Activities of Daily Living and Quality of Life After Orthopaedic Surgery for Haemophilic Arthropathy: A Scoping Review

ABSTRACT

Introduction

Haemophilic arthropathy causes chronic pain and disability. While orthopaedic surgery aims to alleviate these, post‐surgical activities of daily living (ADL) and quality of life (QOL) outcomes lack systematic review.

Aim

To assess ADL and QOL outcomes after orthopaedic surgery for haemophilic arthropathy and identify research gaps.

Methods

This scoping review searched MEDLINE, Embase, CENTRAL, CINAHL, and PEDro for studies reporting ADL or QOL outcomes in patients undergoing lower‐limb orthopaedic surgery for haemophilic arthropathy. Data were qualitatively synthesised.

Results

Sixteen studies were included, predominantly on total knee arthroplasty (n = 11). The SF‐36 was widely used, whereas haemophilia‐specific tools were rare. Despite heterogeneous designs, varied follow‐ups, and inconsistent mental health results, most studies demonstrated significant post‐surgical improvements in physical function and QOL.

Conclusion

While standardised, disease‐specific assessments are needed, this review confirms surgery consistently improves ADL and QOL. However, it should be noted that it is well known that, in a disease as rare as haemophilia, it is very difficult to conduct Level I evidence studies on orthopaedic surgery (prospective, double‐blind, randomized trials). In fact, this study confirms what was already well known empirically: that orthopaedic surgery is effective in relieving or eliminating joint pain thereby improving the QOL for patients with advanced haemophilic arthropathy who have not responded to non‐surgical treatment.

1. Introduction

Haemophilia, an inherited blood clotting disorder, affects approximately 30,000 to 33,000 individuals in the United States, with an incidence of 1 in 5000 male births [1]. Severe haemophilia often progresses to arthropathy (haemophilic arthropathy) due to repeated joint bleeding, leading to chronic pain and functional disability [2]. It primarily affects knees, elbows, and ankles, causing synovial hypertrophy, cartilage damage, and bone destruction [3, 4]. In a study of 71 individuals with haemophilia, an average of four joints showed major pain, primarily in ankles (45%) and knees (39%), with 50% of participants experiencing daily pain without treatment [5]. Pain significantly impacts quality of life (QOL), predominantly affecting daily activities (89%) and mood (85%) [5]. The pathogenesis involves haemoglobin release and iron deposition in joints, triggering inflammatory changes in synovial tissue and cartilage [3, 6]. Management strategies include prophylactic clotting factor replacement, radiosynovectomy, and surgical interventions such as synovectomy and joint arthroplasty [4]. Radiosynovectomy is an effective, safe, minimally invasive, well‐tolerated long‐term procedure for the treatment of chronic haemophilic synovitis [7], and the WFH clinical scale revealed an improvement of around 19% [8]. Recent advancements include extended half‐life recombinant drugs and non‐replacement therapies, which have improved treatment adherence and patient QOL. A randomized controlled trial involving the use of 180‐degree immersive virtual reality video to visualize movement in patients with haemophilic ankle arthropathy showed improvements in pain and range of motion [9]. Despite these advances, chronic arthropathy remains a significant challenge, necessitating further research into underlying mechanisms and targeted treatments [3, 10].

Surgical interventions for haemophilic arthropathy include arthroscopic synovectomy, alignment osteotomy, joint debridement, radial head removal, ankle arthrodesis, and total joint arthroplasty [11]. Although surgery improves activities of daily living (ADL) and QOL, patients with haemophilia remain at increased risk of bleeding and infection [11]. Late infection and arthrofibrosis are common complications in this population following joint arthroplasty, possibly due to frequent intravenous self‐infusion and immune suppression. Despite these challenges, orthopaedic procedures yield high levels of patient satisfaction [4]. Total knee arthroplasty (TKA) for haemophilic arthropathy has been associated with overall improvement in satisfaction and functional scores six months after surgery, reduction in pain and functional limitations, improvement in knee range of motion, few complications, and no reported infections or implant failures [12]. However, reports on ADL and QOL after orthopaedic surgery for haemophilic arthropathy remain limited, and the actual situation is unclear. Haemophilia requires long‐term management rather than a single treatment, and orthopaedic surgery requires follow‐up to assess outcomes related to ADL and QOL, as well as physical and joint function.

The aim of this scoping review was to assess outcomes of ADL and QOL after orthopaedic surgery for haemophilic arthropathy, to clarify the current situation and identify future research implications.

2. Materials and Methods

This review followed the protocol described on the Open Science Framework [13], in accordance with the Preferred Reporting Items for Systematic Reviews and Meta‐analysis (PRISMA) extension for scoping reviews (PRISMA‐ScR) statement [14]. The scoping review framework by the Joanna Briggs Institute (JBI) was also used, following a five‐stage approach [15].

2.1. Stage 1: Identifying the Research Question

This review was focused on outcomes of ADL and QOL after orthopaedic surgery for haemophilic arthropathy, and the research questions are as follows:

1. What are the outcomes for ADL and QOL After orthopaedic surgery for haemophilic arthropathy?

2. When are ADL and QOL outcomes measured After orthopaedic surgery for haemophilic arthropathy?

3. How does orthopaedic surgery for haemophilic arthropathy affect ADL and QOL?

2.2. Stage 2: Identifying Relevant Studies

The Inclusion Criteria Were Defined Using the Population, Concept, and Context (PCC) Framework [15]

2.2.1. Participants

Patients with haemophilia and haemophilic arthropathy who underwent orthopaedic surgery on the lower limb joints (hip, knee, and ankle) were included. In this review, haemophilic arthropathy was defined as a condition in which repeated intra‐articular haemorrhages (haemarthrosis) in patients with haemophilia lead to joint damage, resulting in chronic inflammation and deformity. Orthopaedic surgery for haemophilic arthropathy was defined as any surgical treatment aimed at pain reduction, improvement of joint function, and prevention of joint bleeding. Procedures included synovectomy, arthroplasty (osteotomy), total joint replacement, and arthrodesis.

2.2.2. Concept

Existing literature on ADL and QOL outcomes following orthopaedic treatment for haemophilic arthropathy was reviewed. ADL and QOL were defined as measures of activity and participation based on the International Classification of Functioning model, excluding outcomes related to body functions and structures. Regarding QOL assessment, both disease‐specific and generic questionnaires were available [16]; however, in this review, evaluation tools related to ‘body functions’ [17] were excluded. Evaluation items for ADL [18] and QOL [19] derived from haemophilia or haemophilic arthropathy, rather than from surgical conditions such as TKA, were included.

All published randomised controlled trials, crossover trials, cluster‐randomised trials, quasi‐randomised trials, and non‐randomised trials were included, along with observational studies, with or without control. Studies with any duration of follow‐up were included. Case reports, case series, conference abstracts, and review articles were excluded.

2.2.3. Context

The following databases were searched: MEDLINE, Embase, Cochrane Central Register of Controlled Trials (CENTRAL), CINAHL and PEDro. In addition, ongoing trials were identified through the World Health Organization International Clinical Trials Registry Platform and ClinicalTrials.gov. References of included articles were also screened to identify relevant publications. Studies from all countries, institutions, follow‐up durations, and languages were included. Detailed search strategies for each database are provided in the Appendix.

2.3. Stage 3: Study Selection

Study selection followed the PRISMA flow diagram [20]. Using the PCC framework, two researchers carried out screening and selection, comparing their lists and resolving any discrepancies by discussion or through arbitration by a third researcher.

2.4. Stage 4: Charting the Data

Data extraction was carried out by one researcher using standardised data extraction forms that included outcomes, study type, number of participants, control, and post‐operative period. Extracted data were confirmed by other researchers. When necessary, study authors were contacted.

2.5. Stage 5: Collating, Summarising, and Reporting the Results

Search results were presented using the PRISMA flow diagram. Extracted data were organised and described as qualitative synthesis.

3. Results

3.1. Search Selection

A total of 5105 records were extracted across all databases between 11 and 13 May 2025. (MEDLINE: 12 May 2025; Embase:11 May, 2025; CENTRAL:13 May, 2025; CINAHL:12 May, 2025; and PEDro: 12 May, 2025). After removing 1412 duplicates, 3693 records remained for title and abstract screening. Reports sought for retrieval totalled 217, of which 3 could not be retrieved. Full‐text screening was conducted on the remaining 214 reports, and 198 were excluded for the following reasons: wrong outcome (not measuring ADL and QOL based on the ICF model) (n = 80), wrong study design (case reports and case series) (n = 59), wrong publication type (conference abstracts and review articles) (n = 54), and wrong population (patients with haemarthrosis not caused by haemophilia) (n = 5). Ultimately, 16 studies were deemed eligible [21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36]. The PRISMA flow diagram showing the selection process is presented in Figure 1.

FIGURE 1.

PRISMA 2020 flow diagram. A total of 5105 records were identified through database searches. After removing 1412 duplicates, 3693 records were screened. Of these, 3476 were excluded, and 216 reports were sought for retrieval. Three reports could not be retrieved, and 213 were assessed for eligibility. Finally, 16 studies were included in the review after excluding 197 reports due to wrong outcome (n = 80), study design (n = 59), publication type (n = 53), or population (n = 5). CENTRAL, Cochrane Central Register of Controlled Trials; MEDLINE, MEDical Literature Analysis and Retrieval System Online; Embase, Excerpta Medica Database; CINAHL, Cumulative Index to Nursing and Allied Health Literature; PEDro, Physiotherapy Evidence Database; PRISMA, Preferred Reporting Items for Systematic reviews and Meta‐Analyses.

3.2. Study Characteristics

Surgical interventions included TKA in 11 studies [21, 24, 26, 27, 28, 29, 30, 31, 34, 35, 36], total ankle replacement (TAR) in two studies [25, 32], synovectomy of the knee in two studies [23, 33], and ankle joint distraction in one study [22]. Retrospective cohort studies were the most common, accounting for ten studies [23, 24, 25, 26, 28, 29, 30, 32, 34, 36], followed by five prospective cohort studies [21, 22, 31, 33, 35] and one prospective randomised clinical trial [27] on postoperative drainage status following TKA. No randomised controlled trials, non‐randomised comparative studies, or longitudinal studies on the presence or absence of surgical treatment were identified. Participants’ ages ranged from 30 to 50 years; however, one knee synovectomy study [33] included a younger participant aged 16 years. Follow‐up durations varied widely, from 1 year [27] to 12 years [31] (Table 1).

TABLE 1.

Type of ADL and QOL outcome.

Surgical method	Author, year	Study type	Participants (Mean Age [range] /Number of cases per site)	Control	Pre / post‐operative period [range]	ADL, QOL outcome
TKA	Jenkins PJ, 2013 [30]	Retrospective cohort	43.2 ± 10.0 [27–60]/ 13	NA	78 months [17–116]	SF‐12 (PCS, MCS)
TKA	Cho K, 2013 [34]	Retrospective cohort	36.5 [26.0–55.0] / 22	NA	40.6 months [24.0 – 65.0)	SF‐36 (PCS, MCS)
TKA	Kim K, 2016 [29]	Retrospective cohort	38.4 [28.0–55.0] / 29	NA	5 years [3.0 – 7.0]	SF‐36 (PCS, MCS)
TKA	Zygogiannis K, 2024 [21]	Prospective cohort	50.37 ± 7.63 [40.0‑65.0] / 19	NA	12 months after surgery	SF‐36 (PCS, MCS)
TKA	Bae JK, 2020 [36]	Retrospective cohort	38.7 ± 8.3 [26.0–69.0] / 102	NA	10.2 ± 2.2 years [5.2 – 13.4]	SF‐36 (PCS, MCS)
TAR	Preis M, 2017 [25]	Retrospective cohort	51.4 ± 10.2 [32.9–63.7] / 14	NA	5.8 ± 2.3 years [2.0 –9.2]	SF‐36 (PCS, MCS)
TKA	Schick M, 1999 [24]	Retrospective cohort	44.0 [23.0–68.0] / 13	NA	4 years and 2 months [ 8 months to 8 years and 10 months]	SF‐12
TAR	Eckers F, 2017 [32]	Retrospective cohort	43.0 [27.4–57.6] / 17	NA	9.3 years [2.2–17.8]	SF‐36 (PCS, MCS)
Synovectomy of the knee	de Almeida AM, 2015 [33]	Prospective cohort	16.1 [9.6–25.0] / 9	NA	5.0 years [1.1–6.9]	SF‐36 (Overall score, PCS, MCS)
TKA	Mortazavi SMJ, 2017 [27]	Prospective randomised clinical trial	With drain: 34.1 [23.0–51.0] / 106 Without drain: 35.8 [25.0–57.0] / 108	With / Without drain	Post operation 3 weeks, 3, 6 and 12 months	SF‐36 (PCS, MCS)
TKA	Beckers G, 2024 [35]	Prospective cohort	51.0 [26.0–87.0] / 20	NA	55.3 months [6.0–128.0]	Haemo‐Qol‐A, HAL
TKA	Mortazavi SMJ, 2016 [26]	Retrospective of PWH	Unilateral TKA: 35.06 ± 9.69 / 19 Bilateral TKA: 36.50 ± 7.04 / 8	Unilateral / bilateral TKA:	Unilateral TKA: 26 months Bilateral TKA: 29 months	SF‐36 (PCS, MCS)
Synoviorthesis and synovectomies of the knee	Tussell JM, 2002	Retrospective cohort	Average 21.6, median 22 (including all patients with haemophilia)	NA	NA	SF‐36 (PCS)
TKA	Ernstbrunner L, 2017 [31]	Prospective cohort	58 (SD ± 8) / 21	NA	Mean 18 years (SD ± 8 years, minimum follow‐up: 12)	SF‐36 (PCS, MCS)
TKA	Liu Y, 2023 [28]	Retrospective cohort	38.4 ± 7.9 [21.0–63.0] / 78	NA	8.70 ± 1.97 years [4.3–15.2]	SF‐36 (PCS, MCS)
Ankle joint distraction	van Bergen EDP, 2022 [22]	Prospective cohort	33.5 ± 11.5 [21.0–53.0]/ 8	NA	34.5 ± 4.2 months [24.0–36.0]	HAL

Abbreviations: ADL, activity of daily living; HAL, Haemophilia Activities List; MCS, mental component summary; NA, not available; PCS, physical component summary; QOL, quality of life; TAR, total ankle replacement; TKA, total knee arthroplasty

3.3. ADL and QOL Outcome

Regarding ADL and QOL outcomes, the 36‐Item Short Form Health Survey (SF‐36) was the most frequently used measure, reported in twelve studies [21, 23, 25, 26, 27, 28, 29, 31, 32, 33, 34, 36], followed by the SF‐12 in two studies [24, 30], and the Haemophilia‐Specific Quality of Life Questionnaire for Adults (Haem‐A‐QoL) [35] and Haemophilia Activities List (HAL) [22] in one study each. Regarding outcome changes, seven studies showed statistically significant improvements in SF‐36 scores following TKA [21, 26, 27, 28, 29, 34, 36], while statistical analysis was not performed in one study [31]. For TAR, both studies used the SF‐36; one study showed significant improvements in both physical and mental scores [25], while the other, without statistical analysis, showed the scores as equivalent to the standard population [32]. In the two reports on knee synovectomy, improvements were observed in the SF‐36 overall and physical component scores but not in the mental component score [33], and one study noted values lower than those of the reference population [23]. For the SF‐12, physical scores significantly improved, whereas mental scores did not [30]. The other SF‐12 study did not show preoperative values [24]. Regarding Haem‐A‐QoL, scores were significantly lower (indicating improved QOL) more than 5 years after TKA compared with preoperative values [35]. The report describing HAL outcomes up to 36 months post ankle joint distraction surgery indicated improvements from 12 months postoperatively for lower extremity basic functions, and from 24 months for complex functions compared with preoperative levels [22] (Tables 1 and 2).

TABLE 2.

Changes in ADL and QOL after orthopaedic surgery.

ADL / QOL Outcome	Surgical method	Author, year	Changes in outcome
SF‐12	TKA	Jenkins PJ, 2013 [30]	PCS: 25.3 [23.1–27.6] (Pre‐operative) vs 36.7 [25.1–48.0] (Post‐operative, p = 0.017) MCS: 42.1 [37.0–45.4] (Pre‐operative) vs 40.7 [35.1–55.2] (Post‐operative, p = 0.859)
SF‐36 (PCS, MCS)	TKA	Cho K, 2013 [34]	PCS: 24.9 ± 15.2 (Preoperative) vs 71.0 ± 14.2 (24 months postoperative, p < 0.001) MCS: 43.2 ± 24.7 (Preoperative) vs 73.4 ± 20.2 (24 months postoperative, p < 0.001)
SF‐36 (PCS, MCS)	TKA	Kim K, 2016 [29]	PCS: 25.71 ± 5.94 (Preoperative) vs 59.89 ± 2.63 (Last follow up, p < 0.001) MCS: 18.09 ± 4.26 (Preoperative) vs 67.17 ± 2.41 (Last follow up, p < 0.001)
SF‐36 (PCS, MCS)	TKA	Zygogiannis K, 2024 [21]	PCS‐12: 29.2 ± 5.1 (Pre‐operative) vs 48.4 ± 5.8 (Post‐operative, p < 0.001) MCS‐12: 37.6 ± 10.8 (Pre‐operative) vs 53.3 ± 9.4 (Post‐operative, p < 0.001)
SF‐36 (PCS, MCS)	TKA	Bae JK, 2020 [36]	PCS: 25.4 ± 14.8 (Preoperative) vs 72.2 ± 14.0 (Last follow up, p < 0.001) MCS: 36.8 ± 20.5 (Preoperative) vs 72.9 ± 12.9 (Last follow up, p < 0.001)
SF‐36 (PCS, MCS)	TAR	Preis M, 2017 [25]	PCS: 38.1 ± 5.3 [29.5–44.9] (Preoperative) vs 77.7 ± 5.2 [70.6–86.0] Postoperative, p < 0.001) MCS: 51.4 ± 5.2 [40.8–57.5] (Preoperative) vs 78.9 ± 3.9 [70.0–82.8] Postoperative, p < 0.001)
SF‐12	TKA	Schick M, 1999 [24]	MCS: 51 [35–62], Physical dimension: 42[28–52]
SF‐36 (PCS, MCS)	TAR	Eckers F, 2017 [32]	PCS: 47.0 ± 13.0 [16.0–59.0] (described ‘vs standard population: 47.0’) MCS: 57.0 ± 5.0 [50.0–65.0] (described ‘vs standard population: 52.0’)
SF‐36 (Overall score, PCS, MCS)	Synovectomy of the knee	de Almeida AM, 2015 [33]	SF‐36 overall score: 94.70 [53.4–124.6] (Preoperative) vs 122.60 [88.5–135.8] (Postoperative, p = 0.021) SF‐36 PCS: 31.15 [20.4–51.8] (Preoperative) vs 44.08 [31.8–54.5] (Postoperative, p = 0.023) SF‐36 MCS: 55.04 [34.9–65.2] (Preoperative) vs 57.30 [37.2–65.3] (Postoperative, p > 0.05)
SF‐36 (PCS, MCS) Haemo‐Qol‐A, HAL	TKA	Mortazavi SMJ, 2017 [27]	With drain	PCS: 35.14 ± 8.14 (Preoperative) vs 60.82 ± 8.16 (Postoperative 12 months, p < 0.05)
			Without drain	MCS: 34.38 ± 9.83 (Preoperative) vs 68.57 ± 6.39 (Postoperative 12 months, p < 0.05)
SF‐36 (PCS, MCS)	TKA	Beckers G, 2024 [35]	Haemo‐Qol‐A: 37.8 [10.7–66.8] (Preoperative) vs 29.1 [14.6–57.6] (>5 years; Last follow‐up, p < 0.001) HAL (Total score): 55.6 [28.6–84.0] (Preoperative) vs 66.5 [51.9–85.2] (>5 years; Last follow‐up, p = 0.014)
SF‐36 (PCS)	TKA	Mortazavi SMJ, 2016 [26]	unilateral TKA PCS: 27.64 ± 7.84 (Preoperative) vs 46.65 ± 8.34 (Postoperative, p < 0.001) MCS: 35.89 ± 9.23 (Preoperative) vs 50.02 ± 9.55 (Postoperative, p < 0.001) bilateral TKA PCS: 26.48 ± 5.12 (Preoperative) vs 49.50 ± 7.44 (Postoperative, p < 0.001) MCS: 34.32 ± 14.03 (Preoperative) vs 54.47 ± 8.03 (Postoperative, p < 0.001)
SF‐36 (PCS, MCS)	Synoviorthesis and synovectomies of the knee	Tussell JM, 2002	SF‐36: significantly lower compared to the Spanish reference population
SF‐36 (PCS, MCS)	TKA	Ernstbrunner L, 2017 [31]	PCS: 32 ± 19 (12 years), MCS: 54 ± 9 (12 years)
HAL	TKA	Liu Y, 2023 [28]	PCS: 27.3 ± 13.9 (Preoperative) vs 69.13 ± 14.2 (Final follow up, p < 0.001) MCS: 31.48 ± 6.53 (Preoperative) vs 60.4 ± 7.12 (Final follow up, p < 0.001)
SF‐12	Ankle joint distraction	van Bergen EDP, 2022 [22]	HAL (lower extremity basic functions): Higher than preoperative values 12–36 months after surgery HAL (lower extremity complex functions): Higher than preoperative values 24–36 months after surgery

Abbreviations: ADL, activity of daily living; SF, short form health survey; HAL, Haemophilia Activities List; MCS, mental component summary; NA, not available; PCS, physical component summary; QOL, quality of life; TAR, total ankle replacement; TKA, total knee arthroplasty

4. Discussion

This review clarified the current evidence regarding the assessment of ADL and QOL following surgical treatment for haemophilic arthropathy. Regarding Research Question 1, while most studies showed favourable outcomes, especially in physical function, only a few involved the use of validated generic questionnaires. In response to Research Question 2, the timing of ADL and QOL assessments varied widely across studies, ranging from a few weeks to more than a decade postoperatively, with no standardised follow‐up intervals identified. For Research Question 3, although improvement in ADL and QOL was consistently observed across different surgical interventions, the available evidence remains heterogeneous in terms of surgical type, patient characteristics, and follow‐up duration. These findings highlight the need for more standardised and haemophilia‐specific evaluation protocols in future studies.

Among studies on ADL and QOL after surgical treatment, those involving the use of TKA and the SF‐36 were the most frequently reported. The SF‐36, developed as part of the Medical Outcomes Study, is a generic health status assessment scale [37] used not only in haemophilic arthropathy but also in various other contexts [38]. Its use as a QOL outcome measure in patients with haemophilia has also reached a certain level of consensus, as even individuals with mild haemophilia have been reported to show lower SF‐36 scores than healthy controls [39]. The SF‐36 is therefore frequently used both in postoperative orthopaedic settings and among patients with haemophilia. However, the SF‐36 was used postoperatively in only 11 studies in this review, with substantial differences in age and follow‐up duration. Therefore, while the SF‐36 has gained some consensus as a generic QOL measure in the context of surgical treatment for haemophilic arthropathy, the current evidence base may still be insufficient to establish definitive conclusions.

Evaluations of postoperative outcomes in haemophilic arthropathy varied widely across studies, from very short‐term assessments to decades‐long follow‐ups. Most investigations were clustered around 1–5‐year follow‐ups, ensuring patients had sufficient time for rehabilitation and stabilisation of joint function before ADL and QOL assessments. These extended timelines highlight a keen interest in not only the short‐term gains but also the durability of surgical benefits and the occurrence of late complications. At the same time, shorter‐term evaluations have also been documented. Some studies included multiple checkpoints within the first post‐operative year to trace the trajectory of recovery. For instance, a randomised trial on TKA with and without suction drains tracked outcomes up to 12 months post‐surgery, capturing early improvements by 6–12 months [27]. Similarly, a prospective study on health‐related QOL at baseline and at 1 year post‐TKA showed significant gains by the one‐year mark [26]. Overall, the timing of outcome measurement ranged from a few weeks to over 15 years postoperatively across studies. This wide range underlines the importance of evaluating both short‐term improvements and long‐term sustainability of ADL and QOL outcomes. Early follow‐ups capture the initial functional recovery and any acute complications, whereas intermediate and long‐term follow‐ups enable assessment of sustained pain relief, joint function, prosthesis survival, and patients’ ability to maintain an independent, active life.

Across the reviewed studies, orthopaedic surgery led to marked overall improvements in both ADL and QOL in patients with haemophilic arthropathy. Nearly all investigations showed significant gains in patient‐reported outcome scores from preoperative baseline to final postoperative follow‐up. In the field of orthopaedics, the SF‐36 is often used as an outcome measure postoperatively for conditions including arthropathy [40]. Improvements in SF‐36 scores have been reported in studies of total hip replacement (THR) [41], systematic reviews and meta‐analyses of primary THR and TKA, and investigations of four surgical procedures (THR, TKA, partial meniscectomy, and ACL revision) [40]. These findings suggest that successful orthopaedic intervention can restore a level of function and life quality in patients with haemophilia that is close to ‘normal’ for their age, a remarkable outcome given these patients’ severe baseline joint damage. TKA is an effective procedure for improving ROM and decreasing functional deficits resulting from haemophilic arthropathy [42]. Despite the anatomical challenges, the mechanical survival of total knee replacements in patients with haemophilia is quite good. However, the prevalence of infection after the total knee replacements was high. The prevention of late infection would substantially improve the long‐term outcome of total knee replacements in this patient population [43]. The mechanical survival of total knee replacements in haemophiliacs is very good, confirming that this is a reproducible procedure in haemophilia, even in HIV‐positive patients with a CD4 count > 200 cells/mm³ and in those with inhibitors [44]. Clinical outcomes of the primary TKAs in this series were good prosthetic survival and excellent pain relief [45]. A systematic review comparing the outcomes of arthrodesis and TAR for haemophilic ankle arthropathy revealed that both procedures significantly improved pain and function [46]. However, the QOL outcomes described in that review were based on studies also included in this review [25, 32], potentially reflecting an even greater paucity of evaluations comparing other procedures with TKA. Furthermore, synovitis and synovectomy in haemophilia are expected to offer benefits such as ‘minimally invasive intervention, early recovery, reduced postoperative bleeding, shorter hospital stay, and improved range of motion’, [47] and arthroscopic synovectomy was effective in reducing recurrent haemarthrosis and maintaining range of motion; however, joint deterioration continued to occur although probably at a slower rate [48]. Additionally, arthroscopic knee debridement can delay total knee replacement in painful moderate haemophilic arthropathy of the knee in adult patients, where Knee Society pain scores improved from 39 preoperatively to 66 postoperatively, and function scores improved from 36 to 52. Range of motion improved on an average from −15° of extension and 90° of flexion before surgery, to −5° of extension and 110° of flexion at the last follow‐up [49]. Yet no reviews focusing on ADL or QOL outcomes have been reported. For lower limb joints, synovitis and synovectomy are often performed prior to TKA or TAR and are more frequently undertaken in relatively younger patients, which may explain the lack of long‐term QOL investigations in this population.

Haemophilia‐specific tools such as the HAL and Haem‐A‐QoL [18, 19] are used for assessing ADL and QOL. However, in the postoperative context, these instruments were used in only one study each, indicating their limited application. While it is not possible to determine which tool is superior, from a surgical perspective, ADL and QOL measures tend to be used based on body function, which were excluded under our study criteria [50]. Haemophilia‐specific assessments, which are designed to focus on long‐term disease burden and bleeding control, are often not applied during postoperative follow‐up, suggesting that these two types of scales may not be integrated. This also implies a lack of ADL and QOL assessment batteries that address both surgical and haemophilia‐specific aspects. For example, the Haem‐A‐QoL can capture physical aspects of QOL that are often overlooked by generic measures [51]. Therefore, in the postoperative evaluation of haemophilic arthropathy, haemophilia‐specific ADL and QOL assessments may be preferable to generic QOL measures.

The findings of this review indicate that orthopaedic surgery can substantially improve ADL and QOL in patients with haemophilic arthropathy, even in those with advanced joint damage. Despite the review's emphasis on the clinical importance of timely surgical referral and appropriate procedure selection, supported by adequate perioperative management and rehabilitation, notable evidence gaps remain. Haemophilia‐specific instruments such as the HAL and Haem‐A‐QoL have rarely been applied postoperatively, with most studies relying on generic tools that may not fully capture the disease‐specific burden. Follow‐up timing and assessment protocols vary widely, and procedures other than TKA are underrepresented. Future studies should adopt standardised and integrated evaluation methods that address both surgical recovery and haemophilia‐specific challenges, with longer‐term follow‐ups.

This study has some limitations. ADL and QOL outcomes were defined as those specifically targeting ‘Activity’ and ‘Participation’ according to the ICF model. However, some measures categorised under ‘Body Structure and Function’ also include items or evaluation content related to ‘Activity’ and ‘Participation’. The absence of a globally standardised assessment for ADL and QOL in haemophilic arthropathy, combined with the predominance of evaluations conducted within the orthopaedic field, may explain our findings. Based on these results, there is a clear need to establish unified assessment criteria. Furthermore, none of the included articles were randomised controlled trials or other high‐level evidence studies, making it difficult to present a definitive consensus even in the context of a scoping review. By its nature, this review included studies from all countries; however, the prevalence of haemophilia and approaches to patient follow‐up vary regionally. Therefore, outcome changes in this study should be interpreted with caution.

5. Conclusion

This scoping review highlights that orthopaedic surgery, including TKA, TAR, synovectomy, and ankle distraction, can substantially improve ADL and QOL in patients with haemophilic arthropathy. While physical function outcomes generally improve postoperatively, the evidence base remains limited by heterogeneity in surgical procedures, follow‐up durations, and assessment tools. Notably, haemophilia‐specific instruments for evaluating ADL and QOL have rarely been applied in postoperative settings. Future research should be focused on developing standardised and haemophilia‐specific outcome measures, as well as conducting high‐quality prospective and comparative studies to better understand long‐term functional and QOL trajectories in this population. However, it should be noted that it is well known that, in a disease as rare as haemophilia, it is very difficult to conduct Level I evidence studies on orthopaedic surgery (prospective, double‐blind, randomized trials). In fact, this study confirms what was already well known empirically: that orthopaedic surgery is effective in relieving or eliminating joint pain and thus improving the QOL for patients with advanced haemophilic arthropathy who have not responded to non‐surgical treatment.

Funding

This work was supported by the Health and Labour Sciences Research Grants (HLSRG) from the Ministry of Health, Labour and Welfare, Japan (Grant Number 24HB2004).

Ethics Statement

Not applicable as this scoping review was conducted using data from previous studies.

Conflicts of Interest

The authors declare no conflicts of interest.

Supporting information

Supporting Information: hae70311‐sup‐0001‐Appendix.docx

Data Availability Statement

No new datasets were generated or analysed during the current study.