Total Knee Replacement in Classic Hemophilia – A Challenge Worth taking?

Background: Hemophilia is a sex linked recessive disorder. It is characterized either by low level of factor VIII popularly known as hemophilia A or classic hemophilia and Hemophilia B or factor IX deficiency or Christmas disease. Factor VIII levels in normal individuals range from 0.6 to 1.5 U/ml. In hemophilia these levels are less than 0.5 U/ml. Repeated intra articular bleeding is common. This bleeding causes a classical arthropathy. The pathophysiology of hemophilic arthropathy is multifactorial. Accumulated blood inside the joint is phagocytosed by the synovium, which undergoes inflammatory changes similar to those seen in rheumatoid arthritis, the hypertrophied synovium secrets hydrolytic enzymes which destroy the cartilage. Increased intra articular pressure and repeated immobilization also play a significant par tin the destruction of the joint. Management of hemophiliac arthropathy is difficult and needs multi-specialty approach. I am presenting a complex case of one such hemophlia induced knee arthropathy.

Case discussion: A 45 year old male sales executive presented with inability to walk for 7 months following pain and deformities of both knees. He was a known hemophiliac and surprisingly had never received a factor VIII transfusion before. On examination he had 30 degrees of fixed flexion deformity on each side with further flexion of only 20 degrees. There was synovial thickening on both sides. There was marked muscle wasting on both sides. He was unable to take even one step. He was bed ridden for 7 months. His X-rays picture showed gross porosis too.

Treatment Options: There were 3 options and these were discussed with the patient in detail.
Option 1: Bilateral TKR, ideal option but the cost implications were very high considering the fact that he might require augments or wedges or even a LCCCK knee prosthesis and not to forget the factor VIII burden. It was also explained to the patient that the risk of infection was high in such cases.

Option 2: Bilateral Knee Arthrodesis, cheapest and safest option but obvious disadvantage of traveling by public transport etc. Patient refused this outright.

Option 3: One TKR and one arthrodesis: compromise between the first 2 options but financially ideal. Patient accepted this mainly on the financial grounds.

Problems, which were anticipated:

Presence of antibodies (inhibitors) to factor VIII: Inhibitors develop in approximately 15% to 20 % of patients treated with factor VIII. Fortunately this patient had no antibodies since he had never received any transfusions before. A patient with inhibitor level above 20 Bethesda units responds poorly if at all to treatment with factor VIII concentrate.

HIV status: Many of these patients are HIV positive again due to transfusions. The risk on infection increases many folds if a patient id positive, however our luck continued on this front as well since the patient was HIV negative. In HIV positive patients a preop CD 4 count is a must and the incidence of postoperative infection is low in patients with counts > 400 x 10 ?.
Haemolytic anaemia: A Haemolytic reaction to infused anti A or anti B antibodies may occur in patients with blood groups A, B or AB who receive a large amount of factor concentrate. Haemolytic anaemia, which requires treatment postoperatively, is managed by replacement with washed, packed, type O RBC’s and factor concentrates ( monoclonal and recombinant).

Planning details:

Patient’s factor levels were determined to classify the severity of hemophilia and were found to be less than 0.01 U/ml. This classified him as severe deficiency of factor VIII. Incidentally the severity of hemophilia is based on the factor levels as well as frequency and the severity of bleeding episodes. Factor levels >5 indicate a mild disease whereas levels between 1 to 5 characterize a moderate disease. 

• The requirement of factor VIII are as follows for a TKR
• 100% level for the surgery and the next 48 hours.
• 60 % level for the next 3 days
• 40% for the next 3 days
• 20% for physiotherapy (4 to 6 weeks post operative)

Source Risk of Hepatitis Risk of HIV Risk of haemolysis

• Plasma + + 0
• Cryo + + 0
• Factor VIII concentrate 
• Heated ++++ 0 ++++
• Solvent - - ++++
• Monoclonal - - -
• Recombinant - - -
• Factor IX heated ++++ ? -
• Activated ++++ - -

Day of Surgery:

Factor VIII levels were sent at 8 AM. Result received at 1pm that the levels were 100%. Patient taken up for surgery at 2PM. General anaesthesia was used. Right TKR done first under tourniquet and the intraoperative pictures are shown below. Bone was very soft but luck favored us again and we could implant a standard cruciate sacrificing TKR. It took us 90 minutes to complete the surgery i.e. 20 minutes more than a standard TKR. We then arthrodesed his right knee using 2 A-O 4.5mm LCDCP plates at 90 degrees to each other. This surgery took us 70 minutes. Since suture removal would be hazardous we took subcuticular ethilon sutures on both sides. Drains were inserted on both sides.

Postoperative protocol:

We did not opt for a routine physiotherapy protocol as the factor requirement would go up and we were on a shoestring budget! Wounds healed well without ant infection or breakdown. However he developed a large blister underneath the tourniquet on right side and required factor for more time than usual. His blister did heal with repeated dressings and did not require any debridement. He walked with the help of a walker and his replaced knee at the end of 16 days and was very happy. He achieved a range of 0 to 84 degrees of flexion, which was excellent because the average flexion reported in various series world over has been 74 degrees.

Conclusions:
Replacement surgeries or for that matter any surgery in hemophiliacs is a huge ask but if the required facilities are available then the outcome is very pleasing. These surgeries are to be done only in centers where there is a good hematology backup and excellent theatre facilities for performing joint replacement surgeries. Careful follow up of these patients is mandatory and infections even in remote places should be treated aggressively. Routine prophylaxis for dental work is recommended.

Intra op photo showing haemosiderin deposits on the patella

Post operative X-ray

Preoperative X-ray showing gross destruction

References:

1. Arnold WD,Hilgenreiner MW, Hemophilic Arthropathy Journal of Bone and Joint Surgery, Am 59:287,1977
2. Thompson HC III, Wilson FC, Lachiewicz: Knee Arthroplasty in Hemophiliacs: CORR 360,1999
3. Wiedel and Luck, Total Knee Arthroplasty in Hemophiliac patients: Evaluation of long term results
4. Lachiewicz and Insall: Total knee arthroplasty in hemophilia, JBJS 1985,Am.
5. Surgery of the knee. Textbook by Insall-Scot 3rd Edition

Dr. Shreedhar Archik , "Care Clinic" 4,Shankar Niwas, Dadasaheb Rege marg, Shivaji Park, Mumbai 4000028 Tel: 24445608