SCIWORA Syndrome (Spinal Cord Injury without Radiologic Abnormality)

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 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 SCIs (1)
  • Occurs in all populations—90% in pediatric patients (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):
      • 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 (1,5).
    • Horizontally oriented facet joints permit more translational motion in the coronal (AP) plane.
    • Anterior wedging of vertebral bodies
    • Ligament and joint capsule elasticity 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–C3
  • Location
    • Cervical: upper > lower
    • Thoracic: protected and splinted by ribs preventing forced flexion and extension
    • Lumbar: rare and usually fatal (1)

Risk Factors

  • History of trauma
  • Age <8 years
  • Male: female
    • Adult; 4.5:1 (3), children; 2:1 (3)
  • Improper seatbelt wear

Diagnosis

History

  • MVA/MVC
  • Sports-related injury
  • Fall
  • Child abuse

Physical Exam

  • Assess for sensorimotor deficit.
  • Abnormal neurologic findings are not accounted for by known/visible injuries.
  • Musculoskeletal exam abnormalities increase risk of SCI.

Differential Diagnosis

  • End-plate cartilage fracture
  • Transverse myelitis
  • Intramedullary hemorrhage
  • Anterior spinal artery syndrome
  • Disseminated encephalomyelitis
  • Atlantoaxial dislocation
  • Central cord syndrome
  • Brown-Séquard syndrome

Diagnostic Tests & Interpretation

  • Screening CT of the entire spinal column is recommended (4)[C].
  • MRI of the region of suspected neurologic injury (4)[C]
  • MRI within 24 hours of injury; consider repeat if normal (1)[C].
  • Assess spinal stability in a SCIWORA patient using flexion–extension radiographs in the acute setting and at late follow-up, even if MRI shows no extraneural injury (4)[C].
  • Spinal angiography and myelography are not recommended for the evaluation of SCIWORA patients (4)[C].
  • CT scan can reliably rule out fracture.

Follow-Up Tests & Special Considerations
  • Adults are less prone to SCIWORA due to decreased flexibility and mobility in comparison to pediatric patients. SCIWORA is still possible in the setting of acute trauma or cervical spondylosis.
  • A normal MRI does not rule out SCIWORA (1,2).
  • MRI abnormalities correlate closely with neurologic injury and prognosis (3)[C].
  • MRI can 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 somatosensory evoked potentials (SSEPs) in cases of suspected SCIWORA with normal MRI (4)[C].

Treatment

General Measures

  • Immobilize unconscious patients until plain x-ray and CT radiographs are obtained.
  • Immobilize until able to assess neurologic and pain status.
  • Immobilize patients with transient findings, such as numbness or a history of trauma following SCI protocol.
  • Supportive care and serial neurologic and musculoskeletal exams
  • Blood pressure support (4)[C]
  • Corticosteroid use is controversial and is not considered standard practice in pediatric cases (5)[C].

Additional Therapies

  • Rigid external immobilization of the spinal segment for 12 weeks (day and night) (1)[C]
  • Immobilization for 12 weeks even after the return of normal neurologic function minimizes SCIWORA recurrence (1,2,4)[C].
  • Avoidance of “high-risk” activities for up to 6 months following SCIWORA (2,4)[C]
  • Initial treatment is nonoperative. Surgery may be required for spinal cord compression or spine instability secondary to extraneural injury (1,2)[C].
  • Surgery for adults is often warranted as disc and ligamentum flavum pathology is common (2)[C].
  • Early discontinuation of external immobilization is recommended when patients are asymptomatic and spinal stability is confirmed by flexion and extension radiographs (4)[C].

Admission, Inpatient, and Nursing Considerations

  • Admission criteria/initial stabilization
    • Unexplained neurologic findings (2)[C]
    • Neurologic injury 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

Ongoing Care

  • SCIWORA can initially present after trauma with no symptoms. Subsequent neurologic deterioration is consistent with the diagnosis.
  • Reassess neurologic function within 24 to 48 hours in patients presenting with no initial symptoms after a concerning traumatic event (latency period and secondary injury).
  • Clinical symptoms may take 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 correlate with prognosis.
  • Favorable
    • Initial mild to moderate injury
    • Normal or mild edema on initial MRI (1)
    • Resolution of changes on 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)
    • Concomitant traumatic brain injury (concussion)

Complications

Iatrogenic, delayed, and recurrent SCIWORA have been reported; therefore, careful handling of trauma patients and close follow-up is warranted.

Additional Reading

  • Piatt JH Jr, Campbell JW. Spinal cord injury without radiographic abnormality and the Chiari malformation: controlled observations. Pediatr Neurosurg. 2012;48(6):360–363. [PMID:23920472]
  • 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. [PMID:21301913]

Codes

ICD-10

  • S14.101A Unsp injury at C1 level of cervical spinal cord, init encntr
  • S14.102A Unsp injury at C2 level of cervical spinal cord, init encntr
  • S14.103A Unsp injury at C3 level of cervical spinal cord, init encntr
  • S14.104A Unsp injury at C4 level of cervical spinal cord, init encntr
  • S14.105A Unsp injury at C5 level of cervical spinal cord, init encntr
  • S14.106A Unsp injury at C6 level of cervical spinal cord, init encntr
  • S14.107A Unsp injury at C7 level of cervical spinal cord, init encntr
  • S14.108A Unsp injury at C8 level of cervical spinal cord, init encntr
  • S14.109A Unsp injury at unsp level of cervical spinal cord, init
  • S14.2XXA Injury of nerve root of cervical spine, initial encounter

ICD-9

  • 952.00 C1-C4 level with unspecified spinal cord injury
  • 952.05 C5-C7 level with unspecified spinal cord injury
  • 952.9 Unspecified site of spinal cord injury without evidence of spinal bone injury
  • 953.9 Injury to unspecified site of nerve roots and spinal plexus

SNOMED

  • 11807002 injury at C5-C7 level with spinal cord injury AND without bone injury (disorder)
  • 129137006 Nerve root injury
  • 24392008 injury at C1-C4 level with spinal cord injury AND without bone injury (disorder)
  • 405754008 cervical spinal cord injury (disorder)
  • 90584004 Spinal cord injury (disorder)

Clinical Pearls

  • Consider SCIWORA in patients presenting with a history of trauma and neurologic symptoms with negative x-ray and CT findings.
  • The appearance of the spinal cord on MRI helps provide prognostic information.
  • Treat SCIWORA with early immobilization, continue for 12 weeks, and avoid high-risk activities for an additional 12 weeks. Immobilization for 12 weeks is superior to 8 weeks.
  • Consider serial MRI to assess for delayed presentation of pathology.

Authors


Patrick M. Carey, DO
Jason B. Alisangco, DO, FAAFP

Bibliography

  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. [PMID:15805953]
  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.  [PMID:18580514]
  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.  [PMID:25807412]
  4. Rozzelle CJ, Aarabi B, Dhall SS, et al. Spinal cord injury without radiographic abnormality (SCIWORA). Neurosurgery. 2013;72(Suppl 2):227–233.  [PMID:21160237]
  5. 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.  [PMID:23417193]


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