Keywords
spontaneous spinal epidural hematoma - cervical spine
Palavras-chave
hematoma epidural espinhal espontâneo - coluna cervical
Introduction
Spontaneous spinal epidural hematoma (SSEH) is a very rare condition that requires
an urgent diagnosis. The incidence of SSEH is estimated to be 1 patient per 1,000,000
individuals. Typically, SSEH presents an acute onset of severe back and neck pain,
and signs of compression of the spinal cord develop rapidly. Although hemiparesis
due to SSEH is a relatively uncommon symptom compared with quadriparesis, to the best
of our knowledge, a few cases of hemiparesis due to SSEH have been reported until
now. Patients with hemiparesis due to SSEH have often been misdiagnosed as presenting
with a cerebral infarction and treated with antiplatelet or anticoagulation therapy.
This is one of the pitfalls in emergency stroke cases. In the present study, we present
a case report of a 57-year-old female with controlled hypertension who presented with
SSEH mimicking a cerebrovascular accident (CVA) and was successfully treated with
surgical decompression.
Case Report
A 57-year-old woman with a medical history of hypertension presented to our emergency
department complaining of a sudden onset of weakness in the right upper and lower
extremities. There was no history of trauma, infection, or drug administration. Upon
neurological examination, weakness of grade 3/5 was noted in her right upper and lower
extremities, while there was no motor weakness of the right facial muscles. Her sensory
function was completely preserved. All of the blood count, blood urea nitrogen (BUN),
creatinine, international normalized ratio (INR), prothrombin time (PT), partial thromboplastin
time (PTT), electrolytes and troponin titer were within the normal limits. Aspirin
was administered by a neurologist with a strong suspicion of a transient ischemic
attack (TIA). Imaging studies of the brain showed no hemorrhagic lesion, no tumor
or arteriovenous malformation. A magnetic resonance imaging (MRI) exam of the cervical
spine revealed an epidural hematoma extending from level C5 to level C7, causing spinal
cord compression. ([Fig. 1]) Aspirin was discontinued and the patient was submitted to a surgical treatment
for decompression laminectomy. A bilateral C5 to C7 laminectomy was performed. The
patient was informed that her condition would be published anonymously and signed
a written informed consent. During the operation, an epidural hematoma was evacuated.
([Fig. 2]) Postoperatively, the power in both limbs improved to grade 5/5 just after the surgery.
Fig. 1 MRI and MR myelogram from C5 to C7 epidural hematoma. (A) MR myelogram cut off. (B) Axial T2 image which shows right side epidural hematoma. (C) Sagittal image of C5 to C7 epidural hematoma.
Fig. 2 Intra-operative image of right side clotted blood in epidural space.
Discussion
Spontaneous spinal epidural hematoma is rare, and the annual incidence is 1 case per
1,000,000 individuals.[1] The spontaneous development of SEHs is most frequent after the fourth or fifth decade
of life. However, it has been reported to occur in all age groups, and it is a very
rare clinical entity in children. The male/female ratio is 1.4:1.[1] Although SSEH is an accumulation of blood in the vertebral epidural space in the
absence of trauma or iatrogenic procedures such as lumbar puncture, there is still
no consensus regarding its definition. Some authors include hematomas secondary to
coagulopathy, vascular malformations, hemorrhagic tumors and also pain control procedures.[2]
[3] Other authors claim, however, that hematoma can be labeled as spontaneous only when
it is of idiopathic origin.[2] Patients often present with a sudden onset of back or neck pain around the affected
vertebrae, with corresponding dermatomal radiculopathy, which then rapidly progresses
to symptoms and signs of spinal cord compression. This represents a neurosurgical
emergency. Without prompt diagnosis and treatment, there will be permanent neurological
deficits or even death.[4]
[5] The most common cause of hemiparesis is a cerebrovascular incident, such as cerebral
infarction. Although hemiparesis due to spinal cord disorders is rare, injury of the
unilateral corticospinal tract in the cervical spinal cord causes hemiparesis. Therefore,
cervical SSEH, in which quadriparesis is a common symptom, can cause hemiparesis.[6]
Patients with hemiparesis due to SSEH have often been misdiagnosed as having cerebral
infarction and treated with antiplatelet or anticoagulation therapy. As a result,
the hemiparesis worsened, and only then were they diagnosed with SSEH. How can one
distinguish the symptoms of SSEH with hemiparesis from cerebral infarction? Typically,
SSEH is characterized by the sudden onset of neck and back pain followed by motor
and sensory dysfunction.[6] As you see in the case reported in the present study, the patient did not present
with acute neck pain, and the only symptom was progressive hemiparesis, which made
a neurologist colleague suspect of TIA and administer anticoagulant therapy.
The preferred diagnostic tool for SSEH is an MRI exam, which can reveal the location
and extent of the hematoma, the degree of spinal cord compression and the signal in
the spinal cord, although a computed tomography (CT) scan may be useful for cases
in which MRI is contraindicated. The MRI appearance of the hematoma depends on the
hematoma stage.[5]
[7] It typically shows biconvex hematomas in the epidural space with well-defined borders
tapering superiorly and inferiorly.[1] In the present case, the MRI revealed a heterogeneous hyperintense extradural lesion
in the T1 sequence and a heterogeneous hypointense lesion in the T2 sequence from
the fifth to the seventh cervical vertebrae. No vascular or tumor lesion were detected
during operation.
The differential diagnosis of SSEH includes acute herniated intervertebral disc, acute
ischemia of the spinal cord, epidural tumor or abscess, spondylitis, transverse myelitis,
or even a dissecting aortic aneurysm and acute myocardial infarction.[1]
Spontaneous spinal epidural hematoma is an urgent surgical condition, and the most
effective treatment is decompressive laminectomy and prompt drainage of the hematoma
.[7] The postoperative recovery from SSEH depends, predominantly, on the interval between
the onset of the symptom and the surgical decompression.[2] However, conservative treatment has also been documented, and it was employed only
when the neurological deficits improved in the early phase or with the coexistence
of coagulopathy. Multilevel acute epidural hematomas may be difficult to treat operatively
in patients with coagulopathy. Although the functional recovery of these patients
may not be complete, the SSEH can be treated without surgery, and the patient should
be protected from the significant risk of surgical intervention.[1] We performed an urgent decompression surgery by bilateral laminectomy and medial
facetectomy followed by the evacuation of about 5 ml of clotted extradural hematoma.
Conclusion
Hemiparesis or hemiplegia without signs and symptoms of cerebral involvement, such
as facial hemiparesis or speech disturbance, could be a sign of cervical cord pathology,
and in the subset of hemorrhagic lesions, an accurate diagnosis has great importance
because anticoagulant therapy as a routine treatment for ischemic CVA could be life-threatening.
A high degree of suspicion, meticulous history taking, and physical examination are
of great importance in these rare conditions.
