Cleidocranial dysplasia- a case report and literature review

ABSTRACT

Cleidocranial dyspalsia is a rare congenital skeletal disorder, associated with hypoplasia or aplasia of clavicles, delayed closure of cranial sutures and fontanels, frontoparietal bossing, delayed exfoliation of primary dentition, delayed or failure of eruption of permanent teeth, and presence of multiple supernumerary teeth. The disorder is caused by mutation in the CBFA1 gene, on the short arm of chromosome 6p21. Estimated prevalence ofcleidocranial dysplasia is one per million, without gender or ethnic predilection. A multidisciplinary approach is often required for dental management of multiple supernumerary teeth. We report clinical and radiographic presentation and surgical management of multiple supernumerary teeth in an 11-year-old child with cleidocranial dysplasia.