title: Obstructive lung disease is common in children with syndromic and congenital scoliosis: a preliminary study.
abstract: 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.
  
  
  METHODS
  Level IV, prognostic study investigating the effect of a patient characteristic on the outcome of disease.
authors.affiliation: 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.
authors.forename: Gary L
authors.initials: GL
authors.lastname: McPhail
authors.forename: Sacha A
authors.initials: SA
authors.lastname: Howells
authors.forename: Richard Paul
authors.initials: RP
authors.lastname: Boesch
authors.forename: Robert E
authors.initials: RE
authors.lastname: Wood
authors.forename: Mathew
authors.initials: M
authors.lastname: Ednick
authors.forename: Barbara A
authors.initials: BA
authors.lastname: Chini
authors.forename: Viral
authors.initials: V
authors.lastname: Jain
authors.forename: Steven
authors.initials: S
authors.lastname: Agabegi
authors.forename: Peter
authors.initials: P
authors.lastname: Sturm
authors.forename: Eric
authors.initials: E
authors.lastname: Wall
authors.forename: Alvin
authors.initials: A
authors.lastname: Crawford
authors.forename: Greg
authors.initials: G
authors.lastname: Redding
mesh_terms: D001999:Bronchoscopy; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008173:Lung Diseases, Obstructive / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D008297:Male; D015995:Prevalence; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012600:Scoliosis / Q000150:complications* / Q000503:physiopathology; D013904:Thoracic Vertebrae / Q000000981:diagnostic imaging / Q000503:physiopathology; D014057:Tomography, X-Ray Computed