Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Spinal deformity in Marfan syndrome.

J G Birch, J A Herring

    Journal of Pediatric Orthopedics
    |September 1, 1987
    PubMed
    Summary

    Spinal fusion for Marfan syndrome patients with spinal deformities shows mixed results. While solid fusion is achievable, complications like pseudarthrosis are common, necessitating aggressive surgical techniques and careful monitoring.

    Related Concept Videos

    You might also read

    Related Articles

    Articles linked to this work by shared authors, journal, and citation graph.

    Sort by
    Same author

    Comparison of Moseley and Rotterdam straight-line graphs in predicting leg lengths and leg-length discrepancy at maturity.

    Journal of children's orthopaedics·2019
    Same author

    Somatosensory evoked potential monitoring of peripheral nerves during external fixation for limb lengthening and correction of deformity in children.

    The Journal of bone and joint surgery. British volume·2012
    Same author

    Mutations responsible for Larsen syndrome cluster in the FLNB protein.

    Journal of medical genetics·2006
    Same author

    Peripheral nerve monitoring during surgery for upper extremity lengthening.

    Techniques in hand & upper extremity surgery·2006
    Same author

    Adolescents undergoing surgery for idiopathic scoliosis: how physical and psychological characteristics relate to patient satisfaction with the cosmetic result.

    Spine·2001
    Same author

    Efficacy of perioperative halo-gravity traction in the treatment of severe scoliosis in children.

    Journal of pediatric orthopedics·2001

    Area of Science:

    • Orthopedics
    • Genetics
    • Spine Surgery

    Background:

    • Marfan syndrome is a genetic disorder affecting connective tissue.
    • Spinal deformities, including scoliosis and kyphosis, are common in Marfan syndrome.
    • Conservative and surgical treatments aim to correct spinal deformities and prevent progression.

    Purpose of the Study:

    • To evaluate the outcomes of spinal deformity treatment in patients with Marfan syndrome.
    • To assess the effectiveness of conservative management and surgical fusion.
    • To identify factors influencing successful spinal fusion and potential complications.

    Main Methods:

    • Retrospective review of 14 Marfan syndrome patients treated after 1975.
    • Analysis of outcomes for patients undergoing conservative (brace) treatment or spinal fusion.
    • Assessment of fusion success, correction loss, pseudarthrosis, and kyphotic deformity development.

    Main Results:

    • Brace treatment was successful in only one of nine patients.
    • Nine patients underwent spinal fusion, with three achieving solid fusion.
    • Four patients developed pseudarthrosis requiring revision, often associated with kyphotic deformity.
    • Successful fusion requires substantial bone grafting, robust internal fixation, and vigilant monitoring.

    Conclusions:

    • Conservative brace treatment has limited success for spinal deformities in Marfan syndrome.
    • Spinal fusion can be successful but carries a high risk of pseudarthrosis and correction loss.
    • Kyphotic deformities in Marfan syndrome may necessitate anterior fusion approaches.
    • Aggressive surgical techniques and meticulous follow-up are crucial for managing spinal deformities in Marfan syndrome.

    Related Experiment Videos