Cleidocranial dysplasia: case report of three siblings.

Background: A family case report of cleidocranial dysplasia(CCD) with varied manifestations from father to threesiblings is presented. CCD ( MIM # 119600) is a rareautosomal dominant skeletal dysplasia caused by CBAF1gene ( OMIM 600211) with a wide range of variability. Inall the cases generalized dysplasia in bone, prolongedretention of primary teeth and delayed eruption of permanentteeth were evident. Interestingly, there were nosupernumerary teeth present. There was mandibularprognathism which was intercepted by occipital chin cuptherapy.Aims and objective: To present the clinical manifestations,diagnostic imaging and treatment modalities along withdermatoglyphics in CCD patients.Conclusion: Cleidocranial dysplasia is an uncommondisorder however its clinical and radiological features arecharacteristic. In addition the CCD patients may bedistinguished by specific dermatoglyphic markers. It carrieswith it several implications in terms of complications likeskeletal malocclusion, dental caries, etc. Medical treatmentis mainly directed at orthopedic and dental correction. Ateam approach to the management of dental abnormalitieson a long-term basis with the overall goal to provide anesthetic facial appearance and functioning occlusion by lateadolescence or early adulthood should be focused.