Spinal Cord Injuries in Children

Spinal Cord Injuries in Children

Introduction

Spinal cord injuries (SCI) in children present unique challenges due to the developing anatomy and physiology of pediatric patients. These injuries can lead to devastating long-term consequences, making early recognition, appropriate management, and comprehensive rehabilitation crucial. This document provides an overview of pediatric SCI, covering epidemiology, etiology, pathophysiology, clinical presentation, diagnostic approaches, management strategies, and long-term outcomes.

Epidemiology

Pediatric SCI is relatively rare, with an estimated incidence of 1.99 to 2.79 per 100,000 children per year. However, the impact on patients, families, and healthcare systems is profound. Key epidemiological features include:

  • Age distribution: Bimodal, with peaks in early childhood (0-5 years) and adolescence (15-17 years).
  • Gender: Higher incidence in males, particularly in adolescence.
  • Mortality: Highest in the first year post-injury, especially for high cervical injuries (C1-C4).
  • Incomplete injuries: More common in children compared to adults, offering better potential for neurological recovery.

Etiology

The causes of pediatric SCI vary by age group and geographical location. Common etiologies include:

  1. Motor vehicle accidents (MVAs): Leading cause in adolescents.
  2. Sports and recreational activities: Particularly in adolescents (e.g., diving, gymnastics, football).
  3. Falls: More common in younger children.
  4. Nonaccidental trauma (child abuse): Especially in infants and young children.
  5. Gunshot wounds and other violence: More prevalent in certain geographical areas.
  6. Medical/surgical complications: Such as birth injuries or complications of spine surgery.

Specific pediatric conditions predisposing to SCI include:

  • SCIWORA (Spinal Cord Injury Without Radiographic Abnormality): More frequent in children due to the elasticity of their spinal columns.
  • Congenital anomalies: Such as Klippel-Feil syndrome or os odontoideum.
  • Skeletal dysplasias: Increasing risk of cervical spine instability.
  • Juvenile rheumatoid arthritis: Potential for atlantoaxial instability.

Pathophysiology

Understanding the pathophysiology of pediatric SCI is essential for developing targeted therapeutic interventions. The injury involves both primary and secondary mechanisms:

Primary Injury

This results from the initial mechanical insult, causing immediate tissue damage:

  • Direct compression or transection of the spinal cord.
  • Disruption of blood vessels leading to ischemia.
  • Axonal shearing and neuronal membrane disruption.

Secondary Injury

A cascade of events following the primary injury that can exacerbate damage:

  1. Vascular changes: Hemorrhage, vasospasm, thrombosis leading to ischemia.
  2. Excitotoxicity: Excessive release of excitatory neurotransmitters (e.g., glutamate).
  3. Ionic imbalances: Accumulation of intracellular calcium and sodium.
  4. Free radical production: Causing lipid peroxidation and DNA damage.
  5. Inflammation: Release of pro-inflammatory cytokines and chemokines.
  6. Apoptosis: Programmed cell death of neurons and oligodendrocytes.

Children's response to SCI differs from adults due to their immature nervous and musculoskeletal systems:

  • Increased neural plasticity may allow for better functional recovery.
  • The developing spine can lead to delayed onset of spinal deformities (e.g., scoliosis).
  • Ongoing growth can result in tethered cord syndrome years after the initial injury.

Clinical Presentation

The clinical manifestations of pediatric SCI depend on the level and completeness of the injury. A thorough neurological examination is crucial, but may be challenging in young or uncooperative children.

Key Assessment Points:

  1. Level of consciousness and cognition.
  2. Cranial nerve function (particularly for high cervical injuries).
  3. Motor function: Strength, tone, reflexes.
  4. Sensory function: Light touch, pinprick, proprioception.
  5. Autonomic function: Heart rate, blood pressure, temperature regulation, sweating.
  6. Respiratory status: Diaphragm and accessory muscle function.
  7. Bowel and bladder function.

ASIA Impairment Scale (AIS)

The International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI) should be used when possible:

  • Grade A: Complete. No sensory or motor function preserved in sacral segments S4-S5.
  • Grade B: Incomplete. Sensory, but not motor, function preserved below the neurologic level including S4-S5.
  • Grade C: Incomplete. Motor function preserved below the neurologic level; most key muscles below the level have muscle grade < 3.
  • Grade D: Incomplete. Motor function preserved below the neurologic level; most key muscles below the level have muscle grade ≥ 3.
  • Grade E: Normal. Sensory and motor functions are normal.

Spinal Shock

This phenomenon, characterized by flaccid paralysis and loss of reflexes below the level of injury, can mask the true extent of SCI in the acute phase. It may last from several days to weeks, complicating early prognostication.

Associated Injuries

Children with SCI often have concomitant injuries that require attention:

  • Traumatic brain injury (TBI).
  • Fractures of the extremities or pelvis.
  • Abdominal or thoracic visceral injuries.
  • Brachial plexus injuries (especially in birth-related SCI).

Diagnostic Approaches

Accurate and timely diagnosis of pediatric SCI is critical. The diagnostic workup should be tailored to the patient's age, mechanism of injury, and clinical presentation.

Imaging

  1. Plain Radiographs:
    • Often the initial imaging modality.
    • Assess alignment, bone integrity, and soft tissue swelling.
    • Limited in evaluating ligamentous injuries and the spinal cord itself.
  2. Computed Tomography (CT):
    • Excellent for detecting bony injuries.
    • Useful for patients with altered mental status or inconclusive radiographs.
    • Consider radiation exposure, especially in young children.
  3. Magnetic Resonance Imaging (MRI):
    • Gold standard for evaluating the spinal cord, ligaments, discs, and soft tissues.
    • Can detect cord edema, hemorrhage, compression, and SCIWORA.
    • May require sedation in young children.
  4. Dynamic Imaging:
    • Flexion-extension radiographs or CT may be used to assess instability.
    • Must be performed under medical supervision to prevent further injury.

Electrophysiological Studies

These can provide valuable information about spinal cord function, especially when clinical examination is limited:

  • Somatosensory evoked potentials (SSEPs).
  • Motor evoked potentials (MEPs).
  • Electromyography (EMG) and nerve conduction studies (NCS).

Laboratory Studies

While not specific for SCI diagnosis, they are important for overall management:

  • Complete blood count (CBC).
  • Coagulation profile.
  • Arterial blood gas (for respiratory assessment).
  • Renal and liver function tests.

Acute Management

The primary goals in the acute phase are to prevent secondary injury, maintain adequate perfusion to the spinal cord, and address life-threatening complications.

Prehospital Care

  • Immobilization of the spine with age-appropriate devices.
  • Airway management with in-line stabilization.
  • Maintenance of adequate oxygenation and circulation.

Emergency Department Management

  1. Primary and secondary surveys following ATLS (Advanced Trauma Life Support) guidelines.
  2. Respiratory support: Intubation may be necessary for high cervical injuries.
  3. Hemodynamic stabilization:
    • Treat neurogenic shock (bradycardia and hypotension due to loss of sympathetic tone).
    • Avoid hypotension (maintain age-appropriate systolic blood pressure).
    • Use of vasopressors (e.g., norepinephrine) may be required.
  4. Corticosteroids: The use of high-dose methylprednisolone remains controversial in pediatric SCI. Decision should be individualized based on current evidence and risk-benefit analysis.
  5. Early consultation with pediatric neurosurgery and/or orthopedic spine surgery.

Surgical Management

Indications for surgery include:

  • Progressive neurological deterioration.
  • Irreducible deformity or instability.
  • Significant spinal cord compression.
  • Open injuries or CSF leaks.

Timing of surgery is individualized, but early decompression (within 24 hours) may improve neurological outcomes in certain cases.

Intensive Care Management

  1. Respiratory:
    • Close monitoring of vital capacity and negative inspiratory force.
    • Aggressive pulmonary hygiene to prevent atelectasis and pneumonia.
    • Non-invasive ventilation or tracheostomy may be needed for high-level injuries.
  2. Cardiovascular:
    • Continuous cardiac monitoring.
    • Management of autonomic dysreflexia (paroxysmal hypertension, bradycardia, sweating above the level of injury).
  3. Gastrointestinal:
    • Stress ulcer prophylaxis.
    • Early nutritional support (preferably enteral).
    • Bowel program initiation to prevent impaction.
  4. Genitourinary:
    • Indwelling catheter placement with strict aseptic technique.
    • Monitoring for urinary tract infections.
  5. Skin:
    • Regular turning and positioning.
    • Use of appropriate pressure-relieving surfaces.
    • Vigilant skin checks to prevent pressure ulcers.
  6. Thermoregulation:
    • Monitor for poikilothermia (fluctuations with environmental temperature).
    • Prevent hypothermia or hyperthermia.

Rehabilitation

Rehabilitation should begin as early as possible, even in the intensive care setting. A multidisciplinary team approach is essential, involving physiatrists, physiotherapists, occupational therapists, speech therapists, psychologists, social workers, and other specialists as needed.

Key Components of Pediatric SCI Rehabilitation:

  1. Physical therapy:
    • Range of motion exercises to prevent contractures.
    • Strengthening of preserved muscle function.
    • Mobility training (transfers, wheelchair skills, gait training if appropriate).
  2. Occupational therapy:
    • Activities of daily living (ADLs) training.
    • Adaptive equipment prescription.
    • Environmental modifications.
  3. Speech and language therapy:
    • For patients with concomitant TBI or those requiring ventilatory support.
    • Augmentative and alternative communication devices if needed.
  4. Psychological support:
    • Addressing adjustment to injury, body image issues, and developmental concerns.
    • Family counseling and education.
  5. Educational reintegration:
    • Collaboration with schools for appropriate accommodations.
    • Vocational planning for adolescents.

Assistive Technology

Rapidly evolving technological advances offer new possibilities for enhancing function and independence:

  • Computer-based augmentative communication.
  • Environmental control systems.
  • Functional electrical stimulation (FES).
  • Robotic exoskeletons for gait training.

Spasticity Management

Spasticity often develops weeks to months after injury. Management options include:

  1. Physical interventions: Stretching, casting, orthotics.
  2. Oral medications: Baclofen, diazepam, dantrolene.
  3. Focal interventions: Botulinum toxin injections.
  4. Surgical options: Intrathecal baclofen pump, selective dorsal rhizotomy.

Long-term Complications and Management

Children with SCI require lifelong follow-up to monitor for and manage potential complications:

Musculoskeletal

  1. Spinal deformity:
    • Scoliosis is common, especially in children injured before the adolescent growth spurt.
    • Regular radiographic monitoring and early intervention (bracing or surgery) are crucial.
  2. Heterotopic ossification:
    • Can lead to joint contractures if not recognized early.
    • Management includes physical therapy, NSAIDs, and sometimes radiation therapy or surgical excision.
  3. Osteoporosis:
    • Results from immobility and loss of weight-bearing.
    • Increases fracture risk, often from minor trauma.
    • Consider calcium and vitamin D supplementation, and possibly bisphosphonates in severe cases.

Neurological

  1. Syringomyelia:
    • Development of a fluid-filled cavity within the spinal cord.
    • Can cause progressive neurological deterioration.
    • MRI for diagnosis; may require neurosurgical intervention.
  2. Tethered cord syndrome:
    • The spinal cord becomes abnormally attached to surrounding tissues, leading to stretching and further neurological injury as the child grows.
    • Surgical detethering may be necessary.
  3. Neuropathic pain:
    • Can be challenging to manage.
    • Multimodal approach including medications (gabapentin, pregabalin, tricyclic antidepressants), physical modalities, and psychological interventions.

Urological

  1. Neurogenic bladder:
    • Regular urodynamic studies to assess bladder and sphincter function.
    • Clean intermittent catheterization is often required.
    • Anticholinergic medications or botulinum toxin injections for detrusor overactivity.
  2. Recurrent urinary tract infections:
    • Meticulous catheter hygiene.
    • Avoid prophylactic antibiotics to prevent resistance.
  3. Renal deterioration:
    • Monitor renal function and perform regular upper tract imaging.
    • Early management of high intravesical pressures and vesicoureteral reflux.

Gastrointestinal

  1. Neurogenic bowel:
    • Establish a regular bowel program (timed evacuation, suppositories, digital stimulation).
    • Dietary management and adequate fluid intake.
  2. Constipation and impaction:
    • Common problems requiring vigilant prevention and prompt treatment.

Respiratory

  1. Recurrent pneumonia:
    • Aggressive pulmonary hygiene (chest physiotherapy, assisted coughing techniques).
    • Annual influenza vaccination and pneumococcal vaccination as indicated.
  2. Sleep-disordered breathing:
    • Polysomnography for diagnosis.
    • Non-invasive ventilation may be required.

Cardiovascular

  1. Autonomic dysreflexia:
    • Life-threatening hypertensive episodes triggered by noxious stimuli below the level of injury.
    • Educate patients, families, and caregivers about recognition and emergency management.
    • Prompt identification and removal of the triggering stimulus (often related to bladder or bowel distention).
  2. Orthostatic hypotension:
    • Gradual postural changes, compression stockings, abdominal binders.
    • Medications (midodrine, fludrocortisone) in severe cases.

Psychosocial and Developmental

SCI has profound effects on a child's psychosocial development and family dynamics:

  • Regular psychological assessments to address evolving needs.
  • Peer support programs and adaptive sports can enhance self-esteem and social integration.
  • Transition planning for adolescents moving to adult healthcare services.
  • Attention to sexuality and reproductive health as the child matures.

Emerging Therapies

Research in SCI is rapidly evolving, with several promising avenues:

  1. Neuroprotective agents:
    • Targeting secondary injury mechanisms (e.g., riluzole, minocycline).
  2. Neuroregenerative therapies:
    • Stem cell transplantation (neural, mesenchymal, induced pluripotent).
    • Biomaterials and scaffolds to support axonal regrowth.
  3. Neuromodulation:
    • Epidural stimulation to activate spinal circuits below the injury level.
  4. Brain-computer interfaces:
    • Allowing direct brain control of assistive devices.
  5. Gene therapy:
    • Targeting growth factors and inhibitory molecules.

While these therapies show promise, most are still in experimental stages. It's crucial to counsel families realistically about current limitations while nurturing hope for future advancements.

Prevention

Given the devastating impact of pediatric SCI, prevention strategies are paramount:

  • Public education on road safety, proper use of car seats and seat belts.
  • Legislation and enforcement of safety measures (e.g., helmet laws for bicycling and contact sports).
  • Water safety programs (supervision, pool fencing, diving safety).
  • School-based violence prevention programs.
  • Child protection services to prevent non-accidental trauma.
  • Sports safety guidelines, including rules modifications and proper coaching techniques.

Further Reading

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