Familial Occurrence of Cleidocranial Dysostosis: A Case Report

Cleidocranial dysostosis is a rare congenital skeletal disorder, associated with total or partial absence of clavicles, delayed closure of cranial fontanelles and brachycephalic skull. There is delayed exfoliation of primary dentition and eruption of permanent teeth. Presence of multiple supernumerary teeth and morphologic abnormalities of the maxilla and mandible are also observed. The incidence of this autosomal dominant disorder is reported as 1:10,00,000 and the prevalence is 0.5:1,00,000, with no gender or ethnic group predilection. It was first described by Marie and Sainton in 1898. This paper presents the case of a seven year old male patient who reported to the department of Paediatric and Preventive Dentistry with a chief complaint of decayed teeth. Following thorough clinical and radiographic examination and investigations along with 3D images, the diagnosis was confirmed as cleidocranial dysostosis. A familial pattern was observed as patient’s father and older sister had similar clinical features.