Chest-wall reconstruction for spondylocostal dysostosis.

A child with respiratory insufficiency due to a lethal syndrome known as spondylocostal dysostosis is described. Severe chest-wall deformities with resultant paradoxical chest-wall motion were successfully corrected by surgical reconstruction of the right thorax. We conclude that patients with the severe lethal form of the syndrome may benefit from aggressive surgical correction of the thoracic abnormality.