Special Features of Total Knee Replacement in Hemophilia

Abstract and Introduction

Abstract

Total knee replacement is an operation frequently needed by hemophilia patients, which greatly improves their quality of life. This operation, however, carries a higher risk of bleeding and infection for hemophiliacs than it does for osteoarthritis sufferers. It is advisable to implant prosthetic components using antibiotic-loaded cement. It is essential to maintain a level of 100% of the replacement clotting factor for 2 weeks. Hematological treatment must be established, depending on the patient's factor levels and other pharmacokinetic parameters such as recovery and half-life, optimal doses and treatment time. It is preferable to use general anesthesia due to the risk of spinal bleeding. The lifespan of total knee replacement in hemophilic patients is shorter than in patients with osteoarthritis because of the increased risk of infection.

Introduction

From birth, hemophilia patients suffer from a congenital clotting defect that makes them prone to multiple joint bleeds (hemarthrosis). These may be spontaneous or caused by minimal trauma, and fundamentally affect the knees, ankles and elbows. Such recurrent hemarthrosis of the knees will cause joint degeneration (hemophiliac arthropathy) at a very early age (20–30 years) (Figure 1).^[1]

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Figure 1.

Recurrent hemarthroses of the knee will cause joint degeneration (hemophiliac arthropathy) at a very early age (20–30 years). Severe hemophiliac arthropathy in a hemophilia patient, very painful and disabling: anteroposterior image (A), and lateral radiograph of the same patient (B).

The only way to combat this degeneration – although it cannot be completely prevented – is through primary prophylaxis (before the first hemarthrosis or after 1–2 hemarthroses) before the first hemarthrosis takes place.^[2] That is to say, it is essential to protect the joints of hemophiliac patients through the use of primary prophylaxis.

At present, 70–80% of patients do not receive adequate treatment due to financial and economic constraints. Hemophilia patients with very painful and/or incapacitating knees as a result of the above-mentioned degenerative arthropathy frequently require total knee replacement (TKR) if there is to be a significant improvement in their quality of life.^[3–6]

Before deciding on TKR, the possibility of arthroscopic debridement must be taken into account, provided that the knee has normal axial alignment (i.e., without varus or valgus deformities). In this article, the author will provide recommendations regarding TKR in hemophilia.

Table 1. Prosthetic lifespan in hemophiliac patients (percentage of primary total knee replacement removal-free survival).
Study (year)	Percentage of TKR removal-free survival	Ref.
Solimeno et al. (2009)	81 at 7 years	[20]
Cohen et al. (2000)	88.4 at 10 years	[21]
Sikkema et al. (2011)	94 at 20 years	[22]

TKR: Total knee replacement.

Box 1. Risk factors of infection following total knee replacement in general population.
Patient-related risk factors (immunosuppression treatment and previous infections in the knee) Lack of systematic preoperative screening by swab (nose and any skin lesion(s) during the month preceding surgery, recent history of surgery) Inadequate preoperative antibiotic prophylaxis Inappropriate surgeon's preparation and unclean air in the operating room. Inadequate skin preparation and dressings Intravenous therapy