Interdisciplinary Management for Total Knee Replacement in Patients with Hemophilia and Severe Arthropathy.
Authors: Gerhardt Andrea , Stahlschmidt Kristina , Werner Andreas , Krauspe Ruediger , Scharf Ruediger E. 
Published: April 2020

Abstract
Severe hemophilia is complicated by spontaneous joint bleedings, leading to severe secondary arthrosis. Hemophilic arthropathy with symptoms of incapacitating knee pain, not responding to medical treatment, associated with an impaired function is a clear indication for total knee replacement (TKR). Primary goals of total knee arthroplasty (TKA) are pain relief and satisfactory function which depends on adequate joint stability and motion. A well-balanced hemostasis is a basic requirement for successful interventions in hemophilic patients. Furthermore, the postoperative range of motion (ROM) depends on many factors, including surgical technique, preoperative motion, and appropriate rehabilitation.
We report on our experience with 7 arthroplasties of knee joint arthropathies in 7 patients with severe hemophilia A (n = 4) and severe hemophilia B (n = 3). Indication for total knee replacement was arthropathy in stage III–V, progressive joint destruction, flexion contracture, axial malalignment, and pain refractory to conservative treatment. The average age of the patients at the time of arthroplasty was 35 years (range 18–42 years). Three patients were seropositive for HIV and HCV, 1 patient for HIV, 1 patient for HCV, and 2 patients had no viral infections. Mean hospitalization was 16.7 days (range 14 – 21 days). Six of 7 patients received a non-constrained bicondylar TKA (LCS complete n=5, Rotaglide n=1) and one patient a constrained bicondylar TKA (custom-made prostheses). The TKAs were inserted using bone cement containing gentamycin. Implantation was performed after arterial closure via medial dissection. Surgery was covered by appropriate factor VIII or IX replacement therapy with episodic bolus injections (factor VIII activity aimed at 100%). Median consumption of coagulation factor was 113.742 units (range 55.000 – 157.000 units). The postoperative blood loss was approximately 831 ml blood (range 250–1200 ml) in the low-vacuum drainage systems, the mean range of preoperative and postoperative haemoglobin (13.8 g/dl vs. 9.3 g/dl) was 4.5 g/dl. Transfusion of red blood cells was not required in any of the 7 individuals. Postoperative thromboembolic prophylaxis with low molecular weight heparin (4000 IE/day) was performed in all patients. The short-term results after a mean follow-up interval of 22 month (range 2–59 months) revealed no peri- and postoperative thromboembolic or bleeding complications and no infections. In 6 of 7 patients the knee extension improved with an average from 29.3° preoperatively to 5° postoperatively. One patient with reduced compliance (left hospital contrary to medical advice, rejected rehabilitation) suffered from postoperative articular fibrosis which made an open arthrolysis 3 and 8 month postoperative necessary and brought benefit in ROM from flexion/extension pre-operative 100/30/0 to 80/0/0 postoperative. In summary, our interdisciplinary treatment protocol demonstrates that total knee arthroplasty can be performed in high- risk hemophilic patients with a low rate of complications and in improvement in quality of life because of pain-relief and increase of motility and function in all patients. The high-dose replacement therapy is justified by the clinical outcome and benefit to the patients.