Scoliosis (Scoliosis, Congenital)

• lateral curvature of the spine,
• abnormal if the curve is more than 10 degrees.
• 85% Idiopathic and the condition usually is diagnosed during adolescence.
• 5% Congenital vertebral anomalies
• 10% other skeletal anomalies (eg, rib fusion)
  • 5%  neuromuscular conditions (eg, CP, polio, spinal muscular atrophy, muscular dystrophy, myelomeningocele, spinocerebellar degeneration, spinal anomalies: hemivertebrae)
  • 5% misc: hereditary disorders (marfan, ehlers-danlos, neurofibromatosis, etc), compensatory scoliosis (eg, leg length discrepancy).
• Positive family history is a significant risk factor, no direct genetic link has been established.

Infantile Scoliosis

•  <3 years; males>females; classically left thoracolumbar curve; resolves as child grows
• lateral curve is associated with vertebral rotation (thoracic spine), chest wall deformity (rib hump), or flank prominence (lumbar spine). May also see shoulder assymetry, unilateral scapular prominence, waist assymetry, small chest, paralumbar bumps.
• In infants, hold baby prone on examiners hand.
• affected infants rapidly develop plagiocephaly with flattening of the head on the concave side of the curve and corresponding prominence on the opposite side of the head.
• must eval for GU tract anomalies esp unilateral renal agenesis (increased incidence 20-30%, with congenital vertebral anomalies)
• may also see Klippel-Feil syndrome, Sprengel deformity of scapula, neuromuscular conditions

Juvenile Idiopathic Scoliosis: 4-10 years; males=females; 1/3-1/2 have abnormality on MR

Adolescent Idiopathic Scoliosis: >11 years; more common in females; right thoracic most common; left thoracic curve assoc. with underlying abnormality in 75% -MRI strongly suggested

Idiopathic scoliosis should be PAINLESS

Adolescent idiopathic scoliosis typically is:

•  a mild-to-moderate deformity
• that involves a radiographic curve or Cobbs angle of less than 30 degrees
• and is associated with no complications or long-term disability.
• Except for severe curvatures of greater than 45 degrees , the risk for curve progression ends with skeletal maturity. Other factors associated with curve progression include the magnitude of the curve at diagnosis, a thoracic curve, and the presence of a double curve. Idiopathic curves with a Cobbs angle greater than 45 degrees are at risk for progression in adulthood, and surgical treatment may be recommended.

The Adams test is used to inspect the spine for scoliosis.

• Adam's forward-bending test can be done in kids starting from age 6, until the end of puberty.
• The clinician stands behind the patient, who bends forward at the waist 90 degrees, keeping the knees extended.
• An elevation or "hump" indicates asymmetry of one side compared with the other.
• A scoliometer, essentially a protractor that resembles a level, provides a quantification of the asymmetry and aids in identifying progression or the need for further evaluation.
• A scoliometer reading of 7 degrees or greater correlates with a radiographic curve or Cobbs angle of 20 degrees.
• Radiographic curves between 20 and 30 degrees have a higher risk of progression in premenarcheal, skeletally immature girls compared with girls 2 years postmenarche.

Evaluation:

• Obtain a PA standing x-ray of the lower cervical spine, the entire thoracic and lumbar spine and the pelvis on a single film <- the gold standard for evaluation of the degree of curvature. Use a 36 inch cassette.
• <25 degrees: follow, refer to ortho
• progressive curves of 25-40 degrees: brace if child has remaining growth (TLSO).  Bracing will not correct the curvature, although it may help to slow the progression in skeletally immature patients whose curves are less than 40 degrees.
• >40 degrees: surgery, spinal fusion
• Referral to an orthopedic surgeon should be considered for any premenarcheal girl who has a Cobbs angle greater than 20 degrees and any adolescent who has a Cobbs angle greater than 40 degrees.
• Peak height velocity usually occurs after Sexual Maturity Rating stage 2 and before menarche. Accordingly, at this age the growth spurt is probably ongoing and there is an increased risk of progression of the spinal curvature prior to epiphyseal closure.
• The daughters of mothers who have scoliosis are at increased risk, although the severity of the daughter's scoliosis does not correlate with maternal severity.

References:
Killian JT, Mayberry S, Wilkinson L. Current concepts in adolescent idiopathic scoliosis. Pediatr Ann. 1999;28:755-761
Thompson GH, Scoles PV. Idiopathic scoliosis. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 16th ed. Philadelphia, Pa: WB Saunders Co; 2000:2083-2086

CHLA Board Review 2005