J Korean Soc Spine Surg.  2001 Dec;8(4):482-490.

Scoliosis Associated with Marfan Syndrome

• Lim CK
• Chang BS
• Lee DH
• Jeong BY
• Lim ST
• Lee CK

Abstract

OBJECTIVES
To evaluate characteristics and results of treatment of scoliosis associated with Marfan syndrome
METHODS
The clinical data of 57 patients diagnosed as Marfan syndrome from January 1989 to June 1999 were reviewed. Thirty one patients had major curves more than 10 degrees by Cobb's method on whole spine standing film and their curve patterns and treatment methods were analyzed. Twelve cases of Marfan scoliosis who underwent posterior instrumented fusion were compared with randomly-sampled cases with idiopathic scoliosis about flexibility, correctability and correction loss of curves.
RESULTS
Scoliosis was identified in 31 of 57 patients (54.4%) and among them 15 patients had the curve more than 40 degrees. Twelve curves were double major, 10 were thoracic, and 8 were double thoracic. Seventy-five per cent of the 57 patients had congenital heart disease and 40% had lens dislocation. Twelve of 31 cases underwent posterior fusion with pedicle screw instrumentation. Marfan scoliosis (mean flexibility; 39% in thoracic, 52% in lumbar) were significantly less flexible than idiopathic scoliosis (60% in thoracic, 93% in lumbar) (P<0.05). Mean correctability of Marfan scolisis of operation (61% in thoracic, 55% in lumbar) was poorer than that of idiopathic scoliosis (72% in thoracic, 67% in lumbar), however, this was not significant statistically (P>0.05). Mean correction loss in Marfan syndrome was 3.2% at the mean 4.1 year follow-up in 9 cases.
CONCLUSIONS
The prevalence of scoliosis in Marfan syndrome shows positive correlation with severity of the disease. As compared to idiopathic scoliosis, the curve associated with Marfan syndrome was larger and less flexible. After posterior fusion using pedicle screw instrumentation, the correctability of Marfan curve was poorer than that of idiopathic curve, which is, however, insignificant statistically. So it was considered to be a good method to treat Marfan scoliosis.

Keyword

Marfan syndrome; Scoliosis

MeSH Terms

Follow-Up Studies
Heart Defects, Congenital
Humans
Lens Subluxation
Marfan Syndrome*
Pliability
Prevalence
Scoliosis*
Spine

Figure

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  Fig. 1-A,B. A 22-year old girl with scoliosis associated with Marfan syndrome. She underwent an operation for heart disease and had been taking Coumadin(anticoagulant). The magnitudes of right thoracic and left lumbar curves were 78˚ and 68˚ retrospectively.

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  Fig. 2-C,D. Side-bending radiographs. The thoracic and lumbar curves were corrected to 54˚ (31%) and 27˚ (60%).

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  Fig. 3-E,F. Postoperative standing radiographs. The thoracic and lumbar curves were corrected to 29° (63%) and 28° (59%).

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