Scoliosis and Cardiopulmonary Outcomes in Osteogenesis Imperfecta Patients

STUDY DESIGN.Retrospective clinical study of individuals with osteogenesis imperfecta (OI). OBJECTIVE.To assess the relationship between severity of scoliosis and pulmonary function, and to assess the relationship between restrictive lung disease and self-reported quality of life in individuals with...

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Bibliographic Details
Published in:Spine (Philadelphia, Pa. 1976) Pa. 1976), 2019-08, Vol.44 (15), p.1057-1063
Main Authors: Bronheim, Rachel, Khan, Sobiah, Carter, Erin, Sandhaus, Robert A, Raggio, Cathleen
Format: Article
Language:English
Subjects:
Adolescent
Adult
Child
Female
Fractures, Compression - complications
Humans
Lung - physiopathology
Lung Diseases - etiology
Lung Diseases - physiopathology
Male
Osteogenesis Imperfecta - complications
Quality of Life
Radiography
Respiratory Function Tests
Respiratory Insufficiency - etiology
Respiratory Insufficiency - physiopathology
Retrospective Studies
Scoliosis - complications
Scoliosis - diagnostic imaging
Scoliosis - etiology
Spinal Fractures - complications
Thoracic Wall - physiopathology
Vital Capacity
Young Adult
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Summary:STUDY DESIGN.Retrospective clinical study of individuals with osteogenesis imperfecta (OI). OBJECTIVE.To assess the relationship between severity of scoliosis and pulmonary function, and to assess the relationship between restrictive lung disease and self-reported quality of life in individuals with OI. SUMMARY OF BACKGROUND DATA.OI is a heritable connective tissue disorder characterized by osteopenia and a predisposition to fracture. Respiratory insufficiency is a leading cause of mortality. Literature on pulmonary function in this population has shown a negative correlation between percent-predicted vital capacity and severity of scoliosis. However, it has been suggested that decreased pulmonary function in OI may be due to intrinsic pulmonary disease, in addition to the impact of vertebral compression fractures and scoliosis. METHODS.Anterior-posterior spine radiographs and pulmonary function tests from 30 individuals with OI were reviewed. Radiographs were evaluated for scoliosis, defined as a curve ≥ 10°. If more than one curve was present, the largest curve was used. Pulmonary function was defined as the forced expiratory volume in 1 second (FEV1)/forced vital capacity (FVC) ratio. Restrictive pulmonary disease was defined as FEV1/FVC > 80%, while obstructive disease was defined as FEV1/FVC 
ISSN:0362-2436
1528-1159
DOI:10.1097/BRS.0000000000003012