Traumatic spinal cord injury (SCI) refers to a trauma to the spinal cord leading to impaired motor, sensory, and/or autonomic function.
Causes of traumatic SCI include the following:
- Automobile accidents
- Violence such as gunshots or penetrating wounds
- Sports injuries
- Diving accidents
Unique etiologies of pediatric SCI include the following:
- Lap belt injuries.
- Spinal cord injury without radiographic abnormalities (SCIWORA)1,2that are traumatic.
- Higher cervical injuries related to:
- Skeletal dysplasia, which increase the risk of SCI as in rheumatoid arthritis, due to atlanto-axial instability resulting from synovitis of the facets and destruction of the dens
- Achondroplasia which the child develops myelopathy and central apnea due to a small foramen magnum
- Osteogenesis imperfecta, with its complication of basilar invagination, though SCI due to this condition is rare
The age-specific etiologies are summarized below as follows:3
|Age (y)||Motor Vehicle Injury (%)||Violence (%)||Sports (%)||Falls (%)||Medical Surgical (%)||Other (%)|
Epidemiology including risk factors and primary prevention
- Annual incidence of SCI in the U.S is 40 cases per 1 million population or 12,000 new cases each year.3
- Children younger than 15 years constitute 3% to 5% of SCIs (300-500 cases annually), and patients younger than 20 years comprise 20% of all SCIs (2000 cases annually).3
- Like adults with SCI, males more commonly sustain SCI than females during adolescence. However, the preponderance of males becomes less marked as age of injury decreases, such that females equal males in those 3 years of age or younger.4
- For children 0-8 years old 70% are paraplegic and approximately 66% have complete lesions.4
- For children >8 years and older, half of the children are paraplegic and about half of them have complete lesions.4
Children have greater spinal mobility and less spinal stability than adults do. The cervical spine and its ligaments take 8 to 10 years to mature and achieve stability. The spinal ligaments are generally more elastic and the facet joints have a shallow and horizontal orientation compared with the adult spine. The incompletely ossified vertebral bodies have relative anterior wedging. Moreover, a child’s head is relatively large compared with the strength of the neck muscles.
The fulcrum of movement on flexion and extension is higher, at the C2-3 level, and the increased mobility results in more frequent high cervical injuries than in adults. SCIWORA1 is explained based on the differential stretch hypothesis: higher water content allows pediatric intervertebral disks to stretch and expand. This permits significant distraction of the spinal column and cord, leading to injury. Traumatic injury to the vascular supply of the spinal cord results in cord ischemia. Injury to the spinal cord via force transmission through the intact spinal column can also occur.
Disease progression including natural history, disease phases or stages, and disease trajectory – clinical features and presentation over time
Affected children are more likely to have complete injuries.5 Magnetic resonance imaging (MRI) findings correlate with neurologic recovery. Complete injuries have shown extensive hemorrhage and cord disruption with poor prognosis for recovery.6 Motor and sensory recovery depends on the nature of the injury. At 1-year post-injury, cases of incomplete SCI show a higher degree of motor recovery than those of complete SCI. Motor recovery is also greater in people with tetraplegia compared with that of people with paraplegia. Functional recovery in SCI occurs by compensation and neural plasticity rather than by repair.7
Specific secondary or associated conditions and complications
Spasticity, autonomic dysreflexia, pain, neurogenic bladder, urinary tract infections, neurogenic bowel, pressure ulcers, depression, osteoporosis, sexual dysfunction, joint contractures, and heterotopic ossification are all seen in children. Immobilization hypercalcemia is unique to adolescent boys and occurs in the first 3 months after injury.3 Pathologic fractures of long bones, latex allergy, syringomyelia, and hip instability are common in children.<sup>8</sup>
Children with SCI are at unique risk for several orthopedic complications that do not occur in the skeletally mature adult. The first is scoliosis. Almost every child who sustains an SCI before skeletal maturity develops scoliosis, and approximately two-thirds of these children require surgery.9 Thoracolumbar-sacral orthotic bracing has been shown to significantly slow the rate of curve progression and delay the need for surgery in children with SCI.10 Close surveillance and referral to surgery when appropriate is essential to caring for pediatric spinal cord patients. General indications for spine fusion surgery in children with neurogenic scoliosis secondary to SCI include curves greater than 40° by Cobb angle, age greater than 10 years, rapid progression of the curve, and functional problems or pain in a mature patient.
2. ESSENTIALS OF ASSESSMENT
Inquire about the nature of trauma, pain, new numbness/paresthesia, weakness, and function. Information as to the location, severity, and nature of symptoms, which may involve upper and lower extremities, should be included. Inquire as to whether the symptoms are transient, evolved over time, or permanent. Inquire about new-onset incontinence in a previously continent child. The presence of neck and back pain suggests the possibility of spinal injury, although back pain in children is rare.
Physical examination should include a general exam and an exam specific to SCI. A general physical exam for the respiratory tract (especially in patients with tetraplegia or high paraplegia), abdominal examination, skin to rule out decubitus, and cranial nerves should be included. A physical exam specific to SCI is important in determining the level and extent of injury to the upper motor neurons. The exam involves testing all dermatomal levels from C2-S5 using both touch and pinprick sensation, key muscle groups from C5-T1 and L2-S1, as defined by the American Spinal Injury Association (ASIA), and reflexes (bulbocavernosus, abdominal, deep tendon, and Babinski). Neurologic level of injury should be determined, as outlined by the ASIA guidelines.
Studies have demonstrated that the ASIA motor and sensory exams most likely do not have utility in children less than 6 years of age and hence, may not be an appropriate method to determine neurological consequence of SCI in infants and toddlers. While examinations can be performed in children as young as 4 years of age the interrater reliability is poor prior to age 6. Children injured at a young age and those who are not toilet trained prior to injury, with limited experience with volitional bowel movements, have diﬃculty with the anal motor exam. Clinicians may want to explain to parents how standardized testing for neurological classiﬁcation is performed and, because of their child’s young age, classiﬁcation as complete or incomplete may not be reliably determined and only an estimated neurological level can be provided from the examination.11,12
Currently there is no validated alternative methods of assessment of neurological impairment in babies with SCI. Monitoring physiological variables such as heart rate and blood pressure during sensory testing or the use of electrodiagnostics such as nerve conduction studies may also be eﬀective techniques in assessing the neurological consequence of SCI in young children. Correlating the relationships between images of the injured spinal cord on CT scan or MRI combined with ASIA motor and sensory scores may be useful for younger children or for older children who are unable to cognitively fully participate in the ASIA examination.11,12
Functional assessment should include mobility for transfers, potential for ambulation (in cases of incomplete injury), and self-care. Bowel and bladder function and toileting skills should be assessed. A neuropsychologic evaluation is essential if concomitant brain injury is suspected. The Functional Independence Measure (FIM) or Wee FIM II (in infants, children, and younger adolescents) and the Spinal Cord Independence Measure III may be used to measure progress.13,14
Complete blood count, comprehensive metabolic profile, serum calcium, phosphate, parathyroid hormone, and urinalysis with culture are the minimum laboratory tests to be included during admission.
Plain radiographs and computed tomography images of the spine are essential to investigate for fractures, dislocations, bleeding, and other associated injuries.
MRI, with or without contrast, is recommended in patients when SCI is suspected. In SCIWORA, MRI may be normal (~35%)2,15 or abnormal. It is useful for detecting both extraneural and intraneural damage to the cord, and for determining prognosis. The contrast study would delineate ligamentous or soft tissue injury, scarring, or disk herniation. MRI of the brain is indicated if brain injury is suspected.
Supplemental assessment tools
The use of somatosensory evoked potentials in comatose patients who cannot provide an adequate response to enable an assessment of cord injury is justified with suspected SCIWORA.
Early predictions of outcomes
Younger patients tend to have more complete and severe injuries.
At the time of initial injury, high-energy mechanisms, thoracic involvement, younger age, and complete injury portend a poor prognosis.1 In patients with complete injuries, any neurologic recovery is rare. Patients with incomplete injuries tend to have a good prognosis7 in terms of motor and sensory function that can translate to ambulation and self-care. MRI showing intraneural hemorrhage is typically accompanied by complete injury with permanent deficits in SCIWORA. A normal MRI is typically indicative of a complete recovery.
Environmental barriers need to be identified and addressed in terms of accessibility issues at home and in the community. Examples include the following: entrance/exit ramps and doorway clearance for wheelchair accessibility, grab bars in the bathroom for easier access, and wheelchair transportation options for community and recreational activities.
Social role and social support system
Assess the support system and social roles to address functional needs and provide support at home, for discharge, and for community reintegration. Inquire about the school setting, family roles, and necessary support systems, and ensure that they are available and that modifications are made.
Identify and/or consider specific issues relevant to ethics, quality of life, professionalism, and safety.
Delayed onset of neurologic findings, normal x-rays of the spine, and unfamiliarity with SCIWORA may lead to the inappropriate discontinuation of spinal precautions and bracing. Such decisions could worsen the injury, leading to poor functional outcomes and medicolegal situations.
3. REHABILITATION MANAGEMENT AND TREATMENTS
Available or current treatment guidelines
No current specific treatment guidelines are available. A review of the current standard of medical and rehabilitation care has been previously described.16
Rehabilitation must be developmentally based.3-6 Goals should address growing children’s needs regarding health maintenance and the restoration of function and productivity to improve quality of life and life satisfaction. Interventions encompass training in mobility, activities of daily living, skin care, bladder and bowel programs, recreation, psychosocial counseling, education, vocational support, and community reintegration. Devices, which may assist with mobility, vary according to age, growth, and size. They may range from parapodia, strollers, and wheelchairs to gait orthoses, functional electric stimulation, modifications for sports, and driving.
Long-term community ambulation is dependent on several factors including ASIA Impairment Scale score, age, body size, and compliance with the treatment program. Community ambulation is most likely in cases involving young patients, L3 or lower lesions, or impairment scores of D.17,18
At different disease stages
Treatment consists of:
- Spine immobilization to prevent further neurologic injury.
- Supportive care for spinal and neurogenic shock.
- Deep vein thrombosis and GI prophylaxis.
- Pain control.
- Use of high dose methylprednisolone to improve neurologic recovery in the pediatric population is not currently recommended.19
Subacute Stage/Rehabilitation Phase
- Physical/occupational/speech therapy to learn mobility, self-care, and wheelchair management.
- Bowel program: Bowel programs are initiated at the developmentally appropriate age of 2 to 4 years, or earlier if they are experiencing diarrhea or constipation. Children who have hand function that is adequate to perform independent bowel care should begin their own bowel programs when they are 5 to 7 years old.3,20
- Bladder program: Clean intermittent catheterization (IC) is the standard bladder management for children and adolescents with SCI. IC is initiated when the child is approximately 3 years old, or earlier if the child is having recurrent urinary tract infections or is starting to develop renal impairment. Children who have hand function that is adequate to perform self-catheterization should begin self-catheterization when they are 5 to 7 years old.3,5,21-23
- Prophylaxis of venous thromboembolism: Deep venous thrombosis (DVT) is very rare between birth and 5 years of age (0%) and from 6 to 12 years of age (2%). The incidence increases from age 13 to 15 years (8%) and age 16 to 21 years (9%). Due to the low incidence of DVT in children who are injured before reaching adolescence, it may be reasonable to limit the use of anticoagulants to those who have other risk factors for DVT such as concurrent fracture.24,25
- Hypercalcemia: This most commonly involves adolescents and young adult males during the first 3 months after injury. Patients with hypercalcemia typically present with abdominal pain, nausea, vomiting, lethargy, malaise, polydipsia, polyuria, and dehydration. Serum calcium and ionized calcium are elevated above age-appropriate normal values. Complications may include nephrolithiasis, urolithiasis, and renal failure. Treatment may include pamidronate and hydration with intravenous normal saline.26-28
- Pressure Ulcers: Pressure ulcers are one of the most common complications for children and adolescents with SCI. Prevention of pressure ulcers with relief techniques may include wristwatches with automatic resetting timers to remind children about pressure relief. As the patient grows, properly fitting wheelchairs and adequate cushions must be prescribed with pressure mapping to reduce the risk of pressure injury. Pressure ulcer prevention must be developmentally appropriate and responsibility must be progressively shifted from the parents to the patient as they grow up.29
- Pulmonary complications: As in the case of adult patients with SCI, pulmonary dysfunction is a major complication during both the acute and chronic phases of SCI. Children with high cervical injuries require lifelong ventilatory support.5,30
- Pain control with medical management: Chronic pain can be a significant co-morbidity in children and adolescents with SCI. Pain can cause disability and affect school, work, and social interactions.31
- Psychologic and behavioral support during challenging transition times.
- Spasticity management: Compared with adults, a smaller percentage of children with SCI experience spasticity. The goals of managing spasticity are to improve function, prevent complications, and alleviate pain and incontinence.32
- Autonomic dysreflexia: Noxious stimuli must be identified and minimized. Due to lower blood pressures in children with SCI secondary to both age and neurologic level, it is important that a baseline blood pressure is recorded in this population. A blood pressure elevation of 20 to 40 mmHg above this baseline may be considered autonomic dysreflexia. The varying cognitive and verbal communication abilities of children with SCI may result in less clear communication of symptoms as compared to adults.33,34
- Hyperhidrosis: Commonly seen in SCI patients with tetraplegia or thoracic paraplegia. It should be treated if it impairs function, increases the risk of developing a pressure ulcer, or is uncomfortable for the patient. Management should start with the avoidance and alleviation of precipitating factors, and if this fails, medications such as propantheline or transdermal scopolamine should be considered.35,36
- Provide family/caretaker training and education for necessary modifications for discharge along with community resources.
Health maintenance and prevention of secondary complications associated with neurogenic bowel and bladder, management of spasticity and pain, prevention of pressure ulcers, screening for osteoporosis with a dual-energy x-ray absorptiometry scan, and immunizations are all critical during the chronic phase.
Other aspects include the following:
- Annual assessments with the ASIA exam to evaluate for changes/recovery.
- Continued education for the identification and management of autonomic dysreflexia.
- Ongoing family/caretaker training as needs change.
- Identification and utilization of available community resources for school, educational, vocational, and leisure activities.
Coordination of care
A multidisciplinary team consisting of the patient, family, physiatrist, nursing, physical, occupational and speech therapists, rehabilitation psychologist, social worker, child-life support/recreation therapist, discharge coordinator, and other medical/surgical specialties must work together to achieve successful rehabilitation and discharge to the community. An education consultant is recommended to assist with school reentry.
The transition to adulthood is a major goal of caring for children with spinal cord injuries.37-39 The importance of early transition planning is central to future employment in adult life, as well as future life satisfaction.40,41
Patient & family education
Care for the pediatric spinal cord patient must be oriented around the family due to the central role of parents and family in the care of a child or adolescent.42 Education of the patient and family is vital for medical management, including autonomic dysreflexia and skin care, and activity participation is warranted. Families should receive training in mobility, self-care, and bowel and bladder programs, depending on the deficits and developmental age of the child, readiness for training, and availability of community resources. Equipment transitions with assistive technology, such as progressive mobility needs (parapodium to manual or power wheelchair), will facilitate community integration.43 Anticipatory guidance, a term that refers to the education of children and parents regarding the future implications of disability, is critical to enabling successful transitions through each developmental stage and eventually into adulthood.
Tracking includes the following:
- Neurologic recovery via the ASIA exam.
- Functional changes in FIM and Wee FIM II scores.
- Success in community reintegration.
- Participation in academic, athletic, and recreational activities.
- Participation in community activities.
Translation into practice: practice “pearls”/performance improvement in practice (PIPs)/changes in clinical practice behaviors and skills
A unique opportunity arises to educate primary care pediatricians regarding the identification and management of the conditions to which the SCI population is prone, such as obesity, hyperlipidemia, metabolic syndrome, and aging. The promotion of wellness programs with accessibility and advocacy is critical.
4. CUTTING EDGE/EMERGING AND UNIQUE CONCEPTS AND PRACTICE
Cutting edge concepts and practice
One practice involves epidural stimulation of the lumbosacral spinal cord for voluntary movement, standing, and assisted stepping.44
Activity-based restoration therapies in promoting recovery of function are promising.45
A neuroprosthesis, if applicable, may allow for increased functional independence.46
Functional electric stimulation for mobility, activities of daily living, and bladder function can be made more available.47
Body weight-supported gait training and robotic therapies for functional and vocational activities have been explored.48
GAPS IN THE EVIDENCE-BASED KNOWLEDGE
Gaps in the evidence-based knowledge
The clear role of methylprednisolone in acute SCI should be reinvestigated.
Platelet-rich plasma treatments for pressure ulcer treatments need to be studied.
More research is needed to evaluate the role of assistive technology to promote self-care and mobility with functional electric stimulation and robotics.
Telemedicine for preventative care, such as for pressure sores, should be studied.
The use of bisphosphonates such as Zoledronic Acid to improve bone density in pediatric spinal cord injured patients needs further investigation.49
- Pang D. Spinal cord injury without radiographic abnormality in children, 2 decades later. Neurosurgery. 2004;55(6):1325-42; discussion 1342-3.
- Yucesoy K, Yuksel KZ. SCIWORA in MRI era. Clin Neurol Neurosurg. 2008;110(5):429-433. doi: 10.1016/j.clineuro.2008.02.004 [doi].
- Vogel LC. DM. Pediatric spinal cord injury issues: Etiology, demographics, and pathophysiology. Top Spinal Cord Inj Rehabil. 1997(3):1-8.
- Vogel L, Betz R, Mulcahey M. Pediatric spinal cord disorders. In: Kirshblum S, Campagnolo D, DeLisa J, eds. Spinal cord medicine. 1st ed. ed. Lippincott Williams & Wilkins; 2001:438-462.
- Massagli TL. Medical and rehabilitation issues in the care of children with spinal cord injury. Phys Med Rehabil Clin N Am. 2000;11(1):169-182.
- Machino M, Yukawa Y, Ito K, et al. Can magnetic resonance imaging reflect the prognosis in patients of cervical spinal cord injury without radiographic abnormality? Spine (Phila Pa 1976). 2011;36(24):E1568-72. doi: 10.1097/BRS.0b013e31821273c0 [doi].
- Anderson C, Vogel L, De Vivo M, Betz R, McDonald C. Pediatric spinal cord injury: Evidence-based practice and outcomes. J Rehabil. 2004(10):69-78.
- Curt A, Van Hedel H, Klaus D, Dietz V. EM-SCI study group. recovery from a spinal cord injury: Significance of compensation, neural plasticity, and repair. J Neurotrauma. 2008(25):677-685.
- Dearolf WW,3rd, Betz RR, Vogel LC, Levin J, Clancy M, Steel HH. Scoliosis in pediatric spinal cord-injured patients. J Pediatr Orthop. 1990;10(2):214-218.
- Mehta S, Betz RR, Mulcahey MJ, McDonald C, Vogel LC, Anderson C. Effect of bracing on paralytic scoliosis secondary to spinal cord injury. J Spinal Cord Med. 2004;27 Suppl 1:S88-92.
- Mulcahey M, Gaughan J, Betz R, Johansen K. The international standards for neurological classiﬁcation of spinal cord injury: Reliability of data when applied to children and youths. Spinal Cord. 2007(45):452–459.
- Mulcahey MJ, Gaughan JP, Chafetz RS, Vogel LC, Samdani AF, Betz RR. Interrater reliability of the international standards for neurological classification of spinal cord injury in youths with chronic spinal cord injury. Arch Phys Med Rehabil. 2011;92(8):1264-1269. doi: 10.1016/j.apmr.2011.03.003 [doi].
- 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(8):880-885. doi: 10.1038/sc.2011.20 [doi].
- 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(7):431-438. doi: 10.1177/000992289403300709 [doi].
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- Hussey RW, Stauffer ES. Spinal cord injury: Requirements for ambulation. Arch Phys Med Rehabil. 1973;54(12):544-547.
- Vogel LC, Lubicky JP. Ambulation in children and adolescents with spinal cord injuries. J Pediatr Orthop. 1995;15(4):510-516.
- Pettiford JN, Bikhchandani J, Ostlie DJ, St Peter SD, Sharp RJ, Juang D. A review: The role of high dose methylprednisolone in spinal cord trauma in children. Pediatr Surg Int. 2012;28(3):287-294. doi: 10.1007/s00383-011-3012-3 [doi].
- Goetz LL, Hurvitz EA, Nelson VS, Waring W,3rd. Bowel management in children and adolescents with spinal cord injury. J Spinal Cord Med. 1998;21(4):335-341.
- Fernandes ET, Reinberg Y, Vernier R, Gonzalez R. Neurogenic bladder dysfunction in children: Review of pathophysiology and current management. J Pediatr. 1994;124(1):1-7. doi: S0022-3476(94)70245-4 [pii].
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- McLaughlin JF, Murray M, Van Zandt K, Carr M. Clean intermittent catheterization. Dev Med Child Neurol. 1996;38(5):446-454.
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- Kedlaya D, Brandstater ME, Lee JK. Immobilization hypercalcemia in incomplete paraplegia: Successful treatment with pamidronate. Arch Phys Med Rehabil. 1998;79(2):222-225. doi: S0003-9993(98)90304-5 [pii].
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Original Version of the Topic
K. Rao Poduri, MD, Colin D Canham, MD, Woojoong Lee, MD. Traumatic spinal cord injury. 05/12/2013.
Heather Asthagiri, MD
Nothing to Disclose
Justin Weppner, DO
Nothing to Disclose