|Year : 2020 | Volume
| Issue : 1 | Page : 11-14
Spontaneous spinal epidural hematoma presenting with paraparesis: A case report and review of literature
Suresh S Pillai1, Amalraj P Nair2, M Harisankar2
1 Department of Spine Surgery, Baby Memorial Hospital, Kozhikode, Kerala, India
2 Department of Orthopaedics, Baby Memorial Hospital, Kozhikode, Kerala, India
|Date of Submission||31-Oct-2020|
|Date of Decision||10-Nov-2020|
|Date of Acceptance||01-Oct-2020|
|Date of Web Publication||17-Nov-2020|
Amalraj P Nair
Thazhathidathil House, Ayanikkad Post, Kozhikode - 673 521, Kerala
Source of Support: None, Conflict of Interest: None
Spontaneous spinal epidural hematoma (SSEH) is a rare and devastating clinical entity. The incidence is considered to be 1 in million/year. SSEH manifests due to accumulation of blood in the epidural space causing compression of the spinal cord and leading to acute neurological deficit. Although it is a rare condition, it must be considered in nontraumatic patients with sudden onset of neurological deficit. Magnetic resonance imaging (MRI) serves as the modality of choice in diagnosing spinal epidural hematoma. Decompressive laminectomy and hematoma evacuation is considered as the mainstay of treatment, although conservative treatment can be tried in limited cases. Here, we present the case of a 48-year-old male who presented with progressive neurologic deficit. MRI revealed spinal epidural hematoma at T10-L4 level with cord compression which was promptly evacuated. Functional recovery was achieved after the surgical intervention.
Keywords: Decompressive laminectomy, hematoma evacuation, paraparesis, spinal epidural hematoma
|How to cite this article:|
Pillai SS, Nair AP, Harisankar M. Spontaneous spinal epidural hematoma presenting with paraparesis: A case report and review of literature. J Orthop Assoc South Indian States 2020;17:11-4
|How to cite this URL:|
Pillai SS, Nair AP, Harisankar M. Spontaneous spinal epidural hematoma presenting with paraparesis: A case report and review of literature. J Orthop Assoc South Indian States [serial online] 2020 [cited 2020 Dec 3];17:11-4. Available from: https://www.joasis.org/text.asp?2020/17/1/11/300762
| Introduction|| |
Spontaneous spinal epidural hematoma (SSEH) is characterized by blood accumulating in the epidural space and compressing the spinal cord. It is a rare pathology, and the incidence is considered to be 0.1%/100,000/year. The word spontaneous refers to spinal hematoma occurring in the absence of any trauma or iatrogenic procedure. SSEH was first described by Jackson in 1869. The exact cause of SSEH remains unknown in 40%–50% of individuals, but different factors including use of anticoagulants, coagulation disorders, arteriovenous malformation, disc herniation, surgical trauma, epidural catheterization, and conditions causing increased intra-abdominal pressure such as coughing, Valsalva maneuver, and possibly hypertension remain as precipitating factors.,,, Both arterial and venous origins for SSEH had been identified, but many studies point venous source of bleeding as the predominant cause. The clinical manifestation of SSEH varies from severe back pain or neck progressing to radiculopathy and paraplegia or quadriplegia depending on site and extent of hematoma.
Magnetic resonance imaging (MRI) is considered as the diagnostic test in identifying site and extension of SSEH. Decompressive surgery of the spinal cord at level of hematoma and hematoma evacuation remains the effective treatment for SSEH. However, several studies showed that conservative management can be done in patients with no neurological deficits or spontaneously recovering cases. It is considered that the preoperative neurological status remains an important prognostic factor in overall outcome of disease.
In this case report, we present a patient who was admitted with complaints of progressive paraparesis of lower limbs after 2 days of onset of symptoms and whose weakness improved after surgical interventions. [Figure 1], [Figure 2], [Figure 3], [Figure 1]4
|Figure 1: (a and b) Preoperative T2-weighted (magnetic resonance imaging) image taken at the time of admission showing spinal epidural hematoma extending from T10, T11 to L4 level causing maximum spinal cord compression at L2, L3, and L4 levels|
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|Figure 2: Preoperative T1-weighted image taken at the time of admission showing spinal epidural hematoma extending from T11 to L4, L5 levels|
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|Figure 3: (a and b) Repeat magnetic resonance imaging taken 36 h after admission showing spinal epidural hematoma extending from T10, T11 to L5 levels compressing the spinal cord in T2-weighted images|
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| Case Report|| |
A 48-year-old, previously healthy manual laborer came to our hospital with complaints of progressive weakness of both lower limbs for 1 day. It started as severe low backache 2 days back while he was doing manual work. He consulted a nearby hospital and was sent home with analgesics. The pain increased, and he started experiencing progressive numbness and weakness in both lower limbs. The patient had no history suggestive of infection, trauma, drug intake, or recent surgeries. Vitals showed a high blood pressure of 220/120 mmHg, and all other parameters were normal. Laboratory investigations including complete blood count, erythrocyte sedimentation rate, C-reactive protein, liver function test, renal function test, serum electrolytes, and coagulation profile (prothrombin time–international normalized ratio, activated partial thromboplastin time, bleeding time, and clotting time) were send and found to be within normal limits. On neurological examination, weakness was more in the right lower limb compared to the left lower limb and was graded as score B according to the American Spinal Injury Association (ASIA). There was loss of strength of bilateral lower limbs with a power of 3/5 in L2 and L3 domain of the right lower limb and 0/5 in L4, L5, and S1 domain of the left lower limb. Sensory impairment was present in bilateral lower limbs with lax anal sphincter and perianal anesthesia. Bilateral tendon reflexes were reduced, and the patient also had urinary incontinence. There was no motor or sensory impairment in both upper limbs.
MRI of the cervical and dorsal spine showed a lesion in the dorsal and lateral side of the epidural space extending from T10 to L4 level with high-intensity signal in T1-weighted (T1W) images and heterogeneous low-intensity signal in T2-weighted (T2W) images. There was maximum compression of the spinal cord at T11-L3 levels and minimal compression in lower levels.
The patient was admitted to the orthopedic department with a diagnosis of SSEH. After admission, the patient showed mild improvement in neurological status for the next 12–24 h and thereafter there was no significant improvement. A repeat MRI of the whole spine was taken which showed hematoma in the posterior and lateral aspect of the epidural space extending from T10-L5 with prominence at L2, L3, and L4 compressing the spinal cord. He was planned for surgery. Digital subtraction angiography, spinal angiogram, and ultrasound abdomen were done to rule out any vascular malformations and intra-abdominal pathology. The patient underwent posterior approach decompressive laminectomy of L2, L3, and L4 vertebra, and the hematoma was evacuated. Thick clotted blood and prominent epidural hematoma were noted intraoperatively compressing the spinal cord more at the level of L2, L3, and L4. The hematoma was evacuated completely, and the spinal cord was decompressed. The patient showed improvement in neurological status starting from postoperative day 1. As a part of early rehabilitation program, a detailed counseling regarding rehabilitation schedule was given to the patient which included passive joint range of motion training of lower limbs, muscle strengthening enhancing program, and position change training. The patient was discharged from hospital on postoperative day 5. On discharge, the patient was able to walk with mild limping and the other symptoms such as urinary retention and numbness also improved. In follow-up after 2 weeks, the patient came with walking without any swaying or limping. Bowel habit was normal, and the patient had postvoid residual urine of 280 mL, so he was put on Foley's catheter and sent home.
| Discussion|| |
SSEH is usually seen in patients in the fourth/fifth decades of life, with a slightly higher incidence in males (1.4:1). Of all spinal lesions, it accounts for <1% with an annual incidence of 1/million. It is a devastating clinical entity which may result in morbidity with delayed treatment or no treatment.
The cause of SSEH is still a controversial topic. In majority of the cases, the cause remains unknown. In statistical studies, almost 40% of SSEH cases are idiopathic where there is no history of recent trauma, iatrogenic procedures, or precipitating factors. In remaining 60% of cases, the use of anticoagulants, factor IX deficiency, hemophilia, neoplasm, AV malformation, cavernous hemangioma, therapeutic thrombolysis, and herniated disc had been identified as precipitating factors. SSEH had been also found in associations with conditions causing increased intra-abdominal and intrathoracic pressures. Hypertension as a precipitating factor is still in debate. Many authors suggest the association between hypertension and SSEH, whereas meta-analysis has suggested no increased risk of SSEH in hypertensive individuals.
The source of epidural hematoma as a venous origin or an arterial origin is still a dilemma. Studies point that these hematomas develop from rupture of epidural veins, epidural arteries, or from vascular malformations. According to many studies, slow progression of the disease in most cases and the anatomical location of the hematoma supports venous origin as the main source of bleeding., However, in case of rapidly progressing symptoms, an arterial source should be considered. Furthermore, a venous source of bleeding cannot be applicable in cervical epidural hematoma cases because of rapid deterioration of symptoms and due to high intrathecal pressure of this region compared to venous pressure.
The hypothesis of venous origin as the main source of bleeding predominates since venous system lack valves are more prone to underlying pressure changes from thoracic and abdominal cavities. The association of SSEH with conditions increasing intrathoracic and intra-abdominal pressures such as coughing, sneezing, bending, Valsalva maneuver, and coitus supports the hypothetical cause of hematoma as a venous source of bleeding. However, the source of bleeding of hematoma is only an academical concern. In clinical practice, once the epidural space is filled with blood, either due to arterial/venous system, the prognosis and treatment remains the same, and the source of bleeding has no role in prognosis once bleeding happened. Although SSEH can occur at any level of the spinal cord, most of the cases reported cervicothoracic (C5-T2) and thoracolumbar (T10-L2) as the predominant sites. The spinal segment which undergoes more mobility produces more tension in epidural veins which subsequently rupture. This increased mobility in the cervicothoracic and thoracolumbar region is the reason for being the most common sites of epidural hematoma. There are many studies showing the relation between site of hematoma and outcome of disease.
The dorsal and lateral epidural space is affected more with SSEH than ventral since posterior and lateral compartments have rich venous plexus than anterior and also the dural sac is firmly adherent to posterior longitudinal ligament anteriorly making it more resistant to rupture.
Signs and symptoms of SSEH depend on the site of hematoma formation, source of bleeding, extend of hematoma, and the severity of cord compression. It may vary from severe neck and back pain to paraplegia or quadriplegia. The sensory system is also usually affected along with motor. In severe cases, the epidural hematoma extends to the cauda equina in which the patient manifests with cauda equina syndromes.
SSEH is difficult to establish before the appearance of neurological symptoms. In the abscence of neurological deficit, pain will be the predominant symptom and so often sseh may be misdiagnosed as pulmonary emboli,, pneumothorax and acute myocardial infarction and in the presence of neurological symptoms one may confuse sseh with transverse myelitis, guillain-barre symdrome and epidural subarachanoid bleeding. The preoperative ASIA score is an accurate marker for the postoperative functional outcome and subsequent prognosis of the disease, so that the ASIA score should be immediately checked on presentation.
In case of suspicion of SSEH, MRI serves as the imaging modality of choice. The intensity of hematoma differs according to time since presentation. At about 24 h from onset of symptoms, the hematoma appears isointense in T1W images and hypointense in T2W images – progressing to hyperintense lesions in both T1W and T2W images after 24 h of onset of symptoms. It is seen that the hematoma looks hypointense in both T1W and T2W images in case of chronic epidural hematomas.,, MRI also gives a picture of site of hematoma, extension, and the magnitude of spinal cord compression. If MRI is not available, computed tomography can be used as an alternative.
Once the diagnosis of SSEH is made, surgical intervention remains the mainstay of treatment. Various studies suggest that in case of patients with no or minimal neurological deficit or in patients with spontaneous recovery of symptoms, conservative management can also be tried as well. Dynamic MRI should be done as follow-up in such cases.
Decompressive laminectomy at the site of hematoma and subsequent hematoma evacuation is the standard surgical modality in case of confirmed SSEH. Many of the studies suggest that surgery should be done within 12–24 h of symptom onset for effective neurological improvement. It has been seen that the preoperative neurological status and ASIA score play important prognostic factors in the outcome of surgery and neurological improvement. Patients presenting with rapidly progressing symptoms, large hematoma involving large number of spinal segments has the worst prognosis. Furthermore, lack of sensory sparing in comparison to some sparing of sensation also accounts for poor prognosis.
In our case, we found improvement of neurological symptoms in the patient within the first 24 h but thereafter no improvement, so he underwent surgical decompression and hematoma evacuation which showed excellent results. The interesting thing about our case is that there is no predisposing factor other than a minor lifting incident as the cause of SSEH.
| Conclusion|| |
SSEH occurs as a rare disease in adult population. Of all, 30%–40% are idiopathic, and for the remaining cases, varying predisposing factors have been identified. The source of bleeding is still in debate in many cases: whether an arterial or venous origin. In a case of SSEH, immediate diagnosis and prompt surgical management is essential. Any patient with severe neck pain/back pain or progressive weakness should be evaluated for SSEH. MRI serves as an important imaging modality in suspected cases. Although conservative management can be tried out in patients with no or minimal neurological deficit, surgical intervention with emergency decompressive laminectomy and hematoma evacuation is the mainstay of treatment. Preoperative neurological deficit and severity serve as important prognostic factors in overall outcome disease. The rarity of this disease limits our knowledge regarding the cause of hematoma, predisposing factors, and prognosis; this makes the way for further studying. This case report should be useful for working doctors in identifying the signs and consequences of SSEH. This report should also emphasize the importance of considering SSEH as a differential diagnosis in patients coming with paraparesis without any history of trauma or other predisposing factors.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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