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Congenital Scoliosis Q76.3


Congenital Scoliosis

Congenital Scoliosis Etiology, Natural History

  • failure of segmentation(unilateral bar), failure of formation(hemivertebrae) or mixed.
  • unilateral unsemented bar is common and progressive.
  • fully segmented hemivertebra=high risk of progression, nonsegmented of fused hemivertebrae=low risk
  • most severe scoliosis in each region of the spine is caused by a a unilateral unsegmented bar with contralateral hemivertebrae at the same level. The next most severe scoliosis is caused by a unilateral segmented bar alone

Congenital Scoliosis Associated Anomalies

  • genitourinary(25%)
  • cardiac(10%)
  • dysraphism(25%)

Congenital Scoliosis Treatment

  • Renal, cardiac, and neurologic evaluation is indicated in infants with congential scoliosis (Basu PS, Spine 2002;27:2255).
  • bracing contraindicated because congenital curves are generally rigid.
  • semi-segmented hemivertebra generally do not exceed 40 degrees at skeletal maturity and treatment is not usually required except occasionally when the hemivertebra is at the lumbosacral junction
  • Surgery:convex growth arrest(anterior hemiepiphysiodesis and posterior hemiarthrodesis), posterior fusion, A/PF +/- I, hemivertebra excision.

Congenital Scoliosis Refereneces

  • Mcmaster MJ, David CV: Hemivertebra as the cause of scoliosis: A study of 104 patients. JBJS 1986; 68B: 588-595.
  • McMaster MJ, Ohtsuka K: The natural history of congenital scoliosis: A Study of two hundred and fifty-one patients. JBJSA 1982;64:1128-1147.
  • Hedequist D, JAAOS 2004;12:266