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The spine in osteogenesis imperfecta

D R Benson, D H Donaldson, E A Millar

    The Journal of Bone and Joint Surgery. American Volume
    |October 1, 1978
    PubMed
    Summary
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    Osteogenesis imperfecta patients frequently develop scoliosis, with incidence rising sharply after age five. Bracing treatments like the Milwaukee brace proved ineffective and potentially harmful for spinal curvature in these patients.

    Area of Science:

    • Orthopedics
    • Genetics
    • Pediatrics

    Background:

    • Osteogenesis imperfecta (OI) is a genetic disorder characterized by brittle bones.
    • Spinal deformities, particularly scoliosis, are a common complication in OI patients.
    • Understanding the progression and treatment of scoliosis in OI is crucial for patient management.

    Purpose of the Study:

    • To investigate the incidence, pattern, and progression of spinal curvature in a large cohort of patients with osteogenesis imperfecta.
    • To evaluate the effectiveness and complications of the Milwaukee brace in treating scoliosis in OI patients.
    • To identify factors associated with the development and severity of scoliosis in OI.

    Main Methods:

    • Retrospective analysis of a large series of patients diagnosed with osteogenesis imperfecta.

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  • Documentation of spinal curvature incidence, degree, and progression over time.
  • Assessment of treatment outcomes and complications associated with the Milwaukee brace.
  • Correlation of spinal curvature with patient age, ambulatory status, chest deformities, and bone type.

    • Main Results:

    • A high incidence of spinal curvature was observed, reaching nearly 80% in patients over twelve years old.
    • Scoliosis incidence was 26% in children aged 1-5 years, increasing significantly thereafter.
    • Curvature severity was greater in older children and related to bone type.
    • The Milwaukee brace demonstrated ineffectiveness in treating scoliosis and caused detrimental complications, including rib cage deformation.
    • Patients with chest deformities and those who were not ambulatory showed a predilection for scoliosis.

    Conclusions:

    • Spinal curvature is highly prevalent and progresses predictably in osteogenesis imperfecta patients.
    • Current bracing methods, such as the Milwaukee brace, are not effective for scoliosis management in OI and can be harmful.
    • Factors like non-ambulatory status and chest deformities are associated with increased scoliosis risk in OI.