Total Hip Replacement in Severe Haemophilia A: Challenges and Feasibility

Abstract

The management of haemophilic patients is complicated due to multiple comorbidities. A dedicated haemophilia treatment centre with a multidisciplinary team can plan and execute elective orthopaedic surgery in such haemophilic individuals. A cementless total hip arthroplasty (THA) was performed in a 26-year-old male patient with haemophilic arthropathy of the right hip under factor VIII replacement therapy based on activated partial thromboplastin time levels. The patient received a preoperative dose of recombinant anti-haemophilic factor. Venous thromboembolic event prophylaxis was not given. Postoperative radiographs demonstrated successful prosthesis placement. The patient could bear weight and walk unassisted two weeks after surgery. THA in patients with haemophilia leads to significant improvement in joint function with a relatively low incidence of complications with modern techniques and haematological management.

Introduction

Haemophilia A is an X-linked recessive disease due to a deficiency of clotting factor VIII. Haemophilia A is quite prevalent with approximately one in 5000 live male births [1]. The joints most frequently affected by haemophilic arthropathy (HA) are the knee, elbow, and ankle [2]. The severity of haemophilia can range from severe (factor levels less than 1%) to moderate (factor levels of 1-5%) to mild (factor levels of 6-30%) based on the deficiency of coagulation factor VIII in the blood. It is expressed in percentage (100% represents the normal concentration for the investigated coagulation factor). The management of haemophilic patients is complicated due to multiple comorbidities. Joint problems developing due to recurrent haemarthrosis, such as chronic synovitis and degenerative arthritis, serve as a significant cause of morbidity. The incidence of haemarthrosis increases with age, with 21% of haemophiliac children reportedly belonging to age one to six years, which increases to 50% in those aged 10- 17 years, followed by up to 60% in those aged 18-65 years [3-5]. A dedicated haemophilia treatment centre with a multidisciplinary team can plan and execute elective orthopaedic surgery in such haemophilic individuals. The first reported hip arthroplasty in a haemophiliac patient was done in 1967 [6]. However, the fixation methods of components (i.e., cemented or uncemented) have been controversial for haemophilic hips [7]. The team should include a haematologist, an expert arthroplasty surgeon, a rehabilitation physician, and a physiotherapist. The most prevalent issues are postoperative bleeding, poor bone quality, and muscular contracture [8,9]. Here, we report a cementless total hip arthroplasty (THA) performed in a 26-year-old patient with HA of the right hip under factor VIII replacement therapy based on activated partial thromboplastin time (APTT) levels. Written informed consent was obtained from the patient after informing the purpose in his local language. The surgical challenges arising in THA in haemophilia are abnormal bleeding, concurrent viral disease, difficulty in achieving successful fixation at the bone and implant interface, abnormal femoral and acetabular anatomy soft tissue contractures, and poor bone quality [10].