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Duke Orthopaedics
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Wheeless' Textbook of Orthopaedics

Spinal Anomalies in Marfan's Syndrome


- See:
    - scoliosis menu
    - scoliosis menu

- Discussion:
    - scoliosis occurs in the majority of patients w/ Marfan's syndrome, (over 60%) but curvatures significant enough to require treatment 
          occur in only 20% of pts;
    - risk of progression:
          - scoliosis often occurs before age of 10 & may progress rapidly;
          - progression is more likely w/ curves greater than 20 deg in growing patients, and is more likely w/ curves more than 30-40 deg in 
                 adults;
    - associated conditions:
          - high-grade spondylolisthesis is additional spinal deformity that reportedly occurs with this syndrome.
          - dural ectasia and anterior myelomeningocele may be present & are thought to be caused by CSF pulsations against weakened dura;

- Bracing: (see discussion of bracing)
    - scoliosis in Marfan's generally does not respond to bracing;
    - bracing in Marfan's is complicated by thoracic lordosis;
    - however, if curve progression is less than 45 deg w/o thoracic lordosis or lumbar kyphosis, orthosis is used;

- Steroids:
    - progesterone and estrogen therapy to induce puberty and control progressive scoliosis has not been successful.

- Arthrodesis:
    - is recommended for adolescents w/ curves > 45 deg, painful curves, or rapidly progressing curves, or adults w/ curves > 50 deg;
    - consider Ant Approach w/ diskectomy is used for rigid curves;
    - spinal fusion is often complicated by pseudarthrosis.

- Thoracic Lordosis:
    - is more of a problem than scoliosis because of decr pulmonary f(x);
    - w/ thoracic lordosis or lumbar kyphosis, max flexion & extension x-rays are obtained before surgery with the patient lying supine are 
         advised to assess sagittal flexibility;

- Arthrodesis:
    - post fusion w/ sublaminar wires corrects thoracic lordosis;
    - flexible curves respond to post fusion, whereas rigid curves may be Rx'ed w/ ant diskectomy & fusion followed by post fusion


Scoliosis in Marfan's syndrome. Its characteristics and results of treatment in thirty-five patients.

The thoracolumbar spine in Marfan syndrome.



Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Monday, May 21, 2012 3:51 pm