Original Research

Total Knee Arthroplasty in Hemophilic Arthropathy

Am J Orthop. 2015 December;44(12):E503-E507

Hemophilic arthropathy of the knee is common among patients with hemophilia and is a major cause of severe joint pain and functional disability requiring total knee arthroplasty (TKA).

We evaluated TKA outcomes and complications with a special focus on prosthetic survival and infection. We retrospectively reviewed the medical records of 74 patients with chronic hemophilic arthropathy of the knee treated with TKA (N = 88) over a 13-year period. The same type of implant was used in all cases. Fourteen patients had 2-stage bilateral TKAs.

Mean patient age was 38.2 years (range, 24-73 years). Fourteen patients were positive for human immunodeficiency virus, and 32 for hepatitis C virus. Mean follow-up was 8 years (range, 1-13 years). The prosthetic survival rate with implant removal for any reason regarded as final endpoint was 92%. Causes of TKA failure were prosthetic joint infection (6.8%) and aseptic loosening (2.2%).

Clinical outcomes of the primary TKAs in this series were good prosthetic survival and excellent pain relief. TKA infection continues to be a major concern for patients with hemophilia relative to patients without hemophilia.

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References

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Chronic hemophilic arthropathy, a well-known complication of hemophilia, develops as a long-term consequence of recurrent joint bleeds resulting in synovial hypertrophy (chronic proliferative synovitis) and joint cartilage destruction. Hemophilic arthropathy mostly affects the knees, ankles, and elbows and causes chronic joint pain and functional impairment in relatively young patients who have not received adequate primary prophylactic replacement therapy with factor concentrates from early childhood.^1-3

In the late stages of hemophilic arthropathy of the knee, total knee arthroplasty (TKA) provides dramatic joint pain relief, improves knee functional status, and reduces rebleeding into the joint.^4-8 TKA performed on a patient with hemophilia was first reported in the mid-1970s.^9,10 In these cases, the surgical procedure itself is often complicated by severe fibrosis developing in the joint soft tissues, flexion joint contracture, and poor quality of the joint bone structures. Even though TKA significantly reduces joint pain in patients with chronic hemophilic arthropathy, some authors have achieved only modest functional outcomes and experienced a high rate of complications (infection, prosthetic loosening).^11-13 Data on TKA outcomes are still scarce, and most studies have enrolled a limited number of patients.

We retrospectively evaluated the outcomes of 88 primary TKAs performed on patients with severe hemophilia at a single institution. Clinical outcomes and complications were assessed with a special focus on prosthetic survival and infection.

Patients and Methods

Ninety-one primary TKAs were performed in 77 patients with severe hemophilia A and B (factor VIII [FVIII] and factor IX plasma concentration, <1% each) between January 1, 1999, and December 31, 2011, and the medical records of all these patients were thoroughly reviewed in 2013. The cases of 3 patients who died shortly after surgery were excluded from analysis. Thus, 88 TKAs and 74 patients (74 males) were finally available for evaluation. Fourteen patients underwent bilateral TKAs but none concurrently. The patients provided written informed consent for print and electronic publication of their outcomes.

We recorded demographic data, type and severity of hemophilia, human immunodeficiency virus (HIV) status, hepatitis C virus (HCV) status, and Knee Society Scale (KSS) scores.¹⁴ KSS scores include Knee score (pain, range of motion [ROM], stability) and Function score (walking, stairs), both of which range from 0 (normal knee) to 100 (most affected knee). Prosthetic infection was classified (Segawa and colleagues¹⁵) as early or late, depending on timing of symptom onset (4 weeks after replacement surgery was the threshold used).

Patients received an intravenous bolus infusion of the deficient factor concentrate followed by continuous infusion to reach a plasma factor level of 100% just before surgery and during the first 7 postoperative days and 50% over the next 7 days (Table 1). Patients with a circulating inhibitor (3 overall) received bypassing agents FEIBA (FVIII inhibitor bypassing agent) or rFVIIa (recombinant factor VII activated) (Table 2). Patients were not given any antifibrinolytic treatment or thromboprophylaxis.

Surgery was performed in a standard surgical room. Patients were placed on the operating table in decubitus supinus position. A parapatellar medial incision was made on a bloodless surgical field (achieved with tourniquet ischemia). The prosthesis model used was always the cemented (gentamicin bone cement) NexGen (Zimmer). Patellar resurfacing was done in all cases (Figures 1A–1D). All TKAs were performed by Dr. Rodríguez-Merchán. Intravenous antibiotic prophylaxis was administered at anesthetic induction and during the first 48 hours after surgery (3 further doses). Active exercises were started on postoperative day 1. Joint load aided with 2 crutches was allowed starting on postoperative day 2.

Mean patient age was 38.2 years (range, 24-73 years). Of the 74 patients, 55 had a diagnosis of severe hemophilia A, and 19 had a diagnosis of severe hemophilia B. During the follow-up period, 23 patients died (mean time, 6.4 years; range, 4-9 years). Causes of death were acquired immune deficiency syndrome (AIDS), liver cirrhosis, and intracranial bleeding. Mean follow-up for the full series of patients was 8 years (range, 1-13 years).

Descriptive statistical analysis was performed with SPSS Windows Version 18.0. Prosthetic failure was regarded as implant removal for any reason. Student t test was used to compare continuous variables, and either χ² test or Fisher exact test was used to compare categorical variables. P < .05 (2-sided) was considered significant.

Results

Prosthetic survival rates with implant removal for any reason regarded as final endpoint was 92%. Causes of failure were prosthetic infection (6 cases, 6.8%) and loosening (2 cases, 2.2%). Of the 6 prosthetic infections, 5 were regarded as late and 1 as early. Late infections were successfully sorted by performing 2-stage revision TKA with the Constrained Condylar Knee (Zimmer). Acute infections were managed by open joint débridement and polyethylene exchange. Both cases of aseptic loosening of the TKA were successfully managed with 1-stage revision TKA using the same implant model (Figures 2A–2D).