> Table of Contents > Sciwora Syndrome (Spinal Cord Injury Without Radiologic Abnormality)
Sciwora Syndrome (Spinal Cord Injury Without Radiologic Abnormality)
Patrick M. Carey, DO
Jason B. Alisangco, DO, FAAFP
image BASICS
Also called spinal cord injury without radiographic evidence of trauma (SCIWORET), spinal cord injury without CT evidence of trauma (SCIWOCTET), or spinal cord injury without neuroimaging abnormality (SCIWONA)
DESCRIPTION
  • SCIWORA occurs after trauma; it is an acute spinal cord injury (SCI) and nerve root trauma resulting in transient or permanent sensory, motor, or combined sensorimotor deficits.
  • Neural injuries occur without a fracture or misalignment visible on radiographic imaging (x-ray, CT).
  • SCIWORA has a broad presentation, from minor neurologic symptoms to complete quadriplegia.
EPIDEMIOLOGY
Incidence
  • Variable: reported to be 19-34% of pediatric spinal cord injuries (1)
  • Occurs in all populations but is primarily observed in pediatric patients (90%) (1)
  • Bimodal: affects children <8 years old and adults >60 years old; rarely occurs between 16 and 36 years (2)
  • There is no association between Chiari malformation type 1 and SCIWORA.
ETIOLOGY AND PATHOPHYSIOLOGY
  • Trauma (3)
    • Motor vehicle collision (MVC) (most common cause); either unrestrained passengers, pedestrians, or bicyclists struck by motor vehicles
    • Sports-related injury
    • Significant fall
    • Child abuse
  • Mechanism
    • Traumatic neural (edema, hematomyelia, cord disruption) and extraneural (disc injury or ligament disruption) injury occurs after (2,4,5) the following:
      • Hyperextension
      • Hyperflexion
      • Longitudinal distraction
      • Ischemic damage
      • Secondary injury from inflammatory response to tissue damage
  • Age: Pediatric patients have a higher incidence of SCIWORA than adults due to anatomic differences and increased mobility and flexibility (1,4,6).
    • Horizontally oriented facet joints permit more translational motion in the coronal (AP) plane.
    • Anterior wedging of vertebral bodies
    • Elasticity of ligaments and joint capsules permits increased intersegmental movement and disc protrusion.
    • In patients age <8 years, head size-to-trunk ratio is disproportionately large.
    • Weaker nuchal musculature
    • Uncovertebral joints are absent.
    • Pseudosubluxation of C2 to C3
  • Location
    • Cervical: upper > lower
    • Thoracic: protected and splinted by ribs, preventing forced flexion and extension
    • Lumbar: rare, usually fatal (1)
RISK FACTORS
  • History of trauma
  • Age <8 years
  • Male: female
    • Adult; 4.5:1 (3,7), Children; 2:1 (3)
  • Improper wear of seatbelt
image DIAGNOSIS
PHYSICAL EXAM
  • Assess for sensorimotor deficit.
  • Abnormal neurologic findings are not accounted for by known/visible injuries.
  • Abnormalities on musculoskeletal exam increases risk of SCI.
DIFFERENTIAL DIAGNOSIS
  • End-plate cartilage fracture
  • Transverse myelitis
  • Intramedulllary hemorrhage
  • Anterior spinal artery syndrome
  • Disseminated encephalomyelitis
  • Atlantoaxial dislocation
  • Central cord syndrome
  • Brown-Sequard syndrome
DIAGNOSTIC TESTS & INTERPRETATION
  • Radiographic screening with CT of the entire spinal column is recommended (5)[C].
  • MRI of the region of suspected neurologic injury (5)[C]
  • MRI within 24 hours of injury and consider repeat if normal (7)[C]
  • Assessment of spinal stability in a SCIWORA patient is recommended with flexion-extension radiographs in the acute setting and at late follow-up, even in the presence of a magnetic resonance imaging (MRI) negative for extraneural injury (5)[C].
  • Neither spinal angiography nor myelography is recommended in the evaluation of patients with SCIWORA (5)[C].
  • CT scan can reliably rule out fracture.
Follow-Up Tests & Special Considerations
  • Adult considerations: Adults are less prone to SCIWORA due to decreased flexibility and mobility in comparison to pediatric patients, although SCIWORA is possible in the setting of acute trauma or cervical spondylosis.
  • A normal MRI does not rule out SCIWORA (1,2,7).
  • MRI abnormalities correlate closely with neurologic injury and prognosis (3)[C].
  • MRI may be able to demonstrate neural and extraneural injuries: cartilaginous end-plate fracture, edema, herniation, and interspinous ligamentous injury (1,2)
Diagnostic Procedures/Other
  • Diagnosis is based on clinical findings of neurologic dysfunction or MRI abnormality.
  • Consider diffusion-weighted MRI and somatosensoryevoked potentials (SSEPs) in cases of suspected SCIWORA with normal MRI (5,8)[C].
image TREATMENT
GENERAL MEASURES
  • Immobilize unconscious patients until plain x-ray and CT radiographs are obtained.
  • Immobilize until neurologic and pain assessment can be made.
  • Transient findings, such as numbness, and a history of trauma should be treated with immobilization following SCI protocol.
  • Supportive care and serial neurologic and musculoskeletal exams
  • Blood pressure support (5)[C]
  • Corticosteroid use is controversial and is not considered standard practice for pediatric cases (6)[C].
P.947

ADDITIONAL THERAPIES
  • Rigid external immobilization of the spinal segment for 12 weeks (day and night) (1)
  • Immobilization for 12 weeks even after the return of normal neurologic function to minimize SCIWORA recurrence (1,2,5)[C]
  • Avoidance of “high-risk” activities for up to 6 months following SCIWORA (2,5)[C]
  • Initially, treatment is nonoperative. Surgery may be required for spinal cord compression or spine instability secondary to extraneural injury (1,2).
  • Surgery for adults is often warranted as disc and ligamentum flavum pathology is common (2)[C].
  • Early discontinuation of external immobilization is recommended for patients who become asymptomatic, and spinal stability is confirmed with flexion and extension radiographs (5)[C].
INPATIENT CONSIDERATIONS
Admission Criteria/Initial Stabilization
  • Unexplained neurologic findings (2)[C]
  • Neurologic injury found via MRI (2)[C]
  • Suspected ligamentous lesion (2)[C]
  • Trauma and spinal injury protocol
  • Rigid immobilization
  • Consultation with a spine surgeon
Discharge Criteria
  • Neurologic exam without any deficits
  • Resolution of transient neurologic symptoms
image ONGOING CARE
  • SCIWORA can present with no initial symptoms after trauma followed by subsequent neurologic deterioration.
  • Reassess neurologic function within 24 to 48 hours if patient presents with no initial symptoms after a traumatic event, as there may be a latency period and secondary injury.
  • Clinical symptoms may take as long as 10 days to develop (3).
  • Follow-up MRI before discharge
PROGNOSIS
  • Initial clinical instability, injury severity, MRI findings, neurologic features, injury location, patient age, and persistence of symptoms have a direct correlation with prognosis.
  • Favorable
    • Initial mild to moderate injury
    • Normal or mild edema on initial MRI (1)
    • Resolution of changes as evidenced by follow-up MRI
  • Unfavorable
    • Initial severe neural injury
    • MRI findings of spinal cord transection and significant hemorrhage
    • Intramedullary hemorrhage seen on MRI is predictive of complete cord injury (1).
    • Follow-up MRI findings of persistent SCI
    • Higher cervical injuries
    • Patients age <8 years (1)
    • Concominant traumatic brain injury (concussion)
REFERENCES
1. Launay F, Leet AI, Sponseller PD. Pediatric spinal cord injury without radiographic abnormality: a meta-analysis. Clin Orthop Relat Res. 2005;(433):166-170.
2. Kasimatis GB, Panagiotopoulos E, Megas P, et al. The adult spinal cord injury without radiographic abnormalities syndrome: magnetic resonance imaging and clinical findings in adults with spinal cord injuries having normal radiographs and computed tomography studies. J Trauma. 2008;65(1):86-93.
3. Boese CK, Oppermann J, Siewe J, et al. Spinal cord injury without radiologic abnormality in children: a systematic review and meta-analysis. J Trauma Acute Care Surg. 2015;78(4):874-882.
4. Trigylidas T, Yuh SJ, Vassilyadi M, et al. Spinal cord injuries without radiographic abnormality at two pediatric trauma centers in Ontario. Pediatr Neurosurg. 2010;46(4):283-289.
5. Rozzelle CJ, Aarabi B, Dhall SS, et al. Spinal cord injury without radiographic abnormality (SCIWORA). Neurosurgery. 2013;72(Suppl 2):227-233.
6. Easter JS, Barkin R, Rosen CL, et al. Cervical spine injuries in children, part II: management and special considerations. J Emerg Med. 2011;41(3):252-256.
7. Boese CK, Lechler P. Spinal cord injury without radiologic abnormalities in adults: a systematic review. J Trauma Acute Care Surg. 2013;75(2): 320-330.
8. Shen H, Tang Y, Huang L, et al. Application of diffusion-weighted MRI in thoracic spinal cord injury without radiographic abnormality. Int Orthop. 2007;31(3):375-383.
Additional Reading
&NA;
  • Parikh RN, Muranjan M, Karande S, et al. Atlas shrugged: cervical myelopathy caused by congenital atlantoaxial dislocation aggravated by child labor. Pediatr Neurol. 2014;50(4):380-383.
  • Piatt JH Jr, Campbell JW. Spinal cord injury without radiographic abnormality and the Chiari malformation: controlled observations. Pediatr Neurosurg. 2012;48(6):360-363.
  • Shah LM, Zollinger LV. Congenital craniocervical anomalies pose a vulnerability to spinal cord injury without radiographic abnormality (SCIWORA). Emerg Radiol. 2011;18(4):353-356.
Codes
&NA;
ICD10
  • S14.109A Unsp injury at unsp level of cervical spinal cord, init
  • S14.2XXA Injury of nerve root of cervical spine, initial encounter
  • S14.102A Unsp injury at C2 level of cervical spinal cord, init encntr
Clinical Pearls
&NA;
  • If a patient presents with a history of trauma and neurologic symptoms but has negative x-ray and CT findings, consider SCIWORA.
  • Spinal cord appearance on MRI provides prognostic information (3)[C].
  • Treat SCIWORA with early immobilization, continue for 12 weeks, and avoid high-risk activities for an additional 12 weeks (2)[C].
  • Immobilization for 12 weeks is superior to 8 weeks (1)[C].
  • Consider serial scans or short-interval follow-up MRI to assess for delayed presentation of pathology (3)[C].