Spinal deformity, pulmonary compromise, and quality of life in osteogenesis imperfecta.
METHODS
A cross-sectional radiologic and clinical study of patients with osteogenesis imperfecta.


OBJECTIVE
To determine whether pulmonary compromise is more closely correlated with scoliosis, kyphosis, or chest wall deformity in the population with osteogenesis imperfecta, and to assess the impact of spinal deformity, chest wall deformity, and pulmonary function on quality of life.


BACKGROUND
The incidence of scoliosis in osteogenesis imperfecta is between 39% and 80%. Up to 60% of patients with osteogenesis imperfecta have significant chest wall deformities. Pulmonary compromise is the leading cause of death in adults with osteogenesis imperfecta.


METHODS
Fifteen patients with osteogenesis imperfecta between the ages of 20 and 45 were evaluated with sitting or standing anteroposterior and lateral radiographs of the entire spine, pulmonary function testing, and a validated health self-assessment questionnaire (Short Form-36). Radiographs were evaluated for thoracic scoliosis, thoracic kyphosis, and chest wall deformity. Correlation analysis was performed.


RESULTS
Thoracic scoliosis was strongly correlated with decreased predicted vital capacity (r = -0.76). Significant diminution in vital capacity below 50% occurred at a curve magnitude of 60 degrees. Kyphosis and chest wall deformity were not predictive of decreased pulmonary function. Physical health (PCS) was closely correlated with predicted vital capacity (r = 0.65; P < 0.01) and with scoliosis (r = -0.52; P < 0.05).


CONCLUSIONS
Thoracic scoliosis of more than 60 degrees has severe adverse effects on pulmonary function in those with osteogenesis imperfecta. This finding may partly explain the increased pulmonary morbidity noted in adult patients with osteogenesis imperfecta and scoliosis compared with that in the general population.
Widmann
R F
RF
Department of Orthopaedics and Radiology, Hospital for Special Surgery, New York, NY, USA. widmannr@hss.edu
Bitan
F D
FD
Laplaza
F J
FJ
Burke
S W
SW
DiMaio
M F
MF
Schneider
R
R
D000328:Adult; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D007738:Kyphosis / Q000000981:diagnostic imaging / Q000209:etiology / Q000503:physiopathology; D008168:Lung / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D010013:Osteogenesis Imperfecta / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology*; D011788:Quality of Life; D013902:Radiography, Thoracic; D012600:Scoliosis / Q000000981:diagnostic imaging / Q000209:etiology / Q000503:physiopathology; D013121:Spinal Curvatures / Q000000981:diagnostic imaging* / Q000209:etiology*; D013131:Spine / Q000000981:diagnostic imaging; D013909:Thorax