Marfan's

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