Scoliosis

Scoliosis is a structural alteration of the vertebral column characterized by a lateral spinal curvature of greater than 10 degrees in the coronal plane. Scoliosis can be classified as idiopathic (in most cases) or secondary to underlying conditions. Other classifications are according to etiology, location, and severity. Patients present with spinal asymmetry and often have associated pain. Diagnosis is initially clinical and is confirmed later by X-ray. Management can be conservative or surgical, depending on the severity.

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Overview

Definition

Scoliosis is a lateral spinal curvature of the spine greater than 10 degrees in the coronal plane, usually accompanied by rotation of variable amounts.

  • Dextroscoliosis: spinal curvature to the right 
  • Levoscoliosis: spinal curvature to the left 

Epidemiology

  • Incidence: 10 in 200 children by age 10–15 
  • Only 1 in 200 patients become symptomatic.
  • 70%–90% of cases are idiopathic (unknown cause).
  • Girls have an increased likelihood of progression (rapid growth → increased curvature change).
  • Most common type is idiopathic, followed by congenital.
  • Most common type in the pediatric population: adolescent idiopathic scoliosis (AIS) 

Classification

  • Scoliosis is broadly divided into primary (idiopathic) and secondary to underlying conditions: 
    • Type I: primary degenerative (de novo) scoliosis
    • Type II: progressive AIS with secondary degenerative changes
    • Type III: scoliosis secondary to another pathologic condition:
      • Marfan syndrome
      • Chiari malformation
      • Syringomyelia
    • Type IIIA: lumbosacral scoliosis caudal to a previous spinal fusion
    • Type IIIB: scoliosis secondary to metabolic bone disease (e.g., osteoporosis) combined with asymmetric degenerative disease and/or vertebral fracture
  • Scoliosis can also be classified according to reversibility:
    • Structural: Structural curves do not resolve with movement.
    • Functional: Curvature can be reduced with side- or forward-bending motions.
    • Change from functional to structural:
      • Progressive soft tissue shortening
      • Body changes
      • Disc degeneration
      • Fibrosis of ligaments and fascia
      • Decreased active and passive range of motion (ROM)
  • Classification according to severity:
    • Mild: 10–15 degrees
    • Moderate: 20–45 degrees
    • Severe: > 50 degrees
  • Classification according to cause:
    • Idiopathic
    • Congenital
    • Acquired/secondary
    • Neuromuscular
Scoliosis

Scoliosis

Image: “Blausen 0785 Scoliosis 01” by Blausen. License: CC BY 3.0

Pathophysiology

Scoliosis can develop de novo or as a continuation of congenital, idiopathic, or early-developed scoliosis. The uneven loading of the spine produces and exacerbates the pathological curvature seen in scoliosis. 

  • Congenital scoliosis:
    • Congenital structural defect of the spine 
    • Failure of vertebrae to form or merge 
    • Hemivertebrae, neural tube defects (spina bifida)
    • Can be unilateral or bilateral
  • Acquired scoliosis:
    • Tissue or mechanical alterations force the vertebral column adapt to a pathological position that produces spinal curving:
      • Osteomalacia
      • Sciatica
      • Psoas syndrome
      • Healed leg or hip fracture
      • Cerebral palsy
    • Asymmetric degeneration of intervertebral disc and facet joints due to osteoarthritis
    • Neuromuscular:
      • Leg length discrepancy
      • Neurofibromatosis
      • Quadriplegia

Clinical Presentation

History

  • Pain:
    • Sharp and/or burning pain 
    • Associated with movement
    • Resolves spontaneously
  • Patient complaints will vary depending on the age group:
    • Children: juvenile idiopathic scoliosis
      • Often asymptomatic
      • More progressive than adolescent idiopathic scoliosis (AIS)
      • Check between ages 10 and 15 when rapid bone growth is occurring: Asymmetry may be noted.
    • Adolescents complain of their clothes not fitting.
    • Adults present with: 
      • Symptoms of osteoarthritis
      • Back and neck pain
      • Headache
      • Some patients may report chest pain.
  • Important history questions to address:
    • Age of onset
    • Rate of progression
    • Features of pain
    • Neuromuscular complaints
    • Dyspnea 
    • Family history of scoliosis
    • Current pubertal stage
    • Onset of menarche (girls)
    • History of limb trauma, infection, arthritis

Physical examination

  • Document the height of the patient.
  • Stage of puberty
  • Neurological examination for weakness and gait
  • Skin: Check for café au lait spots associated with neurofibromatosis.
  • Musculoskeletal:
    • Adams forward bend test: Typical “double curve” in AIS is right thoracic and left lumbar.
    • Hyperlaxity of joints: possible Ehler-Danlos syndrome 
    • Feet:
      • Flat feet
      • High arches
      • Hammer/claw toes
    • Symmetry:
      • Upper limbs
      • Level of occiput, shoulders, iliac crests, posterior superior iliac spine, posterior inferior iliac spine, and greater trochanters
      • Lower limb length
  • Scoliometer: 
    • Measures the angulation of the spine while the patient bends forward:
      • Have a consistent, standardized way to measure every time.
      • Always use the same type of instrument on follow-up exams.
    • Cobb angle will be greater than the angle of trunk rotation.

Diagnosis

Diagnosis is initially made clinically and later confirmed by X-ray, which will show the presence of spinal curvature. Idiopathic scoliosis is a diagnosis of exclusion.

  • Screening programs in schools 
  • Spine X-ray: if screening is abnormal
    • Standing posteroanterior (PA) view and measuring the Cobb angle:
      • Draw a line at the superior border of the highest vertebra inclined toward the concavity and then draw a line perpendicular to this.
      • Draw a line at the inferior border of the lowest vertebra inclined toward the concavity and then draw a line perpendicular to this.
      • The Cobb angle is the degrees measured at the intersection of the 2 perpendicular lines.
    • Lateral-bending X-rays
    • Note the details for future comparison:
      • Direction of curvature (dextro or levo)
      • Location: level of cervical, thoracic, or lumbar spine
      • Other radiologic findings such as masses, congenital vertebral lesions, or vertebral body lucencies
  • MRI spine indications:
    • Patients under age 10 with rapid progression
    • In patients with:
      • Increased pain
      • Associated neurologic symptoms
      • Abnormal findings on the plain radiographs
  • Labs: requested according to clinical suspicion of underlying etiologies, according to the age group

Management

Management ranges from conservative to surgical depending on the degree of scoliosis.

Treatment

  • Conservative therapy: 
    • Goal is to optimize the patient’s function.
    • Observation with exam and X-ray (PA view) every 4–6 months
    • Bracing to slow progression
    • Osteopathic manipulative therapy
    • Home exercise program
    • Physical therapy and chiropractic are ineffective.
  • Orthopedic referral:
    • If unable to measure the Cobb angle 
    • For a Cobb angle 20–29 degrees in a premenarchal girl or boy aged 12–14
    • For a Cobb angle > 30 degrees in any patient
    • Rapid progression of Cobb angle more than 5 degrees
  • Goals of surgery: 
    • Primary: utilizing spinal fusion for prevention of curve progression
    • Secondary: Partial curve correction is frequently achieved.
  • Surgical indications:
    • At diagnosis, the patient has a Cobb angle of ≥ 50 degrees
    • Sometimes, skeletally immature patients with Cobb angle between 40 and 50 degrees may need surgery.
  • Surgical procedures: bone grafting and instrumentation
    • Has evolved from the Harrington rods (cephalad and caudad spinal connections with hooks) to modern segmental instrumentation (hooks, screws, wires, rods)
    • Most important part of the surgery is obtaining bony fusion: autograft or allograft
    • Postoperative period:
      • No sports for 9–12 months until the spine has fused
      • No collision sports after spine has fused
      • Potential complications of surgery: chronic pain, infection of the hardware

Complications

  • Progression of the deformity is untreated.
  • Sacral base unleveling
  • Cardiac and pulmonary restriction
  • Chronic back pain
  • Cosmetic issues
  • Nerve injury
  • Spinal disc herniation and lumbar radiculopathy
  • Ankylosing spondylitis

Prognosis

The prognosis of scoliosis is dependent on the underlying cause, comorbidities, and deformity at the time of presentation. 

  • Progression:
    • Seen in up to 70% of adult scoliosis patients 
    • Untreated scoliosis: 0.5 to 1 degree/year
    • Progression is not affected by pregnancy.
    • Pregnancy is not affected by scoliosis.
  • Back pain:
    • Slightly increased occurrence of back pain in adults with AIS, whether treated or untreated
    • Patients with AIS treated with bracing or surgery may also have an increased incidence of degenerative disc changes.

Clinical Relevance

The following conditions are associated with scoliosis:

  • Marfan syndrome: a genetic condition with autosomal dominant inheritance. Marfan syndrome affects the elasticity of connective tissues throughout the body, most notably in the cardiovascular, ocular, and musculoskeletal systems. The skin, lungs, and CNS are also affected.
  • Chiari malformation: a group of CNS conditions characterized by the underdevelopment of the posterior cranial fossa, with subsequent protrusion of neural structures through the foramen magnum. 
  • Cerebral palsy (CP): a syndrome of motor impairment caused by a non-progressive CNS injury. Cerebral palsy is classified according to muscle tone, its distribution, and the presumed time of injury (spastic diplegia is the most common), and is present in about 3 of 1,000 live births. 
  • Spinal disc herniation: a condition characterized by the degeneration of the annulus fibrosus and the herniation of the nucleus pulposus of the intervertebral discs. Spinal disc herniation usually presents as radiculopathy (most commonly lumbar) due to the pressure exerted by the herniated nucleus pulposus. 
  • Ankylosing spondylitis: a spondyloarthropathy characterized by chronic and indolent inflammation of the axial skeleton. The condition is most often seen in young men and is strongly associated with HLA-B27. Severe disease can lead to fusion and rigidity of the spine. 
  • Juvenile idiopathic arthritis: formerly known as juvenile rheumatoid arthritis, a heterogeneous group of inflammatory diseases characterized by arthritis and the most common pediatric rheumatic disease.
  • Osteoblastoma: a benign, bone-forming tumor most commonly located at the posterior elements of the spine causing symptoms of nerve root compression.
  • Neurofibromatosis type 1: a neurocutaneous disorder that causes the growth of tumors in the nervous system and skin and is characterized clinically by café au lait spots on the skin. 

References

  1. Browner, B., et al. (2020). Skeletal trauma: basic science, management, and reconstruction. Philadelphia, Pa: Elsevier.
  2. Hey, L.A. (2018). Scoliosis in the adult. UpToDate. Retrieved July 12, 2021, from https://www.uptodate.com/contents/scoliosis-in-the-adult
  3. LeFever, D., & Menger, R. P. (2021). Infantile and juvenile scoliosis. StatPearls. Retrieved July 12, 2021, from http://www.ncbi.nlm.nih.gov/books/NBK519498/
  4. Menger, R.P., & Sin, A.H. (2021). Adolescent and idiopathic scoliosis. StatPearls. Retrieved July 12, 2021, from http://www.ncbi.nlm.nih.gov/books/NBK499908/
  5. Scherl, S.A. (2020). Adolescent idiopathic scoliosis: Clinical features, evaluation, and diagnosis. UpToDate. Retrieved July 12, 2021, from https://www.uptodate.com/contents/adolescent-idiopathic-scoliosis-clinical-features-evaluation-and-diagnosis

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