Pseudoaneurysms in haemophilia

Abstract

The incidence of bleeding as a result of a pseudoaneurysm in haemophilia is very low. The diagnosis should be suspected if the patient has a history of arterial trauma. Pseudoaneurysms can appear anywhere where trauma occurs. This can include arterial access for catheterization, blunt trauma or penetrating trauma. The diagnosis should be confirmed using Duplex ultrasonography, computed tomography (CT) angiogram or conventional angiogram. Many options exist for the treatment of pseudoaneurysms. Although surgery was the gold standard treatment in the past (surgical ligation with or without distal bypass), several less invasive treatment options are popular today. They include covered stent, ultrasound probe compression and ultrasound-guided thrombin injection. So far, only 14 pseudoaneurysms have been reported in patients with haemophilia: nine were located in the musculoskeletal system (four in the hand, four in the knee, one in the ankle), whereas five were non-musculoskeletal. Early diagnosis and treatment of this complication is vital. Endovascular treatment offers a minimally invasive treatment option. If arterial embolization fails to solve the pseudoaneurysm, open vascular surgery with surgical ligation with or without distal bypass should be performed. Whatever the procedure, a correct surgical haemostasis must be achieved by the infusion of factor concentrate (recombinant or plasma-derived) at the right dose and tranexamic acid. The advent of activated prothrombin complex concentrates and recombinant factor VIII (rFVIIa) has made invasive procedures possible in haemophilia patients with high-titre inhibitors.

Persistent Vascular Remodeling and Leakiness are Important Components of the Pathobiology of Re‐bleeding in Hemophilic Joints: Two Informative Cases

“…Vascular structures on PD assumed large confluent, pulsatile shapes that were described previously and appear unique to hemophilia in that they are absent in normal joints and osteoarthritis and less pronounced in rheumatoid arthritis . Of note, distorted synovial vascular structures resembling pseudoaneurysms in bleeding hemophilic joints were described before in case series during angiography and embolization, providing additional evidence that not only neoangiogenesis but also vascular remodeling is an important feature of progressive hemophilic arthropathy .…”

Contemporary Approaches to Hemophilia

“…The first results from this study were published recently [28]. The study was a multicenter, prospective, randomized comparison of HD (200 IU/kg/d) and LD (50 IU/kg three-times per week) FVIII regimens in 115 'good-risk' subjects with severe hemophilia A and high-titer inhibitors.…”

“…The missense mutations were associated with a low risk (odds ratio: 0.5) with a prevalence of 0.39%, whereas nonsense and entire F9 deletions had significantly higher risks (odds ratio: 11.0 and 32.7) with a prevalence of 14.3 and 44.5%, respectively [28]. Owing to the low prevalence of FIX inhibitors, very few data exist on the risk factors for inhibitor development in HB patients.…”

“…The diagnosis should be confirmed using duplex ultrasonography, a computed tomography angiogram or a conventional angiogram. Early diagnosis and treatment of this complication is vital [28]. Many options exist for the treatment of pseudoaneurysms.…”

“…In total, 14 nonserious and 27 serious adverse events occurred that were likely or possibly related to the infusion of the product. In total, 16 of the serious events were development of FVIII inhibitors and the other 11 involved catheter-related infections or hematomas [28].…”