Spinal Anomalies in Marfan's Syndrome
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- scoliosis menu
- 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
- 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;
- progesterone and estrogen therapy to induce puberty and control progressive scoliosis has not been successful.
- 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;
- 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