Neurocrit Care

https://doi.org/10.1007/s12028-019-00819-z

SPINAL CORD COMPRESSION

Emergency Neurological Life Support: Spinal

Cord Compression
Ryan Martin1*, Sarah Peacock2, Megan Corry3, Kerri L. LaRovere4 and Safdar A. Ansari5

© 2019 Neurocritical Care Society

Abstract
Spinal cord compression (SCC) is an acute neurological emergency that requires prompt diagnosis and management
to limit irreversible neurological injury. Patients typically present with back pain and a spinal level below which there is
neurological dysfunction. Evaluation and treatment of respiratory insufficiency are vital in the setting of quadriplegia
to prevent respiratory collapse. A brief history of the patient’s risk factors to suggest an etiology should be obtained.
Unless contraindicated, a magnetic resonance imaging of the spine should be done promptly to confirm the diagno-
sis and initiate treatment. Empiric treatment of the most likely diagnosis should be initiated if imaging is dependent
on transfer and will be delayed beyond a reasonable time frame. Here we outline the different etiologies of SCC and
provide a protocol for its rapid recognition, diagnosis, and treatment.
Keywords: ENLS, Spinal cord compression, Myelopathy, Traumatic spinal injury

Introduction patients with terminal cancer [2]. It is important to expe-

Spinal cord compression (SCC) is an emergent neu- dite workup, diagnose SCC, and deliver definitive treat-
rological condition with catastrophic sequelae if not ment, since neurological disability becomes permanent if
recognized, triaged, and managed appropriately and not treated within 48 to 72 h [3] (Fig. 1). Untreated spi-
immediately. The clinical presentation of SCC is acute nal cord injury (SCI) can lead to permanent paraplegia or
neurological dysfunction at or below the level of com- quadriplegia, with significant short- and long-term com-
pression, and the most common etiologies for SCC are plications and a devastating impact on quality of life. This
trauma, malignancy, degenerative spinal disease, epi- module is meant to give a broad framework for the prin-
dural abscess, and hematomas. All of these etiologies ciples of diagnosis and emergent management of SCC,
are mass lesions leading to extrinsic cord compression which can be adapted to reflect global and regional vari-
and can have similarities in pathophysiology, clinical ations based on the local availability of diagnostic tools
presentation, and management [1]. These disorders can and treatments.
sometimes damage the spinal vertebrae, ligaments, and
intervertebral discs, leading to compromised structural Prehospital Considerations
integrity and spinal instability. Prehospital care in the setting of neurological emer-
The true population incidence of SCC is difficult to gency varies across the world. Low-resource countries
ascertain due to a lack of accurate diagnosis and system- may rely on families to transport patients for medical
atic recording. Neoplastic SCC is considered to be the care. In countries where prehospital providers are avail-
most common etiology, and it is reported in 2.5–5% of able, care may be tailored to improve outcome. Prehos-
pital providers may encounter a patient with SCC in the
field at the time of acute presentation, or during emer-
*Correspondence: [email protected] gent transfer from an emergency department (ED) to
1
Departments of Neurological Surgery and Neurology, Davis School a specialty care facility. Initial prehospital care focuses
of Medicine, University of California, Sacramento, CA, USA
Full list of author information is available at the end of the article on determining nature of injury or illness and the need
 Fig. 1 ENLS SCC protocol

for spinal motion restriction. Immediate assessment will streamline the transition of care along the contin-
of consciousness (Glasgow coma scale) and airway, uum from prehospital to ED environments. A patient
breathing, and circulation (ABCs) will determine the who has already been diagnosed with an SCC in an ED
need for field interventions such as ventilatory support may require emergent transfer to a specialty acute care
for patients in respiratory failure or fluid/vasopressors facility. In the emergent transfer setting, the priority is
for neurogenic shock. Continuous assessment during patient-centered communication between prehospital
transport and early notification of the receiving facility personnel and the members of the transfer team.
Clinical Presentation Impairment Scale (ASIA) which was devised for trau-
The classical presentation of SCC includes back/neck matic SCC [5].
pain, bilateral weakness, or paralysis of the limbs, urinary
retention, obstipation, and a “sensory level,” defined as Acute Management
a loss of sensation below the dermatomal level of com- The checklist for the management of SCC within the first
pression. The typical acute presentation is that of “spinal hour is given in Table 2. Immediately after recognition
shock,” with flaccid paralysis, loss of reflexes, and sensa- or suspicion for SCC, spinal motion restriction should
tion below the level of compression [1]. Compression of be instituted with a cervical collar and thoracolumbar
the cauda equina can cause a similar clinical presentation motion restriction if warranted. Spinal motion restriction
with concomitant perineal (saddle) anesthesia and radic- and precautions during patient care (and transport) pre-
ular pain [4]. SCC at or above T6 can lead to hemody- vent further cord compression and injury. While optimal
namic instability secondary to loss of sympathetic tone; blood pressure is not known, spinal cord blood flow is
this leads to “neurogenic shock” with systemic hypoten- often impaired in the setting of a compressive lesion, and
sion and relative bradycardia. Common causes of SCC hypotension should be avoided.
are given in Table 1. Those patients with quadriplegia should be monitored
Spinal cord compression of the cervical segments can closely for respiratory distress and failure. Frequent
lead to quadriplegia, whereas compression below these bedside pulmonary function testing (negative inspira-
levels causes paraplegia, which is far more common. tory force and forced vital capacity), detection of dys-
Quadriplegia is the most disabling presentation of SCC phonia, and tachypnea are vital to diagnose impending
and, when present in an alert and responsive patient, respiratory collapse [6]. The patient’s own assessment of
should suggest a cervical spinal cord lesion. Cervical their respiratory status is frequently accurate but may
spinal involvement can lead to respiratory compromise be unreliable in the presence of analgesia or sedation. A
and failure, which warrants close respiratory monitoring low threshold should be maintained for placement of a
and airway support. The level of injury in SCC is deter- definitive airway and mechanical ventilation, particularly
mined by the lowest spinal cord segment with completely if rapid progression of motor weakness is observed (see
intact sensorimotor function. The most widely used ENLS Acute Non-traumatic Weakness algorithm for indi-
severity scale is the American Spinal Injury Association cations on intubation [7]).

Table 1 Common etiologies of SCC and when to suspect them

Etiology When to suspect

Trauma Sudden onset

History of recent trauma
Neoplastic disease History of cancer—particularly lung, breast, prostate, lymphoma
Nocturnal back pain
Subacute onset
Acute hemorrhage Coagulopathy—examples include:
Antiplatelet use
Anticoagulant use
Liver or renal failure
Other bleeding diathesis
Recent spinal surgery
Acute severe midline back pain
Acute disc herniation Myelopathy or radiculopathy
Improved pain when recumbent
Infection Fever
History of diabetes, cancer, immunosuppression, renal failure,
alcohol abuse, intravenous drug use
Indolent onset
Intrinsic lesions of the spinal cord Myelopathy without evidence of compression on imaging
Table 2 Checklist of SCC management in the first hour
Checklist

☐ Brief history of the patient

☐ Spinal motion restriction (motion restriction)
☐ Ensure proper ventilation, especially in the presence of quadriplegia
☐ Laboratories: CBC, Chemistries, INR/PT, PTT, platelet function assay (e.g., VerifyNow platelet reactivity profile)
☐ Obtain emergent spine imaging (MRI unless contraindicated)
☐ Alert spine surgeon
☐ Suspected neoplastic disease: administer corticosteroids (dexamethasone 10 mg IV load and 4 mg every 6 h IV/PO), contact radiation oncology
☐ Suspected epidural infection: check ESR and start antibiotics
☐ Initiate interfacility transfer if anything cannot be performed at your facility

Imaging workup of the spine should be obtained in an should include gram-negative organisms (consider anaer-
emergent fashion, enabling determination of etiology of obic coverage). Antibiotic administration should not be
SCC and definitive surgical planning and treatment. Com- delayed for imaging, transfer, or diagnostic confirmation.
puted tomography (CT) and magnetic resonance imaging The acute clinical management of patients with SCC
(MRI) are the highest yield imaging studies of the spine [8]. also includes urinary catheterization for patients with
Initial imaging should be ordered from two spinal segments urinary retention and adequate IV hydration. Appropri-
above the suspected level of injury and continue down to ate analgesia and initiation of a bowel regimen should be
the conus. Patients presenting with back pain and a clini- considered as early as possible to minimize discomfort
cal suspicion for SCC should have imaging of the complete and prevent constipation [1, 13].
spine [9–12]. CT is the diagnostic study of choice in trau-
matic SCC, as it reveals bony injuries in an efficient and Emergent Transfer
cost-effective manner. In non-traumatic SCC, CT can be Due to the rapid progression of some acute SCC syn-
limited in diagnostic ability, and MRI is the imaging study dromes, transfer agreements between EDs and other acute
of choice. Acute care and regional facilities without round- care facilities should be preestablished to avoid prolonged
the-clock MRI availability should transfer the patient to a attempts to find a facility when time is of the essence.
higher level of care in an expedited fashion. MRI with gado- Emergent transfer is warranted when the acute care facility
linium contrast can diagnose structural, infectious, inflam- treating the SCC patient is unable to provide definitive care
matory, and neoplastic pathologies and demonstrate soft (Table 2). Advanced imaging as well as specialists in neurol-
tissue abnormalities in discrete regions of the spinal canal ogy, neurosurgery, radiation oncology, and infectious dis-
(extradural, intradural, extramedullary, and intramedul- eases may be needed to ensure the best possible outcome
lary). Additionally, MRI allows the evaluation of long seg- [14]. If the circumstance arises in which there is no feasible
ments of the spine and spinal cord in a single examination. way to transfer a patient, or if the transfer will be delayed
In patients with contraindications to MRI, CT myelography significantly, the spine should be immobilized to prevent
may be used to identify SCC [9–12]. When imaging is not worsening of SCC, and empiric treatment with corticoster-
available, empiric treatment for the most likely diagnosis oids or antibiotics for the most likely diagnosis should be
should be initiated as outlined below. implemented without delay. The medical complications of
Patients presenting with clinical SCC and a history of SCI will also need to be monitored and treated, including
cancer have a high suspicion for metastases to the spine respiratory and hemodynamic instability, constipation, uri-
and should be given empiric steroids. Administration of nary retention, pain, deep vein thrombosis, and bed sores.
steroids should not be delayed for imaging, transfer, or During hospital-to-hospital transfer, communication
diagnostic confirmation (dosing discussed below under between providers is essential. Ideal communication is
neoplastic disease). Patients presenting with clinical between physicians or advanced practice providers at each
SCC and evidence of infection (fever, leukocytosis, etc.) hospital and nurse to nurse to ensure a smooth transition
or a history of intravenous drug use should be started on of care. To minimize transfer times, the acute care facility
empiric broad spectrum antibiotics after initial blood cul- should have a contractual arrangement with a transport-
tures are drawn. Antimicrobial coverage should include ing agent stipulating a maximum arrival time of less than
staphylococcus, streptococcus, and methicillin-resistant 30 to 60 min. The transporting ambulance should be able
staphylococcus aureus (MRSA). If the patient has had a to provide advance life support, including advanced air-
recent neurosurgical procedure, antimicrobial coverage way management, ventilatory, and hemodynamic support,
if needed. Some distances may be far enough away to war- of pain and temperature sensation [16]. Early cranial or
rant transport via aircraft. Empiric treatment with corti- spinal nerve dysfunction and signs of meningeal irrita-
costeroids or antibiotics should be started, even if imaging tion are suggestive of leptomeningeal metastases. This
is unavailable and diagnosis is unconfirmed. A checklist occurs in up to 8% of cancer patients and typically por-
for acute care of SCC in the first hour of recognition is tends a poor prognosis [17]. Symptoms may include
given in Table 2. A communication checklist (and sam- motor or sensory deficits in several non-contiguous sites
ple provider to provider sign-out) facilitating transfer to a in the central nervous system. These patients often have
higher level of care is given in Table 3. abnormal spinal fluid studies demonstrating pleocytosis,
elevated protein, and hypoglycorrhachia. Multiple large
Neoplastic Disease volume cerebral spinal fluid (CSF) samples for cytology
Malignant SCC is an important cause of morbidity and are required to make a diagnosis in such cases.
mortality in patients with cancer. Patients with malig- SCC can be the presenting symptom of cancer in 20%
nant SCC often endorse a long history of back pain of patients, so a preexisting knowledge of malignancy is
(initial symptom in 85–95%), with or without radicular not always evident [18]. Cancer of the lung, breast, and
radiating pain [1]. Malignant SCC-associated back pain prostate, as well as lymphoma, are the most common neo-
is worse with recumbency due to lengthening of the plasms causing malignant SCC [1]. Primary spinal cord
spine and stretch on the epidural venous plexus [15]. tumors such as ependymomas and astrocytomas are asso-
Patients with intramedullary tumors can present with ciated with intrinsic SCC, though they are significantly
symptoms of a hemicord (Brown-Sequard) syndrome, less common than metastatic extrinsic SCC. Metastases
which typically includes ipsilateral hemiplegia, loss of can occur in the intramedullary space and mimic primary
vibratory sense, and fine touch with contralateral loss spinal cord tumors; however, this is extremely rare.
Tumors like meningiomas and nerve sheath tumors
(schwannomas and neurofibromas) can arise in the intra-
Table 3 Communication checklist for SCC dural extramedullary space and cause extrinsic SCC.
Arterial hematogenous spread of malignant tumor cells
Communication
to a vertebral body leads to metastases growth and sec-
☐ Age, gender, premorbid conditions, and risk factors ondary extradural compression of the dural sac and spi-
☐ Onset and duration of symptoms nal cord [19]. Less frequently, there is contiguous spread
☐ Paraplegia or quadriplegia from para-spinal tumors; however, this occurs in 15–20%
☐ Spinal level of clinical involvement of lesions. Malignant SCC lesions most frequently pre-
☐ Vital signs sent in the thoracic spine (60%) and less frequently in the
☐ Airway status lumbosacral (30%) or cervical (10%) regions, which cor-
☐ Bowel or bladder involvement responds to the spinal segment mass and volume [20].
☐ Results of laboratory tests SCC is often gradual but can be acute when destruc-
☐ Results of imaging studies if available tion of the vertebral body leads to displaced bone frag-
☐ Medical history (i.e., cancer, intravenous drug abuse, immunosuppres- ments. Animal data indicate that direct compression of
sion) the spinal cord causes demyelination/axonal damage as
☐ Therapies initiated well as secondary damage by vascular compromise. Com-
☐ Discuss further interventions to start now pression of arterial and venous vascular structures (spe-
Sample Sign-off Narrative cifically the epidural venous plexus) leads to vasogenic
“I am transferring to you a patient who I suspect has a spinal cord spinal cord edema, blood–spinal barrier breakdown, and
compression secondary to an epidural abscess. He is a 67-year-old male
secondary SCI [15]. This supports treatment paradigms
with a history of hypertension, diabetes, and intermittent intravenous
drug use who presented 1 h ago with mid-back pain over the last targeting spinal perfusion pressure optimization.
2 weeks. Over the last 4 days, his legs have been weak, and today he Diagnosis of malignant SCC is established with a con-
was unable to walk. He appears paraplegic with a T-10 sensory level.
He has had low-grade fevers and chills since his back pain started. On
trast-enhanced MRI of the spine, which is reported to
exam, he is afebrile, blood pressure is within normal limits and he has have 100% sensitivity [1]. Upon identification of a com-
no evidence of respiratory insufficiency. Strength in his arms is normal, pressive spinal cord lesion, patients without a previous
but he is unable to lift his legs off the bed. His bladder was distended
history of cancer should have a complete blood count
and a Foley was placed. CBC and coags are normal. ESR is pending. We
do not have any spinal imaging available. Given his history of IV drug (CBC), electrolytes, liver function tests, serum light
use and recent fevers and chills, we suspect this is an epidural abscess chains, immunoglobulins and electrophoresis, urinary
leading to spinal cord compression. Empiric antibiotics were started,
Bence Jones proteins, and a chest radiograph. CT scans
including vancomycin, ceftriaxone and metronidazole. We are initiating
transfer to your facility for emergent MRI of the spine and surgical con- of the chest, abdomen, and pelvis are required to iden-
sultation. Is there anything else we should do before transfer?” tify the primary tumor. Tumor markers are not specific
enough to diagnose cancer; however, they can provide and is always delivered in conjunction with radiotherapy
useful clues if significantly elevated [21]. and surgery. It may be a viable option for patients with
Patients with malignant SCC and acute neurologi- leptomeningeal metastasis when delivered intrathecally.
cal dysfunction should receive corticosteroids. Empiric Some solid tumor metastasis like lymphoma, breast can-
treatment with corticosteroids is recommended in cer, and neuroblastoma can be chemosensitive [30, 31].
patients with known malignancy and acute SCC, even if Despite aggressive therapy, the overall prognosis for
unconfirmed by MRI spine imaging. Optimum dosing of patients with metastatic SCC is poor. While there are a
corticosteroids is not well established. An initial prospec- number of clinical scales like the Oswestry spinal risk
tive randomized trial of 57 patients randomized to high- index (OSRI) [32], Tokuhashi score, Baur score, Tomita
dose dexamethasone (96 mg IV load, followed by 96 mg score, Linden score, Rades score, and Katagiri score [33]
orally for 3 days, then a 10-day taper) vs no steroids that can be used to plan treatment and predict survival,
showed significant improved clinical outcomes (pain and prediagnosis motor function remains the most important
ambulation) in the treatment group [22]. Two smaller tri- indicator of outcome [1, 32].
als comparing high- vs low-dose corticosteroids indicate
that a loading dose of 10 mg dexamethasone (followed by Acute Hemorrhage
16 mg daily) is safer and just as effective, and has been Spinal hematomas can present with rapidly develop-
adopted at many centers [23, 24]. Given the safety pro- ing paraparesis or quadriparesis with local or radicular
file and efficacy of the lower dose of dexamethasone, we pain. Spinal epidural hematomas are the more common
recommend a dexamethasone 10 mg IV loading dose fol- compressive etiology, but intramedullary hemorrhage
lowed by 4 mg oral/IV every 6 h as maintenance. (hematomyelia) can also occur and presents similarly.
Once imaging and diagnosis are confirmed, a combina- The most common causes of non-traumatic spinal cord
tion of surgical treatment, radiotherapy, and chemother- hematoma include vascular malformations, coagulopa-
apy is recommended [25, 26]. Surgical removal of tumor thy, inflammatory myelitis, spinal tumors, and syrin-
and spinal decompression is the primary and emer- gomyelia [34]. Traumatic causes include penetrating
gent treatment for malignant SCC. This practice is sup- injuries and high-impact, blunt-force trauma, or may be
ported by a landmark prospective clinical trial involving iatrogenic as a result of postsurgical bleeding. In rare
101 patients that compared surgery and radiotherapy to cases, spinal hematoma can be a late complication after
radiotherapy alone that was stopped at interim analysis. radiotherapy, presumably due to bleeding from telangiec-
The trial showed that early surgery with circumferential tasia caused by the radiotherapy [35]. MRI with and with-
removal of the tumor, decompression of the spinal cord, out gadolinium is the modality of choice for diagnosing
and stabilization of the spine significantly improved clini- spinal hematoma, as it will demonstrate both the hema-
cal outcomes (ambulation and pain), quality of life, and toma and any associated underlying pathology, including
reduced need for narcotics and corticosteroids, with spinal cord edema [8, 36]. Conventional angiogram of the
a nonsignificant improved survival rate [27]. This trial spine may also be necessary to evaluate an underlying
used early surgery (within 24 h) and reinforces the need vascular lesion in the setting of spontaneous hemorrhage
for timely referral and surgical evaluation [27]. This trial without a clear etiology.
has been supported with more recent meta-analyses Given the relative rarity of spinal hematomas, very lit-
and systematic reviews [20, 28]. Newer, minimally inva- tle high-level evidence is available to guide therapy, with
sive neurosurgical procedures for tumor removal, spi- most approaches being empiric and based on case series
nal decompression, and stabilization offer potentially and case reports. Coagulopathy should be identified and
improved therapeutic benefit and also have a palliative reversed promptly, and while surgical decompression is
role [29]. the treatment modality of choice, conservative manage-
Radiotherapy is often used in conjunction with surgery ment can be offered in patients who make a quick and
and is also recommended alone in patients with mini- complete neurological recovery [37–39]. There is consid-
mal neurological dysfunction and high degree of tumor erable variation among surgeons regarding the timing of
responsiveness to radiation. Hematologic tumors such as surgical intervention, but several series suggest that delay
lymphoma, myeloma, and seminoma are highly radiosen- in diagnosis [40, 41] and delays in surgery more than 12 h
sitive, while breast, lung, and prostate have intermediate are associated with worse neurological outcome [37, 42,
radiosensitivity [1]. There have been significant advance- 43]. The greatest predictor of neurological recovery is the
ments in radiosurgical techniques, and all malignant SCC severity of neurological deficits upon diagnosis, but when
patients should be referred for evaluation. Chemotherapy managed promptly, between 40 and 50% of patients can
is not a mainstay for acute treatment of malignant SCC make a complete neurological recovery [37, 43].
Acute Disc Herniation osteomyelitis can also occur. Most epidural abscesses
Acute disc herniation is usually associated with spinal occur in the posterior epidural space, which contains
trauma. Non-traumatic acute disc herniation causing fat, small arteries, and the venous plexus. The anterior
myelopathy is extremely uncommon though reported epidural space is a potential space with tightly adherent
[44]. Acute disc herniation can cause myelopathy and dura and is therefore more difficult to invade. A detailed
present as hemicord (Brown-Sequard) syndrome [45]. history for risk factors for infection should be obtained,
Non-traumatic acute disc herniation more commonly including a history of diabetes, cancer, immunosuppres-
causes back pain and radiculopathy. Radiculopathy is sion, renal failure, alcohol abuse, and intravenous drug
associated with reduced sensory and/or motor function, use [1]. Staphylococcus aureus is the most commonly
at the level of injury and in a radicular pattern. Back pain reported pathogen [1, 49], though gram-negative organ-
in this setting is typically worse when standing/sitting isms, anaerobes, and Mycobacterium tuberculosis can
and improved in the recumbent position. (This is distinct also cause a spinal epidural abscess [1, 50].
from back pain associated with malignant SCC, which is It can be difficult to distinguish infections from lep-
typically worse when the patient lies down.) tomeningeal metastases, particularly in immunosup-
Acute myelopathy from disc herniation is most com- pressed patients or those with lymphoma who may be
mon in the cervical and thoracic spine and is caused by susceptible to both. Patients with infectious processes
direct compression of the spinal cord as well as compres- tend to develop cranial nerve and spinal abnormalities
sion of its blood supply. MRI demonstrates the herniated later in the course of illness as compared to patients with
disc as well as signal changes in the injured cord on T2 leptomeningeal neoplasia. Plain radiographs of the spine
signal sequences. may show abnormalities that involve two vertebral bod-
Steroids are often given to treat pain and inflammation ies across a disc space. This is suggestive of an infectious
in patients with acute disc herniation-associated myelop- process, as it is rare for metastatic tumor to cross the disc
athy and radiculopathy. Due to the rarity of this condition space.
and paucity of data, there are no well-established guide- The workup for suspected epidural abscess includes
lines on dosing for corticosteroids. Due to the absence of CBC, erythrocyte sedimentation rate (ESR), blood cul-
data, and risks of high-dose corticosteroid therapy, the tures, and preoperative laboratory studies. However,
authors recommend using a similar dosing regimen as abnormalities of the CBC and ESR are non-specific.
malignant SCC (dexamethasone 10 mg IV once, followed Two-thirds of patients have a leukocytosis, and some
by 4 mg IV/PO every 6 h). may have a highly elevated ESR [48]. However, an ESR of
Surgical evaluation should be offered in a timely and less than 20 has excellent sensitivity for excluding a diag-
urgent manner. Though there is no consensus regard- nosis of spinal epidural abscess [51]. CSF studies may
ing timing of surgery or type of decompression, most show elevated protein and leukocyte levels but may also
surgeons prefer early surgery to prevent permanent be within normal ranges. Accordingly, lumbar puncture
neurological compromise [46]. Minimally invasive thora- is generally not recommended given low sensitivity to
coscopic approaches (both anterior and lateral) have detect infection and the risk of introducing infection
reduced surgical morbidity and promise improved out- into the cerebrospinal fluid [1].
comes [46, 47]. Like spinal hematomas, little high-level evidence
exists to guide treatment strategies in patients with a
Infectious Lesion spinal epidural abscess and there is considerable vari-
Suppurative infections of the spinal epidural space can ability with regard to surgical management. However,
cause neurological injury directly, by compressing the because surgical decompression and drainage of the
spinal cord, or indirectly, by compromising blood flow. abscess are often indicated, surgical evaluation should
Unfortunately, diagnosis is often delayed because symp- be requested promptly. Antibiotics alone may be con-
toms develop with an indolent onset over weeks [1]. sidered in patients either who are neurologically intact
The classic triad of fever, back pain, and neurological or who have had complete weakness for more than
dysfunction is not seen in the majority of patients [48], 48–72 h [49, 52, 53]. In these patients, close observa-
and other symptoms may include localized back pain, tion for neurological worsening is advised given the
radiculopathy, weakness, sensory changes, and sphincter high failure rates of medical management (6–49%) [52],
dysfunction. particularly in patients with risk factors for neurologi-
The mechanism of spread is often hematogenous, but cal worsening (diabetes, C-reactive protein > 115, white
only half of patients have a site of infection remote to blood cell > 12, age > 65, positive blood cultures, MRSA)
the spine [1]. Direct spread of infection from vertebral [49, 52]. In patients with neurological deficits, early
decompression, irrigation, and debridement are the Spinal cord injury without radiographic abnormal-
mainstay of treatment [49]. ity (SCIWORA) is a clinical diagnosis made in the set-
Abscesses may be multi-microbial; therefore, vancomy- ting of trauma and spinal cord dysfunction without an
cin and a third or fourth generation cephalosporin should MRI abnormality. The cervical spinal cord is most likely
be started to cover MRSA and gram-negative organisms, affected due to increased mobility of the cervical spine.
respectively. Anaerobic coverage with metronidazole Children are high risk of this injury due to the relatively
may also be considered. In patients with negative blood large head-to-body ratio in childhood. Treatment is
cultures, CT-guided aspiration of the epidural space is non-surgical due to the absence of a surgical lesion. Sup-
occasionally used to obtain cultures and decompress portive measures include spinal motion restriction with
mass effect. Such cultures should be sent for aerobic and collars, braces, or orthosis for up to 3 months. The use of
anaerobic bacteria, fungi, and tuberculosis. However, corticosteroids in this setting should be carefully evalu-
cultures remain negative in 20–40% of cases. The recom- ated with inherent risks and used judiciously [55, 56].
mendation to delay antibiotics until cultures are obtained
in patients who have a mechanically stable spine and a Pediatric Considerations
stable neurological examination is controversial [54]. There are some unique etiologies of SCC in children.
Genetic conditions associated with cervical instability
Intrinsic Lesions of the Spinal Cord (No Extrinsic from odontoid hypoplasia or ligamentous laxity include
Compression) trisomy 21, Klippel–Feil syndrome, skeletal dysplasia, and
Patients with acute symptoms of spinal cord dysfunction mucopolysaccharidoses. Atlantoaxial instability can pro-
can have intrinsic or intramedullary (non-compressive) gress to subluxation/dislocation, myelopathy, and quad-
abnormalities of the spinal cord on MRI. These include riparesis. Children with these genetic conditions may
spinal infarct, inflammatory/demyelinating myelitis, have multiple pathologies that can affect spine function,
infectious, and para-infectious disease. such as syringomyelia, tethered cord, myelomalacia, or
Spinal cord ischemia may require further imag- segmental cord atrophy. For this reason, a pediatric neu-
ing with spinal angiography to identify arteriovenous rosurgeon and orthopedic surgeon may operate together
malformation, arterial occlusion, or other vascular in order to achieve safe and adequate decompression
abnormalities. Serum and CSF studies should be sent and stabilization. Malignant SCC can result from vari-
for evidence of autoimmune and infectious vasculitis. ous types of pediatric cancers including neuroblastoma,
Serum studies should include serology for Lyme, syphi- lymphoma, leukemia, osteosarcoma, posterior fossa or
lis, human immunodeficiency virus (HIV), and nutri- brain stem tumors, metastatic tumors, pheochromocy-
tional deficiencies including vitamin B12. CSF should be toma, and spinal cord tumors. Back pain in a child with
tested for viral studies including: herpes simplex virus a known history of malignancy should be considered a
1, 2 polymerase chain reaction (HSV PCR), cytomeg- red flag symptom for malignant SCC until proven other-
alovirus (CMV) PCR, and varicella–zoster virus (VZV) wise. A 1–2 mg/kg bolus of dexamethasone IV should be
IgM and IgG. Immunoglobulin indices should be evalu- given once the diagnosis is confirmed radiographically,
ated including (but not limited to): IgG index, albumin followed by 0.25–0.5 mg/kg oral/IV every 6 h. Decom-
index, IgG synthesis rate and, oligoclonal bands. Urine pressive surgery should be considered for severe spinal
should be screened for copper deficiency (see ENLS instability, rapidly progressive symptoms, or progressive
Acute Non-Traumatic Weakness protocol [7]). symptoms despite therapy.
Spinal trauma due to abusive mechanisms is another
Negative Imaging unique cause of SCC in children. Mechanisms of spinal
Negative spinal MRI in a patient with acute disturbance injury in child abuse include acceleration/deceleration
of motor and or sensory function (quadriplegia or para- rotational forces on the brain and neck from shaking
plegia) should prompt evaluation for acute neuropathy, with or without direct impact. Non-accidental inflicted
neuromuscular junction disorders, and myopathy. Clini- trauma results in a constellation of distracting injuries,
cal presentation, CSF, and electrophysiologic studies may including orthopedic and solid organ injuries. The most
establish a diagnosis of Guillain–Barre syndrome (acute information about brain and spine injury is often gleaned
polyradiculoneuropathy), Myasthenia Gravis, Lambert– from MRI. The presence of spinal subdural hematoma
Eaton syndrome, or motor neuron disease (see ENLS (SDH) on imaging is highly specific for an abusive etiol-
Acute Non-Traumatic Weakness protocol [7]). ogy [57].
 The child with acute SCC requires a multi-disciplinary by serial assessment of blood pressure, perfusion, and
team that includes pediatric specialists in orthopedic hematocrit. Appropriate vasopressors, inotropes, and
surgery, neurosurgery, neurology, anesthesiology, and anesthetic and sedative medications should be selected
critical care medicine who are familiar with these unique to avoid worsening of any hemodynamic instability.
etiologies. These experts have experience in managing Given compromised spinal cord blood flow in the setting
the airway, hemodynamic changes, and complications of cord compression, hypotension should be avoided by
that often accompany SCC in children. Once this diag- targeting a mean arterial pressure (MAP) at least at the
nosis is suspected, the child should be transported to the 50th percentile for age, which can be calculated using
closest facility with these capabilities. the formula MAP = 55 + (1.5 × age in years) [58]. Similar
When stabilizing the cervical spine in a neutral posi- to adults, the mainstay of treatment of SCC in children
tion in the prehospital setting, infants and young children depends on the underlying etiology, restriction of spinal
should have a pad placed under their back up to the scap- cord motion, avoiding secondary insult, and operative
ulae to account for their disproportionately large occiput. management in select cases.
This maneuver will help prevent neck flexion which can
result from resting on a flat surface. The more cephalad Nursing Communication
the compression, the more likely there will be concern Nursing care plays an important role in the manage-
for compromise of respiratory and cardiovascular func- ment of SCC. After the diagnosis of this emergency, the
tion. Since cardiac output is more heart rate dependent role of the nurse is to assist the providers with stabiliz-
in children compared to adults, bradycardia that accom- ing the patient’s clinical status and discussing strategies
panies SCI above T6 should be treated aggressively. to prevent further complications. Nurses should be edu-
Medications such as atropine, dopamine, or epinephrine cated on how to perform a complete neurological assess-
increase heart rate (the catecholamines also increase vas- ment. Establishing a baseline neurological examination is
cular tone) and are preferred in this clinical scenario. essential to detect clinical improvement or deterioration.
Similar to adults, the initial priorities for a child with In addition to closely monitoring the patient’s neurologi-
acute SCC are airway maintenance with spinal motion cal status, nursing care for the patient with SCC includes
restriction, breathing and ventilation, and circulation ensuring spinal motion restriction, monitoring respira-
with hemorrhage control. Any child who is unable to tory and hemodynamic status, assessing bowel and blad-
open their eyes or verbalize should be considered a can- der dysfunction, and taking proper precautions with
didate for airway intubation because of concern about patient movement.
absent or impaired airway protective reflexes. Precau- Neurological examination monitoring should be per-
tions to restrict cervical spine motion associated with formed with a consistent grading tool to be able to moni-
intubation should be taken. Spinal trauma due to abusive tor and communicate any changes in the examination
mechanisms or a complete SCI above C3 can result in between the nurse and the providers. The examination
apnea and cardiac arrest. After airway and ventilation are should be performed hourly when the patient is first
confirmed as being adequate, circulation must be opti- admitted, any time the patient has been moved out of
mized with attention to identifying shock (e.g., hypov- bed, or if the nurse suspects any type of deterioration.
olemic, cardiogenic, and neurogenic). Neurogenic shock If the patient requires spinal motion restriction, nurses
may be present in cases of SCC above T6 and reflects should use proper precautions when moving the patient
sympathetic denervation of the heart and vasculature and always use the log-roll technique with an adequate
with resulting decreased inotropism, chronotropism, amount of staff to maintain proper spinal alignment.
and arterial and venous dilation. Lack of compensatory Patients who present with quadriplegia or cervical SCC
tachycardia is also a useful way to differentiate neuro- are high risk of respiratory impairment. Close monitoring
genic shock from hypovolemia in children. In children, by the nurse of the patient’s respiratory status including
hypotension is defined as systolic blood pressure (SBP) respiratory rate, pattern of breathing, depth of respira-
below the 5th percentile for age or by clinical signs of tions, and cough strength and effectiveness are essential
shock. The 5th percentile for age of SBP can be estimated to recognize patients whose respiratory status is decom-
by: 70 mm Hg + [2 × age in years]. If necessary, an intra- pensating and those who may require endotracheal intu-
venous bolus of 20 mL/kg 0.9% sodium chloride solu- bation. SCC at or above the T6 level is at risk to develop
tion should be administered as soon as vascular access is hemodynamic instability and requires close monitoring
obtained. Subsequent boluses of fluid should be guided
of the patient’s vital signs including heart rate and blood 7. Caulfield AF, Flower O, Pineda JA, Uddin S. Emergency neurological life
support: acute non-traumatic weakness. Neurocrit Care. 2017;27(Suppl
pressure with prompt communication with the provider 1):29–50.
team if any change in values. 8. Distefano D, Aless Cianfoni R. Imaging of spinal cord compression. Imag-
ing Med. 2014;6(1):89–116.
9. Bammer R, Fazekas F. Diffusion imaging of the human spinal cord and the
Clinical pearls
vertebral column. Top Magn Reson Imaging. 2003;14(6):461–76.
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• Empiric treatment for infectious or malignant causes of SCC (with spinal cord compression: a study of progression from first symptom
appropriate antibiotics or corticosteroids) is recommended if delay in until diagnosis in 153 patients. Eur J Cancer (Oxford, England: 1990).
MRI imaging is unavoidable 1994;30A(3):396–8.
• The authors recommend a dexamethasone 10 mg IV loading dose 13. Loblaw DA, Perry J, Chambers A, Laperriere NJ. Systematic review of
followed by 4 mg oral/IV every 6 h as maintenance, in the setting of the diagnosis and management of malignant extradural spinal cord
malignant SCC (can also be considered in acute disc herniation) compression: the Cancer Care Ontario Practice Guidelines Initiative’s
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1
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The authors are grateful for the contributions and insight provided by the surgery versus conventional radiotherapy for the treatment of metastatic
following reviewers: Scott Thomas May, PharmD, BCPS, BCCCP; and Christi spinal epidural disease. Neuro-oncology. 2005;7(1):64–76.
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Publisher’s Note 22. **Sørensen S, Helweg-Larsen S, Mouridsen H, Hansen HH. Effect of
Springer Nature remains neutral with regard to jurisdictional claims in pub- high-dose dexamethasone in carcinomatous metastatic spinal cord
lished maps and institutional affiliations. compression treated with radiotherapy: a randomised trial. Eur J Cancer
(Oxford, England: 1990). 1994;30A(1):22–7. This is a landmark trial showing
significantly improved clinical outcomes (pain and ambulation) in patients
with malignant SCC treated with high-dose corticosteroids.
23. Graham PH, Capp A, Delaney G, et al. A pilot randomised comparison
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