Definition:

Raine syndrome or lethal osteosclerotic bone dysplasia is an autosomal recessive disorder characterized by generalized osteosclerosis and characteristic facial dysmorphism. Death in the neonatal period is due to respiratory failure. Radiographic studies have shown generalized increase in the density of all bones and the basal structures of the skull, which leads to the characteristic facial features including microcephaly, proptosis, hypoplastic nose and midface, small jaw, and cleft palate.