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The clinical manifestations of severe haemophilia, an X-linked hereditary bleeding disorder, include spontaneous and trauma-related articular and muscular bleeds. Recurring bleeds in the target joints lead to haemophilic arthropathy and the need for endoprothesis. We report a case of a sudden intra-articular pseudoaneurysm formation in a synovial artery after endoprosthesis surgery of a knee. This event occurred in a haemophilia A patient with an inhibitor to factor VIII (FVIII).A 29-year-old man with a diagnosis of severe haemophilia A underwent an elective endoprosthesis reoperation of his left knee. The patient had developed a FVIII inhibitor in his youth, but had never undergone immune tolerance induction therapy. He had been treated with recombinant FVIIa (rFVIIa, NovoSeven Ò ; Novo Nordisk Inc, Princeton, NJ, USA) on an on-demand basis. According to his history, activated prothrombin complex concentrate (aPCC, Feiba Ò ; Baxter AG, Volketswil, Switzerland) had not cured his bleeds effectively. The patient had chronic hepatitis C infection that was eradicated successfully in 2006. He experienced recurrent joint haemorrhages, particularly into the knees and ankles, but also into his elbows and hips. In 2005, he had undergone his first endoprosthesis operation of the left knee under activated rFVIIa coverage. The insertion of the endoprothesis was complicated and an extension defect of 15°remained after the operation. Furthermore, in association with his rehabilitation he experienced an intra-articular bleed and a 20°extension deficit led to a functionally unsatisfactory result.To correct the extension deficit, a revision arthroplasty was performed in May 2009. In preoperative laboratory tests no anaemia or thrombocytopenia was found and his liver enzymes and prothrombin time were normal. All other coagulation factors, with the exception of FVIII (<1%), were normal. The inhibitor titre was 3 BU mL )1 prior to the operation and it remained at that level. At 30 min prior to surgery, the patient received 7 mg (115 lg kg )1 , patientÕs weight was 60 kg) of rFVIIa and 500 mg tranexamic acid (Caprilon Ò ; Leiras, Finland) intravenously. Throughout surgery and during the following two postoperative days, he received repetitive infusions of rFVIIa 5 mg (90 lg kg )1 ) every 2 h and tranexamic acid 500 mg three times a day. The operation and early recovery period were uneventful. There were no perioperative bleeding problems and pain was manageable. On the first postoperative day his haematocrit was 30% and he received two units of red blood cells.During postoperative days 3-6, the patient developed a progressive pain in the operated knee upon mobilization. He was receiving rFVIIa 5 mg (90 lg kg )1 ) every 3 h and tranexamic acid 500 mg three times a day. At first, an intra-articular bleed was suspected and he was switched to 5000 IU aPCC 3 h after the administration of rFVIIa. The pressure and pain on leg lowering continued during the postoperative day 6 and during the following day. He could

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