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Pathophys and Cause

Cause:Genetic, at least in 30% (positive family hx); autosomal dominant; polygenic

Pathophys:Perhaps due to unequal development of vertebral growth plates, or unequal muscle and ligamentous balance

Epidemiology

Female/male = 4:1. 10% of population have minor abnormalities, 1.5% have significant abnormalities, 0.5% need rx. Worldwide. Increased in ballet-trained children (Nejm 1986;314:1348, 1379)

Signs and Symptoms

Sx:Onset age 9-13 yr, usually asx

Si:Most have thoracic curve convex to R, lumbar to L; hump appears on one side when bent over (razor back deformity) with high, usually R shoulder; asymmetric scapulae; plumb line from posterior neck when standing misses gluteal cleft; when standing, space between arm and body is asymmetric R to L

Screen for lateral hump when bent over using leveling device across back (scoliometer; measures rib rotation); refer to orthopedist if >20° and still in early puberty; screening efficacy unclear (Jama 1999;282:1427)

Course

Gradual progression in curvature until skeletal maturity by age 18 yr; after maturity, curvature progresses more slowly; progression greater in girls (J Bone Joint Surg Am 1995;77:823)

Complications

Deformity; respiratory compromise; r/o leg length discrepancy

Lab and Xray

Xray:Rarely indicated, can be used to follow care despite radiation; only if considering tx (curve 40-100°)

MRI if onset < age 8 yr or progression >1°/month (Am Fam Phys 2002;65:1817)

Treatment

Rx:

Minor (<15-25° by scoliometer): recheck q3mo

Intermediate (25-45°): to prevent increase, esp in preadolescent girls, thoracolumbarsacral orthosis or Milwaukee brace 8-24 hours/d; adv effects: protrusion of incisors, poor compliance

Severe (45): surgical placement of Harrington rods/bone grafting