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 ra
tios and angles obtained from lateral head films. Setting: The CCD ind
ividuals were referred to the Center for Craniofacial Anomalies for di
agnostic workup and treatment recommendations. Subjects: The sample co
nsisted of 14 Caucasians, The inclusion criterion for the young, prepu
bertal group (A) was complete primary dentition, and for the adult, po
stpubertal group (B), the eruption of all four first molars was requir
ed. Interventions: No treatment other than extraction or surgical remo
val of selected primary or supernumerary teeth was performed. Results:
Both groups showed significantly smaller anterior upper face height c
ompared with controls. Group B subjects demonstrated significantly sma
ller face height values than the controls in the A point-nasion-B poin
t (ANB) angle, facial axis, mandibular plane angle, palatal plane/mand
ibular plane angle, and genial angle. No significant differences were
found between group A individuals and the controls for these measureme
nts. The older group had shorter anterior lower face height compared w
ith both anterior upper face height and posterior lower face height. C
onclusions: Whereas young CCD subjects showed relatively normal jaw pr
oportions and morphology of the mandible, older CCD individuals tended
to have short lower face height, acute genial angle, anterior inclina
tion of the mandible, and mandibular prognathism. These differences ca
n be attributed to pronounced horizontal mandibular growth resulting f
rom lack of vertical maxillary growth and impaired eruption of permane
nt teeth.