Spontaneous intradural extramedullary hematoma after mild exercise in Von Willebrand disease: A rare clinical presentation and literature review.

BACKGROUND

Von Willebrand disease (VWD) is the most common inherited bleeding disorder. Spinal intradural extramedullary hematoma (SIEH) is an exceptionally rare manifestation.

CASE DESCRIPTION: A 28-year-old woman with VWD developed spontaneous SIEH following mild exercise, presenting with back pain, progressive lower limb weakness, and double incontinence. Magnetic resonance imaging (MRI) demonstrated a T3/4 intradural extramedullary hematoma. Urgent surgical evacuation through T3/4 fenestration was performed, followed by targeted hemostatic therapy with recombinant von Willebrand factor (Vonicog Alfa). The patient made a complete neurological recovery within 3 months and remained asymptomatic at 14-month follow-up.

CONCLUSION

SIEH should be considered in VWD patients presenting with acute spinal symptoms, even without trauma. Early MRI, prompt decompression, and tailored coagulation management are critical to optimal outcomes.