Restrictive lung disease and cor pulmonale secondary to polyostotic fibrous dysplasia.
Polyostotic fibrous dysplasia is a rare benign pathological condition of bone in which proliferation of fibrous and osteoid elements results in expansile deformities of the skeleton. We present a case of polyostotic fibrous dysplasia in a young man in whom the severe deformities of the chest wall and spine produced restrictive lung disease, cor pulmonale and respiratory failure.
Narayan
Rajeev L
RL
Maldjian
Pierre D
PD
D000328:Adult; D004452:Echocardiography; D005359:Fibrous Dysplasia, Polyostotic / Q000150:complications / Q000000981:diagnostic imaging*; D006801:Humans; D008171:Lung Diseases / Q000000981:diagnostic imaging* / Q000209:etiology; D008297:Male; D011660:Pulmonary Heart Disease / Q000000981:diagnostic imaging* / Q000209:etiology