LETTERS TO THE EDITORS
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Total ankle arthroplasty in patients with inherited bleeding disorders _ I K O W S K I , * P . A M B R O Z I A K , * M . K U C H A R Z E W S K I ‡ and A . K O T E L A , * † J . L O R K O W S K I , * P . ZB I. KOTELA*§ *Department of Orthopaedic Surgery and Traumatology, Central Clinical Hospital of the Ministry of Interior,, Warsaw; †Department of Orthopaedics and Traumatology of Musculoskeletal System, 1st Faculty of Medicine, Medical University of Warsaw, Warsaw; ‡Department of Descriptive and Topografic Anatomy, Medical University of Silesia, Zabrze; and §Department of Physiotheraphy, Kochanowski University of Humanities and Scienes, Kielce, Poland
Inherited bleeding disorders include a group of rarely occurring coagulation disturbances caused by dysfunction of the clotting cascade factors, and are characterized by spontaneous bleeding. More than 90% of bleeding episodes in haemophilic patients involve the musculoskeletal system, and 80% of these occur within joints [1]. Almost 80% of all articular haemorrhages affect the knees, elbows and ankle joints [1,2]. As a result of recurrent bleedings, irreversible joint destruction and clinically symptomatic arthropathy may be present even at the age of 20–30. The treatment of end-stage ankle arthropathy remains a controversial problem. Two surgical approaches have been described in the recent literature: total ankle replacement (TAR) and ankle arthrodesis. Although fusion of the ankle is still perceived to be the standard treatment option, TAR continues to evolve, with increasingly better outcomes and survival rates of approx. About 80% at 10 years [3]. According to Barg et al. [4], TAR is a valuable alternative to ankle fusion for patients with haemophilic osteoarthritis. In this paper, we report on series of three patients scheduled to undergo TAR due to severe haemophilic arthropathy of the ankle joint. A 28-year-old male with a moderately severe form of haemophilia B treated for recurrent haemorrhages into the joint and haemophilic arthropathy. Conservative treatment, rehabilitation and intraarticular administration of hyaluronic acid did not reduce the symptoms. Because of intraarticular haemorrhages, he had a radiosynovectomy with 186Re, resulting in substantial reduction in intraarticular bleeding, however, without pain relief. The ankle joint pain was mainly due to large anterior osteophytes. First, ankle joint arthroscopy, synovectomy and removal of anterior osteophytes have been performed. However, this only brought about a temporary improvement. Despite the young age, the patient was qualified for TAR. Preoperative data are Correspondence: Andrzej Kotela, Department of Orthopaedic Surgery and Traumatology, Central Clinical Hospital of the Ministry of Interior, Warsaw, Poland. Tel.: +48 22 508 13 70; fax: +48 22 508 13 81; e-mail: [email protected] Accepted after revision 21 February 2015 © 2015 John Wiley & Sons Ltd
shown in Table 1. The postoperative period and healing of the surgical wound were uneventful. A 38-year-old male with a moderately severe form of haemophilia A treated for pain and effusion in the right ankle joint following sprain injuries. Radiographs showed haemophilic arthropathy. Previous conservative treatment failed. The patient was qualified for TAR and operated on following appropriate haematological premedication (supplementation of factor VIII). His postoperative course was uneventful. A 47-year-old female with von Willebrand disease type 3 treated for recurrent haemorrhages into the ankle joint with increasing arthropathy of the joint (Fig. 1). In view of the lack of response to conservative treatment, she was qualified for TAR. Postoperatively, in all three patients a significant pain relief and functional improvement were observed (Table 1). Conventional radiographs showed correct position of prosthesis components without any signs of loosening. In all three patients we used the uncemented threecomponent prosthesis (Mobility, DePuy International, Leeds, UK) through the standard anterior approach. A thigh tourniquet was used until finishing the prosthesis implantation. Suction drainage was applied postoperatively for 48 h and the patients were immobilized in a lower leg cast. Rehabilitation was started following
Table 1. Clinical assessment: pre- and postoperative results. Before operation Case 1 AOFAS Dorsiflexion Plantarflexion VAS-pain Case 2 AOFAS Dorsiflexion Plantarflexion VAS-pain Case 3 AOFAS Dorsiflexion Plantarflexion VAS-pain
After 6 months
After 12 months
60 10° 20° 70
87 20° 40° 19
91 20° 40° 12
38 15° 30° 81
88 15° 45° 22
90 20° 45° 18
52 15° 30° 60
80 10° 30° 6
84 10° 30° 8
AOFAS, American Orthopaedic Foot and Ankle Society’s scalce; VAS, Visual Analogue Scale.
Haemophilia (2015), 21, e223--e259
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(a)
(b)
Fig. 1. Total ankle arthroplasty in patient with von Willebrand disease: preoperative (a) and postoperative (b) view.
2 weeks of immobilization of the limb and full weight bearing was allowed after 8 weeks. Each of our patients was closely monitored by a haematologist in the perioperative period and clotting factor supplementation was carried out according to an algorithm proposed by the haematologist. Reports of TAR procedures in patients with congenital blood disorders are extremely rare. However, despite numerous controversies, the results of available studies are promising. Van der Heide et al. [5]. analysed the results of five TARs performed in three patients. After a median follow-up of 5.6 years (range = 1–8.7), they found good to excellent clinical results and no signs of loosening for all cases. Barg et al. [4]. reported on eight patients with 10 TARs followed up for an average of 5.6 years (range = 2.7–7.6). In this study, the mean American Orthopaedic Foot and Ankle Society’s scalce (AOFAS) score increased significantly from 38 preoperatively to 81 postoperatively, the pain level decreased from 7.1 preoperatively to 0.8 postoperatively, and all domains of the SF-36 score showed significant improvements in quality of life. Radossi et al. [6] described two cases of TAR with painless ankle movement, improved ability to walk and no signs of prosthetic loosening or infection after 4 years (case 1) and 7 years (case 2) of follow-up.
Haemophilia (2015), 21, e223--e259
Our findings are comparable to those made in other studies and, in our opinion, promising (the results of preoperative and follow-up examinations are presented in Table 1). The mean AOFAS score in our patients increased from 50 preoperatively to 88 postoperatively, and the pain level decreased from 7.0 preoperatively to 1.2 postoperatively. The mean range of motion improved and no intra- or perioperative complications were observed. All patients were fully satisfied with the surgical outcomes and stated that they would have the surgery performed again. On the other hand, we do acknowledge the general concerns from studies reporting higher rates of aseptic loosening and deep infections after hip and knee replacement in this group of patients [4]. According to Barg et al. [4], TAR is a valuable alternative to ankle fusion for patients with haemophilic osteoarthritis. In our opinion, TAR is a more physiological solution as it preserves the ankle motion and results in less gait impairments than ankle arthrodesis. Supporting these conclusions is indirect evidence from gait mechanics studies [7–9] which describe optimization of the gait pattern following TAR. The development of ankle arthroplasty together with improved understanding of the kinematics, criteria for selecting patients and surgical techniques may thus have led to increased interest in ankle arthroplasty [10]. However, a prerequisite for achieving such outcomes is a carefully conducted qualification process, properly performed procedure, appropriate haematologic monitoring and subsequent appropriate rehabilitation. In our opinion, TAR is a valuable alternative to ankle fusion for patients with inherited bleeding disorders and advanced ankle destruction. Good outcomes of ankle arthroplasty rely on careful patient selection, appropriate haematologic monitoring and surgical experience. Further long-term clinical studies are required to clarify the role of TAR as treatment option in patients with end-stage haemophilic ankle osteoarthritis.
Acknowledgements Andrzej Kotela, performed the research, analysed the data, wrote the manuscript; Jacek Lorkowski, performed the research, analysed the data, wrote _ the manuscript; Piotr Zbikowski, performed the research, analysed the data; Paweł Ambroziak, performed the research, analysed the data; Marek Kucharzewski, analysed the data, viewed the manuscript; Ireneusz Kotela, performed the surgery, designed the study, reviewed the manuscript.
Disclosures The authors stated that they had no interests which might be perceived as posing a conflict or bias.
© 2015 John Wiley & Sons Ltd
LETTERS TO THE EDITORS
References 1 Rodriguez-Merchan EC. Aspects of current management: orthopaedic surgery in haemophilia. Haemophilia 2012; 18: 8– 16. 2 Kelly D, Zhang CQ, Soucie MJ, MancoJohnson M, Dimichele D. Prevalence of clinical hip abnormalities in haemophilia A and B: an analysis of the UDC database. Haemophilia 2013; 19: 426–31. 3 Gougoulias N, Khanna A, Maffulli N. How successful are current ankle replacements? A systematic review of the literature. Clin Orthop Relat Res 2010; 468: 199–208.
© 2015 John Wiley & Sons Ltd
4 Barg A, Elsner A, Hefti D, Hintermann B. Haemophilic arthropathy of the ankle treated by total ankle replacement: a case series. Haemophilia 2010; 16: 647–55. 5 van der Heide HJ, Novakova I, de Waal Malefijt MC. The feasibility of total ankle prosthesis for severe arthropathy in haemophilia and prothrombin deficiency. Haemophilia 2006; 12: 679–82. 6 Radossi P, Bisson R, Munari F et al. Total ankle replacement for end-stage arthropathy in patients with haemophilia. Haemophilia 2008; 14: 658–60. 7 Brodsky JW, Polo FE, Coleman SC, Bruck N. Changes in gait following the Scandina-
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vian Total Ankle Replacement. J Bone Joint Surg Am 2011; 93: 1890–6. 8 Ingrosso S, Benedetti MG, Leardini A, Casanelli S, Sforza T, Giannini S. GAIT analysis in patients operated with a novel total ankle prosthesis. Gait Posture 2009; 30: 132–7. 9 Valderrabano V, Nigg BM, von Tscharner V, Stefanyshyn DJ, Goepfert B, Hintermann B. Gait analysis in ankle osteoarthritis and total ankle replacement. Clin Biomech (Bristol, Avon), 2007; 22: 894–904. 10 Steck JK, Anderson JB. Total ankle arthroplasty: indications and avoiding complications. Clin Podiatr Med Surg 2009; 26: 303–24.
Haemophilia (2015), 21, e223--e259
Copyright of Haemophilia is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
e257
Total ankle arthroplasty in patients with inherited bleeding disorders _ I K O W S K I , * P . A M B R O Z I A K , * M . K U C H A R Z E W S K I ‡ and A . K O T E L A , * † J . L O R K O W S K I , * P . ZB I. KOTELA*§ *Department of Orthopaedic Surgery and Traumatology, Central Clinical Hospital of the Ministry of Interior,, Warsaw; †Department of Orthopaedics and Traumatology of Musculoskeletal System, 1st Faculty of Medicine, Medical University of Warsaw, Warsaw; ‡Department of Descriptive and Topografic Anatomy, Medical University of Silesia, Zabrze; and §Department of Physiotheraphy, Kochanowski University of Humanities and Scienes, Kielce, Poland
Inherited bleeding disorders include a group of rarely occurring coagulation disturbances caused by dysfunction of the clotting cascade factors, and are characterized by spontaneous bleeding. More than 90% of bleeding episodes in haemophilic patients involve the musculoskeletal system, and 80% of these occur within joints [1]. Almost 80% of all articular haemorrhages affect the knees, elbows and ankle joints [1,2]. As a result of recurrent bleedings, irreversible joint destruction and clinically symptomatic arthropathy may be present even at the age of 20–30. The treatment of end-stage ankle arthropathy remains a controversial problem. Two surgical approaches have been described in the recent literature: total ankle replacement (TAR) and ankle arthrodesis. Although fusion of the ankle is still perceived to be the standard treatment option, TAR continues to evolve, with increasingly better outcomes and survival rates of approx. About 80% at 10 years [3]. According to Barg et al. [4], TAR is a valuable alternative to ankle fusion for patients with haemophilic osteoarthritis. In this paper, we report on series of three patients scheduled to undergo TAR due to severe haemophilic arthropathy of the ankle joint. A 28-year-old male with a moderately severe form of haemophilia B treated for recurrent haemorrhages into the joint and haemophilic arthropathy. Conservative treatment, rehabilitation and intraarticular administration of hyaluronic acid did not reduce the symptoms. Because of intraarticular haemorrhages, he had a radiosynovectomy with 186Re, resulting in substantial reduction in intraarticular bleeding, however, without pain relief. The ankle joint pain was mainly due to large anterior osteophytes. First, ankle joint arthroscopy, synovectomy and removal of anterior osteophytes have been performed. However, this only brought about a temporary improvement. Despite the young age, the patient was qualified for TAR. Preoperative data are Correspondence: Andrzej Kotela, Department of Orthopaedic Surgery and Traumatology, Central Clinical Hospital of the Ministry of Interior, Warsaw, Poland. Tel.: +48 22 508 13 70; fax: +48 22 508 13 81; e-mail: [email protected] Accepted after revision 21 February 2015 © 2015 John Wiley & Sons Ltd
shown in Table 1. The postoperative period and healing of the surgical wound were uneventful. A 38-year-old male with a moderately severe form of haemophilia A treated for pain and effusion in the right ankle joint following sprain injuries. Radiographs showed haemophilic arthropathy. Previous conservative treatment failed. The patient was qualified for TAR and operated on following appropriate haematological premedication (supplementation of factor VIII). His postoperative course was uneventful. A 47-year-old female with von Willebrand disease type 3 treated for recurrent haemorrhages into the ankle joint with increasing arthropathy of the joint (Fig. 1). In view of the lack of response to conservative treatment, she was qualified for TAR. Postoperatively, in all three patients a significant pain relief and functional improvement were observed (Table 1). Conventional radiographs showed correct position of prosthesis components without any signs of loosening. In all three patients we used the uncemented threecomponent prosthesis (Mobility, DePuy International, Leeds, UK) through the standard anterior approach. A thigh tourniquet was used until finishing the prosthesis implantation. Suction drainage was applied postoperatively for 48 h and the patients were immobilized in a lower leg cast. Rehabilitation was started following
Table 1. Clinical assessment: pre- and postoperative results. Before operation Case 1 AOFAS Dorsiflexion Plantarflexion VAS-pain Case 2 AOFAS Dorsiflexion Plantarflexion VAS-pain Case 3 AOFAS Dorsiflexion Plantarflexion VAS-pain
After 6 months
After 12 months
60 10° 20° 70
87 20° 40° 19
91 20° 40° 12
38 15° 30° 81
88 15° 45° 22
90 20° 45° 18
52 15° 30° 60
80 10° 30° 6
84 10° 30° 8
AOFAS, American Orthopaedic Foot and Ankle Society’s scalce; VAS, Visual Analogue Scale.
Haemophilia (2015), 21, e223--e259
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LETTERS TO THE EDITORS
(a)
(b)
Fig. 1. Total ankle arthroplasty in patient with von Willebrand disease: preoperative (a) and postoperative (b) view.
2 weeks of immobilization of the limb and full weight bearing was allowed after 8 weeks. Each of our patients was closely monitored by a haematologist in the perioperative period and clotting factor supplementation was carried out according to an algorithm proposed by the haematologist. Reports of TAR procedures in patients with congenital blood disorders are extremely rare. However, despite numerous controversies, the results of available studies are promising. Van der Heide et al. [5]. analysed the results of five TARs performed in three patients. After a median follow-up of 5.6 years (range = 1–8.7), they found good to excellent clinical results and no signs of loosening for all cases. Barg et al. [4]. reported on eight patients with 10 TARs followed up for an average of 5.6 years (range = 2.7–7.6). In this study, the mean American Orthopaedic Foot and Ankle Society’s scalce (AOFAS) score increased significantly from 38 preoperatively to 81 postoperatively, the pain level decreased from 7.1 preoperatively to 0.8 postoperatively, and all domains of the SF-36 score showed significant improvements in quality of life. Radossi et al. [6] described two cases of TAR with painless ankle movement, improved ability to walk and no signs of prosthetic loosening or infection after 4 years (case 1) and 7 years (case 2) of follow-up.
Haemophilia (2015), 21, e223--e259
Our findings are comparable to those made in other studies and, in our opinion, promising (the results of preoperative and follow-up examinations are presented in Table 1). The mean AOFAS score in our patients increased from 50 preoperatively to 88 postoperatively, and the pain level decreased from 7.0 preoperatively to 1.2 postoperatively. The mean range of motion improved and no intra- or perioperative complications were observed. All patients were fully satisfied with the surgical outcomes and stated that they would have the surgery performed again. On the other hand, we do acknowledge the general concerns from studies reporting higher rates of aseptic loosening and deep infections after hip and knee replacement in this group of patients [4]. According to Barg et al. [4], TAR is a valuable alternative to ankle fusion for patients with haemophilic osteoarthritis. In our opinion, TAR is a more physiological solution as it preserves the ankle motion and results in less gait impairments than ankle arthrodesis. Supporting these conclusions is indirect evidence from gait mechanics studies [7–9] which describe optimization of the gait pattern following TAR. The development of ankle arthroplasty together with improved understanding of the kinematics, criteria for selecting patients and surgical techniques may thus have led to increased interest in ankle arthroplasty [10]. However, a prerequisite for achieving such outcomes is a carefully conducted qualification process, properly performed procedure, appropriate haematologic monitoring and subsequent appropriate rehabilitation. In our opinion, TAR is a valuable alternative to ankle fusion for patients with inherited bleeding disorders and advanced ankle destruction. Good outcomes of ankle arthroplasty rely on careful patient selection, appropriate haematologic monitoring and surgical experience. Further long-term clinical studies are required to clarify the role of TAR as treatment option in patients with end-stage haemophilic ankle osteoarthritis.
Acknowledgements Andrzej Kotela, performed the research, analysed the data, wrote the manuscript; Jacek Lorkowski, performed the research, analysed the data, wrote _ the manuscript; Piotr Zbikowski, performed the research, analysed the data; Paweł Ambroziak, performed the research, analysed the data; Marek Kucharzewski, analysed the data, viewed the manuscript; Ireneusz Kotela, performed the surgery, designed the study, reviewed the manuscript.
Disclosures The authors stated that they had no interests which might be perceived as posing a conflict or bias.
© 2015 John Wiley & Sons Ltd
LETTERS TO THE EDITORS
References 1 Rodriguez-Merchan EC. Aspects of current management: orthopaedic surgery in haemophilia. Haemophilia 2012; 18: 8– 16. 2 Kelly D, Zhang CQ, Soucie MJ, MancoJohnson M, Dimichele D. Prevalence of clinical hip abnormalities in haemophilia A and B: an analysis of the UDC database. Haemophilia 2013; 19: 426–31. 3 Gougoulias N, Khanna A, Maffulli N. How successful are current ankle replacements? A systematic review of the literature. Clin Orthop Relat Res 2010; 468: 199–208.
© 2015 John Wiley & Sons Ltd
4 Barg A, Elsner A, Hefti D, Hintermann B. Haemophilic arthropathy of the ankle treated by total ankle replacement: a case series. Haemophilia 2010; 16: 647–55. 5 van der Heide HJ, Novakova I, de Waal Malefijt MC. The feasibility of total ankle prosthesis for severe arthropathy in haemophilia and prothrombin deficiency. Haemophilia 2006; 12: 679–82. 6 Radossi P, Bisson R, Munari F et al. Total ankle replacement for end-stage arthropathy in patients with haemophilia. Haemophilia 2008; 14: 658–60. 7 Brodsky JW, Polo FE, Coleman SC, Bruck N. Changes in gait following the Scandina-
e259
vian Total Ankle Replacement. J Bone Joint Surg Am 2011; 93: 1890–6. 8 Ingrosso S, Benedetti MG, Leardini A, Casanelli S, Sforza T, Giannini S. GAIT analysis in patients operated with a novel total ankle prosthesis. Gait Posture 2009; 30: 132–7. 9 Valderrabano V, Nigg BM, von Tscharner V, Stefanyshyn DJ, Goepfert B, Hintermann B. Gait analysis in ankle osteoarthritis and total ankle replacement. Clin Biomech (Bristol, Avon), 2007; 22: 894–904. 10 Steck JK, Anderson JB. Total ankle arthroplasty: indications and avoiding complications. Clin Podiatr Med Surg 2009; 26: 303–24.
Haemophilia (2015), 21, e223--e259
Copyright of Haemophilia is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
