Open Access, Peer-reviewed
pISSN 2093-4378
eISSN 2093-4386
    J Korean Soc Spine Surg. 2001 Dec;8(4):482-490. Korean.
    Published online Dec 31, 2001.
    https://doi.org/10.4184/jkss.2001.8.4.482
    Copyright © 2001 Korean Society of Spine Surgery
    Original Article

    Scoliosis Associated with Marfan Syndrome

    Chang-Kyun Lim, M.D., Bong-Soon Chang, M.D., Dong-Ho Lee, M.D., Beom-Young Jeong, M.D., Soo Taek Lim, M.D. and Choon-Ki Lee, M.D.
      • Department of Orthopedic Surgery, College of Medicine, Seoul National University, Seoul, Korea.
    • Address reprint requests to Choon-Ki Lee, M.D. Department of Orthopedic Surgery, College of Medicine, Seoul National University, #28 Yongon-dong, Chongno-gu, Seoul 110-744, Korea. Tel: 82-2-760-2336, Fax: 82-2-764-2718, Email: choonki@plaza.snu.ac.kr

    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.

    Keywords
    Marfan syndrome; Scoliosis

    Figures

    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.

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

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

    Tables

    Table 1
    Summary of operated cases of Scoliosis Associated with Marfan Syndrome

    Table 2
    Summary of operated cases of Idiopathic Scoliosis (sample)

    Table 3
    Accompanied diseases of Marfan Scoliosis

    Table 4
    Curve pattern of Marfan Scoliosis

    References

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    MeSH Terms
    Follow-Up Studies
    Heart Defects, Congenital
    Humans
    Lens Subluxation
    Marfan Syndrome*
    Pliability
    Prevalence
    Scoliosis*
    Spine
    Metrics
    Share
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    Tables
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