Articular erosions
3. Periarticular osteopenia
4. Multiple subchondral cysts
5. Enlarged epiphysis
6. Narrowing of joint space
7. Gross incongruence of articulating bone ends
8. Joint ankylosis-
One close Differential diagnosis would be JRA but unlike in JRA bony ankylosis and growth retardation does not occur in haemophilic arthritis.
Unique features of haemophilicarthropathy are:
• Widening of femoral intercondylar notches
• Enlargement of proximal radius
• Squaring of distal end of patella
Flexion deformities results from joint ankylosis.
Acute Haemarthrosis: Hemarthrosis in the undamaged joint should be managed aggressively with aspiration,regular factor replacement, restorative physical therapy, and clinical follow-up for smoldering low-grade synovitis
- Strict bed rest and immobilization of limbs in extension to a level that patient can tolerate.
- NSAIDs for pain relief: Cox-2 inhibitors and ordinary NSAIDs like ibuprofen.
- Analgesic dose does not affect platelet formation.
- Correction of coagulation defect –Factor replacement/FFP/cryoprecipitate to raise the levels of factor VIII to 25-50% and factor IX to 15-25%. This prevents further episodes.
- If factor levels do not rise with replacement as expected, an inhibitor should be suspected Some patients with active inhibitors respondto factor VIIa as a bypassing agent Fibrin stabilising agents like e-amino caproic acid, tranexamic acid.
- When aspirating a haemarthrosis, instilling bupivacaine with epinephrine as well as corticosteroid may help to arrest the risk of rebleed and the joint inflammatory response
Indications for aspiration of a joint with moderate to advancedarthropathy (ie, grades III and IV) are limited to pain and functional impairment because by then it is too late to prevent joint-surface destruction
Acute haemarthrosis of the immature hip requires special consideration,including factor replacement and aspiration on an emergent basisto reduce the risk of osteonecrosis
Primary prophylaxis is initiated in infancy before or shortly after the patient’sfirst bleed in the hope of preventing future hemarthroses andlong-term disability
Clotting Factor Replacement for Elective Surgery
- Two hours before surgery, the patient is infused with a dose of clottingfactor calculated to attain 100% activity of normal, which isconfirmed with a factor assay before beginning surgery.
- The patient is then begun on a continuous infusion of factor to maintain levels at >60% throughout the surgery.
- Levels are rechecked immediately after surgery.
- Thereafter, levels are checked every 1 or 2 days. A 60% level is maintained by continuous infusion until the patient is discharged
- Source of recurrent haemarthrosis is bleeding from the hypertrophied vascular synovium.
- Hence synovectomy is advised.
- Synovectomy-open or arthroscopic.
- If a joint bleeds more than three times in 6 months, it becomes prone to develop a chronic synovitis
- If synovitis fails to resolve after 3 months, the treatment is by synovectomy With Arthroscopic synovectomy the entire diseased synovium is removed and lesions of articular cartilage can be debrided simultaneously
- Correction of clotting factor deficiency is essential prior to surgery
- Arthroscopic synovectomy can be done in children as young as 3 years If surgical synovectomy is not practical (eg in patients with inhibitors to the clotting factor, advanced HIV, advanced hepatitis, or multiple joint involvement) radiosynovectomy(Synoviorthesis) with Yttrium 90 silicate/Phosphorus 31colloid is used.
- In knee flexion contractures less than 45°, which are not amenable to conservative treatment, hamstring release and posterior transverse capsulotomy may be done.
- Severe cases require supracondylar osteotomy
It is important to follow the following surgical principles (Post and Telfer et al) while operating on a haemophiliac:
- Tight, careful wound closure to avoid dead space; Avoidance of electrocautery because of the tendency of the coagulated areas to slough after surgery;
- Wound suction in deep wounds for a minimum of 24 hours;
- No aspirin or other medications postoperatively that inhibit platelet
- As many procedures in one single surgical session
- No intramuscular injections in the post operative period ·
For patients with advanced mutilated arthritis, joint replacement is indicated.
- Correction of any flexion contracture of more than 30 degrees are recommended before Surgery.
- The patients who require a TKA are relatively young and they require bilateral TKA most often patellarresurfacing is a recommended component of TKA in this population
Elbow: In adults with recurrent Hemarthrosis, radial head impingement,and pain, the surgical procedure of choice is radial head excisioncombined with synovectomy and debridement
Hip: reasonably good results can be obtained with total hip replacement
Shoulder: arthrodesis or prosthetic replacement are viable options.
Arthrodesis may be indicated in chronic arthritis of ankle. Fixed flexion contractures can be corrected by taking appropriate bony wedges at the time of surgery.
- Neurologic: Bleeding into the central nervous system or major peripheral nerves
- Joints: Stiffness, contracture, and arthritis
- Compartment syndrome
- Blood-borne infections
1. Luck JV, Jr, Silva M, Rodriguez-Merchan EC, et al. Hemophilic arthropathy. J Am AcadOrthopSurg 2004;12:234-245
2. Arnold WD, Hilgartner MW. Hemophilic arthropathy. Current concepts of pathogenesis and management. J Bone Joint Surg 1977;59A: 287-305.
3. Lofqvist T, Nilsson IM, Petersson C: Orthopaedic surgery in hemophilia: 20 years’ experience in Sweden. ClinOrthop 1996;332:232–241
4. Post M, Telfer MC: Surgery in hemophilic patients. J Bone Joint Surg 1975; 57A:1136