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Scoliosis in Rett syndrome

T J Huang1, J P Lubicky, K W Hammerberg

  • 1Shriners Hospital for Crippled Children, Chicago Unit, Illinois.

Orthopaedic Review
|December 1, 1994
PubMed
Summary
This summary is machine-generated.

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Scoliosis is a common orthopedic issue in Rett syndrome, often requiring surgical intervention for curves over 40-45 degrees. Early observation and surgical fusion are key for significant spinal deformities.

Area of Science:

  • Orthopedics
  • Neurology
  • Genetics

Background:

  • Scoliosis is the most frequent orthopedic complication in Rett syndrome.
  • It presents as a C-shaped thoracolumbar curve of neurologic origin.
  • Incidence ranges from 36% to 100%, typically appearing before age 8, with rapid progression in the second decade.

Purpose of the Study:

  • To summarize the characteristics and management of scoliosis in Rett syndrome.
  • To outline indications for surgical intervention.
  • To discuss surgical techniques for correction.

Main Methods:

  • Literature review of scoliosis in Rett syndrome.
  • Analysis of age-dependent incidence and progression patterns.
  • Evaluation of treatment outcomes for orthotic and surgical interventions.

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Main Results:

  • Scoliosis onset is typically before age 8, with rapid progression in adolescence.
  • Orthotic treatment does not alter the natural course of spinal deformity.
  • Surgery is indicated for curves >40-45 degrees Cobb angle or those causing pain/functional loss.

Conclusions:

  • Scoliosis and kyphosis in Rett syndrome require close monitoring.
  • Surgical correction, including anterior and posterior fusion, can effectively manage significant spinal deformities.
  • Comprehensive surgical intervention should address both scoliotic and kyphotic segments.