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Spinal cord injury without radiographic abnormality (SCIWORA) in children

Spinal cord injury without radiographic abnormality (SCIWORA) in children
Literature review current through: Jan 2024.
This topic last updated: May 31, 2022.

INTRODUCTION — This topic will review the clinical features and management of SCIWORA in children less than 18 years of age. The approach to ligamentous injury and SCIWORA in adults is discussed separately. (See "Suspected cervical spine injury in adults: Choice of imaging", section on 'Further evaluation with magnetic resonance imaging'.)

TERMINOLOGY — SCIWORA was defined in a series of children as objective signs of acute traumatic myelopathy in the absence of spinal column findings on plain radiographs, flexion-extension radiographs, and/or computed tomography (CT) [1,2]. In this original report, it was noted that patients had neurologic deficits on presentation or a history of transient paresthesia, numbness, or paralysis. Approximately half of patients presented with delayed onset (up to four days after injury) of permanent paralysis.

However, with the advent of magnetic resonance imaging (MRI), approximately two-thirds of cases described as SCIWORA in the literature actually have demonstrable injury to the spinal cord, soft tissue components of the spinal column (ligaments, capsules, or muscles), or vertebral body endplate [3,4]. With the increased availability of MRI, the diagnosis of "real" SCIWORA or spinal cord injury without neuroimaging abnormality is less common. The term "spinal cord injury without computed tomography evidence of trauma" (SCIWOCTET) is sometimes used to describe adult patients with neurologic findings suggestive of spinal cord injury with normal anatomic alignment and no bony abnormalities seen on CT [2].

Nonetheless, it is important to remember that patients with blunt trauma who have a history of transient neurologic symptoms that have resolved by the time of initial evaluation may have a significant injury to the spinal cord and/or spinal column despite a normal physical examination and normal spine radiographs and/or CT.

SCIWORA should not be confused with cervical "burners" or "stingers," which refers to peripheral brachial plexus injuries, typically at the C5-C6 level resulting from trauma to the neck and shoulder (table 1). When children present with transient neurologic symptoms and normal neurologic exams, it may be difficult to distinguish between brachial plexus injuries and SCIWORA, leading to over-reporting of SCIWORA [5]. (See "Overview of cervical spinal cord and cervical peripheral nerve injuries in the child or adolescent athlete", section on 'Cervical burners'.)

PERTINENT ANATOMY — The cervical spine is the most mobile portion of the spinal column and is most prone to traumatic injury. The thoracolumbar spine is partially braced by the rib cage and is less commonly deformed without fracture (figure 1).

Relative to adults, children have several distinct anatomic features that predispose them to hypermobility of the spinal column in the absence of apparent bony injury [3]:

In infants, the ratio of head size and weight to the rest of the body is large, and the neck musculature is weak and limited in its ability to prevent hypermobility of the cervical spine.

The spinal ligaments and capsules are elastic, allowing them to undergo significant stretching without tearing.

The intervertebral disc and annulus have a high water content that allows for longitudinal expansion without rupture during spinal column distraction (as much as 2 inches [5 cm] in the neonate).

The vertebral body facet joints are oriented horizontally and are shallow, which permits significant bony motion during flexion, extension, or rotary neck movements.

Anterior wedging of the vertebral bodies in infants and young children allows for increased forward bony movement.

Children have underdeveloped uncinate processes until approximately 10 years of age and thus, have less restriction of lateral and rotary spinal column movements.

Large diameter of the spinal canal relative to the diameter of the spinal cord, resulting in increased mobility of the spinal cord within the canal.

These anatomic features are most pronounced in infants and children under eight years of age and predispose them to axial cervical spine (occiput through second cervical vertebrae) and spinal cord injuries involving the first through third cervical segments after blunt trauma [6]. Older children and adolescents are more likely to have subaxial cervical spine injuries (fourth through eighth cervical cord segments). Full maturation of the cervical spine is not typically achieved until approximately 16 years of age [3,6].

In addition to these predisposing anatomic features in children, children with genetic skeletal disorders may also be at increased risk for SCIWORA. For example, in a cross-sectional study of 272 children with genetic skeletal disorders, SCIWORA occurred in 10 percent of patients, including those with dysostoses, skeletal dysplasias, disruptions, and osteolysis [7]. For more prevalent genetic skeletal disorders such as Down syndrome, osteogenesis imperfecta, and mucopolysaccharidosis, affected individuals require routine neurologic surveillance for myelopathic signs and symptoms at every well-child visit and are at heightened risk for SCIWORA after blunt trauma [8-10].

Mechanism of injury — Four mechanisms of injury have been described for SCIWORA [3]:

Hyperextension – With hyperextension of the cervical spine, the posterior interlaminar ligaments impinge upon the spinal canal causing up to 50 percent narrowing of the canal diameter. At the same time, spinal cord shortening leads to thickening in the cervical region [11]. Further extension leads to bony displacement that, especially in the infant or young child, may be transient and followed by the spinal column springing back into normal bony position.

Hyperflexion – The mechanism of spinal cord impingement following hyperflexion is similar to that of hyperextension. The anterior interlaminar ligaments impinge upon the cervical spinal canal causing narrowing at a point where spinal cord thickening occurs. Further flexion leads to transient bony displacement with further injury to the spinal cord.

Distraction – Longitudinal distraction is most commonly seen in infants and children younger than five years of age. In these patients, the spinal column can be stretched well beyond the tolerance of the spinal cord without subsequent radiographic bony damage [12]. Distraction with quadriplegia has occurred in neonates after breech extraction [13], in young children restrained only by lap belts who are thrown forward during a front end motor vehicle collision, and in other rapid acceleration/deceleration events.

Spinal cord infarction – Vertebral artery occlusion from transient impingement and/or arterial dissection during deformation of the spinal column may result in ischemia of the spinal cord. This is best evidenced in populations of neonates who experience failure of the atlanto-occipital articulation during delivery and upper cervical spinal cord infarction. Hypotension due to massive blood loss may also result in spinal cord infarction. As an example, delayed thoracic spinal cord infarction was demonstrated in a five year old child who suffered high-energy thoracoabdominal trauma with retroperitoneal injury and hypotension [14].

EPIDEMIOLOGY — SCIWORA has been described in victims of all ages after blunt trauma [1,5,15,16]. Depending upon the study population and reference standard, reported incidence has ranged from 4.5 to 35 percent among children with spinal injuries [3,5,17-19], and approximately 0.2 percent of all pediatric trauma patients [17,19]. Among all cervical spine injuries in children, spinal cord injuries in the absence of other associated vertebral or ligamentous injuries are common, representing up to 15.5 percent of injuries [6].

Most cases of SCIWORA occur in the cervical spine [3,18,20]. Thoracic SCIWORA has also been described but is less common than in the cervical region due to the splinting reinforcement provided by the rib cage. When it occurs, thoracic SCIWORA is usually associated with extreme traumatic forces (eg, lap-belt complex associated with high-speed motor vehicle collisions) [1,3]. In addition, SCIWORA has been reported in the lumbar region in school-aged children and adolescents, but is rare [21].

Mechanisms of injury associated with SCIWORA vary by age and are associated with neurologic outcome. In young children under eight years of age, SCIWORA is most commonly caused by motor vehicle collisions, falls, and child abuse [3]. In older children, motor vehicle collisions and sports, especially gymnastics, diving, horseback riding, American football, and wrestling, predominate. Worse neurologic outcome is associated with injury during motor vehicle collisions and falls [5].

CLINICAL FEATURES AND DIAGNOSIS — The diagnosis of SCIWORA is made when patients demonstrate clinical findings of spinal cord injury in the absence of spinal column injury on plain radiographs and CT. Approximately two thirds of these patients will have evidence of injury to the spinal cord, soft tissue components of the spinal column (ligaments, capsules, or muscles), or vertebral body endplate on MRI.

Patients with SCIWORA may have definite evidence of spinal cord injury on physical examination as manifested by abnormal vital signs (eg, apnea or bradycardia with hypotension [spinal shock]), neck or back pain, and/or neurologic deficits (eg, paresthesia, paralysis, or loss of pain and/or sensation). (See "Evaluation and acute management of cervical spine injuries in children and adolescents", section on 'History' and "Evaluation and acute management of cervical spine injuries in children and adolescents", section on 'Physical examination'.)

However, although challenging to elicit in young children, transient neurologic symptoms (eg, paresthesia, weakness) by history or resolving during emergency evaluation may also indicate injury to the cervical or thoracic spinal cord [1,22]. Approximately one-quarter of children with SCIWORA may experience delayed onset of neurologic signs minutes to days after injury [23-25], which can range from complete paralysis to partial neurologic deficits. The latent period may range from 30 minutes to four days [1,24,25], making prompt diagnosis difficult [26]. Thus, prompt evaluation with imaging should occur up to four days after the initial injury if transient neurologic symptoms are reported. (See 'Radiologic evaluation' below.)

For this reason, it is critical to have a high index of suspicion and to ask specifically about transient symptoms in any verbal child whose mechanism of injury is consistent with cervical or thoracic spine injury. (See "Evaluation and acute management of cervical spine injuries in children and adolescents", section on 'History'.)

DIFFERENTIAL DIAGNOSIS — SCIWORA is differentiated from cervical spine fracture, dislocation, or subluxation by imaging studies performed during the acute phase of management and evaluation.

When children present with transient neurologic symptoms and normal neurologic exams, it may be difficult to distinguish between cervical brachial plexus injuries (burners or stingers) and SCIWORA. Patients with cervical brachial plexus injuries describe burning pain in the supraclavicular region that radiates down the arm on the affected side in a circumferential, nondermatomal fashion. Unilateral paresthesia, numbness, or weakness may also occur. The discomfort typically resolves within one to two minutes (table 1). (See "Overview of cervical spinal cord and cervical peripheral nerve injuries in the child or adolescent athlete", section on 'Cervical burners'.)

By contrast, patients with SCIWORA have neck pain with reduced range of motion, and bilateral paresthesia or weakness that can involve the lower extremity. Findings can progress to flaccid paralysis and quadriparesis.

RADIOLOGIC EVALUATION — All patients with suspected spine or spinal cord injury should undergo plain radiographs of the cervical spine and any other portion of the spine with evidence of injury and CT of the cervical spine and/or other region based on physical examination and plain radiographs (algorithm 1 and algorithm 2). The approach to imaging of the cervical spine is discussed in detail separately. (See "Evaluation and acute management of cervical spine injuries in children and adolescents", section on 'Cervical spine imaging'.)

Magnetic resonance imaging — MRI should be performed in children who are suspected of having spinal cord injury but have normal plain radiographs (three-view cervical spine or two-view thoracic spine), CT, or both [27-31]. A high index of suspicion should be maintained for patients who present with transient neurologic symptoms but normal neurologic examination if they are younger than eight years or have a high-risk mechanism (eg, axial load biomechanics or high-risk motor vehicle collisions [death of passenger in same vehicle, patient ejected partially or fully, passenger compartment intrusion >12 inches at sides and >18 inches at the roof]) or a predisposing condition [5]. Such patients may warrant MRI even when presenting in a delayed fashion (eg, the next day) after injury.

Small observational studies and case reports suggest that the abnormal spinal cord findings on MRI are seen in up to two-thirds of patients with SCIWORA and that lesions requiring operative intervention (eg, vertebral fracture or ligamentous disruption) are frequently demonstrated [4,6,18,29,32].

Prognosis — Spinal cord appearance on MRI correlates with prognosis as follows [20]:

Spinal cord findings on MRI connotes a higher probability of persistent neurologic deficits. As an example, in a study of 69 children with SCIWORA found that spinal cord abnormalities on MRI were associated with neurologic outcome [5]. Persistent neurologic deficits at hospital discharge were present in 10 of the 15 children with spinal cord findings on MRI (67 percent) whereas only 3 of the 54 children without spinal cord findings on MRI had persistent neurologic deficits at discharge (6 percent).

Absence of signal change within the spinal cord is associated with excellent prognosis. In one series, full neurologic recovery was seen in all 51 patients with a normal MRI, including six children with significant motor impairment at presentation [18]. In another series, full recovery at six months was seen in 23 patients with normal MRI; 21 of these patients had mild deficits at presentation, and two patients had a physical examination suggesting a complete spinal cord disruption [3].

Signal change consistent with edema or microhemorrhages is associated with improvement in neurologic function over time in some patients. In one review, two of five patients with "minor" hemorrhage on initial MRI improved to a mild neurologic deficit at six months while two of nine patients with spinal cord edema fully recovered, and seven had a mild deficit [3].

Hematomyelia (hemorrhage into the spinal cord) and cord disruption are associated with severe, permanent neurologic injury [3,18,28,33].

INITIAL MANAGEMENT — Acute management priorities in children with SCIWORA consist of:

Maintain restriction of spinal motion. Protection of the cervical spine to prevent worsening of an existing spinal cord injury requires restricting motion in both the neck and the body. The neck is placed in a cervical collar, and the body is placed in the supine position, preferably on a soft conforming surface. Infants and young children warrant a cut out in the board or padding under the shoulders to maintain neutral position (figure 2). (See "Pediatric cervical spinal motion restriction", section on 'Techniques'.)

Provide emergency support of airway, breathing, and circulation while treating other life-threatening injuries. The cervical spine should be manually stabilized during airway management to prevent exacerbation of a spinal cord injury (figure 3). Initially, the airway can be opened with the jaw-thrust maneuver (figure 4). Orotracheal intubation with in-line manual stabilization provides the best approach to secure the airway in trauma victims without causing further cervical spine injury. (See "Pediatric cervical spinal motion restriction", section on 'Manual in-line stabilization of the cervical spine' and "Technique of emergency endotracheal intubation in children", section on 'Cervical spine immobilization'.)

Transient loss of spinal cord function can occur following spinal injury and result in vasodilatory shock. However, clinicians must first assume that hypotension following trauma results from hemorrhage and ensure appropriate fluid resuscitation. (See "Trauma management: Approach to the unstable child", section on 'Circulation'.)

Any injury associated with a neurologic deficit requires emergency consultation with a pediatric spine surgeon or a spine surgery team (often staffed by pediatric orthopedists and pediatric neurosurgeons) to guide supportive care and definitive management of the injury. If consultation is not available onsite, immediate transfer must be arranged to a pediatric trauma center that can provide these services.

Definitive therapy should be based on the MRI findings and provided in consultation with a pediatric spine team.

DISPOSITION — Most children with SCIWORA warrant hospital admission. Individual patients who meet all of the following criteria may be discharged from the emergency department in a padded hard cervical collar (eg, Miami J or Aspen cervical collar):

A history of transient cervical neurologic symptoms that have fully resolved upon presentation

Low-risk mechanism of injury and no other serious injuries

A normal detailed neurologic examination

Normal plain radiographs and/or CT

Assured neurosurgical follow-up within one to two weeks

The parent or caregiver and, when applicable, the patient must fully understand the need for restriction of any activity (eg, sports, aggressive play, bicycle riding) that might lead to reinjury.

Close follow-up of neurologic function by a neurosurgeon is necessary in all children with SCIWORA. Ongoing treatment in patients without operative lesions consists of prolonged external immobilization to permit healing of the presumed ligamentous injury and to prevent exacerbation of the myelopathy (eg, Guilford brace for cervical SCIWORA, thoracolumbar orthosis for thoracolumbar SCIWORA) [23].

The duration of bracing in children with SCIWORA is debated, and recommendations vary from no bracing in patients with transient symptoms and an initial normal MRI to a Guilford brace or thoracolumbar prosthesis for three months in patients with persistent deficits [3,18,20,34]. Evidence supporting bracing and restriction of activities for three months derives from one case of recurrent injury with permanent neurologic deficit at 10 weeks [3] and a meta-analysis of cases of SCIWORA, which found that recurrent injury was significantly more common in patients braced for 8 weeks versus 12 weeks (17 versus 0 percent) [34]. In contrast, a single hospital series of 189 children with SCIWORA reported 21 cases of recurrent SCIWORA [18]. The 85 patients treated with more rigid bracing had recurrent SCIWORA more frequently than did the 97 patients treated with a soft collar or no bracing (15 versus 7 percent, odds ratio [OR] 2.3; 95% CI 0.9-6.0). All patients with recurrent SCIWORA had full neurologic recovery.

One reasonable approach is to vary the duration of bracing depending upon the presence of neurologic deficits, findings on MRI, and results of nerve conduction studies as follows [3]:

Persistent neurologic deficit, positive MRI, or when performed, abnormal somatosensory evoked potentials – Rigid brace for three months

Transient resolved neurologic deficit or symptoms only and normal MRI or somatosensory evoked potentials – Padded hard collar (eg, Miami J or Aspen cervical collar) for one to two weeks and reevaluation by a neurosurgeon

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Cervical spine injury".)

SUMMARY AND RECOMMENDATIONS

Terminology – The term SCIWORA is commonly used to describe patients with neurologic findings suggestive of spinal cord injury with normal anatomic alignment and no bony abnormalities seen on plain film and/or CT of the spine. However, with the advent of MRI, approximately two-thirds of children with SCIWORA actually have demonstrable injury to the spinal cord, soft tissue components of the spinal column (ligaments, capsules, or muscles), or vertebral body endplate. (See 'Terminology' above.)

Pertinent anatomy – Relative to adults, children have several distinct anatomic features that predispose them to hypermobility of the spinal column in the absence of apparent bony injury. (See 'Pertinent anatomy' above.)

Children with genetic skeletal disorders such as Down syndrome, osteogenesis imperfecta, and mucopolysaccharidoses are at higher risk of SCIWORA and warrant a higher index of suspicion for injury after blunt trauma.

Epidemiology – SCIWORA typically involves the cervical spine and occurs with greater severity in children younger than eight years of age. Motor vehicle collisions, falls, and sports account for most instance of SCIWORA in children. (See 'Epidemiology' above.)

Clinical presentation – SCIWORA may present as definite evidence of spinal cord injury on physical examination or subtle transient neurologic symptoms (eg, bilateral paresthesia, weakness). Approximately one-quarter of affected children may experience delayed onset of neurologic signs minutes to days after injury, which can range from complete paralysis to partial neurologic deficits. (See 'Clinical features and diagnosis' above.)

Diagnosis – The diagnosis of SCIWORA is made when patients demonstrate clinical findings of spinal cord injury in the absence of spinal column injury on plain radiographs and CT and differentiates it from cervical spine fracture, subluxation, and dislocation. When children present with transient neurologic symptoms and normal neurologic exams, it may be difficult to distinguish cervical brachial plexus injuries (burners or stingers) from SCIWORA. The table provides a comparison of important features for each condition (table 1). (See 'Clinical features and diagnosis' above and 'Differential diagnosis' above.)

Radiologic evaluation – MRI should be performed in children who are suspected of having spinal cord injury but have normal plain radiographs (three views) and/or CT of the cervical spine. A high index of suspicion should be maintained for patients that present with transient neurologic symptoms, but normal neurologic examination if they are younger than eight years and have a high-risk mechanism (falls from a height or motor vehicle collisions). (See 'Radiologic evaluation' above.)

Initial management – Acute management priorities in children with spinal cord injury without radiographic abnormality (SCIWORA) consist of (see 'Initial management' above):

Spinal motion restriction – Protection of the cervical spine to prevent worsening of an existing spinal cord injury requires restricting motion in both the neck and the body. The neck is placed in a cervical collar, and the body is placed in the supine position, preferably on a soft conforming surface. Infants and young children warrant a cut out in the board or padding under the shoulders to maintain neutral position (figure 2). (See "Pediatric cervical spinal motion restriction", section on 'Techniques'.)

Stabilization – Provide emergency support of airway, breathing, and circulation while treating other life-threatening injuries. The cervical spine should be manually stabilized during airway management to prevent exacerbation of a spinal cord injury (figure 3). Initially, the airway can be opened with the jaw-thrust maneuver (figure 4). Orotracheal intubation with in-line manual stabilization provides the best approach to secure the airway in trauma victims without causing further cervical spine injury. (See "Pediatric cervical spinal motion restriction", section on 'Manual in-line stabilization of the cervical spine' and "Technique of emergency endotracheal intubation in children", section on 'Cervical spine immobilization'.)

Transient loss of spinal cord function can occur following spinal injury and result in vasodilatory shock. However, although hypotension can result from neurologic injury, clinicians must first assume that hypotension following trauma results from hemorrhage and ensure appropriate fluid resuscitation. (See "Trauma management: Approach to the unstable child", section on 'Circulation'.)

Emergency specialty consultation – Any injury associated with a neurologic deficit or spinal column instability requires emergency consultation with a pediatric spine surgeon or a spine surgery team (often staffed by pediatric orthopedists and pediatric neurosurgeons) to guide supportive care and definitive management of the injury. If consultation is not available onsite, immediate transfer must be arranged to a pediatric trauma center that can provide these services.

Definitive treatment – Definitive therapy should be based on the MRI findings and provided in consultation with a neurosurgeon with pediatric expertise. (See 'Initial management' above and 'Disposition' above.)

Disposition – Most children with SCIWORA warrant hospital admission. Criteria for discharge of individual patients from the emergency department in a padded hard cervical collar (eg, Miami J or Aspen cervical collar) are provided above. (See 'Disposition' above.)

ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges Alison Chantal Caviness, MD, who contributed to an earlier version of this topic review.

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Topic 15603 Version 18.0

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