{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,10]],"date-time":"2026-06-10T08:23:22Z","timestamp":1781079802820,"version":"3.54.1"},"reference-count":34,"publisher":"SAGE Publications","issue":"4","license":[{"start":{"date-parts":[[2020,8,1]],"date-time":"2020-08-01T00:00:00Z","timestamp":1596240000000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Journal of Children's Orthopaedics"],"published-print":{"date-parts":[[2020,8]]},"abstract":"<jats:sec>\n                    <jats:title>Purpose<\/jats:title>\n                    <jats:p>Scoliosis, chest wall deformities and pulmonary involvement are common features of Marfan syndrome (MFS). We aimed to assess the impact of spinal and chest wall deformities on pulmonary function in paediatric MFS patients with a surgically managed spinal deformity.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>In this multicentre retrospective study, spirometry, lung volumes and radiographic imaging were performed on 26 MFS patients between the ages of seven and 18 years who were undergoing planned spinal fusion surgery for spinal deformity. A correlation analysis assessed the relationship between radiographic measurements of spinal and chest wall deformities and predicted total lung capacity (TLC), forced vital capacity (FVC) and the ratio between forced expiratory volume in one second and FVC (FEV1\/FVC).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>In total, 18 patients (70%) had impaired pulmonary function. Thoracic kyphosis (mean 19.3\u00b0; -32\u00b0 to 54\u00b0) had a strong positive correlation with FEV1\/FVC (r = 0.65; p &lt; 0.001). Significant decrease in FEV1\/FVC below 80% occurred at kyphosis under 15\u00b0 (p = 0.004). Kyphosis had a moderate negative correlation with FVC (r = -0.43; p = 0.03). Chest wall deformity had a strong negative correlation with FEV1\/FVC (r = -0.61; p = 0.001). The magnitude of the thoracic curve (mean 55.2\u00b0; 28\u00b0 to 92\u00b0) had a significant moderate negative correlation with TLC (r = -0.45; p = 0.04).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>In MFS, three factors correlate with decreased pulmonary function measures: hypokyphosis, increasing chest wall deformity and increasing coronal curve magnitude. Hypokyphosis and increased chest wall deformity correlated with diminished FEV1\/FVC; increasing thoracic spinal curvature with diminished TLC. Further analysis with a larger cohort will help better define the relationship between these deformities and pulmonary function in this unique population.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Level of Evidence<\/jats:title>\n                    <jats:p>IV<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1302\/1863-2548.14.200076","type":"journal-article","created":{"date-parts":[[2020,7,7]],"date-time":"2020-07-07T05:02:58Z","timestamp":1594098178000},"page":"343-348","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":15,"title":["The correlation between spinal and chest wall deformities and pulmonary function in Marfan syndrome"],"prefix":"10.1177","volume":"14","author":[{"given":"Hila","family":"Otremski","sequence":"first","affiliation":[{"name":"Pediatric Orthopaedic Department, Dana Dwek Children's Hospital, Tel Aviv Medical Center, Affiliated to the Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roger F.","family":"Widmann","sequence":"additional","affiliation":[{"name":"Pediatric Orthopaedic Department, Hospital for Special Surgery, New York, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mary F.","family":"Di Maio","sequence":"additional","affiliation":[{"name":"Department of Pediatric Medicine, Hospital for Special Surgery, New York, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dror","family":"Ovadia","sequence":"additional","affiliation":[{"name":"Pediatric Orthopaedic Department, Dana Dwek Children's Hospital, Tel Aviv Medical Center, Affiliated to the Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2020,8,1]]},"reference":[{"key":"bibr1-1863-2548.14.200076","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-015-0369-8"},{"key":"bibr2-1863-2548.14.200076","doi-asserted-by":"publisher","DOI":"10.1038\/352337a0"},{"key":"bibr3-1863-2548.14.200076","first-page":"1993","volume-title":"GeneReviews\u00ae [Internet]","author":"Dietz 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