Spontaneous spinal epidural hematoma in the setting of Evans syndrome: a case report and literature review.

BACKGROUND

Spontaneous spinal epidural hematoma (SSEH) is a rare condition with potential for devastating neurologic outcomes. The etiology of SSEH is not well understood. However, it has been linked with blood dyscrasias and anticoagulant administration. The treatment for SSEH is dependent upon patient presentation and consists of surgical decompression or close observation and treatment of any underlying coagulopathy. In cases where there is progressive or severe neurologic deficit, time to decompression has been shown to influence patient outcomes. Immune thrombocytopenia (ITP) is an autoimmune hematologic disorder resulting in decreased production and altered function of platelets. Evans syndrome (ES) is a rare variant of ITP diagnosed in <5% of ITP cases. Platelet counts of less than 30,000 have been associated with increased risk of bleeding events in those with ITP. Skin, oral, and nasal mucosal surfaces are the most common locations of spontaneous bleeding. The central nervous system accounts for less than 1% of spontaneous bleeds. To our knowledge, this is the first reported case of SSEH in a patient with ES.

CASE DESCRIPTION: In the case at hand, a 69-year-old male patient with ES presented with acute, severe, atraumatic pain in the lower cervical region. His examination did not reveal neurologic deficit. Advanced imaging demonstrated a large epidural hematoma in the cervicothoracic region with mass effect on the adjacent spinal cord. The patient underwent urgent surgical decompression without complication along with medical treatment of his thrombocytopenia. Postoperatively, his neurologic status remained preserved without any other known bleeding events. He currently receives immunotherapy for ES management.

CONCLUSIONS

SSEH is a rare pathology that can result in catastrophic neurologic injury. There are only a few previously reported cases of SSEH in the setting of ITP. In this instance, initial treatment is dependent upon patient presentation, followed by appropriate workup/management of the underlying blood dyscrasia.