Plastic bowing of the ribs in children

Four cases of plastic bowing of the ribs are presented. In three patients with Werdnig-Hoffman disease, plastic curvatures were associated with chronic pneumonia and atelectasis. We postulate that intrapulmonary retractive forces can deform ribs thinned by muscular atrophy. In turn, thoracic collapse can perpetuate lobar and segmental atelectasis. In one case of osteogenesis imperfecta without pneumonia, we believe normal muscle forces bent ribs weakened by deficiency of normal cortical architecture.