From Pain to Paralysis - Spontaneous Spinal Epidural Hematoma: A Case Report and Review of the Literature

Spontaneous spinal epidural hematoma (SSEH) is a rare clinical condition with a reported incidence of 0.1 per 100,000 patients annually (8). SSEH typically affects adults in the age group between 40 to 60 years and often presents as severe pain with rapidly progressive neurological deterioration, overall presenting as an acute spinal cord compression syndrome (9). The bimodal age distribution of SSEH shows peaks in the second and seventh decades of life. In our case, the patient presented with neck pain followed by progressive sensory and motor deficits, a presentation consistent with previously reported cases (10). The spinal location of SSEH shows a bimodal distribution as well, with peaks at C6 and T12 levels (11).

The exact etiology of SSEH remains unclear, though some cases are associated with the use of anticoagulants, some coagulopathic conditions, and vascular anomalies (12). In some cases, few authors have described the association of cavernous hemangioma and Paget’s disease with this disease (3) (4). However, in our patient, there was no identifiable cause which corresponds to the fact that 40-50% of cases are idiopathic (3). In such cases where there is no history of trauma or use of anticoagulants, it is hypothesized that the origin of hematoma can be due to some rupture in vessels (13). The origin of hematoma can be either arterial or venous (14), but it is mostly attributed to a venous cause because the venous plexus in the cervical and thoracic regions lacks valves (3). Increased venous pressure in these areas can potentially rupture the corresponding vessels leading to bleeding and the formation of hematoma (13). Increased venous pressure can be due to straining, coughing, sneezing, and constipation (3). This may explain the occurrence of SSEH in otherwise healthy individuals, although further research is warranted to clarify the exact etiology.

Prompt diagnosis of SSEH is critical to execute early surgical intervention thus limiting the potential neurological complications (2). Particularly in severe motor deficits, timely intervention is crucial for better neurological outcomes and uneventful recovery. MRI is recognized as the diagnostic modality of choice, as it accurately identifies the site and extent of hematoma (8). In our patient, T2-weighted hyperintensity identified the presence of hematoma with significant spinal cord compression, supporting the urgency of surgical intervention. As seen in our patient with compression extending from C4 to C6, rapid MRI assessment and urgent laminectomy are considered crucial for the management of cases with severe neurological involvement (5).

The standard treatment for SSEH is urgent decompressive laminectomy and hematoma evacuation, especially in patients presenting with severe and progressive symptoms (7). It has been reported that timely surgical intervention is associated with favorable outcomes and significant recovery (15). However, cases with minimal symptoms can be managed conservatively but carry risks of permanent neurological impairment as the hematoma can enlarge over time (16). Since our patient had hematoma associated with a rapidly progressive onset of symptoms and significant clinical deterioration, conservative management was not followed.

Many surgical cases report only partial recovery (7). The likelihood of poor outcome of surgery depends upon the prognostic factors including the thoracic location, large size of the hematoma, poor neurological status of the patient at presentation, delayed surgery beyond 24 hours, and use of anticoagulants (1). Our patient underwent C4-C6 decompressive laminectomy, and the hematoma was surgically evacuated relieving the pressure on the spinal cord. Even after the surgery, the patient had a post-operative hyperintense signal in the spinal cord with significant residual compression which is associated with the poor outcome. This highlights the importance of postoperative imaging in evaluating potential residual lesions or re-occurrence of hematoma.

In our case, the patient had poor prognostic factors including severe neurological symptoms on presentation, involvement of the cervical spine, large size of the hematoma, and delayed intervention due to presentation on the 3rd day of illness. Upon discharge, his pain had subsided with improvement in limb strength. Although the compression was relieved after surgery, there were persistent signal changes in the cord due to primary cord compression, resulting in compromised neurological recovery. This case emphasizes the role of post-operative care and monitoring as well as the importance of follow-up and rehabilitation plans. Based on the existing research, most of the reported cases of SSEH who underwent timely decompression and laminectomy showed quick recovery with complete neurological recovery (8).