Cleidocranial dysplasia

Cleidocranial dysplasia (CCD) is a disorder that affects mostly those bones which derived from endochrondal and intramembranous ossification, such as the cranium and the clavicles. CCD is present at a frequency of one in one million individuals. Over 500 cases have been reported. A 13 years old boy reported in the Paediatric Endocrinology outpatient department, Bangladesh Institute of Research and Rehabilitation on Diabetes, Endocrine and Metabolic Disorders (BIRDEM) on 6th April 2006 with complaints of not growing according to his age, a wide gap over the head since birth and delayed shedding of primary teeth and also eruption of secondary teeth. The boy had elongated facies with midfacial hypoplasia, depressed nasal bridge with hypertelorism. His anterior fontanelle was open about 3/1.5 cm. He had short clavicle and his shoulders could be easily apposed. Radiological findings showed anterior fontanellle and sutures were open and widened, all decidual teeth were intact and all secondary teeth were impacted beneath the primary teeth and hypoplastic clavicle. He was diagnosed as a case of Cleidocranial Dysplasia on the basis of history, clinical examination and investigation.