• Yuki Taniguchi, Yoshitaka Matsubayashi, So Kato, Toru Doi, Norifumi Takeda, Hiroki Yagi, Ryo Inuzuka, Yasushi Oshima, and Sakae Tanaka.
• Department of Orthopaedic Surgery, The University of Tokyo Hospital, Tokyo, Japan.
• Spine. 2021 Aug 1; 46 (15): 1020-1025.

Study DesignA retrospective study of the prospective cohort.ObjectiveTo demonstrate the accurate distribution of the severity of scoliosis in patients with Marfan syndrome, and to identify the predictive physical features for progression of scoliosis in Marfan syndrome.Summary Of Background DataTo date, no study has unveiled the risk factors for the progression of scoliosis in Marfan syndrome.MethodsWe retrospectively obtained data from a prospective cohort of the Marfan syndrome clinic at our institute. We enrolled patients whose whole spine radiographs in the standing position were evaluated at the age of 15 or above, from January 2014 to March 2020. The collected variables were physical manifestations defined as in the systemic score of the revised Ghent nosology. We classified the degree of scoliosis into four categories: "not apparent," "mild" (10° ≤ Cobb < 25°), "moderate" (25° ≤ Cobb < 40°), and "severe" (40° ≤ Cobb or surgery conducted). To identify the risk factors for progression of scoliosis in Marfan syndrome, we conducted univariate and multivariate association analyses between severe scoliosis and each physical manifestation.ResultsWe identified 131 eligible patients (61 men and 70 women) with a mean age of 31.2 years. Scoliosis with a Cobb angle of ≥10° was identified in 116 patients (88.5%). Moderate scoliosis was identified in 33 patients (25.2%) and severe scoliosis in 53 patients (40.5%). The prevalence of each physical manifestation was equivalent to that reported in previous studies. Multivariate logistic regression analysis revealed that female sex (odds ratio, 3.27) and positive wrist sign (4.45) were predictive factors for progression of scoliosis into severe state in patients with Marfan syndrome.ConclusionsThe present study demonstrated the accurate distribution of the severity of scoliosis and identified the predictive factors for progression of scoliosis in patients with Marfan syndrome.Level of Evidence: 3.Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.

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