A rare bone dysplasia and chronic lung disease

Objective: 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. Results: 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. Conclusion: 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.