Common and New Orthopaedic/Physical Therapy Recommendations. Mauricio Silva MD

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1 Common and New Orthopaedic/Physical Therapy Recommendations Mauricio Silva MD

2 Hemophilia Journal WFH Website

3 Recommendations regarding the clinical management of people with hemophilia Practice statements are in bold in text. All such statements are supported by the best available evidence in the literature. Grading was performed professionally as per 2011 Oxford Centre for Evidence-Based Medicine

4 Levels of evidence Oxford Center for Evidence-Based Medicine 2010

5 WFH Guideline Me FAQ

6 WFH Intention Useful to those initiating and maintaining hemophilia care programs. Extensive review of the literature Encourage practice harmonization around the world Stimulate studies where practice recommendations lack adequate evidence

7 Hemophilia Sex-linked genetic disorder Deficiency of clotting factor Hemophilia A: Factor VIII Hemophilia B: Factor IX 1:7,500 live male births

8 Hemophilia: Clinical Progression Normal Joint Acute Bleeding Chronic Synovitis Early Arthritis End Stage Arthritis

9 Approximate Frequency of Bleeding at Different Sites Haemophilia, (2013), 19, e1-e47

Fitness and Physical Activity Physical activity should be encouraged to promote physical fitness and normal neuromuscular development, with attention paid to muscle strengthening,

10 Fitness and Physical Activity Physical activity should be encouraged to promote physical fitness and normal neuromuscular development, with attention paid to muscle strengthening, coordination, general fitness, physical functioning, healthy body weight, and self-esteem. (Level 2) [15] 15. Gomis M, Querol F, Gallach JE, Gonzalez LM, Aznar JA. Haemophilia 2009; 15:

11 Bone density may be decreased in people with hemophilia. [16,17] For patients with significant musculoskeletal dysfunction, weight-bearing activities that promote development and maintenance of good bone density should be encouraged, to the extent their joint health permits. (Level 3) [16] Fitness and Physical Activity 16. Iorio A, Fabbriciani G, Marcucci M, Brozzetti M, Filipponi P. Thromb Haemost 2010; 103: Wallny TA, Scholz DT, Oldenburg J et al. Haemophilia 2007; 13:

12 Hemophilia: Clinical Progression Normal Joint Acute Bleeding Chronic Synovitis Early Arthritis End Stage Arthritis

13 Hemophilia: Principles of Care Haemophilia, (2013), 19, e1-e47

14 Definition of Response to Treatment of Acute Hemarthrosis Definitions in hemophilia. Recommendation of the scientific subcommittee on factor VIII and factor IX of the scientific and standardization committee of the International Society on Thrombosis and Haemostasis. JTH 2012 (in press).

Human Inhibition articular cartilage of Proteoglycan Synthesis 125

15 Proteoglycan synthesis (%) Common and New Orthopaedic/PT Recommendations Acute Hemarthrosis: Induction of Cartilage Damage Human Inhibition articular cartilage of Proteoglycan Synthesis Cont Roosendaal G. et al. Arthritis Rheum, 1999.

16 Induction of Cartilage Damage Mononuclear Cells Pro-inflammatory Cytokines IL-1-ẞ IL-6 TNFα Red Blood Cells Chondrocyte Iron Roosendaal G et al. J Rheumatol 1997 Roosendaal G et al. Arthritis Rheum 1999 Hooiveld M et al. Am J Pathol 2003 Hooiveld M et al. Rheumatology (Oxford) 2003 Bates E et al. Annals of the Rheumatic Diseases 1984, Oxygen Metabolites Toxic Hydroxyl Radicals

17 Acute Bleeding - Factor Replacement Therapy Definitions in hemophilia. Recommendation of the scientific subcommittee on factor VIII and factor IX of the scientific and standardization committee of the International Society on Thrombosis and Haemostasis. JTH 2012 (in press).

18 No. of Joint Bleeds per Year Common and New Orthopaedic/PT Recommendations Prevention of Hemophilic Arthropathy Randomized trial 65 boys (<30 months of age) % Normal Joints by MRI 0 Episodic (n=32) Prophylactic (n=33) 0 Manco-Johnson MJ. et al, N Eng J Med, 2007.

19 Tradeoff Between Initiation of Treatment and Joint Pathology

20 Chronic Synovitis Hypervascularity Chronic synovial inflammation

21 % ROM Limitation Common and New Orthopaedic/PT Recommendations Arthropathy: Natural History Severe Moderate Mild Age (Yr) Source JM. et al, Blood, 2004.

22 Pettersson score (max 78) Common and New Orthopaedic/PT Recommendations Arthropathy: Natural History Cumulative no. of joint bleeds Fig.3. Mean Pettersson scores according to cumulative number of joint bleeds. Fischer, K. et al, Acta Radiologica, #43, 2002.

Arthrocentesis Removal of blood from a joint: may be considered in the following situations: A bleeding, tense, and painful joint, which shows no improvement 24 h after conservative treatment Joint

23 Arthrocentesis Removal of blood from a joint: may be considered in the following situations: A bleeding, tense, and painful joint, which shows no improvement 24 h after conservative treatment Joint pain that cannot be alleviated Evidence of neurovascular compromise of the limb Unusual increase in local or systemic temperature and other evidence of infection (septic arthritis) (Level 3) [4,9,10] 4. Hermans C, et al Haemophilia 2011; 17: Ingram GI et al, Ann Rheum Dis 1972; 31: Rodriguez-Merchan EC. Haemophilia 2012; 18:

24 Prevention of Hemophilic Arthropathy Mononuclear Cells MR16-1 (anti IL-6) TNFα Receptor antagonist IL-10 Pro-inflammatory Cytokines IL-1-ẞ IL-6 TNFα Red Blood Cells Chondrocyte Iron Oxygen Metabolites Toxic Hydroxyl Radicals Sun J. et al. J Thromb Haemost, 2009 Jensen NW et al. B J Haematol, 2008

Prevention of Hemophilic Arthropathy Induction of Cartilage Damage Mononuclear Cells MR16-1 (anti IL-6) TNFα Receptor antagonist IL-10 Pro-inflammatory Cytokines

25 Prevention of Hemophilic Arthropathy Induction of Cartilage Damage Mononuclear Cells MR16-1 (anti IL-6) TNFα Receptor antagonist IL-10 Pro-inflammatory Cytokines IL-1-ẞ IL-6 TNFα Red Blood Cells Chondrocyte Iron Oxygen Metabolites Toxic Hydroxyl Radicals Sun J. et al. J Thromb Haemost, 2009 Jensen NW et al. B J Haematol, 2008

26 Hemophilia: Clinical Progression Normal Joint Acute Bleeding Chronic Synovitis Early Arthritis End Stage Arthritis

27 Synovectomy Synovectomy should be considered if chronic synovitis persists with frequent recurrent bleeding not controlled by other means. Options for synovectomy include chemical or radioisotopic synoviorthesis, and arthroscopic or open surgical synovectomy. (Level 4) Bernal-Lagunas R et al Haemophilia 2011; 17: Caviglia HA et al Haemophilia.2001; 7(Suppl. 2) Yoon KH, et al, Int Orthop 2005; 29:

28 Arthroscopic Synovectomy Arthroscopic Synovectomy Highly effective Widel JD, CORR, 1996 Eickhoff HH et al., CORR, 1997 Rodriguez-Merchan EC et al, CORR, 1997 Dunn AL et al, J. Ped. Orthop, 2004 Major surgery Clotting factor Hospitalization Arthrofibrosis Range of motion Physical therapy Patients with inhibitors

29 Arthroscopic Synovectomy procedures in 44 patients Age range: 4 18 y Mean F/U: 6.6 y 4 10 weeks of clotting factor; 23 patients >6 months Frequency of bleeding decline of 84%

30 Synovectomy Non-surgical synovectomy is the procedure of choice. Radioisotopic synovectomy using a pure beta emitter (phosphorus-32 or yttrium-90) is highly effective, has few side effects, and can be accomplished in an outpatient setting. (Level 4) [18,19] 18. Thomas S, et al, Haemophilia 2011; 17: e Van Kasteren ME, et al, Ann Rheum Dis 1993; 52:

31 Non-Surgical Synovectomy: Before 1990 Au 198 P 32

32 Non-Surgical Synovectomy: Up to 2000 Non-surgical Synovectomy: Up to 2000 Au 198 P 32 Y 90 Re 186 Rifampicin Osmic Acid

33 Non-Surgical Synovectomy: 2010 Au 198 P 32 Y 90 Re 186 Rifampicin Osmic Acid Sm 153

evidence of joint damage 32 P 90 Y 198 Au 86 Re 165 Dy Radiation β β β, γ β, γ β Particle

34 Radiosynovectomy Ideal Candidate Frequent hemarthrosis: 2-3 bleeds/month Target joint Failed conservative treatment with clotting factor replacement and PT No radiological evidence of joint damage 32 P 90 Y 198 Au 86 Re 165 Dy Radiation β β β, γ β, γ β Particle size (μ) Penetration (mm) Half life (days)

35 Radiosynovectomy Procedure Simple Outpatient department Local anesthetic Joint access Drainage Injection of 32 P

No. of cases Bleeds per month Common and New Orthopaedic/PT Recommendations Radiosynovectomy Effectively reduces frequency of bleeding Outcome: Joint Bleeding 50 40 30 50 44 115 primary procedures

36 No. of cases Bleeds per month Common and New Orthopaedic/PT Recommendations Radiosynovectomy Effectively reduces frequency of bleeding Outcome: Joint Bleeding primary procedures 28% with inhibitors Knees Elbows Ankles Subtalars Shoulders % n=115 93% n=115 79% n=115 82% n=78 p< % n=60 80% n=44 87% n=16 74% n=6 Pre Silva M, et al; Hemophilia, 7: 40-49, 2001

37 % of Cases Common and New Orthopaedic/PT Recommendations Radiosynovectomy 100 Outcome: Excellent or good results: 80% Excellent + Good = >75% bleeding reduction Excellent = 100% bleeding reduction Months Silva M, et al; Hemophilia, 7: 40-49, 2001

38 Radiosynovectomy: Safety No excess of malignancies: Compared malignancies in 2,412 Quebec patients who had received RS with an age-matched general population Expected Observed Leukemia Lymphoma Primary Malignancies

39 Embolization Selective therapeutic embolization with gelfoam or polyvinyl alcohol Significant decrease in frequency of bleeding Promising results

Synovitis and Physical Therapy Supervised physiotherapy aiming to preserve muscle strength and functional ability is a very important part of management at this stage.

40 Synovitis and Physical Therapy Supervised physiotherapy aiming to preserve muscle strength and functional ability is a very important part of management at this stage. Secondary prophylaxis may be necessary if recurrent bleeding occurs as a result of physiotherapy. (Level 2) [9,10] 9. Blamey G, Forsyth A, Zourikian N et al. Haemophilia 2010; 16(Suppl. 5): Gomis M, Querol F, Gallach JE, Gonzalez, LM, Aznar JA. Haemophilia 2009;15:

41 Pain Management in Hemophilia Arthropathy - Strategies Pain should be controlled with appropriate analgesics Certain COX-2 inhibitors may be used to relieve arthritic pain (Level 2) [13,14] 13. Rattray B et al, Haemophilia, 2006; 12: Tsoukas C et al, Blood 2006; 107:

42 Hemophilia: Clinical Progression Normal Joint Acute Bleeding Chronic Synovitis Early Arthritis End Stage Arthritis

43 Surgery and Invasive Procedures Surgery for patients with hemophilia will require additional planning and interaction with the healthcare team than what is required for other patients. A hemophilia patient requiring surgery is best managed at or in consultation with a comprehensive hemophilia treatment center. (Level 3) [50,51] The anesthesiologist should have experience treating patients with bleeding disorders. 50. Schild FJ et al, J Thromb Haemost 2009; 7: Kavakli K. Haemophilia1999; 5:

44 Surgery and Invasive Procedures Adequate laboratory support is required for reliable monitoring of clotting factor level and inhibitor testing. Preoperative assessment should include inhibitor screening and inhibitor assay, particularly if the recovery of the replaced factor is significantly less than expected. (Level 4) [52,53] Surgery should be scheduled early in the week and early in the day for optimal laboratory and blood bank support. 52. Serban M et al, Hamostaseologie 2009; 29(Suppl. 1): S Astermark J et al, Haemophilia2010; 16: 747 6

45 Surgery and Invasive Procedures Adequate quantities of clotting factor concentrates should be available for the surgery itself and to maintain adequate coverage postoperatively for the length of time required for healing and/or rehabilitation. If clotting factor concentrates are not available, adequate blood bank support for plasma components is needed. The dosage and duration of clotting factor concentrate coverage depend on the type of surgery performed (Tables 7-1, 7-2).

46 Suggested Plasma Factor Peak Levels and Duration

47 Surgery and Invasive Procedures If these conservative measures fail to provide satisfactory relief of pain and improved functioning, surgical intervention may be considered. Surgical procedures, depending on the specific condition needing correction, may include: Extra-articular soft tissue release to treat contractures. Arthroscopy to release intra-articular adhesions and correct impingement. [31] Osteotomy to correct angular deformity. Prosthetic joint replacement for severe disease involving a major joint (knee, hip, shoulder, elbow). [32] Elbow synovectomy with radial head excision. [33] Arthrodesis of the ankle, which provides excellent pain relief and correction of deformity with marked improvement in function. Recent improvements in ankle replacement surgery may pose an alternative for persons withhemophilia in the future. [34,35]. 31. Wiedel JD. Haemophilia 2002; 8: Goddard NJ, Mann HA, Lee CA. J Bone Joint Surg Br 2010; 92: Silva M, Luck JV Jr. J Bone Joint Surg Am 2008; 90(Suppl. 2 Pt 2): Barg A, Elsner A, Hefti D, Hintermann B. Haemophilia 2010; 16: Tsailas PG, Wiedel JD. Haemophilia 2010; 16:

48 Surgery for Advanced Hemophilic Arthropathy Hip 14% Knee 54% Ankle 14% Elbow 14% Shoulder 4%

49 Total Knee Replacement Capsular Releases

50 Total Knee Replacement Total Knee Replacement Pain relief Decrease in disability Increase in ROM Improvement in quality of life

51 Outcome of TKR in Hemophilia Author Year # Knees Excellent or Good Goddard et al % DVT: 2/70 95% 5% 0% Silva and Luck % - 80% 13% 7% Norian et al % Cohen et al F/U (years) Infection 10% Other Complications Patellar dislocation: 1/21 Arthrofibrosis: 2/21 68% Clinical Results Fair Poor 26% 6% Thomason et al Hemarthrosis: 3/23 17% Nerve Palsy: 1/23 17% Loosening: 2/23 9% 74% Heeg et al % Loosening: 1/9 Hemarthrosis: 1/9 Stiffness: 2/9 78% 11% 11% Unger et al % Hemarthrosis: 2/26 92% 8% 0% Teigland et al % Hemarthrosis: 1/ Kjaersgaard- Andersen et al % Hemarthrosis: 4/12 100% 0% 0%

52 TKR in Hemophilia: Special Considerations High-efficiency particulate air (HEPA)-filtered suits No antithrombotic prophylaxis Longer hospital stay Extensive physical therapy (up to 9 weeks)

53 TKR in Hemophilia: Survival Silva, M. and Luck, J.V., JBJS, 2005

54 TKR in Hemophilia: Outcome KS-Clinical Score % of Knees Excellent (85-100) 53% Good (70-84) 27% Fair (60-69) 13% Poor (<60) 7% KS-Clinical Score Points (Ave) Pain 48 (45-50) Range of Motion 15 (0-23) Stability 24 (15-25) Deductions 7 (0-20)

55 TKR in Hemophilia: Infection Prophylaxis Meticulous antisepsis with self-infusion Regular medical checkups Immediate reporting of any type of infection Prophylactic antibiotic prior to dental work or any other contaminated procedure

56 Elbow Arthropathy Second most frequently affected joint in hemophilia Chronic hemophilic synovitis usually leads to the enlargement of the radial head

57 Elbow Arthropathy: Severe Arthropathy Hemophilic Arthropathy of the Elbow Normal Joint Acute Bleeding Chronic Synovitis Early Arthritis End Stage Arthritis What to do??

58 Elbow Arthropathy: Radial Head Excision Supination Mechanical blockage for rotation Synovial tissue Pain Hemophilia Pronation Impingement

59 Elbow Arthropathy: Radial Head Excision Mechanical blockage Impingement sites Arthritic joint

Elbow Arthropathy: Radial Head Excision 1969-2004 42

60 Elbow Arthropathy: Radial Head Excision radial head excisions 37 patients Mean follow-up: 6 years (1-28)

61 Elbow Arthropathy: Radial Head Excision

62 Elbow Arthropathy: Radial Head Excision

63 Elbow Arthropathy: Radial Head Excision

64 Elbow Arthropathy: Radial Head Excision

65 % of patients Common and New Orthopaedic/PT Recommendations Elbow Arthropathy: Radial Head Excision No early or late post-operative infections Post-operative Pain Severe Mild None

66 Elbow Arthropathy: Radial Head Excision 2.5º p= º p=0.2 Flexion Extension 0.4º p= º p=0.6 Pre-op Post-op Latest F/U 2.9º p= º p= Extension Flexion Flexion Arc

67 Elbow Arthropathy: Radial Head Excision Pronation Supination 36.4º p= Pre-op Post-op Latest F/U 61.1º p< º p< º p= º p= º p= Pronation Supination Arc

68 Elbow Arthropathy: Radial Head Excision

69 19 combined TEA Elbow Arthropathy: Arthroplasty Author # Elbows % Vol. 85-B, No. 8, November 2003 Infection 4 21 Ulnar neuropathy Axilary vein thrombosis Persistent pain 2 10

70 Ankle Arthropathy 3rd most commonly affected joint Onset when children start to walk In severe hemophilia, advanced ankle arthropathy is common by early adulthood

71 Ankle Arthropathy: Arthroplasty Minimal experience in hemophilia n=11 Pain relief Patient satisfaction High risk of infection (20%) Lack of long-term results

72 Ankle Fusion Ankle Arthropathy: Arthrodesis Tibio-talar Tibio-talar + Sub-talar Sub-talar The patient will retain some degree of dorsi-plantar flexion at the mid-foot joints

Ankle Arthropathy: Arthrodesis 1971-2004 39

4.3 years (1-16) Mean age: 36 years (18-60) 25

73 Ankle Arthropathy: Arthrodesis arthrodesis (30 patients) Pain (100%) Mean F/U: 4.3 years (1-16) Mean age: 36 years (18-60) Tibio-talar (TT) TT + Subtalar (ST) ST

distal tibia osteomyelitis 1970 Severe arthropathy TT joint 1971 TT joint fusion

74 Ankle Arthropathy: Arthrodesis No intra-operative or immediate postoperative complications Two late infections (5%) Pin-site infection BK amputation due to distal tibia osteomyelitis 1970 Severe arthropathy TT joint 1971 TT joint fusion (Casted) 1975 Non-union TT fusion 1981 Electric stimulation 1981 Tibial osteomyelitis

75 Ankle Arthropathy: Arthrodesis Non-union 20% TT 27% ST Non-painful Before % TT 50% ST After % TT 22% ST

Iliopsoas Hemorrhage Type of muscle hemorrhage with unique presentation. Signs may include pain in the lower abdomen, groin, lower back and pain on extension, but not on rotation, of the hip joint.

76 Iliopsoas Hemorrhage Type of muscle hemorrhage with unique presentation. Signs may include pain in the lower abdomen, groin, lower back and pain on extension, but not on rotation, of the hip joint. There may be paresthesia in the medial aspect of the thigh or other signs of femoral nerve compression such as loss of patellar reflex and quadriceps weakness. The symptoms may mimic acute appendicitis, including a positive Blumberg s sign. 76

77 Iliopsoas Hemorrhage Type of muscle hemorrhage with unique presentation. Signs may include pain in the lower abdomen, groin, lower back and pain on extension, but not on rotation, of the hip joint. There may be paresthesia in the medial aspect of the thigh or other signs of femoral nerve compression such as loss of patellar reflex and quadriceps weakness. The symptoms may mimic acute appendicitis, including a positive Blumberg s sign. Immediately raise the patient s factor level. Maintain the levels for 5 7 days or longer, as symptoms indicate (refer to Tables 7-1 and 7-2). (Level 4) [20 22] 20. Ashrani AA, et al.haemophilia 2003; 9: Balkan C et al. 2005; 11: Fernandez-Palazzi F et al Clin Orthop Relat Res 1996; 328:

78 Pseudotumors A 6-week course of treatment with factor is recommended, followed by repeat MRI. If the tumor is decreasing, continue with factor and repeat MRI for three cycles. (Level 4) [42,43] 42. D Young AI. Haemophilia 2009; 15: Rodriguez-Merchan EC. Int Orthop 1995; 19:

79 Surgery in Hemophilia: The Future RCT s on continuous prophylaxis Manco-Johnson MJ et al. Prophylaxis versus episodic treatment to prevent joint disease in boys with severe hemophilia. N Engl J Med 2007 Decreased number of joint bleeds Better joint scores Severe Hemophilia A and B Percentage of Patients on Continuous Prophylaxis Age Group Total N (%) (45.9) (56.0) The Universal Data Collection Program, March 2004 Effective reduction in hemophilic arthropathy

80 Surgery in Hemophilia: The Future No further need of joint surgery for hemophiliacs? Inhibitor development Fewer number of cases New challenges Higher risk of intraoperative and postoperative complications

81 FAQ I Do Not Know The Answer!!! 27 hemophilic patients Two cycles of injections VAS, SF-36, WFH, Pettersson score, WOMAC 7 years follow-up Improvement in pain and functional recovery Few patients required TKR for persistent pain 81

82 FAQ I Do Not Know The Answer!!! Short-term efficacy MRI evaluation No evidence of structural benefits Unchanged excretion of urinary CTX-II

83 FAQ I Do Not Know The Answer!!!

Take Home Message Aggressive treatment of acute hemarthrosis in patients without arthritic changes PT for increase in ROM and prevention of

84 Take Home Message Aggressive treatment of acute hemarthrosis in patients without arthritic changes PT for increase in ROM and prevention of contractures If chronic hemarthrosis: RS TKR: excellent choice but beware of risk of infection Radial head excision for advance elbow arthropathy Ankle arthrodesis

85 Hemophilia Journal WFH Website 85

86 Thank you! 86

The Treatment of Flexion Contracture of the Knee Joint in Hemophilic Patients Using Gradual Manipulation and Serial Casting

THE FLEXION IRAQI POSTGRADUATE CONTRACTURE MEDICAL IN HEMOPHILIC JOURNAL PATIENTS VOL. 13,NO.3, 2014 The Treatment of Flexion Contracture of the Knee Joint in Hemophilic Patients Using Gradual Manipulation