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Spinal deformities in Marfan's syndrome

R Savini, S Cervellati, E Beroaldo

    Italian Journal of Orthopaedics and Traumatology
    |April 1, 1980
    PubMed
    Summary

    Marfan syndrome frequently causes severe scoliosis requiring surgical correction. Early surgical intervention for curves over 50 degrees is recommended, with careful follow-up to manage complications like pseudarthrosis.

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    Area of Science:

    • Genetics and Hereditary Diseases
    • Orthopedics and Musculoskeletal Disorders
    • Cardiovascular and Thoracic Surgery

    Background:

    • Marfan syndrome is a hereditary connective tissue disorder with significant cardiovascular, ocular, and skeletal manifestations.
    • Scoliosis is a common skeletal complication, occurring in 40-75% of patients, often progressing more severely than in idiopathic cases.
    • High incidences of double (50%) and triple (23%) curves, along with thoracic lordoscoliosis, are noted, frequently associated with respiratory compromise.

    Purpose of the Study:

    • To review the characteristics and management of scoliosis in Marfan syndrome.
    • To emphasize the limitations of conservative treatment and the indications for surgical intervention.
    • To highlight the importance of post-operative management and potential complications.

    Main Methods:

    • Review of clinical presentations and treatment outcomes for scoliosis in Marfan syndrome patients.
    • Analysis of curve types, progression rates, and associated complications.
    • Evaluation of surgical correction success rates and management of pseudarthrosis.

    Main Results:

    • Conservative treatment is largely ineffective for Marfan scoliosis, except for very mild curves.
    • Surgical correction is indicated for curves measuring 50 degrees or more, achieving 40-50% correction without significant risk.
    • Pseudarthrosis is a common complication requiring systematic review and potential revision of arthrodesis; anterior arthrodesis may be necessary for severe kyphosis.

    Conclusions:

    • Scoliosis in Marfan syndrome often requires surgical management due to its severity and rapid progression.
    • Systematic follow-up and timely surgical intervention are crucial for optimal outcomes.
    • Surgery is contraindicated in patients with severe cardiovascular complications such as aortic insufficiency or aneurysm.

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