Total knee arthroplasty in patients with haemophilic arthropathy is effective and safe according to the outcomes at a mid-term follow-up

TKA is known as the most suitable orthopaedic treatment for end-stage haemophilic arthropathy. Due to the special features of haemophilia, such as a bleeding tendency, prolonged bony deformity, soft tissue contracture, and muscle atrophies [16], the surgical procedure is more difficult than TKA in knee osteoarthritis. However, due to an improvement in haematological management and a deeper understanding of the pathophysiology of haemophilic arthropathy, it is easier to perform TKA in patients with haemophilic arthropathy.

Range of motion and satisfaction

When compared with non-haemophilia patients, patients with haemophilic arthropathy of the knee tend to have a poorer postoperative functional outcome. In a study of 20 patients with haemophilia, the mean ROM at last follow-up was 92° and the mean preoperative flexion contracture was 3° [17]. In a cohort of 116 haemophilic procedures at a single institution, a 5° (5° to 0°) improvement in flexion contracture and a 15° (75° to 90°) improvement in ROM were reported [18]. In our cohort, the average ROM of the knee joint at the last follow-up was 70.3° and the average flexion contracture was 14.3°. Both the clinical score and the functional score of the KSS significantly improved from 33.5 preoperatively to 62.7 at the last follow-up (P < 0.001) and from 41.6 preoperatively to 62.9 at the last follow-up (P < 0.001), respectively. Compared with the above literature, our improvements in maximum postoperative ROM and flexion contracture were not significant. Similar to our study, there was no significant difference between the pre- and postoperative ROM in a study by Westberg et al., with a total ROM of 70° and 79°, respectively [8]. Indeed, Ernstbrunner et al. reported that the total flexion in haemophiliac patients slight decreased postoperatively (from 89° to 87°) [19]. We believe that patient compliance and severe soft-tissue contracture are the two main reasons for the poor postoperative ROM in our cohort. Although our cases showed a 7° improvement in flexion contracture postoperatively, it was still relatively high, which hindered the postoperative rehabilitation process and led to a poor ROM. Also, low patient compliance with postoperative rehabilitation resulted in a poor ROM and high flexion contracture. In this series, the average postoperative VAS score was 4.9, which is connected to irregular prophylaxis with CFs. The average Knee Society Score was also less than in the previous literature [20,21,22]. These results might be associated with the abovementioned restricted ROM, VAS score, and flexion contracture postoperatively.

Surprisingly, 100% of our patients were satisfied with the procedure. In a systematic review, Naoki Nakano et al. [23] concluded that a negative history of mental health problems, severe preoperative radiological degenerative change, no/less postoperative pain, good postoperative physical function, and preoperative expectations being met contributed to better patient satisfaction following a TKA. A study by Noble et al. showed that patients’ expectations were highly correlated with their satisfaction after total knee replacement [24]. With their serious functional impairment and extreme pain preoperatively, the daily needs of our patients were unfulfilled before TKA. For activities of daily living, 65–70° of knee flexion are necessary for the swing phase of normal gait, whereas at least 90° are needed to descend stairs and 105° to rise from a low chair [25]. In our cases, the VAS score decreased from 6.8 to 4.9 and maximum ROM increased from 53.9° to 70.3°. The low expectations of our patients, their strong relief from pain, and their fulfillment of daily activities were considered to be important factors leading to the excellent patient satisfaction at our centre. Besides, our centre had a matching policy to reduce the hospital fees for haemophilia.

Main complications

Interestingly, our study showed that the incidence of complications was 15.6%, which is lower than reported in the literature [26, 27]. We think that this difference is associated with our own unique insights into surgical techniques, infection management, and rehabilitation. Infection, prosthesis loosening, periprosthetic fractures, prosthesis removal, bleeding, neurovascular injury, and inhibitor development are some common problems that occur after TKA in haemophilia patients. We now discuss the main complications that arose in our study.

Knee extensor tendon injury

It was reported that the incidence of knee extensor tendon injury was approximately 1–12% after TKA. Because of the marked adhesions, arthrofibrosis, and decreased ROM in haemophilia patients, patellar subluxation is restricted, making exposure difficult. We usually used a modified quadriceps snip technique that we call Z-shaped quadricepsplasty to resolve knee intra-articular adhesions. Firstly, two small vertical incisions were made on both sides of the rectus femoris muscle. Then, the rectus femoris was divided into two parts after the Z-myotomy. Finally, the two parts of the cut muscle were sutured together. There was a suspected case of partial quadriceps tendon rupture among our follow-up cases. Here are some possible reasons. On the one hand, an incision of the rectus femoris was performed, which could destroy the blood supply to the tendon and easily cause damage to the quadriceps tendon. On the other hand, long-term muscle atrophy, recurrent bleeding, and unfavorable muscle conditions are potential risk factors. Besides, the premature time and the improper intensity of postoperative rehabilitation were harmful to knee extensor tendon.

Prosthesis aseptic loosening

Prosthesis aseptic loosening is a key complication that is worthy of attention, especially in people with haemophilia. The incidence of aseptic loosening after the first TKA is 1–23% for ordinary people without haemophilia [28, 29]. The young age, poor bone quality, microhaemorrhages, and prevalence of osteoporosis [30] in patients with haemophilia are strongly associated with prosthesis loosening. Song et al. [11] reported that there was no wear or loosening after TKA in a study with a mean follow-up of 6.8 years. In our study, a patient's intraoperative and postoperative imaging examinations showed possible prosthesis loosening. Daily low-intensity exercise, anti-osteoporosis drugs, and CF replacement therapy were recommended for patients. When reasonable preventive measures were applied, there was no occurrence of early prosthesis loosening until the last follow-up in this patient.

Infection

Furthermore, infection, an unavoidable topic, was historically considered a catastrophic barrier in haemophilia patients [31]. It was recognized that haemophilia was accompanied by multiple blood-borne viral infections, such as HIV and hepatitis virus, which affects patient immunity and raises the risk of infection [32, 33]. However, some recent studies [34, 35] of TKA in patients with HA have shown that the postoperative infection rate is very low. Our results are consistent with theirs, which indicates that infection is no longer a major obstacle to elaborative and comprehensive management.

Deep vein thrombosis

Deep venous thrombosis (DVT) is one of the most common complications after artificial joint replacement. The use of a tourniquet, immobilization, long-term bed rest, endothelial vascular injury, trauma, and hypercoagulability [36, 37] are associated with DVT. Buckner et al. [38] reported that the incidence of symptomatic venous thromboembolism (VTE; 4.3%) was similar to its estimated incidence in the general population without thromboprophylaxis. A study [39] in Japan did not detect any DVT. Similiarly, there was no DVT in our patients who had undergone TKA. Opinions in the literature on the need for VTE prophylaxis in patients with haemophilia differ [40, 41]. Due to their lack of congenital CFs, haemophilia patients have a low risk of DVT. Even though haemophilia patients were given CFs during surgery, they did not reach a hypercoagulable state. We believe that there is no need for routine pharmacologic prophylaxis after the operation, but mechanical prophylaxis should be employed, such as graduated compression stockings, intermittent pneumatic compression devices, and venous foot pumps.

Undoubtedly, there are some limitations to our study. The principal limitation is the retrospective nature of the work, with no control group. Additionally, the number of patients is relatively small. A large-sample-size study should be performed. Also, this is a single-centre study, so its results may not apply to all centres. However, the follow-up in our study is relatively long compared to those of other studies reported in the literature, and it adds to the available knowledge on the results of TKA in haemophilia patients.

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