Total knee arthroplasty in rare case of type 3 Von Willebrand arthropathy

Introduction This case describes a 43-year-old woman with "type 3 Von Willebrand disease" and severe diffuse arthropathy that in the 2007 underwent left total knee artroplasty. At age of 14, due to recurrent hemarthrosis, the patient underwent left knee synovectomy, the following year underwent right knee synovectomy and after five years underwent left elbow synovectomy. Materials and Methods The patient presented chronic left knee pain, ROM 5°-30°, double support walking, HSS Knee score 38. For the poor conditions of both bone-stock and ACL and collateral ligaments, we chose a constrained prosthesis. Factor VIII (FVIII) was administered during the operation and also in the following 4 weeks to further correct the plasmatic levels of these factors. Considering the high thromboembolic risk (related to surgery, reduction of mobility and the chronic use of estroprogestinic therapy), thrombotic prophylaxis was performed with low molecular weight heparin. Results After 26 months the clinical and radiographic control showed a substantial improvement with pain reduction, ROM 0°-95°, HSS Knee score 94 and perfect prosthesis integration. Discussion Total knee arthroplasty in type 3 Von Willebrand disease patients (prevalence 1:1,000,000) is a complex surgery with high risk of hemorrhagic and infective complications. Conclusions The possibility to improve quality of life in such a satifying manner by surgery, let the patients do not take care of potential problems and complications related to it.