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