|Year : 2022 | Volume
| Issue : 1 | Page : 25-31
Spontaneous spinal epidural hematoma: Institutional experience
Batuk Diyora, Rahul Chajjed, Gagan Dhall
Department of Neurosurgery, LTMG Hospital, Mumbai, Maharashtra, India
|Date of Submission||08-Oct-2021|
|Date of Acceptance||10-Nov-2021|
|Date of Web Publication||9-Mar-2022|
Department of Neurosurgery, Second Floor, College Building, LTMG Hospital, Sion, Mumbai - 400 022, Maharashtra
Source of Support: None, Conflict of Interest: None
Background: Spontaneous Spinal Epidural Haematoma (SSEH) is a rare entity that results from spontaneous, non-traumatic haemorrhage in the spinal epidural space. While erring on the side of caution, few significant clues in the history can lead to an earlier differentiation from more frequently seen pathologies like acute disc herniation and spinal tumors. Once diagnosed, the decision of surgical versus conservative management is entirely clinical and situation-based. In ambiguous scenarios, the study of specific parameters can help to choose one over another.
Objective: We are sharing here our experience of six such patients and review relevant literature, mainly focusing on the variables that can help to determine the best therapeutic plan.
Material and Methods: We studied the parameters like age, gender, location, number of levels involved, drug history, duration to surgery, American Spinal Injury Association (ASIA) impairment scale grade at presentation, therapeutic plan, and their final ASIA score for six patients of SSEH who presented to our department. We also reviewed the published literature focusing on therapeutic decision-making in SSEH.
Results: Five patients were managed surgically and one conservatively. Variables considered while opting for surgical treatment. One patient was managed conservatively based on his improving neurology.
Conclusions: Patients with SSEH need to be cautiously selected for surgical or conservative management for their optimal outcome. The study's various variables highlight a few critical concerns while predicting the outcome and guide one to decide a therapeutic plan for this rare pathology.
Keywords: Coagulopathy and spinal epidural hematoma, compressive myelopathy, spinal epidural hematoma, spontaneous spinal epidural hematoma
|How to cite this article:|
Diyora B, Chajjed R, Dhall G. Spontaneous spinal epidural hematoma: Institutional experience. J Spinal Surg 2022;9:25-31
| Introduction|| |
Blabby first described spontaneous spinal epidural hematoma (SSEH) in 1808. It includes spontaneous, nontraumatic hemorrhage. SSEH practically accounts for less than 1% of all spinal canal space-occupying lesions.,, There are a few peculiar pointers like, history of sudden onset of neck or back pain following coughing, sneezing, and straining activity which can potentially increase the intrathoracic or intra-abdominal pressure., With the history of ongoing anticoagulant or antiplatelet therapy or in cases with known spinal vascular pathology, one must lower the threshold for considering SSEH as one of the possibilities. Symptoms may vary from sudden onset neck or back pain, radiculopathy, paraparesis, or quadriparesis to bladder/bowel involvement. The therapeutic decision of surgical intervention or conservative management is not always straightforward. Surgical treatment is usually urgent, which includes laminotomy/laminectomy followed by evacuation of the clot. Selected cases of SSEH, fulfilling particular prerequisite criteria, can be tried conservative management and are kept under vigilance due to fear of progression and recurrence.
| Materials and Methods|| |
Between “January 2011 to January 2020,” six patients were treated with SSEH. We studied the variables like – age, gender, location, number of spine levels, drug history, duration to surgery, American Spinal Injury Association (ASIA) impairment scale score at presentation, therapeutic decision, their respective clinical outcomes in terms of ASIA score [Table 1] with their postsurgery follow-up of 1 year. We reviewed few cases series based on PubMed MeSH terms to search with the keywords SSEH and ASIA, between the year January 2011 and January 2020 and 4 case series explicitly dedicated to the SSEH, like literature by Raasck K et al., Rajz G et al., Dziedzic T et al., Kim KT et al. We also reviewed a cases series by Maurizio Domenicucci which studied more than 1000 patients of spinal epidural hematomas (SEHs). The aim is to formulate a basic therapeutic decision-making algorithm which can be followed while managing this uncommon but crippling neurological condition. Magnetic resonance imaging (MRI) scan was the investigation of choice. The outcome was assessed based on the ASIA impairment scale at the time of presentation, which was compared with their final ASIA impairment scale [Table 2]. We further classified these patients into good recovery (final score ASIA-D and ASIA-E) and poor recovery (Final score ASIA A, ASIA-B, and ASIA-C). The hematoma was sent for histopathological examination (HPE) in all the cases to rule out any arteriovenous malformation (AVM).
A 55-year-old male presented with sudden onset of severe back pain and weakness in bilateral lower limbs for 4 h. He was on aspirin therapy. Neurological examination revealed the power of Medical Research Council (MRC) Grade 0 in both lower limbs. Sensory examination revealed decreased sensations below D10 dermatome on both sides. He was categorized as ASIA impairment scale B. MRI Thoracic spine revealed biconvex posterior Epidural Hematoma from D8 to D10 vertebral level causing significant spinal cord compression. The hematoma was Isointense to Hyper-intense on T2-weighted images [Figure 1]a and [Figure 1]b. Because of significant neurological deficit, emergency laminectomy and clot removal were performed within 8 h of the onset of symptoms [Figure 1]c, [Figure 1]d, [Figure 1]e. HPE ruled out the existence of any AVM. Postoperatively, his bilateral lower limb power improved to MRC Grade 5 and ASIA B score to ASIA-E, which remained the same at 1-year follow-up.
|Figure 1: Case 1-Magnetic resonance imaging dorsal spine T2 weighted image on sagittal view showing D8-D10 isointense to hyper-intense posterior epidural haematoma (a), T2 weighted axial view showing posterior cord compression (b). Intraoperative image showing epidural haematoma after D8-D10 laminectomy (c-e)|
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A 16-year-old boy presented with sudden onset of upper back pain 2 days before admission. He also complained of weakness in both lower limbs. Initially, he could not lift his limbs against gravity (MRC Grade 2), but his weakness had improved spontaneously over the next 2 days. His initial ASIA impairment scale was B. MRI Thoracic spine showed biconvex posterior Epidural hematoma from D1 to D3 vertebral level compressing the spinal cord. It was Isointense to the spinal cord on T1-weighted [Figure 2]a and Iso to hyper-intense on T2-weighted images [Figure 2]b. Due to improvement in power, he was managed conservatively with intravenous steroids and physiotherapy. His ASIA scale improved to ASIA-D on day two and further improved to ASIA-E on day 7 postadmission. MRI after 1 month showed significant resolution of the hematoma [Figure 2]c and [Figure 2]d.
|Figure 2: Case 2-Magnetic resonance imaging dorsal spine T1 weighted sagittal view (a) and T2 weighted sagittal view (b) showing D1-D3 posterior epidural haematoma, isointense on T1-weighted image and isointense to hyper-intense on T2-weighted images. Same patient follow up Magnetic resonance imaging dorsal spine T1 sagittal view (c) and T2 weighted sagittal view (d) showing decrease in thickness of haematoma|
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A 61-year-old female presented with sudden onset back pain and weakness of bilateral lower limb for 8 days. She had a history of aortic valve replacement and she was on Warfarin therapy. On presentation, her neurological examination showed the power of MRC Grade 2 in bilateral lower limbs, and her ASIA score was ASIA-C. MRI Thoracic spine showed posterior epidural hematoma at D10 to D11 vertebrae level, compressing the spinal cord, which was hyper-intense on T2-weighted image [Figure 3]a. Due to a persistent neurological deficit, she was taken up for emergency surgery on the same day. D10 to D11 laminectomy was performed and epidural clot removed. HPE did not show any features of AVM. Postoperatively, over the next 15 days, her power improved completely in bilateral lower limb to MRC Grade 5, while her ASIA score improved to ASIA-E.
|Figure 3: Case 3-Magnetic resonance imaging dorsal spine T2 weighted sagittal view showing D10-D11 hyper-intense posterior epidural haematoma (a). Case 4-Magnetic resonance imaging lumbar spine T1 sagittal view showing L1-L4 hyper-intense posterior epidural haematoma (b)|
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A 40-year-old female presented with bilateral lower limb weakness for 1 month. She had a history of mitral valve replacement, and she was on Warfarin. She had the power of MRC Grade-1 in the bilateral lower limb, and her ASIA score was ASIA-A. MRI lumbar spine revealed posterior epidural hematoma at L1 to L4 vertebrae level compressing the cord, which was Hyper-intense on T2-weighted images [Figure 3]b. She was operated on the same day (day 30). She underwent L1 - L4 hemilaminectomy and clot removal. HPE did not show any features of AVM. Later, she was rehabilitated with postoperative physiotherapy, but her power did not improve, and her ASIA scale remained the same even after a year.
A 65-year-old female presented with neck pain and weakness in all four limbs 15 days before admission. On presentation, her neurological examination showed the power of MRC Grade 4 in all four limbs. Her ASIA score on presentation was ASIA-D. Her MRI spine showed a posterior epidural mass at C5 to C7 vertebrae level compressing the cord, which was heterogeneously Iso to Hyper-intense on T2 weighted images [Figure 4]a. A spinal Extradural tumor was suspected. She was operated on the 16th day of the presentation. She underwent C5 - C7 laminectomy. Intraoperatively, there was no tumor but only a blood clot [Figure 4]b was found. HPE was clear for any vascular malformation. Postoperatively, her power improved to MRC Grade 5, while her ASIA score improved to ASIA-E.
|Figure 4: Case 5-Magnetic resonance imaging cervical spine T2 weighted images sagittal view showing C5-C7 heterogeneous isointense to hyper-intense posterior epidural haematoma (a) and (b) Intra operative image showing epidural haematoma after C5-C7 laminectomy. Case 6-Magnetic resonance imaging dorsal spine T2 weighted images sagittal view showing D5-D10 heterogeneous isointense to hyper-intense posterior epidural haematoma (c). Intra operative image showing epidural haematoma after D5 to D10 hemi laminectomy (d)|
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A 54-year-old female presented with bilateral lower limb weakness and bladder/bowel incontinence 3 days before admission. On presentation, her neurological examination showed the power of MRC Grade-0 in the bilateral lower limb with no sensation below D-10 dermatome. Blood investigation revealed Thrombocytopenia. Her ASIA score on presentation was ASIA-A. Her MRI dorsal spine showed a posterior epidural hematoma extending from D5 to D10 vertebrae compressing the cord, which was heterogeneously Iso to Hyper-intense on T2 weighted images [Figure 4]c. Due to persistent neurological deficit, she was operated on the same day. D5-D10 Right hemilaminectomy was performed, and the clot was evacuated [Figure 4]d. HPE did not show any features of AVM. Postoperatively, only her sensory component below D-10 improved, while her ASIA score improved to ASIA-B.
| Results|| |
Our series had two male and four female patients, with a mean age of 48.5 years. One patient was of the pediatric (16 years) age group. Five patients underwent surgical intervention, and one was managed conservatively. Out of the five surgically managed cases, three patients had a good outcome (ASIA D and ASIA E). The first patient improved from ASIA C to ASIA-E. The second patient improved from ASIA B to ASIA D. The third patient improved from ASIA D to ASIA E. Rest two of the five surgically treated patients had poor recovery and outcome; the first patient improved from ASIA-A to ASIA-B, and the second patient showed no recovery. One patient was conservatively managed as he was progressively improving at presentation and showed complete recovery with a good outcome from ASIA B to ASIA-E. Three out of six patients were on blood thinners like Aspirin or Warfarin. Out of these three patients, two had a good outcome, and one patient showed no recovery with a poor outcome. Five patients had hematoma involving less than five vertebral levels, of which four patients had a good outcome. One patient had more than five vertebrae levels involvement who presented with ASIA-A and showed poor recovery to ASIA B. Out of Four patients who had hematoma located over the dorsal spine, three of them had good outcomes. Duration from ictus to surgery in our series ranges from 4 h to 30 days. Except for the patient who presented after 1 month of the incident, all other patients showed some improvement. Overall, three patients had complete, two patients had partial, and one had no recovery.
| Discussion|| |
SSEH is a rare neurosurgical emergency with an incidence of 0.1/million/year.,,, Maurizio Domenicucci, in his review of 1010 cases, classified the SEH based on etiology into iatrogenic, noniatrogenic, and multifactorial types. Spontaneous SEH includes a subset of patients from all these three groups - Iatrogenic (drug-induced coagulopathy), Non-Iatrogenic (metabolic inherited coagulopathy, vascular malformations, etc.), and multifactorial. Although SSEH is idiopathic in 40%–60% of cases, there are various other risk factors associated with the development of SSEH, such as underlying coagulopathy, vertebral hemangiomas, pregnancy, leukemia, and even systemic hypertension.,,,, SSEH is most commonly seen in the fourth and fifth decades of life.,, It is uncommon in the pediatric age group., Literature documents around 30 cases of the pediatric SSEH., Our series also had one pediatric case. SSEH is more commonly seen in men than in women (1.4:1)., It is more common in the Cervico-Thoracic or Thoraco-Lumbar regions due to more mobility and thus more tension on the spinal epidural veins.,,,
Most of the authors support the venous source of bleeding as the most common source.,,,, Independent of the source, the amount of compression is more important than the source of the hematoma. There exists a low-pressure valveless system of internal epidural plexus draining the abdomen and thorax, which may rupture when the pressure is increased during any Valsalva-like activity. The dorsal epidural space is more commonly involved, because of its larger size.,, The ventral epidural space is less commonly involved as the ventral epidural veins have support from the posterior longitudinal ligament. All of our six cases had a dorsal epidural hematoma.
Depending on the location, SSEH can present with a spectrum of symptoms ranging from sudden onset severe neck or back pain to paraplegia or even quadriplegia.,, MRI is the investigation of choice. It mainly shows a biconvex hematoma in the epidural space., During the first 24 h, the hematoma appears Isointense on T1-weighted and hyperintense on T2W images. After 24 h it appear hyper-intense on both T1 and T2-weighted images.,,,, Chronic hematomas appear Hypo-intense on both T1 and T2-weighted images.,,
The best management approach remains somewhat unclear. Urgent laminectomy and clot removal within 12 to 48 h of symptom onset has been suggested for the best possible neurological outcome in some studies.,, In our series, only one patient was operated on within 8 h from onset of symptoms, who had excellent and superior outcome (ASIA-B to ASIA-D). While the other four patients were operated after 24 h from onset of symptoms, only 50% of the patient showed good outcome. Similar results were found by Raasck et al. in which one patient who was operated on within 24 h had a good outcome, and 50% of the patients who were operated on after 24 h from the onset of symptoms had a good outcome. However, Raja et al. showed that 43% of patients operated on within 24 h from symptoms had good outcome and 50% of patients who were operated on after 24 h from the onset of symptoms had a good outcome. On the other hand, few studies did not significantly differ in outcomes based on the duration after which surgical intervention was commenced from symptom onset. As most of these studies are case reports and case series based on small numbers, the consensus is emergency or urgent surgical intervention., In our study, 60% of surgically managed cases showed good outcome. Conservative management is considered in patients who are progressively improving or with milder deficits.,, They are kept under vigilance as a spontaneous increase in the size, and worsening may later require surgical intervention.
One out of six patients presented with progressively improving ASIA scores since the onset. This patient was kept under strict observation with systemic steroids, and the patient recovered completely to ASIA-E. A similar algorithm was used by Raasck et al. while managing one of their patient who later showed spontaneous recovery to ASIA E. In the series by Dziedzic et al., five patients were managed on conservative lines; two had a multiorgan failure. The remaining three patients with incomplete cord injury showed complete recovery. Kim et al. also found results on a similar pattern where all their five conservatively managed cases showed good outcome.
The outcome concerning to drugs
Oral anticoagulant use is gaining popularity as an identifiable risk factor for SSEH. In the series published by Domenicucci, about 21.4% of patients had coagulopathy, and most of them were due to drugs. In our series, 67% of patients on drugs showed good outcomes similar to K Raasck et al. and Kim KT et al., whereas Rajz et al. showed 50% of their patients had a good outcome. However, Dziedzic et al., in their studies, stated that long-term ASIA grades were significantly better in non-bleeding disorder cases, as patients with bleeding disorders have more often complete cord injury. They found that only 20% of cases on drugs showed good outcome. This may be probably because all these patients in their series were of ASIA-A on presentation. Similarly, one patient in K Raasck et al., series on drugs showed no recovery as their initial ASIA score was ASIA-A. Favoring the initial ASIA score is more important.
The outcome concerning to vertebrae level
The compression causes damage to the spinal cord by direct pressure on the cord and its blood vessels. The parameters determining the severity of cord injury are multifactorial, including rate, depth, and duration of compression. The larger the area exposed to a hematoma, the greater the compression and poorer is the outcome. However, a thin hematoma which has spread to more vertebrae level will cause less compression. Hence, we believe that the thickness of the hematoma plays a crucial role in the outcome. About 71% of patients of Dziedzic et al., 80% of the patients of K Raasck et al., and 67% of patients of Rajz et al. showed a good outcome when more than five vertebrae levels were involved, which supports the fact that the hematoma has spread more superior-inferiorly rather than anterior-posteriorly (thickness) causing less mass effect on that segment of the spinal cord. However, in our series, we did not find similar results; about 80% of patients showed good outcomes when less than five vertebrae were involved. This may be because the hematoma was thin in all five patients when less than five vertebrae were involved. Similarly, Kim KT et al. found good outcomes in all their patients when less than five vertebrae were involved compared to 62% of patients when more than five vertebrae were involved. Had we had the dimension of the hematoma of our patients, we would have better been able to formulate accurate results.
The outcome concerning to the location
SSEH is mainly seen in the segment which has maximum mobility. The outcome is better in the Lumbar and Sacral spine than other segments, because the Lumbar area is more capacious, so lesser is the compression, and pressure tolerance is much higher at cauda equina. We found 75% of patients whose hematoma was located at the Thoracic region to have a good recovery; Rajz et al. also found somewhat similar results, whereas Dziedzic et al. and K Raasck et al. showed good outcome in patients having hematoma located at the cervicothoracic region. Also, Kim KT et al. showed that all the patients with hematoma located at the cervical or thoracic region showed good outcome, while 80% of patients with the hematoma located at the cervicothoracic region showed good outcome. The variation in results can be due to the difference in pre-surgery ASIA score or time interval of surgery from the onset.
The outcome concerning initial American spinal injury association score
Fifty percent of our patients with an initial ASIA score of A or B showed good recovery (surgery or conservative management), Kim KT et al. showed three patients with good outcome out of their five patients of ASIA A, B score, Dziedzic et al. showed two patients with good outcome out of their six patients of initial ASIA-A, B. K Raasck et al. showed similar results, of which three patients with initial ASIA score of A, B only one patient showed good recovery. Rajz et al. had eight patients with ASIA scores of A, B, but none showed good outcomes. Hence, though early surgical intervention and correction of coagulation profile would give better results, preoperative ASIA score is a more critical parameter determining the overall outcome. Thus, the individual's neurologic status on presentation is the predominant prognostic indicator.,,, Other factors include early surgery (<12–24 h), Lumbar and Sacral segment involvement, hematoma spreading more superior-inferiorly, than spreading anterior-posteriorly, will have higher chances of favorable clinical outcome.
| Conclusions|| |
SSEH is a rare condition that needs urgent attention and diagnosis. The initial clinical picture may be misleading, and in the presence of few flag history points, the attending physician must consider SSEH as one of the differentials and must obtain urgent imaging. Cases with progressive or severe neurological deficit at presentation should be managed with urgent decompression by laminectomy or laminotomy. On the contrary, those without severe neurological deficits or showing recovery during evaluation may be managed with close observation.
Unavailability of the hematoma thickness.
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.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]
[Table 1], [Table 2]