Spinal Cord Injury Without Radiological Abnormality

Disease/Disorder

Definition

Spinal cord injury without radiological abnormality (SCIWORA) involves symptoms of a traumatic myelopathy without evidence of fracture or ligamentous instability on radiograph or computed tomography (CT).¹ The term was coined prior to the availability of magnetic resonance imaging (MRI) yet remains applicable given the typical sequence of imaging in a trauma setting.²

Etiology

In younger children, SCIWORA is most commonly the result of high-energy injuries, such as motor vehicle collisions followed by falls.^3,4 In older children, sports injuries (such as seen in collision sports) may be more common, along with motor vehicle collisions and falls.²

Epidemiology including risk factors and primary prevention

The incidence of SCIWORA in patients with spinal cord injury (SCI) is difficult to elucidate. When considering all spinal injuries, SCIWORA makes up 13-19% of cases in children and 10-12% of cases in adults.² Due to the increasing use of MRI in diagnosing spinal cord injuries and advancements in imaging technology, the number of reported cases of SCIWORA has increased by 64% in recent years.⁵

Up to 90% of documented cases of SCIWORA are in children < 18 years old.⁶  SCIWORA is more common in children 8 years of age and younger and less common in older children and adults.⁷ Younger children are at higher risk due to large head-to-body ratio, weak neck musculature, ligamentous laxity, increased mobility of the cervical spine, and horizontally-oriented facet joints.²

SCIWORA typically involves the cervical spine, and less commonly involves the thoracolumbar spine.³ From birth to age 8, injury tends to occur in the upper cervical spine and after age 9 tends to occur in the lower cervical spine, due to a decrease in head-to-body ratio and strengthening of neck musculature with age which shifts the fulcrum of movement lower in the cervical spine.² Risk factors are essentially the same as with other forms of SCI, such as motor vehicle accidents, sports related injuries, and falls. Tight filum terminale and tethered cord syndrome may be predisposing factors to SCIWORA due to chronic spinal cord traction; however, this is currently still being investigated.⁸  In the pediatric population, similar to the adult population, males are more likely to sustain cervical SCIWORA than females at a ratio of 1.5-2:1.⁹

Primary prevention programs, such as the ThinkFirst Foundation, have helped to lower the overall incidence of SCI in children.

Patho-anatomy/physiology

Four mechanisms of injury are associated with the pathogenesis of SCIWORA: hyperextension, hyperflexion, distraction, and cord ischemia.⁴ The following theories of the etiology are reported:

• Segmental spinal instability: increased elasticity of the pediatric spinal ligaments allow for instability events and damage to the spinal cord, whereas the elastic soft tissues remain intact. Subsequent auto reduction of the spinal column explains the absence of structural abnormalities on imaging. Anatomically, horizontally-oriented facets, anterior vertebral wedging, weak neck musculature and large head-to-trunk ratio contribute to overall spinal instability in the pediatric population.¹
• Differential stretch hypothesis: higher water content allows pediatric intervertebral disks to stretch and expand. This allows significant distraction of the spinal column and cord, leading to injury. The spinal column can stretch up to 7cm but there is injury to the cord itself after 1cm.³
• Vascular injury: traumatic injury of the vascular supply to the spinal cord results in cord ischemia.
• Cord contusion: injury to the spinal cord via force transmission through the intact spinal column.
• Burn injury: injury to the spinal cord from electric current

Disease progression including natural history, disease phases or stages, disease trajectory (clinical features and presentation over time)

One quarter of affected children may experience delayed onset of neurologic signs, ranging from complete paralysis to partial neurologic deficits. The latent period may range from 30 minutes to 4 days.¹⁰ The clinical onset of symptoms typically occurs within the first 48 hours post-trauma in only 50% of patients and is usually delayed.¹ Thus, prompt evaluation with imaging should occur up to 4 days after initial injury if transient neurologic symptoms are reported.The main reported initial symptom is neurologic change in the upper extremity.

• Between 15% and 32% have severe or complete neurologic injury, as defined by the International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI)³
• Children who present with a complete injury are less likely to regain motor, sensory, bowel, and bladder function.
• Children with incomplete (sacral sparing) injury may regain some motor or sensory function.
• Children with mild initial symptoms and those without MRI abnormalities (35%) typically recover fully.⁷
• Recurrent SCIWORA
  • Rare (incidence 1% to 4%).
  • Can occur within hours to years after injury.
  • Usually occurs in children older than 8 years.
  • Often a low energy mechanism with mild initial symptoms.
  • Many patients fully recover,³ although some may have severe neurologic injury.^3,7

Specific secondary or associated conditions and complications

Associated Conditions

• Paralysis of the diaphragm and respiratory distress
• Sensory loss
• Neurogenic bowel and bladder

Complications

• Autonomic dysreflexia
• Paralytic ileus
• Skin breakdown
• Pneumonia
• Urinary tract infections
• Immobilization hypercalcemia
• Loss of muscle mass
• Spasticity and joint contractures
• Chronic pain

Essentials of Assessment

History

A detailed history should focus on the nature of trauma, presence of pain, numbness/paresthesia, and weakness, changes to bladder and bowel function, and preinjury level of function.

Physical examination

A detailed ISNCSCI examination should be completed as age allows. Results of the ISNCSCI exam determine neurologic level of injury and completeness of injury. A general exam, including respiratory, cardiovascular, abdomen, extremities, cranial nerves, and reflexes (bulbocavernosus, abdominal, deep tendon, and Babinski) and an assessment of other associated injuries, such as head injury, long bone, rib, and clavicular fractures, are essential.

SCIWORA may present as definite evidence of neurologic injury on examination, as manifested by abnormal vital signs (e.g., apnea or bradycardia with hypotension [spinal shock]), neck or back pain, and paresthesias, paralysis, or loss of sensation. Transient neurologic symptoms (e.g., paresthesias, weakness) by history may be the only indication that the cervical or thoracic spinal cord is injured.

Functional assessment

Functional assessment includes general mobility, ability to complete transfers, potential for ambulation , self-care skills, bowel and bladder function, and toileting. A neuropsychologic evaluation is essential if concomitant brain injury is suspected. The Functional Independence Measure for Children (WeeFIM) is used to measure functional performance in children between the ages of 3 and 8 years.¹¹ In older children (8-18 years), the WeeFIM or Functional Independence Measure (FIM) may be utilized. Additionally, the third version of the Spinal Cord Independence Measure (SCIM III) may be used to quantify the functional status in older children. The validity and reliability of a fourth edition of the SCIM is currently being studied.¹²

Laboratory studies

There is no laboratory workup specific to SCIWORA. Laboratory studies relevant to SCI should be ordered as clinically indicated.

Imaging

Plain radiographs and CT images of the entire spine are essential to investigate for fracture, dislocation, bleeding, and other associated injuries. However, 90% of SCIWORA cases are spinal cord injuries that appear normal on both plain radiographs and CT scans but show pathology on MRI.¹³  There is evidence to suggest that MRI of the cervical spine should be done 72 hours after trauma, as it is more likely to show abnormalities compared to MRI done on admission due to the damaging effects of delayed secondary inflammatory reactions and edema on the spinal cord.² However, there remains no consensus on the best timing for MRI of the spine and no consensus on if or when a follow up MRI should be done if the initial MRI was negative for abnormalities. MRI of the brain may be indicated if brain injury is suspected.

If standard imaging studies show no fracture or dislocation, flexion-extension radiographs are obtained to detect ligamentous instability.

Supplemental assessment tools

Somatosensory evoked potentials (SSEPs) are adjunct to the previously mentioned studies. In a comatose patient with suspected SCIWORA, SSEPs are helpful to assess for cord injury and posterior column conduction.

Early predictions of outcomes

• Initial injury: high-energy mechanisms, thoracic involvement, younger age, and complete injury portend a poor prognosis. In patients with complete injuries, any neurologic recovery is rare. Patients with incomplete injuries tend to improve with time.
• MRI findings:
  • Absence of MRI abnormalities is associated with better prognosis and recovery rate²
  • Signal change consistent with edema or microhemorrhages is associated with improvement in neurologic function over time in some patients.
• Age at injury: younger patients have worse injuries.
• Early neurologic recovery, within the first three days, may be associated with better long-term outcomes.⁴

Environmental

In anticipation of community reintegration, issues regarding accessibility at home and school should be explored.

Social role and social support system

A family/guardian support system at home, such as caregivers, finances, transportation, and accessibility to medical care, is important for community reintegration.

Professional issues

Among those unfamiliar with SCIWORA, normal structural imaging of the spine may lead to an erroneous conclusion that there is no SCI, leading to inappropriate discontinuation of spinal precautions/bracing. Such decisions could have medicolegal ramifications. Patients with neurologic symptoms after trauma should be evaluated with MRI. Comatose children in whom SCI is a possibility should remain immobilized until an MRI is obtained and an adequate exam can be performed.

Rehabilitation Management and Treatments

Available or current treatment guidelines

• External immobilization is recommended until spinal stability is confirmed by flexion/extension radiographs.²
• Avoidance of high-risk activities, such as gymnastics, diving, down-hill skiing, equestrianism, tackle football, wrestling, and boxing,for up to 6 months, is recommended. ³ Patients with evidence of ligament damage, worsening or no improvement of neurological lesions, compression of the spinal cord, and spinal instability should be considered for surgical intervention. Anterior cervical decompression has been shown to improve the prognosis of  SCIWORA in patients with persistent spinal cord instability and compression on MRI.⁴
• There are no established treatment guidelines specifically for SCIWORA, and the evidence is insufficient to support treatment standards or guidelines.

At different disease stages

Acute and Rehabilitation Admission Phase

• Bracing: There are no established guidelines to determine the length of spine bracing. Spine bracing may be up to 12 weeks, and discontinuation when instability is ruled out and tenderness resolves. A hard cervical collar is recommended for 1-2 weeks after neurological deficits have resolved.
• Medical stabilization.
• Early admission to a specialized CI center can decrease length of stay, severity of complications, and mortality. 
• Physical and occupational therapy for mobility, activities of daily living, and bowel and bladder training for both the patient and the family is needed, based on age and deficits.
• Prevention of secondary complications: education of nurses, the patient, and family about pressure relief, bladder and bowel programs, pain control, autonomic instability, and deep venous thrombosis prophylaxis.
• Necessary modifications to discharge destination for safety and access to available community resources.
• Family education about nature of injury, reasonable expectations, and prognosis.

Subacute to Chronic Management

• Prevention of secondary complications.
• Monitor for resolution of initial presenting deficits.
• Multidisciplinary approach to treatment is necessary for successful community reintegration.
• Social support becomes very important for individuals with permanent deficits. Utilization of reputable online discussion groups and in-person support groups can be an important source of information and support for the patient and their families.
• Health maintenance and return to school and avocational activities.
• The need for repeat MRI for stability monitoring after 3 months of bracing is determined on an individual basis.²

Coordination of care

The treatment team consists of physiatrists, rehabilitation nursing, physical, occupational, and speech therapists, recreational therapists and child life specialists, neuropsychologists, social workers, discharge coordinators, and other medical/surgical specialties to coordinate care. Psychologists should be used for psychosocial support for coping and adjustment for disability and return to home, school, and community.

Patient & family education

Age-appropriate patient education and family training are an integral part of rehabilitation.

Education and training of patients and families depending on deficits are vital to successful rehabilitation.

Emerging/unique interventions

There is no formal measurement of outcomes. Reasonable measures of outcomes are the rate of functional gains in the SCIM III over a period of time, community discharge, return to and success in school and community, and overcoming barriers.

Translation into practice: practice “pearls”/performance improvement in practice (PIPs)/changes in clinical practice behaviors and skills

The treatment endpoints of spinal immobilization and activity restriction for patients are arbitrarily chosen. SCIWORA can recur despite the absence of demonstrable spinal instability and may not be prevented by bracing. There is evidence to suggest there is no significant association between Chiari malformation type 1 and SCIWORA in children, however more research is needed on this topic.¹⁴ There are no data about the role of age in the success or failure of treatments.

Cutting Edge/Emerging and Unique Concepts and Practice

• Functional electric stimulation (FES) cycling: it is beneficial for improving cardiovascular fitness, increasing bone mineral density, building muscle mass, and improving circulation.¹⁵
• Combined FES and activity-based restorative therapy can be beneficial. ¹⁶
• Diaphragmatic pacing for patients dependent on mechanical ventilation.
• Partial body weight suspension gait training can assist with mobility.
• Therapeutic hypothermia has been used in the treatment of central nervous system injuries and has shown some benefit in acute SCI.¹⁷
• Platelet-rich plasma treatments ¹⁸ and electrical stimulation¹⁹ are being used for the treatment of pressure ulcers in SCI.
• Telemedicine is being used for preventive care.
• Use of Riluzole as a neuroprotective agent to minimize the secondary injury cascade.²⁰

Gaps in the Evidence-Based Knowledge

• Stem cell-based treatment for SCIs: currently no effective treatment is available.
• Use of high-dose methylprednisolone is controversial. The efficacy of steroid use such as IV methylprednisolone started within the first 8 hours after traumatic spinal cord injury for 48 hours had been shown to be beneficial, but its effect on SCIWORA treatment still needs to be studied.²
• Assistive technologies:  More research is needed on functional electrical stimulation, robotics and epidural stimulation of the spinal cord.
• Clinical trials are currently being performed on human monoclonal antibodies (NG-101) against Nogo-A, a protein that inhibits the regeneration of damaged nerves.²¹
• KCL-286 is an oral agonist to the retinoic acid receptor (RAR) β2, a transcription factor that promotes axonal growth. Its role in spinal cord injury treatment is currently being studied.²²

References

1. Iaconis Campbell J, Coppola F, Volpe E, Salas Lopez E. Thoracic spinal cord injury without radiologic abnormality in a pediatric patient case report. J Surg Case Rep. 2018 Oct 5;2018(10):rjy250. doi: 10.1093/jscr/rjy250. PMID: 30310640; PMCID: PMC6172700.
2. Atesok K, Tanaka N, O’Brien A, Robinson Y, Pang D, Deinlein D, Manoharan SR, Pittman J, Theiss S. Posttraumatic Spinal Cord Injury without Radiographic Abnormality. Adv Orthop. 2018 Jan 4;2018:7060654. 
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Original Version of the Topic

K. Rao Poduri, MD, Colin D Canham, MD, Woojoong Lee, MD, Jennifer Paul, MD. Spinal Cord Injury Without Radiological Abnormality. 12/02/2013.

Previous Version(s) of the Topic

K. Rao Poduri, MD and Sara Salim, MD. Spinal Cord Injury Without Radiological Abnormality. 4/5/2017

Kaila Yeste, DO, Elizabeth A Barton, MD, Christine A Cleveland, MD, K. Rao Poduri, MD. Spinal Cord Injury Without Radiological Abnormality. 12/8/2020

Author Disclosure

Kimberly C Hartman, MD, MHPE
Nothing to Disclose

Susan Li
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