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Spontaneous iliopsoas muscle hematoma in a patient with von Willebrand disease: a case report
© Keikhaei and Shirazi; licensee BioMed Central Ltd. 2011
Received: 21 September 2010
Accepted: 2 July 2011
Published: 2 July 2011
Iliopsoas hemorrhage is a serious complication of bleeding disorders that occurs most commonly in patients with hemophilia and less commonly in patients with von Willebrand disease. It causes severe pain, muscle dysfunction and occasionally femoral nerve palsy. We describe the case of a patient with von Willebrand disease type 3 with a large iliopsoas hematoma who was treated with a von Willebrand factor concentrate (Humate-P).
A 20-year-old Iranian man was referred to our emergency ward because of the gradual onset of right flank pain. He was known to have been diagnosed with von Willebrand disease type 3 at age two years old. Magnetic resonance imaging showed a mass in the right iliopsoas muscle. The diagnosis of iliopsoas hemorrhage and partial femoral nerve palsy was established, and he responded to medical treatment.
We report a case of von Willebrand disease type 3 with spontaneous iliopsoas hematoma associated with femoral nerve palsy that was well managed with Humate-P treatment.
Von Willebrand disease (vWD) is a hereditary bleeding disorder caused by a quantitative or qualitative defect of von Willebrand factor. The prevalence of this disease is estimated to be 1% to 2% of the general population. Von Willebrand factor is a coagulation factor for initial stages of blood clotting and is also a carrier for factor VIII. Patients with vWD develop coagulation platelet plugs and red coagulation clots are unable to form. vWD consists of a broad variation of clinical manifestations, with one extreme being a minimal bleeding tendency and the other being a severe presentation such as severe hemophilia A. Luckily, most of these patients present with mild to moderate clinical symptoms. The severe type of the vWD, type 3, occurs in less than 5% of patients. The inheritance pattern of this type is usually autosomal recessive. Patients with vWD usually show clinical features such as recurrent nosebleeds, easy bruising, bleeding post-tooth extraction, post-tonsillectomy or other surgical bleeding, and excessive menstrual bleeding.
Musculoskeletal hemorrhage occurs commonly in vWD type 3-like hemophilia A. Iliopsoas hemorrhage is a potentially serious complication because a delay in beginning treatment may result in permanent femoral nerve palsy . The present case report describes a man with vWD type 3 who had a spontaneous iliopsoas hematoma that responded well to Humate-P treatment (CSL Behring, King of Prussia, PA, USA) .
Iliopsoas hemorrhage, whether spontaneous or traumatic, is encountered in a variety of coagulation disorders, such as hemophilia and vWD, and in association with anti-coagulant drug treatments such as heparin, warfarin and ticlopidine; however, most cases of iliopsoas hemorrhage occur in patients with hemophilia , and the first case was reported by Tallroth in 1939 .
The diagnosis of retroperitoneal hematoma is made on the basis of ultrasonography, computed tomography and MRI. The treatment of patients with iliopsoas muscle hemorrhage remains controversial. Some experts consider that surgical decompression (open or percutaneous aspiration) is the treatment of choice because it minimizes neurological deficits. Others have advocated a medical approach. Non-operative treatment consists of complete rest and replacement factor therapy. Iliopsoas hemorrhage occurs mostly in patients with hemophilia after trauma (falling from height), but our patient had no history of trauma. Good results were achieved by administering Humate-P treatment to our patient .
This report describes the case of a patient with vWD type 3 who had a spontaneous iliopsoas hematoma. The diagnosis was established on the basis of MRI, and the patient showed a good response to Humate-P treatment. We could not find any similar case reported in the medical literature, and we think that our case report may be the first of its kind to be published. The publication of this article may raise the clinician's awareness of the treatment of iliopsoas hematoma in patients with vWD.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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