Author(s): Kaila Yeste, DO, Elizabeth A Barton, MD, Christine A Cleveland, MD, K. Rao Poduri, MD
Originally published: December 2, 2013 Last updated: December 8, 2020
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Disease/ Disorder

Definition

SCIWORA is traumatic Myelopathy to the spinal cord without abnormalities on plain radiographic and tomographic imaging evidence of fractures or ligamentous instability of the spine 1.

Etiology

The etiology of SCIWORA varies with age. In children aged 0 to 8 years, there are fewer fractures and subluxations, and hence more SCIWORA. The spines of neonates are vulnerable to distraction injuries, resulting in neonatal SCIWORA. Spinal cord and meningeal ruptures are found within a completely intact vertebral column in infants with complete tetraplegia after traumatic breech extraction.

In younger patients, it is most common as a result of high-energy injuries, motor vehicle accidents, followed by falls 2,3. In older children it is from motor vehicle accidents, followed by sports injuries, collision sports, such as tackle football, wrestling, boxing, and child abuse.

Epidemiology including risk factors and primary prevention

  1. Age: 90% of documented cases of SCIWORA are in children < 18yo. The incidence of SCIWORA in patients with spinal cord injury (SCI) varies significantly because of variations in how SCIWORA is defined and varying levels of awareness of this entity. Of children with SCI, up to 20% to 40% have SCIWORA2,4.The incidence in children is 3.3% to 32%.SCIWORA is most common in children 8 years of age and under, less common in older children, and is rare in adults5.
  2. Location: SCIWORA typically involves the cervical spine, and less commonly involves the thoracolumbar spine2.  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, lending to a better prognosis 6.
  3. Risk factors: Risk factors are essentially the same as with SCIs, 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 7.In the pediatric population, similar to the adult population, males are more likely to suffer a cervical SCI than females at a ratio of 1.5-2:1.16.

Prevention: 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:

  1. Segmental spinal instability: increased elasticity of the pediatric spinal ligaments, allowing for instability events and damage to the spinal cord, whereas the elastic soft tissues remain intact. Subsequent autoreduction 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 population1.
  2. 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 1cm2.
  3. Vascular injury: traumatic injury of the vascular supply to the spinal cord, resulting in cord ischemia.
  4. Cord contusion: injury to the spinal cord via force transmission through the intact spinal column.
  5. 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 minutes to days after injury, ranging from complete paralysis to partial neurologic deficits. The latent period may range from 30 minutes to 4 days8,-10.Thus, prompt evaluation with imaging should occur up to 4 days after initial injury if transient neurologic symptoms are reported11.The main reported initial symptom is neurologic change in the upper extremity1.

  1. Between 15% and 32% have severe or complete neurologic injury, as defined by the American Spinal Injury Association (ASIA)2,12
  2. Patients who present with a complete injury are less likely to regain motor, sensory, bowel, and bladder function.
  3. Patients with incomplete (sacral sparing) injury  may regain some motor or sensory function.
  4. Patients with mild initial symptoms and those without magnetic resonance imaging (MRI) abnormalities (35%) typically recover fully.4,5,13
  5. Recurrent SCIWORA
    • Rare (incidence 1% to 4%).
    • Can occur within hours to years after injury.
    • Usually occurs in patients older than 8 years.
    • Often a low energy mechanism with mild initial symptoms.
    • Patients fully recover,2 although some may have severe neurologic injury2,4.

Specific secondary or associated conditions and complications

Associated Conditions

  1. Paralysis of the diaphragm and respiratory distress
  2. Sensory loss
  3. Neurogenic bowel and bladder

Complications

  1. Autonomic dysreflexia
  2. Paralytic ileus
  3. Skin breakdown
  4. Pneumonia
  5. Urinary tract infections
  6. Immobilization hypercalcemia
  7. Loss of muscle mass
    1.  Spasticity and joint contractures
    2. Chronic pain

Essentials of Assessment

History

A detailed history to elicit nature of trauma, presence of pain, numbness/paresthesia, weakness, bladder and bowel function, and preinjury level of function, location, severity, and nature of symptoms, whether transient or evolved over time, or permanent, new incontinence, presence of neck and back pain, and symptoms for associated injuries are essential.

Physical examination

An evaluation with ASIA Impairment Scale that determines neurologic level and completeness of injury should be performed. A general exam, including respiratory, cardiovascular, abdomen, extremities, and 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, is essential.

SCIWORA may present as definite evidence of SCI 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.14

Functional assessment

Functional assessment includes mobility for transfers, potential for ambulation (if incomplete injury), and self-care, bowel and bladder function and toileting. A neuropsychologic evaluation is essential if concomitant brain injury is suspected. The FIM, WeeFIM, an instrument to measure the need for assistance and the severity of disability in children between the ages of 6 months and 7 years,15 and the third version of the Spinal Cord Independence Measure (SCIM III) 16, to quantify the functional status in older children, can be performed by nursing, physical, speech, and occupational therapists and followed by periodic examination.

Laboratory studies

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

Imaging

Plain radiographs and computed tomography images of the entire spine are essential to investigate for fractures, dislocations, bleeding, and other associated injuries. A full spine survey is essential for suspected thoracic cord and multisystem trauma.

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

MRI of the cervical spine is obtained in patients when SCI is suspected, often in the acute trauma setting 17. In SCIWORA, MRI may be normal (~35%)2,4or abnormal. It is useful for detecting both extraneural, intraneural damage to the cord, and for determining prognosis. A contrast study would delineate ligamentous or soft tissue injury, scarring, or disk herniation. 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.18  However, there remains no consensus on the best timing for MRI of the spine 17 and no consensus on if or when a follow up MRI should be done if the initial MRI was negative for abnormalities17,19. MRI of the brain is indicated if brain injury is suspected.

Supplemental assessment tools

Somatosensory evoked potentials are adjunct to the previously mentioned studies. In comatose patients with suspected SCIWORA, they are helpful to assess for cord injury and posterior column conduction.

Early predictions of outcomes

  1. 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.
  2. MRI: intraneural hemorrhages with cord disruption typically are accompanied by complete injury with permanent deficits.
  3. Absence of signal change within the spinal cord is associated with complete recovery.
  4. Signal change consistent with edema or microhemorrhages is associated with improvement in neurologic function over time in some patients.
  5. Age at injury: younger patients have worse injuries.
  6. Early neurologic recovery, within the first three days, may be associated with better long-term outcomes20.

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

  1. External immobilization is recommended until spinal stability is confirmed by flexion/extension radiographs12.
  2. Avoidance of high-risk activities, such as gymnastics, diving, down-hill skiing, equestrianism, tackle football, wrestling, and boxing 2,for up to 6 months, is recommended.
  3. There are no established treatment guidelines specifically for SCIWORA, and the evidence is insufficient to support treatment standards or guidelines21.

At different disease stages

Acute and Rehabilitation Admission Phase

  1. 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 out12.
  2. Medical stabilization.
  3. Early admission to specialized SCI centers has shown to decrease length of stay, severity of complications, and mortality 22. 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.
  4. 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.
  5. Necessary modifications for discharge destination for safety and access to available community resources.
  6. Family education about nature of injury, reasonable expectations, and prognosis.

Subacute to Chronic Management

  1. Prevention of secondary complications.
  2. Monitor for resolution of initial presenting deficits.
  3. Multidisciplinary approach to treatment is necessary for successful community reintegration.
  4. 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.
  5. Health maintenance and return to school and avocational activities.

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 the 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 topic23.There are no data about the role of age in the success or failure of treatments.

Cutting Edge/ Emerging and Unique Concepts and Practice

  1. Functional electric stimulation (FES) cycling: it is beneficial for improving cardiovascular fitness, increasing bone mineral density, building muscle mass, and improving circulation24,25.
  2. Combined FES and activity-based restorative therapy26.
  3. Diaphragmatic pacing for patients dependent on mechanical ventilation.
  4. Partial body weight suspension gait training can assist with mobility21.Two devices are currently being tested, the “Lokomat”, which includes a robotic assisted exoskeleton, and the “Therastride”, which is used with a treadmill and manual assistance.
  5. Therapeutic hypothermia has been used in the treatment of central nervous system injuries and has shown some benefit in acute SCI 27,28 .
  6. Platelet-rich plasma treatments29, 30 and electrical stimulation31 are being used for the treatment of pressure ulcers in SCI.
  7. Telemedicine is being used for preventive care32,33.
  8. Use of Riluzole as a neuroprotective agent to minimize the secondary injury cascade34.

Gaps in the Evidence-Based Knowledge

  1. Stem cell-based treatment for SCIs: currently no effective treatment is available 35.
  2. Use of high-dose methylprednisolone is controversial. The 2013 guidelines published by neurosurgical surgeons advise against its use36.
  3. Assistive technologies:  More research is needed on functional electrical stimulation, robotics and epidural stimulation of the spinal cord 37,38,39.

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. Pang D. Spinal cord injury without radiographic abnormality in children, 2 decades later. Neurosurgery. 2004;55:1325-1343.
  3. Qi C, Xia H, Miao D, Wang X, Li Z. The influence of timing of surgery in the outcome of spinal cord injury without radiographic abnormality (SCIWORA). Journal of Orthopaedic Surgery and Research. 2020Jun16;15.
  4. Yucesoy K, Yuksel KZ. SCIWORA in MRI era.Clin Neurol Neurosurg. 2008;110:429-433.
  5. Kothari P, Freeman B, Grevitt M, Kerslake R. Injury to the spinal cord without radiological abnormality (SCIWORA) in adults.J Bone Joint Surg Br.2000;82:1034-1037.
  6. Copley PC, Tilliridou V, Kirby A, Jones J, Kandasamy J. Management of cervical spine trauma in children. Eur J Trauma Emerg Surg. 2019 Oct;45(5):777-789. doi: 10.1007/s00068-018-0992-x. Epub 2018 Aug 24. PMID: 30167742; PMCID: PMC6791958.
  7. Liang QC, Yang B, Song YH, Gao PP, Xia ZY, Bao N. Real spinal cord injury without radiologic abnormality in pediatric patient with tight filum terminale following minor trauma: a case report. BMC Pediatr. 2019 Dec 23;19(1):513. doi: 10.1186/s12887-019-1894-8. PMID: 31870344; PMCID: PMC6927174.
  8. Pang D, Pollack IF. Spinal cord injury without radiographic abnormality in children–the SCIWORA syndrome.J Trauma.1989;29:654-664.
  9. Hamilton MG, Myles ST. Pediatric spinal injury: review of 174 hospital admissions. J Neurosurg. 1992;77:700-704.
  10. Osenbach RK, Menezes AH. Spinal cord injury without radiographic abnormality in children. Pediatr Neurosci. 1989;15:168-174.
  11. Hall DE, Boydston W. Pediatric neck injuries. Pediatr Rev. 1999;20:13-19.
  12.  Bosch PP, Vogt MT, Ward WT. Pediatric spinal cord injury without radiographic abnormality (SCIWORA): the absence of occult instability and lack of indication for bracing. Spine. 2002;27:2788-2800.
  13. Mahajan P, Jaffe DM, Olsen CS, et al. Spinal cord injury without radiologic abnormality in children imaged with magnetic resonance imaging. J Trauma Acute Care Surg. 2013 Nov;75(5):843-7.
  14. Brown RL, Brunn MA, Garcia VF. Cervical spine injuries in children: a review of 103 patients treated consecutively at a level 1 pediatric trauma center. J Pediatr Surg.2001;36:1107-1114.
  15. Msall ME, DiGaudio K, Duffy LC, LaForest S, Braun S, Granger CV. WeeFIM. Normative sample of an instrument for tracking functional independence in children. Clin Pediatr (Phila). 1994;33:431-438.
  16. Anderson KD, Acuff ME, Arp BG, et al. United States (US) multi-center study to assess the validity and reliability of the Spinal Cord Independence Measure (SCIM III). Spinal Cord. 2011;49:880-885.
  17. Farrell CA, Hannon M, Lee LK. Pediatric spinal cord injury without radiographic abnormality in the era of advanced imaging. Curr Opin Pediatr. 2017 Jun;29(3):286-290. doi: 10.1097/MOP.0000000000000481. PMID: 28306628.
  18. Liu Q, Liu Q, Zhao J, Yu H, Ma X, Wang L. Early MRI finding in adult spinal cord injury without radiologic abnormalities does not correlate with the neurological outcome: a retrospective study. Spinal Cord. 2015 Oct;53(10):750-3.
  19. Dreizin D, Kim W, Kim JS, et al. Will the Real SCIWORA Please Stand Up? Exploring Clinicoradiologic Mismatch in Closed Spinal Cord Injuries. AJR Am J Roentgenol. 2015 Oct;205(4):853-60.
  20. Asan Z. Spinal Cord Injury without Radiological Abnormality in Adults: Clinical and Radiological Discordance. World Neurosurg. 2018 Jun;114:e1147-e1151. doi: 10.1016/j.wneu.2018.03.162. Epub 2018 Mar 31. PMID: 29614362.
  21. Guidelines for the Management of Acute Cervical Spine and Spinal Cord Injuries: Chapter 13 Neurosurgery. March 2002 – Volume 50 – Issue 3, S100-S104.
  22. Parent S, Barchi S, LeBreton M, Casha S, Fehlings MG. The impact of specialized centers of care for spinal cord injury on length of stay, complications, and mortality: a systematic review of the literature. J Neurotrauma.2011;28:1363-1370.
  23. Piatt JH Jr, Campbell JW. Spinal cord injury without radiographic abnormality and the Chiari malformation: controlled observations. Pediatr Neurosurg. 2012;48(6):360-3.
  24. Dolbow DR, Gorgey AS, Ketchum JM, Moore JR, Hackett LA, Gater DR. Exercise adherence during home-based functional electrical stimulation cycling by individuals with spinal cord injury. Am J Phys Med Rehabil. 2012;91:922-930.
  25. Johnston TE, Smith BT, Mulcahey MJ, Betz RR, Lauer RT.A randomized controlled trial on the effects of cycling with and without electrical stimulation on cardiorespiratory and vascular health in children with spinal cord injury. Arch Phys Med Rehabil.2009;90:1379-1388.
  26. Martin R, Sadowsky C, Obst K, Meyer B, McDonald J. Functional electrical stimulation in spinal cord injury: from theory to practice. Top Spinal Cord Inj Rehabil. 2012;18:28-33.
  27. Ahmad FU, Wang MY, Levi AD. Hypothermia for acute spinal cord injury–a review. World Neurosurg. 2014;Jul-Aug;82(1-2):207-14.
  28. Wang J, Pearse DD. Therapeutic Hypothermia in Spinal Cord Injury: The Status of Its Use and Open Questions. Int J Mol Sci. 2015;Jul 24;16(8):16848-79.
  29. Singh R, Rohilla RK, Dhayal RK, Sen R, Sehgal PK. Role of local application of autologous platelet-rich plasma in the management of pressure ulcers in spinal cord injury patients. Spinal Cord. 2014;Nov;52(11):809-1630.
  30. Biglari B, Reitzel T, Swing T, Büchler A, Gerner HJ, Schmidmaier G, Moghaddam A. A pilot study on the effectiveness of platelet-rich plasma and debridement for the treatment of nonhealing fistulas in spinal cord-injured patients. Adv Skin Wound Care. 2015 Mar;28(3):123-8.
  31. Liu LQ, Moody J, Traynor M, Dyson S, Gall A. A systematic review of electrical stimulation for pressure ulcer prevention and treatment in people with spinal cord injuries. J Spinal Cord Med. 2014; Nov;37(6):703-18.
  32. Houlihan BV, Jette A, Friedman RH, Paasche-Orlow M, Ni P, Wierbicky J, Williams K, Ducharme S, Zazula J, Cuevas P, Rosenblum D, Williams S. A pilot study of a telehealth intervention for persons with spinal cord dysfunction. Spinal Cord.2013 Sep; 51(9):715-20.
  33. Yuen J, Thiyagarajan CA, Belci M. Patient experience survey in telemedicine for spinal cord injury patients. Spinal Cord. 2015; Apr; 53(4):320-3.
  34. Shimizu EN, Seifert JL, Johnson KJ, Romero-Ortega MI. Prophylactic Riluzole Attenuates Oxidative Stress Damage in Spinal Cord Distraction. J Neurotrauma. 2018 Jun 15;35(12):1319-1328. doi: 10.1089/neu.2017.5494. Epub 2018 Mar 20. PMID: 29295647.
  35. Diaz Q, Echeverri K. Spinal cord regeneration: where fish, frogs and salamanders lead the way, can we follow? Biochem J. 2013;451:353-364.
  36. Hurlbert RJ, Hadley MN, Walters BC, et al. Pharmacological therapy for acute spinal cord injury. Neurosurgery. 2013;72 Suppl 2:93-105.
  37. Harkema S, Gerasimenko Y, Hodes J, et al. Effect of epidural stimulation of the lumbosacral spinal cord on voluntary movement, standing, and assisted stepping after motor complete paraplegia: a case study. Lancet. 2011; 377:1938-1947.
  38. Angeli CA, Edgerton VR, Gerasimenko YP, Harkema SJ. Altering spinal cord excitability enables voluntary movements after chronic complete paralysis in humans. Brain. 2014; May;137(Pt 5):1394-409.
  39. Rejc E, Angeli C, Harkema S. Effects of Lumbosacral Spinal Cord Epidural Stimulation for Standing after Chronic Complete Paralysis in Humans. PLoS One. 2015; Jul 24;10(7).

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. Published 4/5/2017

Author Disclosure

Kaila Yeste, DO
Nothing to Disclose

Elizabeth A, Barton, MD
Nothing to Disclose

Christine A. Cleveland, MD
Nothing to Disclose

K. Rao Poduri, MD
Nothing to Disclose