Abstract
BACKGROUND: Spontaneous spinal epidural hematoma (SSEH) is rare in children. Vascular malformation including arteriovenous fistulas and venous malformation is a rare cause of SSEH. CASE DESCRIPTION: A 5-year-old girl presented with 2 episodes of SSEH at the upper thoracic spine and non-hemorrhagic episodes with spontaneous neurological recovery. Diagnostic study with MRI and spinal angiography demonstrated an unusual epidural arteriovenous fistula (AVF) with venous ectasia similar to venous malformation. She underwent embolization of the AVF with NBCA with mild transient neurological deterioration. Follow up angiography showed persistent occlusion of the embolized fistula and inconsistent visualization of another AVF to the patent venous ectasia. CONCLUSIONS: This type of epidural AVF seems to be more common in children and tends to cause multiple neurologic episodes due to SSEH, venous expansion or thrombosis. Endovascular embolization with NBCA should be the first choice of treatment for this disease, unless emergent hematoma evacuation is necessary. Embolization should target at only the fistula site without significant penetration into the venous ectasia. Follow up is necessary for potential reappearance of AVF, even if AVF is occluded at the time of treatment. Time resolved MRI is useful to detect AVFs, thus for diagnosis and follow up of this disease.