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Spinal deformities in Marfan syndrome.

Constantine A Demetracopoulos1, Paul D Sponseller

  • 1Department of Orthopaedic Surgery, Johns Hopkins Medical Institutions, The Johns Hopkins Hospital, 601 North Caroline Street, Room 5253, Baltimore, MD 21287, USA.

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Marfan syndrome causes spinal deformities like kyphoscoliosis, often progressing rapidly and resisting brace treatment. Surgical correction for these Marfan syndrome spinal issues requires careful consideration of unique patient challenges for optimal outcomes.

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Area of Science:

  • Orthopedics
  • Genetics
  • Rheumatology

Background:

  • Marfan syndrome is a genetic connective tissue disorder impacting multiple organ systems.
  • Spinal deformities, including kyphoscoliosis, spondylolisthesis, and atlantoaxial subluxation, are prevalent in Marfan syndrome.
  • Distinctive vertebral morphology in these patients complicates management.

Purpose of the Study:

  • To review the challenges and considerations for managing spinal deformities in Marfan syndrome.
  • To highlight the limitations of conservative treatments and the complexities of surgical interventions.

Main Methods:

  • Literature review of studies on Marfan syndrome and spinal deformities.
  • Analysis of treatment outcomes and complications associated with surgical correction.
  • Discussion of unique biomechanical and morphological factors in Marfan syndrome patients.

Main Results:

  • Minor scoliosis curves in Marfan syndrome may progress rapidly, often inadequately managed by bracing.
  • Surgical correction of spinal deformities in Marfan syndrome is associated with a higher risk of complications, including fixation failure and progressive deformity.
  • Careful surgical planning tailored to the specific challenges of Marfan syndrome can lead to favorable outcomes.

Conclusions:

  • Spinal deformities in Marfan syndrome necessitate specialized management strategies.
  • Effective treatment requires a thorough understanding of the syndrome's impact on spinal anatomy and biomechanics.
  • While challenging, surgical interventions can be successful when adapted to the unique characteristics of Marfan syndrome patients.