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Related Experiment Videos

[Spinal deformities in Marfan disease].

H Robin1, J P Damsin, G Filipe

  • 1Service d'Orthopédie et de Chirurgie Réparatrice de l'Enfant, Hôpital Trousseau, Paris.

Revue De Chirurgie Orthopedique Et Reparatrice De L'Appareil Moteur
|January 1, 1992
PubMed
Summary

Spinal deformities are common in Marfan syndrome. Surgical treatment, particularly with segmental instrumentation, offered better scoliosis correction and spinal balance compared to bracing for severe cases.

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Area of Science:

  • Orthopedics
  • Genetics
  • Cardiovascular Surgery

Background:

  • Marfan syndrome frequently presents with spinal deformities, impacting patient quality of life.
  • Early diagnosis and intervention are crucial for managing skeletal complications in Marfan syndrome.

Observation:

  • A study examined 37 patients with Marfan syndrome, averaging 11 years old, with 32 exhibiting spinal deformities.
  • Treatment modalities included bracing (15 cases), surgery (16 cases), and observation (7 cases).
  • Bracing showed limited efficacy, primarily for mild curves, while surgical outcomes varied with instrumentation techniques.

Findings:

  • Surgical correction of scoliosis achieved 48.2% success rate, with segmental instrumentation showing promise in reducing non-unions and improving spinal balance.

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  • No immediate cardiovascular complications were observed post-operatively.
  • Long-term follow-up revealed one death due to aortic lesions and three patients requiring aortic valve or aorta replacement.

    • Implications:

    • Segmental instrumentation may offer superior outcomes for scoliosis in Marfan syndrome compared to traditional Harrington instrumentation.
    • Close cardiovascular monitoring is essential in Marfan syndrome patients, even after spinal surgery.
    • Multidisciplinary management is key for addressing the complex skeletal and cardiovascular manifestations of Marfan syndrome.