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Obstructive Lung Disease is Common in Children With Syndromic and Congenital Scoliosis: A Preliminary Study

McPhail, Gary L. MD*; Howells, Sacha A. BA; Boesch, Richard Paul DO, MS*; Wood, Robert E. MD, PhD*; Ednick, Mathew DO*; Chini, Barbara A. MD*; Jain, Viral MD; Agabegi, Steven MD; Sturm, Peter MD; Wall, Eric MD; Crawford, Alvin MD; Redding, Greg MD§

Journal of Pediatric Orthopaedics: December 2013 - Volume 33 - Issue 8 - p 781–785
doi: 10.1097/BPO.0000000000000078
Scoliosis

Background: It is well known that restrictive lung disease (RLD) is associated with scoliosis. This study identifies that obstructive lung disease (OLD) is associated with syndromic scoliosis and congenital scoliosis.

Methods: We searched a local database for patients with scoliosis who underwent a pulmonary function testing (PFT) from 2004 to 2009. All patients with congenital scoliosis or syndromic thoracolumbar scoliosis with a Cobb angle of ≥40 degrees and acceptable and repeatable PFT testing were included in the study. OLD was defined as an forced expiratory volume in the first second/forced vital capacity ratio below 95% confidence interval. Bronchoscopy videos and computed tomography scans or magnetic resonance images were reviewed to identify anatomic causes of lower airway disease.

Results: A total of 18 patients met the criteria for inclusion. The median age at diagnosis was 11.3 years. The median primary Cobb angle was 60 degrees. The prevalence of OLD was 33% and RLD was 57%. The 6 children with OLD underwent preoperative bronchoscopy and chest computed tomography or magnetic resonance imaging to identify anatomic causes of lower airway obstruction. The 4 children with OLD and right-sided major thoracic curves had compression of the right mainstem bronchus between the spine (posterior) and the right pulmonary artery (anterior). The 2 children with OLD and left-sided major thoracic curves had compression of the left mainstem bronchus between the spine (posterior) and the descending aorta (anterior) or the left atrium (anterior).

Conclusions: In our study, the prevalence of OLD in children with congenital scoliosis or syndromic scoliosis was 33%, which was elevated when compared with the population prevalence of 2% to 5%. Mainstem airway compression from spine rotation was discovered to be the potential mechanism of disease.

Level of Evidence: Level IV, prognostic study investigating the effect of a patient characteristic on the outcome of disease.

Divisions of *Pulmonary Medicine

Orthopaedic Surgery, Cincinnati Children’s Hospital Medical Center, University of Cincinnati, Cincinnati, OH

WordCraft, Medical Writing Consultants, Los Angeles, CA

§Division of Pulmonary Medicine, Seattle Children’s Hospital, University of Washington, Seattle, WA

The authors declare no conflicts of interest.

Reprints: Gary L. McPhail, MD, Division of Pulmonary Medicine, Cincinnati Children’s Hospital Medical Center, University of Cincinnati, ML 2021, 3333 Burnet Avenue, Cincinnati, Ohio 45229-3039. E-mail: gary.mcphail@cchmc.org.

© 2013 by Lippincott Williams & Wilkins