Successful Surgical Removal of A Massive Iliopsoas Pseudotumor in a Boy With Mild Hemophilia A

Background

Hemophilia A is an inherited genetic disease with a lack of coagulation factor VIII (FVIII). Although the life expectancy since the introduction of factor substitution in 1960s tremendously improved, possible complications are still dreaded. Muscle bleedings are with 30% of all bleeds the second most common bleeding location after the joint (Fernandez-Palazzi F et al., Clinical orthopaedics and related research 1996; 328: 19–24). These bleedings can involve especially the quadriceps, iliopsoas and arm muscles. Approximately 12% of patients with hemophilia A develop an iliopsoas bleed which is considered as a potentially life-threatening event. Initial symptoms of muscle hemorrhage are very similar to joint bleedings with pain, swelling and restrictions in the range of motion. Hence, in the first instance it may be difficult to differentiate between joint and muscle bleed. Over time, patients with muscle bleed can present with chronic pain, nerve compression, compartment syndrome, contracture of the joints, anemia, and recurrent bleedings (Ashrani A et al., Haemophilia 2003; 6: 721–726). The muscle bleedings can evolve to a large size because of less limiting surrounding, unlike the articular capsule in hemarthrosis. Furthermore, hemorrhagic pseudotumors can arise because of ongoing bloody oozing. The prevalence of pseudotumors in patients with hemophilia is approximately 1–3%, independently of the severity of hemophilia (Magallón M et al., American journal of hematology 1994; 2: 103–108).