INTRODUCTION
Spinal cord injury (SCI) has been broadly classified into complete and incomplete SCI. In complete SCI, there is no motor as well as sensory preservation in the sacral segment S4-5. However, in incomplete spinal cord injury, either the motor or the sensory function persists in the sacral segment S4-5.[1] There are four main types of incomplete SCI, namely, Central cord syndrome, Anterior cord syndrome, Posterior cord syndrome, and Brown Sequard syndrome.[2] The ATCCS is the most common type of incomplete SCI. It constitutes one-fourth of all the cases.[345] Central cord syndrome was first proposed by Schneider et al.[6] and it has a disproportionate greater involvement of upper limb motor power than lower limbs, bladder, and bowel involvement and a varied level of sensory involvement. Central cord syndrome has a bimodal presentation, with young patients (<30 years) getting generally affected due to fracture ± dislocation or traumatic disc prolapse, leading to cord compression whereas patients older than 60 years get the injury more often due to low energy trauma as a result of relative neck and head motion than the trunk (whiplash and hyperextension injury) on a background of cervical stenosis.[6] Historically, most ATCCS have shown better outcomes with conservative management. However, another school of thought proposes that early surgical intervention leads to better results.[7] This narrative review is an evidence-based approach that throws light on the gray areas and outlines the management of ATCCS and its present-day consensus.
MATERIALS AND METHODS
A search was conducted in PubMed, Scopus, and Google Scholar from January 1, 1980 to April 1, 2021 using the following search strategy: “central cord syndrome” [All fields] and “trauma” [All fields] and “management” [MeSH terms]; “prognosis” [MeSHterms];“complications” [MeSH terms]. Only journals in English were considered.
On preliminary search, 782 articles were shortlisted, out of which 209 articles were of the topic of interest. Abstracts of all the 209 articles were analyzed. Further appropriate cross-references from full-text articles were retrieved wherever necessary. Totally, 40 articles were shortlisted that dealt with both conservative and surgical management or early and delayed surgical intervention of ATCCS; these were included in the review. They were segregated based on the interest, and content was extracted from the relevant papers.
The authors were blinded to the journals’ and the authors’ names while reviewing. Journals’ scores (Impact Factor) were not considered as exclusion criteria for this review.
Understanding ATCCS
The spinal cord fills approximately 50% of the canal in the cervical and thoracolumbar spine and the rest is filled with CSF, epidural fat, and dura. The cross-section of the spinal cord has a unique arrangement of fibers. Centrally is the gray matter consisting of the lower motor neurons and interneurons. The periphery consists of white matter with fibers of both ascending and descending neurons [Figure 1].[8] In ATCCS, these crossed pyramidal tracts are affected. The outer white matter is more in the cervical region as it consists of fibers from cervical, thoracic, lumbar, and sacral levels. Topographically, the axons of lateral corticospinal tracts are arranged so that the lower limb fibers are lateral whereas the upper limb fibers are placed more medial. This medial portion of crossed pyramidal tracts is affected more. It is postulated that there is relative abundance of fibers with large motor axons innervating the fine motor skills of the hands as compared with neurons causing gross upper limb movements.[910] Hence, ATCCS can present as predominant upper limb involvement with predominance of the hand and forearm weakness in comparison to lower limb involvement. Central cord syndrome can also present as quadriparesis with sacral sparing. Fibers of tract of Goll and Burdach are arranged similar to corticospinal tracts.[8]
Figure 1: The somatotrophic organization of the tracts
Pathophysiology and mechanism of cord insult
The injury to the spinal cord can be broadly divided into primary injury and secondary injury.[2] Primary injury occurs during the accident. Secondary injury constitutes a complex cascade involving the biochemical mediators induced by the primary injury, causing the programmed cell death.
The possible clinical settings of ATCCS occurrence include already existing cervical spondylosis and stenosis, traumatic cervical fractures or dislocation, and acute disc prolapse.[11]
Most of ATCCS results from hyperextension injury in a background of already existing canal stenosis from hypertrophied ligamentum flavum, ossified posterior longitudinal ligament (OPLL), or disc-osteophyte complex.[61213]
Injury can also occur by direct compression from buckling of ligamentum flavum into the stenosed spinal canal. Herniated discs may lead to narrowing of the canal at a focal area. During the injury process, the cord gets pinched bidirectionally. It gets pinched anteriorly due to space-occupying lesions with a possible anterior spinal artery occlusion and posteriorly due to the buckled ligamentum flavum.[12] Younger patients with congenital cervical stenosis also have high chances of sustaining ATCCS by a hyperextension injury mechanism or because of traumatic disc prolapse.[141516] Younger individuals without any preexisting degenerative changes can sustain high-energy mechanism injury.[1113]
Clinical presentation
The ATCCS has bimodal age of presentation, with unique characteristics among the young and the elderly.
- Younger patients sustain ATCCS due to high-velocity injuries such as road traffic accidents or athletic injury. Central cord syndrome in this age group can occur because of an unstable spinal column, traumatic disc prolapse or due to hyperextension neck injury in an already stenosed congenital narrow canal.
- The elderly population present with injury due to relative motion of the neck and head with the trunk (whiplash and hyperextension injury).[6]
Diagnosis is made after a thorough clinical and neurological assessment. Typically, bilateral hand functions are predominantly affected. Pouw et al.[17] proposed that a difference of ≥10 motor points in upper and lower limb score on the ASIA scale supports the diagnosis of ATCCS. Sensory symptoms are variable. Bladder dysfunction typically manifests as urinary retention, and anal tone can be affected. Spasticity is present along with severe neurological deficit.
Investigation
The radiographic evaluation consists of orthogonal views of the cervical spine with an open mouth view to determine any fractures of the C1 and C2 region. Angulation of more than 11° and a translation of >3.5 mm of one vertebra over the adjacent vertebra is considered unstable.[1819] For injuries that are stable on radiological evaluation, stability can be confirmed by dynamic x-rays. Facet dislodgement, local segmental discal kyphosis, increased interspinous distance, and anterolisthesis are the instability criteria given by Roy Camillie.[20] Any fractures that are not seen on the radiographs or doubtful on radiographs can be confirmed with a CT scan. It provides a better understanding of the bony anatomy disruption. MRI scans are more useful in determining the soft tissue involvement and cord changes and are considered the best modality.[21] T2-weighted and STIR sequences are important in evaluating disc injury, posterior ligamentous complex injury, and cord injury, which can be seen as hyperintense signals. The presence of prevertebral intensity can be another sign of instability as well.[22] The presence of a hypointense signal surrounded by a hyperintense halo in T2-weighted images is seen in cord hemorrhage cases, which is a bad prognostic indicator for the neurological outcome.[2324] Newer modalities such as diffusion tensor imaging and fiber tractography are more sensitive than conventional MR scanning.[2526] These are better used for the evaluation of white matter anomalies.[27]
Initial care
Management of the ATCCS should begin at the accident site by proper triage by the caregiver. Resuscitation measures are done according to acute traumatic life support protocols. The neck is stabilized with a hard cervical orthosis. This prevents further cord damage due to excessive movements of the neck. Careful log rolling is carried out while shifting. The principle behind the initial management is to limit the secondary injury cascade and optimize perfusion of the penumbra around the injury site.[28]
Tissue perfusion of the affected cord is managed by maintaining adequate mean arterial pressure. Levi et al.[29] postulated that the mean arterial pressure maintained between 85 and 90 mmHg has a positive impact on neurological recovery. This was supported with similar findings by Vale et al.[30] However, a systematic review by Sabit et al.[31] showed no correlation between the mean arterial pressure and its impact on neurological recovery.
Methylprednisolone use for SCI is an epicenter of controversy for quite some while now. The drug halts the inflammatory cascade by acting as the cell membrane stabilizing agent and prevents lipid peroxidation.[323334] Three hallmark NASCIS trials were advocated by using a high dose of methyl prednisolone. In the NASCIS 2 trial, a dose of 30 mg/kg body weight bolus and 5.4 mg/kg/h of maintenance dose was administered for the next 24 h if the patient came within 8 h of the injury. In the NASCIS 3 trial, the maintenance dose was continued till 48 h if the patient came between 3 and 8 h of the injury. The NASCIS 2 study was a multicentric double-blinded RCT, with subjects randomized into three arms: (1) 30 mg/kg bolus followed by 5.4 mg/kg for 23 h; (2) Naloxone 5.4 mg/kg bolus followed by 0.5 mg/kg/h for 23 h; and (3) placebo. Both the studies had to face a lot of criticism due to the arbitrary cutoff timing of 3 h and 8 h and the adverse effects of high doses of the drug.[3334] However, despite the criticism, the evidence against its use is poor.[32] In their clinical practice guidelines, Fehling et al. recommended the usage according to NASCIS 2 trial with a dosage of 30 mg/kg bolus followed by a maintenance dose of 5.4 mg/kg/h over 24 h.[32] They also recommended against the usage of infusion over 48 h for those who presented after 8 h of injury.
Management
Conservative management
A patient with suspected diagnosis of ATCCS has to be immobilized with hard cervical orthosis. This stabilizes the motion segment and prevents further cord insult. Radiological evaluation is carried out to determine spine instability. In the absence of instability, the cervical orthosis is continued for six weeks or till the neck pain subsides.[35]
Surgical management
Instability determined by radiological evaluation is an absolute indication for surgery. Instability is defined as angular displacement >11° or vertebral translation of >3.5 mm compared with the adjacent vertebra.[36] Instability acts as a dynamic factor causing further cord insult. Management of patients with spine instability begins in the casualty room by applying traction using cervical tongs followed by closed reduction wherever relevant. This provides the earliest form of cord decompression with a positive impact on neurological recovery.[3738]
Patients with a plateau in neurological recovery or deterioration of neurology are considered surgical candidates.[39] The management of ATCCS in a stable spine is controversial.
Surgical options are laminectomy with instrumented fusion, laminoplasty, anterior cervical discectomy and fusion (ACDF), or anterior cervical corpectomy and fusion (ACCF). The type of surgery performed depends on the type of compression and also on the levels of involved segments.
Traumatic herniated nucleus pulposus (HNP) and fracture dislocation, which is reducible, can be managed with ACDF. However, management differs among patients with the degenerative spondylotic spine.
If cervical degenerative changes are in <2 levels, preference is given to the anterior approach. In case of ≥3 level involvement, consideration must be given to spondylotic neck pain and kyphosis. Multiple segments of ACDF or ACCF can be done. If ≥2 level ACCF is done, posterior fixation is contemplated due to possible chances of instrumentation failure. Laminoplasty can be an option in cases with minimal spondylotic neck pain with no or mild kyphotic cervical spine, no or early degenerative disc changes, and those in whom fusion is undesirable such as severe osteoporosis and poor bone healing. Laminectomy without instrumented fusion is not practiced in the modern era because of the possibility of post laminectomy kyphosis and iatrogenic instability, causing further cord compression. Laminectomy with fusion is usually done by the lateral mass fixation (LMF) technique. Fehling et al.[39] compared the outcomes of laminoplasty versus laminectomy and fixation. The latter had an inferior rate of neurological recovery based on the evaluation of Nurick score and patient-related outcome (PRO). Considering this, laminectomy and fixation may be an option in the multiple-level degenerative spine with coexisting mechanical neck pain with mild kyphosis. In case fusion is not necessary, laminoplasty is a better alternative. Cases with significant kyphosis require either anterior or anterior plus posterior approaches.[40]
Comprehensive rehabilitation
Whether managed conservatively or surgically, comprehensive rehabilitation is an essential component of management. Early mobilization and comprehensive rehabilitation is initiated once the patient is medically fit for it. Gait training and hand function restoration are the mainstay of rehabilitation.[535] In the early phase, rehabilitation is to be done on inpatient basis. Rehabilitation on an outpatient basis is contemplated after achieving certain goals. The role of an occupational therapist is pivotal in restoring the hand function.
Outcomes
Even though the neurological outcome is unpredictable, most patients experience spontaneous recovery to some extent regardless of treatment.[563241] Neurological recovery starts with the lower limbs followed by micturition and defecation control, and motor control of upper limbs is the last to improve.[532] Patients with an associated fracture tend to have worse findings on neurological examination at first presentation but they experience significant recovery during the first seven days of the injury. Penrod et al.[35] found improved functional independence and bladder/bowel recovery in the younger population as compared with older counterparts. Aarabi et al.[41] and Chen et al.[21] found that younger patients have good functional recovery. Spasticity, if present, denotes severe neurological injury and has worse functional outcomes.[42]
The most common disability with ATCCS is hand disability, as people with it may or may not recover completely.[35] Gait and hand functions are the major concerns as far as rehabilitation of these patients is concerned.
Yamazaki et al.[42] found that canal diameter and hyperintense signals on T2-weighted MRI scans correlated with poor neurological outcomes. The presence of hemorrhage and the overall extent of pathology noted on sagittal sections of MRI correlate with poorer neurologic prognosis.
Good hand grip, normal tone of muscles, normal cord intensity signal on MRI, and good ASIA motor score are good prognostic factors.[5611214344] Instability, severe cervical stenosis, persistent spasticity, and lack of rehabilitation are bad prognostic indicators.[322424345]
Controversies
Conservative vs operative management
In 1954, Schneider et al.[6] proposed that the natural course of ATCCS led to spontaneous recovery and surgical intervention is not necessary and is possibly harmful. Optimal medical management, early immobilization, and possibly intravenous steroids have improved the overall outcome of ATCCS.
Proper medical assistance of ATCCS includes shifting of a patient to the intensive care unit (ICU) for the initial days, with special care in the monitoring of mean arterial pressure (MAP). Volume resuscitation and ionotropic support supplementation are presumed to improve the neurological outcome by adequate perfusion of the affected cord.
In their study in 1954[6] and 1958[12] with 9 and 12 cases, respectively, Schneider et al. concluded that operative management is not required and may worsen the deficit if operated. Hence, they leaned more towards the conservative approach.
Ishida and Tominaga[11] presented their prospective study on 22 subjects managed without surgery and who had full functional outcome at six months of follow-up.
Pollard and Apple[46] evaluated patients with degenerative spondylotic spine and ATCCS. Patients were treated conservatively or surgically. Both groups demonstrated neurologic improvement without any statistical difference in outcomes. Operated subjects got discharged early and had hastened early functional recovery. However, there was no difference in the outcome in the long run compared with nonoperatively managed subjects.
In their study, Bose et al.[47] compared conservatively and surgically managed cohorts. The conservatively managed cohort did not have spinal instability. The surgically managed cohorts had structural spinal instability or plateaued neurological recovery. The operated subjects did not deteriorate postsurgery neurologically and had greater functional recovery at discharge than the conservative group.
Chen et al.[1321] conducted a retrospective and prospective[21] cohort study with 114 and 37 subjects, respectively. They observed that surgical candidates had a short hospital stay with early and predictable neurological recovery. Articles supporting conservative and surgical management are listed in Tables 1 and 2, respectively.
Table 1: Studies favoring conservative management
Table 2: Studies favoring surgical management
Early vs delayed surgical intervention
Early literature evaluating operative management of ATCCS reported poor outcomes compared with nonoperative management, partly because of lack of present-day sterilization and aseptic procedures. Surgical intervention has continued to become more common in the advent of newer diagnostic modalities, surgical techniques, patient risk profile, and comorbidity optimization.
The timing of operative management is controversial since the adoption of operative strategies in ATCCS. Literature has many varying definitions of early, late, or delayed operative management. However, consensus on early being ≤24 h of the presentation and late being >24 h after presentation is prevalent. Fehling et al.[54] concluded that early surgical intervention (≤24 h of presentation) has a good functional outcome. However, the level of supportive evidence considered in the review was low.
Lenehan et al.[55] believed that early surgery in cases with obvious compression and profound deficit showed positive results as far as functional outcome is considered.
A multicentric prospective study with 313 subjects by Fehling et al.[56] found that early operative management had a positive impact on the ASIA score.
In their study of 42 patients undergoing operative management for ATCCS, Aarabi et al.[41] showed no statistical differences in long-term ASIA motor score, functional outcomes, manual dexterity, and dysesthesia pain at 12 months in patients with early (<24 h) versus late (> 48 h) surgical intervention with regression analysis.
Guest et al.[51] divided the cohort based on injury cause with 50 subjects of ATCCS. The first cohort had subjects with fracture dislocation, or acute HNP, and the other cohort had subjects with already existing canal stenosis. Each cohort was subdivided into those <24 h from injury and those >24 h from injury. The former cohort had a good neurological recovery on early operative management compared with delayed operative management.
Kepler et al.[57] found no statistical difference in ASIA motor score at the seventh day compared with those undergoing early (<24 h) as compared with late surgery (>24 h).
Lenehan et al.[55] concluded that the odds of having an increase of two grades in the ASIA score were 2.8 times greater in the early surgery group than in the late surgery group. However, this study did not analyze ATCCS separately and hence conclusions cannot be generalized.
In their ambisceptive cohort analysis, Zheng et al.[58] could find the positive impact of early surgery within two weeks. Surgery within two weeks reduces the dysfunction of motor neurons at and also distal to the injury site, reduces secondary motor neuron loss, and hence leading to a better surgical outcome.
A systematic review (2019)[59] by the senior author and a consensus statement by the STSG of the International Spinal Cord Society and Spinal Cord Society in 2019 considered the main concerns in the management of ATCCS. The review consisted of 37 articles ranging from 1980 to 2017. The consensus proposed that early operation (<24 h) had a positive outcome in cases with absolute surgical indication. However, evidence supporting early surgery in stable spine and deteriorating neurology is low. The review also brought out the necessity of better quality prospective studies that would resolve the dilemma.
During this narrative review, a thorough literature search was done. However, no prospective studies have been published as far as operative intervention and its timings are concerned after the systematic review just cited.
CONCLUSION
ATCCS is the most common type of incomplete SCI. The presence of instability, plateaued neurological recovery, and progressive neurological deterioration are absolute indications for operative management. Evidence regarding the timing of the surgical intervention is less clear. The studies comparing early with late stabilization have shown mixed results. Since there is only a low level of evidence in favor of early surgery in ATCCS with no instability and deteriorating neurology, the decision of the operative management and its timing should be decided by the surgeon, with each patient being evaluated on merit and the management plan tailored assessing the risk–benefit ratio. This review highlights the need for well-conducted prospective studies to resolve the controversy regarding early surgery versus conservative management and delayed surgery if recovery plateaus or neurological deterioration occurs.
Ethical policy and institutional review board statement
Not applicable.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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