Marfan's Syndrome
Epidemiology
Spinal involvement occurs in approximately 75% of patients with Marfan's syndrome
- scoliosis is the most common spinal deformity
- the cervical spine is almost always normal
Those who develop scoliosis do so by 9 years of age
- one half of the patients develop it by age 6 years
Pathology
Curve patterns resemble idiopathic scoliosis
- frequent progression during adolescence
Despite the patients' generalized ligamentous laxity, many of the curves are quite rigid
Thoracic lordosis
- second most common spinal deformity
- associated with loss of normal lumbar lordosis
Progression
These curves also tend to be painful, progressive, and often cause respiratory problems
Curve progression rates average 7 - 10o per year
- with the most rapid increase occurring during the early adolescent period
Management
Non operative
Brace treatment
Controversial
- most patients fail brace treatment
- orthotics may be used as a holding device for patients who eventually will undergo fusion
Operative
Technique
Posterior spinal instrumentation and fusion
- mainstay of treatment
- a high rate of pseudarthrosis can be expected
- may lower with addition of an anterior fusion, abundant bone grafting, and postoperative immobilization
Complications
Pseudoarthrosis
Cardiac
- high incidence of malformations
- need good pre op work up
Superior mesenteric artery syndrome
- high incidence if put in jacket