Klin Padiatr 2018; 230(06): 333-335
DOI: 10.1055/a-0586-4514
Short Communication
© Georg Thieme Verlag KG Stuttgart · New York

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

Erfolgreiche operative Ausräumung eines massiven Iliopsoas-Pseudotumors bei einem Jungen mit milder Hämophilie A
Alexej Bobrowski
1   Department of General Paediatrics, Adolescent Medicine and Neonatology, Medical Center – University of Freiburg, Freiburg, Germany
,
Annamaria Cseh
2   Department of Pediatric Hematology and Oncology, Medical Center – University of Freiburg, Freiburg, Germany
,
Alexander Pschibul
3   Department of Neuropediatrics and Muscle Disorders, Medical Center – University of Freiburg, Freiburg, Germany
,
David Schorling
3   Department of Neuropediatrics and Muscle Disorders, Medical Center – University of Freiburg, Freiburg, Germany
,
Olaf Moske-Eick
4   Department of Neuroradiology/Neurocentre, Medical Center – University of Freiburg, Germany
,
Jochen Raedecke
5   Department of General and Visceral Surgery, Division for Paediatric Surgery, Medical Center – University of Freiburg, Freiburg, Germany
,
Barbara Zieger
2   Department of Pediatric Hematology and Oncology, Medical Center – University of Freiburg, Freiburg, Germany
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Publikationsverlauf

Publikationsdatum:
27. März 2018 (online)

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).