Total elbow arthroplasty in hemophilia: a high-risk surgical procedure

KEYWORDS:

• Hemophilia
• total elbow arthroplasty
• complications
• results
• prosthetic survival

1.

Hemophilic arthropathy of the elbow can cause significant functional limitations in hemophilic patients who have not been on primary hematologic prophylaxis since the first years of life [1–3].

Such arthropathy may become so advanced (painful and disabling) that the only possible alternative will be to perform a total elbow arthroplasty (TEA) after failure of conservative treatment (hematologic prophylaxis, analgesics, COXIBS, and Physical Medicine and Rehabilitation) and surgical resection of the radial head (joint preserving surgical procedure) [4–7].

In the general population, TEA is a surgical intervention with a high rate of complications (between 16% and 60%) (Table 1, Figure 1); a high rate of need for surgical revision, i.e. need to change the implant (between 6% and 22%); and with an implant survival at 25 years of 78%, i.e. in 22% of patients the TEA must be changed at 25 years (Table 1) [8–15]. Only publications from 2021 to 2023 have been included in Table 1 to take into account only the most modern TEA designs that are currently in use, as the initial and older designs had much lower implant survival and much higher complication and revision rates, which is why they were discontinued.

Figure 1. Complications of total elbow arthroplasty (TEA) in the general population [9].

Table 1. Main results of total elbow arthroplasty (TEA) in the general population from 2021 to 2023.

AUTHORS	YEAR	COMPLICATION RATES	REVISION RATES	IMPLANT SURVIVORSHIP RATE
Macken et al. [8]	2021	21%	NA	NA
Davey et al. [9]	2021	16.3%	14.6%	NA
Borton et al. [10]	2021	NA	20.9%	76.8% at 9.9 years
Parker et al. [11]	2021	60.7%	6.3%	NA
Siala et al. [12]	2021	56%	22%	75% at 3.7 years
Meijering et al. [13]	2022	NA	NA	82% at 10 years
Evans et al. [14]	2022	NA	NA	92% at 10-years (linked TEAs), 86% at 10 years (unlinked TEAs).
Nishida et al. [15]	2023	23%	NA	78.1% at 25 years

NA, not available.

In hemophilia, there are few data on the results of TEA. In fact, only 17 publications on the subject have been found in PubMed, some of them just case reports and others just review articles [4–7,16–28]. In a case report, Nakamura et al. described the hematological treatment employed [18]. They used continuous rFVIIa infusion during and after left TEA in a patient with a high titer of inhibitor to factor VIII. rFVIIa was given as a bolus injection (100 micrograms/kg) at the beginning of the procedure, after which a continuous infusion (10–30 µg/kg/h) was immediately started and continued for 6 days. Tranexamic acid (50 mg/kg/day, p.o.) was also given as an antifibrinolytic treatment. Effective intra- and postoperative hemostasis and normal wound healing of the surgical incisions were accomplished, except for local thrombophlebitis. Nakamura et al. considered that continuous infusion of rFVIIa was a safe and cost-effective alternative to intermittent bolus injections [18].

Table 2 summarizes the case series reported on TEA in hemophilic patients in the literature [4,20–23,25]. A recent publication by Anazor et al. found a complication rate of 42% and a revision rate of 29% in patients with hemophilia [7]. Regarding prosthetic survival in hemophilia patients, it was 87.5% at 15 years in the study by Sorbie et al. [22].

Table 2. Total elbow arthroplasty (TEA) in people with hemophilia.

AUTHORS [REFERENCE]	YEAR	TYPE OF STUDY	METHODS	RESULTS	CONCLUSION
Chapman-Sheath et al. [20]	2003	Case series	Seven TEAs in five patients with severe hemophilia A were performed. Mean follow-up: 42 months.	There was an infection. The prosthesis was revised at 2.5 years in a one-stage procedure and showed no evidence of loosening or infection 3 years later.	All the patients had excellent alleviation of pain and improvement in function.
Marshall Brooks et a.l [21]	2011	Case series	Seven TEAs in six patients.	There were no perioperative complications. One patient needed a revision at 2.5 years. At a mean of 10 years postoperatively, five of six patients continued to experience pain relief and preserved functionality. TEA led to favorable outcomes in six of seven procedures.	The findings of this study supported the viability of TEA, especially to diminish pain and preserve or reestablish functionality.
Sorbie et al. [22]	2011	Case series (the population was varied not specifically for hemophilia)	Sorbie-QUESTOR (SQ) unlinked surface arthroplasty performed by a single surgeon over 15 years at a single center.	ROM improved significantly.	Prosthetic survival was 87.5% at 15 years. The patient groups were hemophilia, rheumatoid arthritis, and ‘other,’ which included osteoarthritis, traumatic arthritis, psoriatic arthritis, and reactive arthritis.
Wang et al. [23]	2012	Case series	6 TEAs in 3 patients	According to the MEPS*, outcomes were good to excellent in 83% of patients.	Despite high complication rates TEA was valuable for accomplishing pain alleviation. Patients reported that risks were outweighed by the benefits.
Vochteloo et al. [25]	2015	Case series	Five patients experienced 8 primary TEAs.	Revision TEA was carried out in 3 TEAs. Two revisions were due to aseptic loosening. The third elbow experienced an excision arthroplasty for a deep infection. The mean follow-up for the primary TEAs still in situ was 9.5 years.	Excellent clinical results and an acceptable survival percentage for TEAs, comparable with the nonhemorrhagic population, can be accomplished in patients with bleeding disorders. Revision TEA in this group of patients yielded good clinical results at medium-run follow-up.
Ernstbrunner et al. [4]	2018	Case series	13 primary TEAs (Coonrad-Morrey design) were performed in 9 patients with an average age of 55 years.	After a mean of 9.1 years, the mean VAS score for pain, total MEPS*, and subjective elbow value were substantially improved. There were 8 complications (62%).	TEA was associated with a significant complication and revision rate. However, even after revision without implant removal, it yielded good functional and subjective long-run outcomes.

ROM, range of motion; MEPS, Mayo Elbow Performance Score; VAS, visual analog scale.

*The MEPS measures elbow function across four domains: pain (45 points), stability (10 points), ROM (20 points), and daily functional tasks (25 points). Scores are categorized as 90–100 = excellent, 75–89 = good, 60–74 = fair, 0–59 = poor.

All the previously mentioned data seem to indicate that TEA in hemophilia has quite poor outcomes, worse than in the general population. Therefore, before indicating a TEA in a hemophilic patient, the advantages and potential risks of the intervention should be carefully weighed, as these patients are usually young at the time of surgical indication.

With this editorial, my aim is to draw attention to the important risks that TEA has in people with hemophilia. It is essential to remember that the fundamental thing is to prevent the problem by avoiding elbow arthropathy through primary hematologic prophylaxis since childhood [1–3]. It should also be remembered how important is an adequate perioperative hemostasis controlled by hematologists with expertise in hemophilia when we find ourselves in the need to perform TEA in a hemophilic patient [29,30].

In summary, TEA in hemophilia is associated with a high risk of complications, which makes its revision rates very high. In addition, prosthetic survival is not too high. Besides, it is important to emphasize that TEA in young individuals (hemophiliacs or non hemophiliacs) makes the upper limb permanently limited in weight lifting for all life because of the great tendency to components loosening, especially in the humeral side. All this should be known by patients and hemophilia expert hematologists, as they are ultimately also part of the group involved in decision-making together with expert orthopedic surgeons in TEA. Therefore, it is important to make clear the high rate of complications with this type of surgery.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

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Peer reviewers on this manuscript have no relevant financial relationships or otherwise to disclose.

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Funding

This paper was not funded.