Introduction

The most common clinical manifestation of haemophilia is arthropathy secondary to recurrent haemarthrosis and chronic synovitis. Surgical treatment of haemophilic ankle arthropathy includes synovectomy, open and arthroscopic debridement, tibiotalar arthrodesis, and total ankle replacement. Surgery is associated with a high incidence of complications in haemophiliacs. Despite the medical and surgical complexities associated with haemophilic arthropathy, orthopaedic procedures have proven effective in terms of patient satisfaction [1]. Joint-surface erosions secondary to chronic synovitis often occur in early childhood, progressing to advanced arthropathy by late adolescence. Ankle arthrodesis (AA) in patients with haemophilia is a joint-non-preserving treatment option often resulting in substantial pain relief and termination of haemarthrosis by osseous consolidating of the joint. However, there is limited availability of studies addressing ankle arthrodesis in young patients with haemophilia. Therefore, the aim of our study was to evaluate the long-term clinical and radiographic results with a minimum follow-up of ten years in young patients with haemophilia who underwent ankle arthrodesis.

Materials and methods

Inclusion and exclusion criteria

This was a retrospective study including young patients who were treated in our reference haemophilia centre by ankle arthrodesis between January 1980 and 2006. Only patients with an available follow-up ≥10 years were included in the study. We searched the orthopaedic department database for the following terms: haemophilia or other coagulation deficiency, ankle arthrodesis. We collected 22 ankle procedures performed in 17 haemophiliacs or patients with severe clotting factor deficiencies. Two patients with haemophilia died from complications of HIV infection and three were lost to follow-up. We report herein the results of 22 isolated tibiotalar arthrodesis cases involving 17 patients with severe clotting deficiencies. All except one had closed growth plates. Ankle arthrodesis was indicated in Stage IV or V (Arnold and Hilgartner classification) haemophilic arthropathy [2] cases when persistent pain with severe disability could not be alleviated by conservative treatment.

The statuses of the ankle joint were followed-up for at least ten years, ensuring long-term results. We received approval from our institutional review board, and all patients provided informed consent for participation in the study.

Surgical technique

The Meary surgical technique was used for tibiotalar arthrodesis in 16 patients. A pneumatic tourniquet was used. The surgical approach was antero-lateral, preserving the superficial fibular nerve. Chisel and gouge were used to remove remaining cartilage of the tibial and talar side to prepare the joint surfaces for ankle arthrodesis. Tibiotalar fixation was achieved using two crossed 4.5-mm screws. One patient, aged six at the time of the procedure, had no fixation, only a cast for six weeks, namely, a short-leg non-weight-bearing plaster cast, applied in the operating room. This patient’s post-operative mobilization protocol comprised non-weight bearing for six weeks followed by partial weight bearing as tolerated after bony union was visible on X-ray. All patients received clotting factors (FVIII, FIX, von Willebrand factor or by-passing agent, according to their status, i.e., haemophilia A or B with or without inhibitors or von Willebrand disease) just before, during, and approximately ten days after the procedure.

Data collection

Ankle evaluation was based on:

  • The calculation of a modified American Orthopaedic Foot & Ankle Society (AOFAS) [3] hindfoot score, adapted as in a similar study [4]. All components of the score relating to ankle motion were removed given that these were eliminated via fusion. The maximum attainable score was lowered from 100 to 78 points (Table 1). To return to a 100-point scale, the scores were calculated as a percentage of the total maximum score.

  • The Ogilvie Harris [5] score, a visual analogue scale of patient satisfaction, was evaluated for every ankle.

  • A complete current radiographic evaluation of the ankle and foot was obtained for every patient to confirm fusion and evaluate subtalar joint and hindfoot alignment. We assessed the frontal tibiotalar angle (FTTA) with the following method: A line was drawn to connect the centre of the tibial articular surface and a point in the middle of the proximal tibial shaft [6].

Table 1 Modified AOFAS hindfoot score [3, 4]
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The data collected also included any need for additional ankle surgeries or subtalar arthrodesis.

Statistical analysis

The statistical analysis was performed using Excel (Microsoft). Data was presented as means and ranges. We used Student’s t-test for paired data to detect any associations linking outcomes for uni- or bilateral arthrodesis. Values of P ≤ 0.05 were considered significant.

Results

The mean follow-up for the 22 AAs was 19.7 ± 8 years. All patients suffered from severe coagulation diseases: 12 with severe haemophilia A, five with inhibitors for six arthrodeses, three with severe haemophilia B with no inhibitors for four arthrodeses, and two with von Willebrand disease (Type 3) for three arthrodeses (Table 2). Five patients had HIV infection. For patients with bilateral arthropathy, the two arthrodesis procedures were performed in two different stages. There were no affected subtalar joints at the time of the ankle procedure.

Table 2. Characteristics of patients undergoing ankle arthrodesis for haemophiliac arthropathy
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The mean age was 15.5 years (6–23) at index surgery. There were no intraoperative or peri-operative complications related to ankle arthrodesis. There were no early or late infections. No tibiotalar non-union was seen (Fig. 1). No re-operations were required. Screw removal was required due to pain in three ankles at a mean of 18 months following the initial operation. During follow-up, no patient developed adjacent arthritis in the talonavicular joint. The mean modified AOFAS score was 83 ± 10.5. We achieved 86% good or excellent results based on the Ogilvie-Harris score (Table 3). For five ankles, the results were excellent, and the patients went on to achieve unlimited walking distance. Of the 22 ankles, 19 (86%) were reported as causing no pain. One patient reported poor outcome at follow-up due to pain originating from a subtalar joint arthritis. Twelve patients had unilateral arthrodesis, five bilateral. The AOFAS mean score was 84.7 ± 8.8 for unilateral arthrodesis and 77.8 ± 11.3 for bilateral arthrodesis. Comparing patients with unilateral and bilateral AA, we found no statistically significant difference in AOFAS score results (p = 0.08).

Table 3 Functional outcomes at last follow-up (18.2 years)
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Fig. 1
figure 1

a Pre-operative view of an ankle with Stage V haemophilic arthropathy. b Long-term X-rays, with good fusion and no signs of hind-foot arthritis

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Radiographic evaluation revealed no mal- or non-unions after ankle fusion. All ankles showed a plantigrade foot with neutral hindfoot position. All FTTA measurements fell between 85 and 95. One showed clinical and radiological signs of subtalar arthritis, with limited walking distance, and pain when moving the hindfoot (Fig. 2). Another patient had a complementary subtalar arthrodesis six years after tibiotalar arthrodesis.

Fig. 2
figure 2

Bilateral ankle fusions at 25 years with signs of sub-talar arthritis on the right side

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Discussion

We achieved 86% good-to-excellent results in the long term for ankle arthrodesis performed in young haemophiliacs. Patients with good results reported plantigrade foot with neutral hindfoot position and minimal activity limitations or gait abnormalities, and were able to walk over long distances. Our results are similar to those in the literature, such as the study by Bluth reporting 75% good results [4]. One ankle (5%) developed symptomatic subtalar arthritis six years after AA, which was treated by subtalar fusion. The surgery-related morbidity for haemophilic patients also appeared well controlled with this technique. We observed no complications even in HIV-positive patients, in line with reports in the literature stating that HIV coinfection is not a risk factor for infection [7]. We believe that this technique represents the treatment of choice for end-stage haemophilic arthropathy of the ankle in children and adolescents. No other study has reported on the surgical treatment of haemophilic ankle arthropathy in children and adolescents. Bilateral arthrodesis was not associated with a difference in functional results.

Our study has several limitations. First, it was a retrospective study, with a small cohort of patients. With only 22 ankles, it was impossible to perform a statistical analysis of our results. Moreover, we did not evaluate the AOFAS modified score pre-operatively, because using the medical records is not reliable. Thus, we were unable to assess the improvement in ankle function. Range of motion was not assessed because it was not part of the AOFAS modified score.

As the ankle is a commonly-affected joint in many individuals with haemophilia, long-term reports on the outcome of surgical treatment of end-stage haemophilic arthropathy is important in order to validate indications and the optimal timing of surgical and non-surgical interventions in ankle arthropathy [8]. Prophylactic administration of clotting factor concentrates is currently the basic approach for treating severe haemophilia A. Nevertheless, these prophylactic regimens, though very effective, do not completely prevent joint disease in the long term [9].

For patients with haemophilia-induced lesions of the foot and ankle, surgical treatment can relieve foot and ankle pain and improve their function [10]. Experiments with clotting factors prior to surgery and substitution therapy during surgery can reduce the risk of severe post-operative haemorrhage [11]. Ankle fusion is indicated only after growth plate fusion, namely, in adolescents. Prior to AA fusion, less invasive surgical options are available, the most common being open or arthoscopic synovectomy or debridement. The long-term results of open synovectomy prove its ability to improve clinical scores, even if radiological scores worsen. This technique delays the progression of arthropathy [12]. In the study by Rodriguez, arthroscopic debridement achieved 94% good-to-excellent results at the five-year follow-up. This should be considered in young haemophiliacs to delay ankle fusion or total ankle replacement, potentially offering the patient several years without intense pain [13]. The main objective for orthopaedic surgeons is to preserve a functional ankle joint as long as possible. Nevertheless, in case of advanced arthropathy and intense pain, only a definitive surgical treatment can be efficient.

Ankle arthrodesis has proven efficient to relieve pain and provide a good functional outcome in adults. It is an appropriate treatment for end-stage haemophilic arthropathy of the ankle [4, 14, 15]. Ankle arthrodesis also achieves good results if associated with subtalar arthrodesis [16]. Arthroscopic ankle arthrodesis is an effective alternative to the open technique, with established advantages in haemophilic arthropathy [17, 18].

Total-ankle replacement (TAR) is a valuable alternative treatment to ankle fusion [19–21], providing significant pain relief and high patient satisfaction. However, due to the increased risk of infection and lack of long-term results, TAR should be indicated cautiously, particularly in patients with severe haemophilia [22]. Furthermore, TAR does not prevent the occurrence of other intra-articular bleeding, and also carries the risk of aseptic joint loosening in young patients. There is some debate in the literature regarding which of the two procedures is more appropriate. A review of the literature performed by Rodriguez clarified this debate, reporting that both TAR and AA provide pain relief and patient satisfaction in haemophilic patients in the short term. There is currently insufficient non-haemophilia data, however, to conclude which treatment is superior [23]. TAR should not yet be considered an appropriate treatment for children with end-stage haemophilic arthropathy.

Conclusion

Over the long term, ankle arthrodesis achieved good functional results in young haemophiliacs, according to both the specialists’ and patient’s own assessment. It appears to represent the current treatment of choice for end-stage haemophilic arthropathy of the ankle in young patients with closed growth plate, and also ensures very limited surgery-related morbidity.