Atypical presentation of spontaneous spinal epidural hematoma

Introduction
Spinal epidural is an uncommon diagnosis, making out less than 1% of all the compressive lesions in the vertebral column (1, 2). Spontaneous spinal epidural hematoma (SSED) designates a condition in which there is blood within the epidural space without any known trauma or iatrogenic triggering event. The incidence has been reported to 0, 1 in 100000 person years (2- 5).

SSEH have the highest incidence among people in the age between 40- 60, with a male predominance (2,3,7). They are often admitted with a sudden debut of acute pain in the vertebral column, that might radiate to the limbs and might involve neurological deficits (1,2-4,7,8).

Deficits often entails pathology in the lower motor neurons, involving hyporeflexia and motor deficits in varying degrees (1). The time span from the onset of pain until deficits develops, might vary from hours to several days or months (1,3,7). It is crucial to recognize the disorder early, as SSEH can potentially lead to severe and permanent deficits or mortality (7).

SSEH can be caused by trauma, surgery and instrumentation (1). Risk factors that predisposes for the SSEH are less understood, which is reflected in that 40- 60% of described cases have no identified cause. Anticoagulants, coagulopathies, hemangiomas, vascular malformations and tumors might be underlying reasons (1,2,4,5, 7 -9). 17- 30% of SSEH are related to use of anticoagulants (5, 8). Other factors are small trauma, pregnancy, hemophilia and leukemia (5, 7).

Case report
The patient was a 57 years old female. She was in life long warfarin treatment due to DVTs. One week prior to admittance, she had experienced an acute onset of pain in the cervical region radiating to the left arm, with no known preceding trauma. The pain exacerbated. She had numbness sensation and paresthesias in her right arm, and had gait disturbances.

She had a slight paresis distally in and sensory deficits in her right arm and a lack of coordination. Reflexes were normal. The motoric and sensory functions deteriorated over the following days, and involved one of her legs and deficits in the thorax as well, and she now had a positive Babinsky`s sign. She also had residual urine.

She had an INR level of 2,9 at the time of admittance.

On the third day after admittance, a MRI of her cervical spine demonstrated a lesion suspect for an intraspinal hematoma, in relation to a cystic formation, which suggested a tumor, that led to an anterior displacement of the spinal cord. The lesion measured 6,3 x 2,2 x 1, 2 cm.

Based on her clinical and radiological findings, she was diagnosed with a medullary compression of uncertain genesis (hematoma, bleeding in a tumor or from a spinal AVM).

She was treated with a posterior cervical decompression with a C4- C6 laminectomy, in which a spinal epidural hematoma was evacuated, without any findings suggesting tumor or AVM. Prior to the operation, she was given Octaplex 1500 units, and her INR was below 1, 5 before surgery.

Postoperatively, the function in her upper right extremity was significantly improved. She has a distal foot clonus in her right lower extremity, and a positive Babinsky` s sign in this extremity, and with rehabilitation, she reported improvement.

To diagnose SSEHs, MRI is the method of choice (7- 9). More cases have been reported recently, which might be related to an increased application of MRI in clinical practice (11). This might lead to less severe cases being recognized, that would previously have gone unnoticed.

The treatment is often an acute decompressive surgery and it is recommended that the surgical procedure is carried out within 24- 36 hours after onset (3, 7). If a coagulopathy is discovered, or if the patient is using anticoagulation, this is corrected prior to surgical treatment (5).

If the patient is not treated surgically, the patient is closely monitored with repeated MRI scans under close observation (8). The outcome for SSEHs with conservative treatment has been described as poor compared with the surgical group (10). Hence, conservative treatment is often recommended for patients that aren’t considered suitable for surgical intervention, and for patients that are asymptomatic or with a stable development of neurological function (8, 9).

The most important factor for the long term outcome in cases of SSEH have been the neurological status prior to surgical intervention (2, 9).

Conclusion

SSEH is an acute emergency finding, that might lead to a fast neurological deterioration with permanent neurological deficits. Coagulopathies, vascular malformations and usage of blood thinners are risk factors. Many patients have none of these present. This is a quite rare diagnosis, which makes it challenging to examine underlying risk factors in a close manner.

Whether surgery is recommended remains a decision based on primary neurological function at the onset, the development and progression of neurological function.

Doctors ought to be aware of the subtleties of the condition as no or trivial antecedent trauma should lead to the indicated examinations and treatment.