Treatment of a compartment syndrome in the leg in an elderly patient with acquired hemophilia type A: A case report

Abstract

Introduction: Acquired hemophilia type A is a rare autoimmune disease characterized by bleeding episodes ranging from mild to severe, leading to significant morbidity and mortality. One of the various presentations is compartment syndrome secondary to a spontaneous hematoma.

Case presentation: A 95-year-old woman arrived at the emergency department with a one-week history of progressive pain in the right leg, without a previous history of trauma. Upon physical examination, she presented tenderness, edema, and bruising on the posterior and lateral aspects of the right leg, without neurovascular deficits. Ultrasound revealed a collection in the posterior compartment of the leg, consistent with a hematoma. Due to worsening clinical condition, urgent drainage and fasciotomy of the posterior and lateral compartments of the leg was performed. After 2 years of follow-up, the patient has shown favorable progression.

Discussion: Hematoma drainage and fasciotomy are the primary management approaches for compartment syndrome secondary to hematoma presence. This etiology should be suspected in patients diagnosed with hemophilia who present to the emergency department with acute limb pain of unclear origin. Treatment should focus on relieving compartment pressure, supplementing the deficient factor, and eradicating inhibitors. None of the globally reported cases present a scenario with such an elderly patient exhibiting positive outcomes.

Conclusion: Compartment syndrome secondary to a spontaneous hematoma in a patient with acquired hemophilia is a diagnosis associated with significant morbidity and mortality, necessitating urgent management to ensure appropriate progression. This requires a multidisciplinary approach to drain the hematoma and rectify the hematologic condition.

Keywords: Acquired hemophilia; Case report; Compartment syndrome; Surgical management.

Fig. 1

Photograph illustrating the documented signs in the patient's right leg.

Fig. 2

Ultrasound showing compartmentalized hematoma in the posterior compartment of the right leg.

Fig. 3

(a) Sagittal, (b) coronal and (c) transversal plain leg CT scan showing hematoma in the posterior and lateral compartments measuring approximately 240 mm × 50 mm with a volume of 295 cm³.

Fig. 4

(a) Marked surgical approach, (b) evidence of hematoma, (c) extracted hematoma, and (d) application of fibrin sealant in the right leg of the patient.