rare bone dysplasia and chronic lung disease

To illustrate the development of chronic lung disease in an 11-year-old child with spondylo-meta-epiphyseal dysplasia-abnormal calcification type. Method: Historical review and radiological investigation by high resolution computerized tomography of the thorax. Severe bronchiectasis with a mosaic perfusion pattern was demonstrated. The onset of lung disease was insidious in the absence of a history of recurrent respiratory infection and symptoms. We postulate that the rigidity of the chest wall led to restrictive lung disease. Children with this type of dysplasia need careful monitoring of their respiratory status during their early years