Cleidocranial dysplasia - A rare case report with review of literature

Cleidocranial dysplasia (CCD), also known as Marie and Sainton's disease is a hereditary bone disorder characterized by partial/complete absence of clavicles, multiple supernumerary teeth, open sagittal sutures and fontanelles. This is a rare syndrome with prevalence of less than 1 per million. CCD shows an autosomal dominant inheritance pattern or spontaneous mutation in gene on 6p21 encoding transcription factor CBFA1 also called as Runx2. In dentistry, the clinical importance of CCD is due to involvement of facial bones, altered eruption pattern and multiple supernumerary teeth. This article presents a classical case of CCD with a detailed description of clinical and radiographic findings along with a discussion of various management modalities.