Ankle fusion in patients with haemophilia.

Ankle fusion in patients with haemophilia is a well-accepted treatment for end-stage arthropathy. However, current published outcome data are based on small sample sizes and generally short-term follow-up. The aim of this study was to evaluate the long-term results of ankle fusion in a large group of haemophilic patients treated at a single institution. The results of 57 ankle fusions performed on 45 patients between 1971 and 2010 were reviewed retrospectively. Data were gathered for type and severity of haemophilia, HIV status, fixation technique, postoperative complications and requirement of additional surgeries. A modified American Orthopaedic Foot & Ankle Society (AOFAS) hindfoot score was calculated for 20 ankles available for follow-up. Patients were followed for a mean of 6.6 years. There were no intra-operative or immediate postoperative complications related to fusion of the ankle. The overall non-union rate was 10.4% for tibio-talar fusion and 8.3% for sub-talar fusion. This rate was reduced to 3.7% and 5.6%, respectively, after the introduction of newer surgical techniques in 1995. None of these non-unions required revision surgery. The modified AOFAS scale demonstrated that 75% had no pain in the operated ankle a mean of 7.2 years following surgery. The remaining 25% scored their average pain as 3 of 10. The functional portion of the score suggested that patients have good alignment, minimal activity limitations or gait abnormalities, and can walk long distances. We conclude that ankle fusion successfully relieves pain and provides a good functional outcome. It is an appropriate treatment for end-stage haemophilic arthropathy of the ankle.

Inter- and intra-observer reliability of radiographic scores commonly used for the evaluation of haemophilic arthropathy.

Although the severity of haemophilic arthropathy is commonly assessed using established radiographic scoring systems, there is limited available information about their inter- and intra-observer reliability. The purpose of the present study was to establish the inter-observer reliability (IEOR) and intra-observer reliability (IAOR) of three different methods available for the classification of haemophilic arthropathy, including the Arnold and Hilgartner classification, a modification to the Arnold and Hilgartner system described by Luck et al., and the classification described by Pettersson et al. Antero-posterior and lateral radiographs of 54 haemophilic joints were included for the analysis. To determine the IEOR for each one of the three radiographic systems, the radiographs were randomly evaluated by four observers, including two orthopaedic surgeons, one orthopaedic resident and one haematologist. For the determination of IAOR, all four reviewers repeated the assessment in a similar fashion, after a period of at least 2 weeks. IEOR and IAOR for the three classification systems was established using kappa (kappa) statistics. A Spearman rank correlation was used to determine the similarities between each reviewer's own interpretative scales. The IEOR was low for the Arnold and Hilgartner system (kappa = 0.35, P < or = 0.001) and the Luck system (kappa = 0.38, P < or = 0.001), but even lower for the Pettersson system (kappa = 0.06, P = 0.1). For the Pettersson system, particularly low kappa values were observed for the presence or absence of osteoporosis (kappa = 0.11, P = 0.0027), enlarged epiphysis (kappa = 0.10, P = 0.0039), erosion of joint margins (kappa = 0.11, P = 0.0018), and joint deformity (kappa = 0.16, P = 0.00001). However, a relatively high Spearman rank correlation for all three scales [r(s) = 0.75 (P < 0.001) for Arnold and Hilgartner system, r(s) = 0.74 (P < 0.001) for the Luck system and r(s) = 0.81 (P < 0.001) for Pettersson system] indicated an overall, general agreement among the reviewers with regard to the severity of the haemophilic arthropathy. There was a moderate IAOR value for both, the Arnold and Hilgartner system (kappa = 0.57, P = 0.00001) and the Luck system (kappa = 0.62, P = 0.00001) with a low IAOR value for the Pettersson system [kappa = 0.22, P = 0.00001). Currently available radiographic scoring systems for haemophilic arthropathy have low inter- and intra-observer reliability rates. Improvements, either through education or modification of the scoring systems, are critical in an era where correlations between clinical and radiographic scores have received significant attention.