Iliopsoas Hemorrhage in Pediatric Patients with Hemophilia, Experience from One Centre

Introduction: Iliopsoas hematoma is a serious, life threatening bleeding event in patients with hereditary clotting disorders and significantly associated with morbidity. It occurs most commonly in patients with severe hemophilia (Balkan et al Haemophilia 2005). In this review, we evaluated our patients with iliopsoas hematoma and compared with literature.

Methods: The eleven pediatric patients with hemophilia and iliopsoas hematoma treated in a research and training hospital in Istanbul, between 2005 and 2015 were enrolled. The clinical characteristics including symptoms, signs, complications, and treatment were analyzed retrospectively.

Results: We evaluated 14 episodes of iliopsoas hematoma from 11 patients (10 hemophilia A and 1 hemophilia B). All patients were male and the median age was 12 (8 -14) years old. Eight patients had one episode, three had two episodes. One patient had a high titer inhibitor against factor VIII. The hematoma was spontaneous and unilateral in all cases. The most common symptoms were hip pain and hip flexion contracture. Iliopsoas hematomas were confirmed by ultrasound, CT or MR scan. The treatment consisted of factor replacement and rehabilitation therapy. One patient needed erythrocytes transfusion. The mean duration of factor replacement therapy was 13.5 +/- 2.1 days. One severe hemophilia A patient with inhibitor treated with recombinant factor VIIa. Long term complications included paresthesia in 3 patients in the distribution of femoral nerve, quadriceps atrophy in 3 patients and permanent abnormal posture in one patient.

Conclusion: The psoas muscles are located in the iliopsoas compartment posterior to the transversalis fascia, which is the posterior boundary of the retro peritoneum. Presentation of iliopsoas hematomas is often non-specific with abdominal, pelvic, back or groin pain or swelling. If they are large, iliopsoas hematomas can present with constipation, urinary frequency or fever. They can even compress the femoral nerve and cause a femoral neuropathy . A delay in treatment may result in permanent femoral nerve palsy (Mannucci N Engl J Med 2004). In this report, we present our experience and treatment modality in 14 episodes of iliopsoas hematoma in pediatric hemophilia patients. Rehabilitation treatment with factor replacement is safe and effective therapy for patients with small hematomas and little or no neurological symptoms. More aggressive recommendations have been made for patients with large hematomas and severe neurological symptoms or hemodynamic instability. An early diagnosis allows early factor replacement therapy, and decreases the risk of recurrence and morbidity.