Cephalometric study of the Apert syndrome in adolescence and adulthood

This paper reports a cephalometric analysis of the craniofacial morphology in adolescents and adults with Apert syndrome. The sample comprised 26 pati ents with Apert syndrome (15 males and 11 females). The control group consi sted of 153 adults (102 males and 51 females). Both lateral and frontal cep halograms were studied. The data were presented as mean plots of the cranio facial region together with data on some of the most significant findings. Marked differences were found in nearly all craniofacial regions except the mandible. The calvaria was increased in height and width but length was de creased. The cranial base showed marked protrusion of the greater wing of t he sphenoid, which contributed to severe reduction of orbital volume and pr otrusion of the eyeglobe. Orbital volume was further reduced by maxillary h ypoplasia in all three planes of space together with retrognathia. Maxillar y height was extremely short and so was the nose. The width of the nasal ca vity, height and depth of the bony nasopharynx, and the nasopharyngeal airw ay were all markedly reduced in size. The mandible was of fairly normal siz e and shape but was posteriorly inclined. Head posture was extended in rela tion to the cervical column. Total facial height was increased, whereas upp er facial height was markedly reduced. Incisor occlusion showed mandibular overjet and open bite. Apert syndrome patients were then compared to a group of Crouzon syndrome p atients. Marked and significant differences were found between the two synd romes in nearly all craniofacial regions, and craniofacial dysmorphology wa s generally more severe in Apert syndrome patients.