Scoliosis

General Information

Description:

  • adolescent idiopathic scoliosis described here, defined as fixed (structural) lateral curvature of spine > 10 degrees in coronal plane with onset near puberty and no known cause
  • kyphosis = posterior curvature, scoliosis = lateral curvature


Types:

  • functional – postural, compensatory, reversible curvature due to another condition, for example, limb length discrepancy or painful muscle spasm
  • structural – not postural, characterized by structural skeletal changes such as vertebral rotation and wedging and rib deformation
    • 70-80% idiopathic, also called familial  – not associated with dysmorphic features, skin lesions, bone fragility or neuromuscular disease
    • 10% congenital scoliosis – errors of formation (hemivertebra) or errors of segmentation (unilateral bar, worsens gradually), commonly associated with genitourinary anomalies, curves present at birth, up to 75% require treatment
    • 15% neuromuscular  – associated with any disease that causes weakness or spastic imbalance of paraspinal muscles in growth child, including cerebral palsy, muscular dystrophy, spinal muscular atrophy, spinal cord injury; may develop at any age in growing child but does not occur after skeletal maturity
    • 5% associated with dysmorphic syndromes – neurofibromatosis, Marfan syndrome, Ehlers-Danlos syndrome, osteogenesis imperfecta, homocystinuria
  • classification by age
    • infantile scoliosis – by age 3 years, more common in boys and Europeans, usually resolves spontaneously
    • juvenile – age 4-10 years
    • adolescent  – age 10 years until skeletal maturity, most significant and prevalent form, can become worse during growth spurt


Organs involved:

  • spine, chest wall


Who is most affected:

  • female (1.5 times), girls age 10-12 years (boys age 14-16 years)
  • females tend to have more severe curves (female:male ratio 8:1 for severe deformity)


Incidence/Prevalence:

  • 1-3% population, most common chest wall disease
  • 0.2-0.3% > 20 degrees


Causes and Risk Factors

Causes:

  • unknown, not bad posture
  • idiopathic most common, congenital anomalies, infection, paralysis (polio), cerebral palsy, syringomyelia, muscular dystrophy, amyotonia congenita, Friedreich’s ataxia, neoplastic, trauma (fracture in growth plate), also see under Types above
  • 13 cases of spine deformity due to rare disorders in retrospective school-screening (Scoliosis 2007 Oct 23;2:15)
  • traumatic scoliosis in case report (Lancet 2005 Mar 5-11;365(9462)


Pathogenesis:

  • gets worse during active growth
  • reduced lung volume, stiff chest wall
  • review of transformation of spinal curvature into spinal deformity can be found in Scoliosis 2006 Mar 31;1:3 full-text


Complications and Associated Conditions

Complications:

  • pulmonary hypertension
  • right ventricular dysfunction
  • cor pulmonale


Associated conditions:

  • Prader-Willi syndrome
    • based on retrospective cohort study
    • scoliosis observed in 43.4% of 145 children with Prader-Willi syndrome
    • Reference – Pediatrics 2008 Aug;122(2):e499

 


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