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Published on: September 30, 2021
Hemophilia orthopedic management with emphasis on developing countries
E Carlos Rodriguez-Merchan1, Michael Heim
1Haemophilia Centre, La Paz University Hospital, Madrid, Spain.
Insights
Early prophylaxis is crucial for children with hemophilia to prevent joint damage. Aggressive treatment of hemarthroses and synovitis is vital when prophylaxis isn't possible, preserving joint function.
Area of Science:
- Orthopedics
- Hematology
- Pediatrics
Background:
- Hemophilia commonly causes hemarthroses from infancy, leading to progressive joint damage.
- Immature skeletons are highly susceptible to hemophilia complications, potentially causing severe structural deficiencies.
- Joint problems in hemophilia can lead to synovitis, recurrent bleeds, and end-stage arthritis.
Purpose of the Study:
- To emphasize the importance of early continuous prophylaxis in managing hemophilia-related joint issues.
- To outline aggressive treatment strategies for hemarthroses and synovitis when prophylaxis is not feasible.
- To review treatment options for established articular complications in adult hemophilia patients.
Main Methods:
- Review of current literature and clinical guidelines on hemophilia joint management.
- Analysis of the impact of prophylaxis versus episodic treatment on joint health.
- Description of conservative and operative interventions for synovitis and advanced joint disease.
Main Results:
- Early continuous prophylaxis significantly reduces the risk of severe structural joint deficiencies.
- Aggressive management of hemarthrosis and synovitis can prevent or delay end-stage arthritis.
- Various surgical interventions are available for established articular complications in hemophilia.
Conclusions:
- Prophylaxis from childhood is the cornerstone of preventing hemophilia arthropathy.
- Prompt and effective treatment of joint bleeds and synovitis is essential for long-term joint preservation.
- A range of surgical options exist for managing advanced joint disease in hemophilia patients.
Abstract:
The articular problems of patients with hemophilia begin in infancy when minor injuries result in hemarthroses. Early continuous prophylaxis (from cradle to college) is of paramount importance because the immature skeleton is very sensitive to the complications of hemophilia; severe structural deficiencies may develop quickly. If continuous prophylaxis is not feasible owing to the expense of the clotting material or lack of venous access, then a major hemarthrosis must be treated aggressively to prevent the development of synovitis, recurrent joint bleeds, and ultimately end-stage arthritis. Once synovitis has developed, which bleeding will make inevitable, the aim should be to treat it as soon as possible. There are conservative (synoviorthesis) and operative (open or arthroscopic synovectomy) methods. Between the second and fourth decades, many patients with hemophilia develop articular complications. Once this has occurred, treatment modalities such as alignment osteotomies, joint debridement, arthrodesis, and arthroplasties are possible. Should surgery be required, one should consider multiple procedures.
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