Oro-facial considerations in a case of cleidocranial dysplasia– A review of the medical and dental management protocols

Cleidocranial dysplasia (CCD) is a rare, hereditary, congenital disorder inherited in an autosomal dominant pattern and is characterised by cranio-facial, skeletal and oro-dental malformations. The typical patient is short, presents a brachiocephalic skull with bossing of the frontal and parietal bones. Cephalic sutures and fontanelles exhibit delayed closure. The mid-face is hypoplastic, giving a misleading appearance of mandibular prognathism. The development of the clavicles is often defective and may range from a small medial gap to total agenesis. Medical issues include delayed attainment of developmental milestones, scoliosis, skeletal, pelvic and thoracic abnormalities. However, oro-dental malformations are important and can be significant. A 17-year-old Malay girl presented to the National Dental centre with complaints of unsightly front teeth. General and radiographic examination revealed features consistent with CCD. Dental clinical findings corroborated radiographically utilising conventional radiography and Cone Beam Computed Tomography uncovered an intact, retained deciduous dentition with multiple unerupted permanent and supernumerary teeth. A diagnosis of CCD was made based on the typical clinical and radiographic features and various management options explored. This condition is of clinical significance to the medical profession and more so for the orthodontist due to involvement of the facial bones, altered teeth eruption patterns and presence of supernumerary teeth. These problems represent a challenge to the clinician who must often seek a multi-disciplinary approach for successful management of this anomaly. Various treatment protocols and their shortcomings with respect to the case are discussed.