Predictive Physical Manifestations for Progression of Scoliosis in Marfan Syndrome. Issue 15 (1st August 2021)

• Record Type: Journal Article
• Authors: Taniguchi, Yuki; Matsubayashi, Yoshitaka; Kato, So; Doi, Toru; Takeda, Norifumi; Yagi, Hiroki; Inuzuka, Ryo; Oshima, Yasushi; Tanaka, Sakae
• Is Part Of: Spine. Volume 46:Issue 15(2021)
• Publication Date: 2021-08-01
• Subjects: Marfan syndrome -- physical feature -- physical manifestation -- progression -- revised Ghent nosology -- risk factor -- scoliosis -- surgery -- systemic score -- wrist sign; Spine -- Abnormalities -- Periodicals; Spine -- Diseases -- Periodicals; Spine -- Surgery -- Periodicals; 616.73005
• DOI: 10.1097/BRS.0000000000003939
• Languages: English
• ISSNs: 0362-2436

Abstract

Abstract : Study Design: A retrospective study of the prospective cohort. Objective: To 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 Data: To date, no study has unveiled the risk factors for the progression of scoliosis in Marfan syndrome. Methods: We 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. Results: We 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. Conclusions: The 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 Abstract : In 131 patients with Marfan syndrome aged 15 or above, severe scoliosis with Cobb angle ≥40° or surgery conducted was identified in 40.5% of the population. Multivariate regression analysis identified female sex and positive wrist sign as predictive factors for progression of scoliosis into severe state in these patients.