Orthopedic Knee Replacement in Hemophilic Patients H.H. Wolf, H. Reichel, O. Dorligshaw

Orthopedic Knee Replacement in Hemophilic Patients

H.H. Wolf, H. Reichel, O. Dorligshaw and H.J. Schmoll

Hemophilic arthropathy is a major complication in hemophilia and may become cause of immobilization and social disability.

After multiple spontaneous or traumatic bleedings acute synovitis may occur.

Deposits of hemosiderin induce chronic synovitis resembling hypertrophy, hyperplasia and hypervascularisation of the synovial membrane followed by intra- articular fibrosis and extra-articular muscle contracture.

Cartilage and bone destruction lead to severe malformation and axial deviation with contractures, chondral atrophy, erosive destruction, and subchondral cysts.

Figure 2 shows radiographs of an hemophilia B patient born in October 1959, who presented at the age of 7 years with pain, swelling, and axial deviation due to hemorrhages into the left knee.

In 1998, the patient has been admitted to our hospital for knee replacement sur- gery.

Radiological examination showed complete destruction of the knee, immobi- lization, and loss of knee function. Furthermore, we found axial deviation as well as shortening of the femur after fracture and inadequate reposition.

Usually, replacement surgery is performed in the elderly. In hemophilic patients, however, severe arthropathy impairs patient’s quality of life in an early age [1].

Fig. 1 Magnetic resonance imaging of the knee: destruction of cartilage and bone structure; hemophilia B patient, 38 years old

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As tendinal and muscular contractures may complicate further orthopedic repla- cement, surgery should be done in an earlier course of disease compared to arthro- pathy patients.

Different protocols exist in hemophilic centres concerning time to undergo knee replacement and perioperative substitution therapy.

Patients` Characteristics

We report the clinical course of 7 hemophilic patients (hemophilia A 6 patients, hemophilia B 1 patient) who underwent knee replacement surgery.

Plasma activity of coagulation factor VIII or IX was < 1 %.

Patients` age was 27 to 62 years, median age 47.3 years.

Hemophilic arthropathy stage IV was found in 1 patient, stage V in 6 patients according to the Arnold and Hilgartner classification.

All patients suffered from severe pain and impairment of knee function.

Stage II or III hemophilic arthropathy of the upper ankle was found.

Fig. 2. Radiographs of the right knee, hemophilia B patient, 7 and 38 years old

Hemarthros with axial deviation in the boy, ossification of the joint in the adult patient

Surgical Therapy

Implantation of the prostheses was performed after arterial closure mostly via medial dissection. However, in 2 patients osteotomy of tuberositas tibiae became necessary.

Endoprostheses of the whole knee with cemented shafts have been performed in 4 joints; prostheses with cementless shafts with minimal fixation in 10 joints.

Intraoperative extension was complete and flexion was at least 80 degrees. Post- operatively, flexion was found to be at least 60 degrees up to day +5, and 90 degrees up to day +14, respectively.

Active training had to be performed using CPM extension.

Only in 1 patient replacement of the second knee was performed consecutively within a 5 month period.

Surgery was performed within 98 to 295 minutes time, depending whether there were one or two surgeons to perform arthroplasty simultaneously.

Orthopedic Knee Replacement in Hemophilic Patients 73

Fig. 3. Severe hemophilic arthropathy, hemophilia A patient

Hemostaseologic Therapy

Substitution therapy with coagulation factor concentrates was performed as bolus injection in 3 patients, as continuous infusion in the following patients. All hemo- philia A patients were treated with recombinant FVIII products.

Continuous infusion therapy and reduction of bleeding were correlated posi- tively.

Postoperative blood loss was 75–940 ml, depending on the modus of admini- stration.

Substitution therapy started the day before surgery by bolus injection of 40 IU/kg body weight. The same dosage was administered immediately before surgery as well as postoperatively.

Continuous infusion was performed in 3 patients with hemophilia A with recom- binant FVIII products, in 1 patient with hemophilia B with plasmatic derivative.

In order to maintain median plasma concentration of 70% substitution of FVIII/

FIX concentrates was done:

Median dosage administered was 50 IU/kg bw until day + 8, 30 IU/kg bw day + 9 to +15, respectively.

The median total dosage of coagulation factor concentrates was 92,000 IU

(range 80,000 to 152,000 IU) depending on modus of administration. The dosage

Fig. 4. Postoperative radiographs of the left knee, hemophilia

A patient.

was lower in patients treated with continuous infusion than with bolus injection regimen.

Thromboembolic prophylaxis was performed with low-molecular heparin sub- cutaneously day 1–15. On day 15, patients started out-patient training.

Results

HSS-Score improved significantly (34.5 to 77.9).

Functional results were found to be good (excellent n= 4, well n=7, sufficient n=2 joints, respectively).

Function of only 1 prosthesis was found to be not sufficient.

Median time of follow-up was 3.7 years (1 up to 7 years). There was no radiolo- gical evidence of instability.

Complications

No postoperative hemorrhages have been found. No development of inhibitors has been seen. 6 years after implantation, late infection of bilateral endoprostheses by staphylococcus species was seen in one patient.

Orthopedic Knee Replacement in Hemophilic Patients 75

Table 1. Functional results after bilateral arthroplasty of the knee

patient age preoperative postoperative difference follow-up

function function

(years) (degree) (degree) (degree) (years)

A 49 left 0-30-45 left 0-30-45 + 45 7.7

right 0-30-50 right 0-5-90 + 65 7.2

B 27 left 0-25-100 left 0-15-80 – 10 5.1

right 0-10-90 right 0-0-90 + 10

G 49 left 0-20-85 left 0-0-110 + 65 4.1

right 0-15-90 right 0-0-110 + 35

D 46 left 0-30-100 left 0-5-110 + 35 3.2

right 0-35-100 right 0-5-100 + 30

E 38 left 0-20-40 left 0-10-100 + 70 1.5

right 0-5-50 right 0-0-110 + 65

Z 62 left 0-30-40 left 0-5-60 + 45 1.3

right 0-20-60 right 0-0-60 + 20

I 50 left 0-40-70 left 0-0-90 + 60 1.1

right 0-15-50 right 0-5-50 + 10

Conclusions

Arthroplasty of the whole knee improved motility and function in younger hemo- philic patients [2].

Simultaneous bilateral arthroplasty was performed without intraoperative bleeding complications in patients with continuous infusion therapy of coagulation factor concentrates [3, 4].

We prefer continuous infusion compared to bolus injection in order to proof stable and sufficient plasma concentrations of coagulation factors [5].

Prospective studies are required to define state of arthropathy for the patients to undergo surgery, modus of surgery (unilateral or simultaneous bilateral arthropla- sty), as well as material of endoprosthesis which has to be used.

Bilateral arthroplasty may be useful concerning postoperative functional trai- ning and cost reduction, but intraoperative hemorrhage could become a complica- tion in case of insufficient substitution.

Data are requested whether continuous infusion or bolus injection provide more safety and comfort to patients and physicians.

References

1. Magone JB, Dennis DA, Weis LD. Total knee arthroplasty in chronic hemophilic arthropathy.

Orthopedics. 1986;9:653-637

2. Wallny T, Saker A, Hofmann P, Brackmann HH, Nicolay C, Kraft CN. Long-term-follow-up after osteotomy for haemophilic arthropathy of the knee. Haemophilia. 2003;9:69-75 3. Beeton K, Rodriguez-Merchan EC, Alöltree J. Total joint arthroplasty in haemophilia.

Haemophilia. 2000;6:474-481

4. Cohen I, Heim M, Martinowitz U, Chechick A. Orthopaedic outcome of total knee replace- ment in haemophilia A. Haemophilia. 2000;6:104-109

5. Reichel H, Birke A, Wolf H-H. Knee endoprosthesis implantation in hemophilia arthropathy:

results, problems and complication. Z Orthop Ihre Grenzgebiete. 2001;139:120-126

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