Alton Daley DO¹; Brandon Klein DO, MBA²; Petros Koutsogiannis DO³; Andrew Tarleton MD⁴; Kanwarpaul Grewal DO^5
1Dartmouth-Hitchcock Medical Center
²Northwell Health at Huntington Hospital, Department of Orthopaedic Surgery, Huntington NY 11743
³Corewell Health’s Beaumont Hospital at Royal Oak, Department of Orthopaedic Surgery, Royal Oak MI 48073 
⁴Orlin & Cohen Orthopedic Group, Garden City NY 11530
⁵Grewal Orthopedic & Spine Care, East Meadow NY 11554

Abstract

Case
A 25-year-old male presented with acute-onset lower extremity motor weakness, loss of sensation below T4, and urinary retention that began an hour prior to presentation. MRI revealed a fluid collection in epidural space extending from C6-T2.  Surgical laminectomy was performed followed by evacuation of the collection. Neurological improvement was noted within one hour of surgery.  Complete return of motor activity and sensation were obtained at 1-month follow up.  

Conclusion
Spontaneous spinal epidural hematomas are a rare etiology of rapid onset neurological deficits in the absence of identifiable cause. Complete recovery of neurological function can be achieved with rapid surgical epidural decompression.

Keywords: Spontaneous Epidural Hematoma, ASIA Scale, Epidural Hematoma, Spine, Trauma

Introduction
Spinal epidural hematomas (SEHs) are a rare. clinical entity, occurring at a rate of only 0.1 patients per 100,000 population (Holtas, 1996). Cited etiologies include coagulopathies, anticoagulant use, arteriovenous malformations, neoplasms, infections, trauma, or iatrogenic causes (Baek, 2008; Baeesa, 2019; Kreppel, 2003; Holtas, 1996). However, in many reported cases, the rupture of an epidural vessel occurs in the absence of underlying cause or risk factor, leading to an idiopathic spontaneous SEH (Munoz, 2015; Zhan, 2008; Golpakrishnan, 2012).  

Spontaneous SEHs have a wide range of clinical presentations.  The majority of cases present with axial pain combined with associated neurological deficits from spinal cord compression. These predominately include upper motor neuron signs, paresis, decreased or absent reflexes, overflow bladder incontinence and/or bowel incontinence (Mutair, 2010; Kato, 2021; Liu, 2007). Magnetic resonance imaging (MRI) is critical for early diagnosis, as literature has demonstrated that maximum neurological recovery is achieved when treatment is initiated within 12 hours of symptom onset (Groen, 1996; Shin, 2006; Raask, 2017).

In most cases, emergent surgical laminectomy is performed, with hematoma evacuation to decompress the involved spinal cord segments. Spinal fusion may also be performed depending on the level of involvement. If deficits are limited, or if the patient exhibits early spontaneous return of neurologic function prior to intervention, literature has demonstrated adequate outcomes with conservative management. In these cases, patients should be serially monitored at facilities with capacity for swift spinal intervention due to potential for hematoma progression (Groen, 2004; Raask, 2017). Prognosis is best when diagnosis is made prior to the development of severe neurological deficits and when time-to-intervention is minimized (Groen, 1996; Shin, 2006; Raask, 2017). 

Here, we describe the presentation and management of a patient with spontaneous SEH focused on the severity of neurological deficits at presentation, the timing of diagnosis and the importance of early intervention. The patient consented that the details of his case be outlined and submitted for publication. 

Case Report
A 25-year-old male, employed as a local orthopaedic x-ray technician, presented with acute-onset lower extremity weakness, numbness, and back pain upon waking, approximately one hour prior to presentation. His symptoms were preceded by a two-week history of back spasms noticed after a resistance training workout. He attributed the spasms to soreness, remained functional, and did not seek out medical evaluation. The patient denied a history of traumatic events, intravenous drug use, infectious symptoms, or a history (personal or familial) of coagulopathic disorders. 

Initial evaluation by our emergency provider occurred two and a half hours after symptom onset, and revealed complete loss of sensation below the level of T4, no muscle tone in the lower extremities, and absent patellar and Achilles reflexes. Laboratory evaluation, including complete blood count, complete metabolic panel, erythrocyte sedimentation rate, c-reactive protein, coagulation studies, and blood cultures were normal. Vital signs were stable except for mild hypertension. One hour after initial evaluation, the patient underwent a non-contrast MRI of the thoracic and lumbar spine for concern of cord compression. The MRI revealed a dorsal epidural mass extending from C6-T2 with peripheral enhancement. The delay in time-from-evaluation to initial imaging was secondary to the lack of a 24-hour in-house MRI technician. 

One hour after the MRI was performed, four and a half hours after onset of symptoms, consultation was called to the orthopaedic surgery department. Complete neurological examination revealed loss of sensation below the nipple line, corresponding to a T4 sensory level.  There was 1⁄5 subjective sensation with complete loss of motor function (0/5) from L2-S1 bilaterally. An MRI of the cervical, thoracic and lumbar spine with and without contrast was then obtained to aid in surgical planning, evaluate for skip lesions, and further evaluate the collection and spinal levels involved.  Findings were negative for skip lesions but confirmed the presence of an iso-to-hyperintense fluid collection in the epidural space from C6-T2 with evident spinal cord compression (Figure 1).  

Figure 1 – T2-weighted MRI findings of an iso-to-hyperintense fluid collection with spinal cord compression at the levels of C6-T2.

Upon confirmation, the patient was consented for surgical laminectomy and spinal cord decompression and potential spinal fusion for stabilization. Preoperative antibiotics were held in order to allow intra-operative cultures to be obtained, and the patient was given dexamethasone per physician preference. A Foley catheter was placed after an elevated post-void residual volume was obtained (502cc).  

The patient was brought to the operating room within 9-hours from initial symptom onset. Neuromuscular monitoring confirmed the absence of motor signals in the lower extremities, with the maintenance of mild sensory signals. The involved levels were exposed using a posterior midline approach (ligamentum nuchae) to visualize lamina from C5-T2. The lateral masses from C5-C7 and transverse processes at the T1-T2 level were exposed and instrumentation tracks were placed for potential cervicothoracic stabilization. 

A clamshell technique was then used to perform laminectomy from C5-T2. After resection of the ligamentum flavum, a gush of approximately 100mL of blood exited the entire length of the decompressed epidural space and an impressive hematoma was visualized and removed (Figure 2). Areas of matured hematoma were identified and evacuated with the use of Woodson elevator and neurolysis technique until the thecal sac was clearly visualized (Figure 3). The wound was irrigated, and multiple cultures were collected, all of which resulted in no growth. 

Figure 2 (left) and Figure 3 (right) demonstrate intraoperative images of the original epidural hematoma (Figure 2) and the decompressed spinal region after exposure, identification and  evacuation (Figure 3).

With a four-level laminectomy crossing the cervicothoracic junction needed to decompresses the hematoma, the decision was made to proceed with posterior instrumented fusion (C5-T2) to help reduce the risk of post laminectomy kyphosis (Cho, 2008. Deutsch, 2003).  (Figure 4). Lateral mass screws were placed from C5-C7 with pedicle screws at T1-T2. Instrumentation was placed under open free hand technique with the assistance of fluoroscopy. Neuromonitoring was used during the whole case, and pedicle screws at T1 and T2 underwent EMG stimulation to rule out any potential breech. Bilateral rods were inserted and set screws were placed. A mixture of autologous lamina collected during laminectomy, and morselized allograft, was used to create a lateral fusion mass. A complex closure of the paraspinal muscles, ligamentum nuchae, posterior cervical and thoracic fascia was performed with interrupted #1 Vicryl suture (Johnson & Johnson, Warsaw, IN). The skin was closed with running 3-0 Monocryl followed by Nylon sutures (Johnson & Johnson, Warsaw IN). A Jackson-Pratt (JP) drain was inserted to prevent re-accumulation of fluid. The patient was successfully extubated and transferred to the ICU for close monitoring. 

Figure 4 – Intra-operative fluoroscopy showing posterior instrumented fusion of the C5-T2 levels. 

One hour postoperatively, the patient exhibited a return of dorsiflexion and plantarflexion of the right foot. On postoperative day 2, motor activity in the left lower extremity improved to 4/5 from L4-S1, he was able to ambulate for a few steps with physical therapy, and sensation was present throughout, but altered throughout the entire lower extremities. The patient was discharged from rehab on postoperative day 5. 

At 1-week, 3-months, 6-months, 9-months, and 1-year postoperatively, the patient was contacted, and a Self-Reported Spinal Cord Independence Measure (SCIM-SR) score was calculated to evaluate his ability to achieve activities of daily living (ADLs) (Fekete, 2012). Scores were calculated on a scale of 0-100, with a 100 indicating the ability to carry out all ADLs without assistance. At 1-month, his score was 98 with limitations only in transiting from the lying position to long (sitting with legs extended) or ring-sit position (sitting with legs bent in a circle position). At 3-months, his score improved to 100, and he reported use of a stationary bike for an hour at a time. At 6-months, he had returned to work, limited only to lifting to less than 20 pounds.  At 1-year, he returned to normal activities and was lifting 50-60 pounds without issue. 

Discussion
Spontaneous spinal epidural hematoma is a rare cause of acute spinal cord compression that occurs in the absence of an identifiable risk factor. While cases of spontaneous SEH have been previously reported, questions remain regarding the idiopathic nature of the disease (Raask, 2017. Liao, 2000). Given a disease-related mortality of 5.7%, and persistent sensorimotor deficits in up to 50% of patients, it is essential to quickly diagnose and risk stratify patients in order to prevent rapid deterioration of neurological status, determine proper treatment decisions, and achieve optimal neurological recovery (Liao, 2004).  

While presenting symptoms vary, the diagnosis of spontaneous SEH should be suspected in patients who present with acute back pain with associated neurological deficits. Neurologic deficits depend on the level of involvement, and may include some combination of paresis, decreased or absent reflexes, and bladder and/or bowel retention leading to overflow incontinence. The neurological status at presentation should be documented by using the American Spinal Injury Association Impairment (ASIA) grading system (Table 1) (Kirshblum, 2011). The ASIA grading system is used to assess the severity and level of spinal cord injury, which may impact management. Our case presented with findings consistent with ASIA grade B, as our patient experienced complete motor loss in the lower extremities with mild preservation of sensation. 

Table 1 – ASIA Grading Scale 

When suspected, MRI should be expedited as time to surgery has been shown to be a strong predictor of post-operative return of neurological function. Previous literature has commonly cited 12-hours as the ideal time interval for decompression to be performed (Shin, 2006. Liao, 2004. Lawton, 1995). In the presented case, the patient was brought to the operating room less than 9-hours after the onset of symptoms, and within 4-hours of orthopaedic surgery consultation. The most common locations for SSEH are the cervicothoracic and thoracolumbar regions due to the high stress observed at junctional zones of the spine (Barwar, 2022.  Foo, 1997). MRI imaging may provide insight on the onset of pathology, as different phases may be determined based on the presence of intracellular oxyhaemoglobin. In the hyperacute (<24 hour) phase, hematoma appears hypointense on T1-weighted sequences and hyperintense on T2-sequences. Beyond this time, T1-sequences become more intense and progress to isointensity, while T2-sequences lose intensity and become hypointense (Moriarty, 2019).  

While no firm clinical guidelines exist, patients with confirmed spontaneous SEH with severe neurological deficits (ASIA A, B, or C) and/or rapid progression of symptoms should be surgically managed with emergent laminectomy and hematoma evacuation to maximize the odds of neurological recovery. Again, early management is vital, as prognosis after surgery relies primarily on preoperative neurological status and ictus to surgery (Shin, 2006. Liao, 2004. Foo, 1981). In their review of 35 patients with SSEH treated operatively, Liao et al reported that 89.9% of patients with incomplete neurological deficits achieved complete recovery compared to 37.5% of those with complete deficits (Liao 2004). These recovery rates are similar to those presented in other literature (Raask, 2017. Lawton, 1995). However, the importance of symptom onset to surgery of ideally within 12-hours cannot be overstated, as even those with complete deficits can achieve full recovery if intervention is performed without delay (Papadopolous, 2002). To this point, early surgical intervention in our case allowed for improvement from an initial ASIA grade of B to an ASIA grade of E at 1-year follow-up, indicating complete neurological recovery.  

Nonoperative management with conservative observation should only be considered in patients with less severe neurological symptoms (ASIA D) or resolving neurological deficits at the time of presentation (Liao, 2009. Raask, 2017). Raask et al reported that of 8 patients with ASIA scores of D treated conservatively, 73% made a full recovery (Raask, 2017). However, due to the risk of hematoma expansion, patients should only be managed conservatively in centers with easy access to MRI and neurosurgical teams as surgical intervention may eventually be required.

Figure 1 | Figure 2 | Figure 3 | Figure 4 | Figure 5 | Table 1

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