Characteristics of Jaw Growth in Cleidocranial Dysplasia

Abstract

Objective

The purpose of this pilot study was to assess craniofacial morphology in young and adult individuals with cleidocranial dysplasia (CCD).

Design

Craniofacial morphology in young individuals (primary dentition) and in young adults was compared with control data using ratios and angles obtained from lateral head films.

Setting

The CCD individuals were referred to the Center for Craniofacial Anomalies for diagnostic workup and treatment recommendations.

Subjects

The sample consisted of 14 Caucasians. The inclusion criterion for the young, prepubertal group (A) was complete primary dentition, and for the adult, postpubertal group (B), the eruption of all four first molars was required.

Interventions

No treatment other than extraction or surgical removal of selected primary or supernumerary teeth was performed.

Results

Both groups showed significantly smaller anterior upper face height compared with controls. Group B subjects demonstrated significantly smaller face height values than the controls in the A point-nasion-B point (ANB) angle, facial axis, mandibular plane angle, palatal plane/mandibular plane angle, and gonial angle. No significant differences were found between group A individuals and the controls for these measurements. The older group had shorter anterior lower face height compared with both anterior upper face height and posterior lower face height.

Conclusions

Whereas young CCD subjects showed relatively normal jaw proportions and morphology of the mandible, older CCD individuals tended to have short lower face height, acute gonial angle, anterior inclination of the mandible, and mandibular prognathism. These differences can be attributed to pronounced horizontal mandibular growth resulting from lack of vertical maxillary growth and impaired eruption of permanent teeth.