Kreiborg S, Aduss H, Cohen M M
School of Dentistry, Department of Pediatric Dentistry, Faculty of Health Sciences, University of Copenhagen, Denmark.
J Craniofac Genet Dev Biol. 1999 Jan-Mar;19(1):1-11.
This paper reports a cephalometric analysis of the craniofacial morphology in adolescents and adults with Apert syndrome. The sample comprised 26 patients with Apert syndrome (15 males and 11 females). The control group consisted of 153 adults (102 males and 51 females). Both lateral and frontal cephalograms were studied. The data were presented as mean plots of the craniofacial 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 decreased. The cranial base showed marked protrusion of the greater wing of the sphenoid, which contributed to severe reduction of orbital volume and protrusion of the eyeglobe. Orbital volume was further reduced by maxillary hypoplasia in all three planes of space together with retrognathia. Maxillary height was extremely short and so was the nose. The width of the nasal cavity, height and depth of the bony nasopharynx, and the nasopharyngeal airway were all markedly reduced in size. The mandible was of fairly normal size and shape but was posteriorly inclined. Head posture was extended in relation to the cervical column. Total facial height was increased, whereas upper 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 patients. Marked and significant differences were found between the two syndromes in nearly all craniofacial regions, and craniofacial dysmorphology was generally more severe in Apert syndrome patients.
本文报告了对患有Apert综合征的青少年和成人颅面形态的头影测量分析。样本包括26例Apert综合征患者（15例男性和11例女性）。对照组由153名成年人组成（102例男性和51例女性）。对侧位和正位头颅X线片进行了研究。数据以颅面部区域的平均图以及一些最重要发现的数据呈现。除下颌骨外，几乎所有颅面部区域均发现明显差异。颅盖骨的高度和宽度增加，但长度减小。颅底显示蝶骨大翼明显突出，这导致眼眶容积严重减小和眼球突出。上颌骨发育不全在三维空间的所有平面上以及下颌后缩进一步减小了眼眶容积。上颌高度极短，鼻子也很短。鼻腔宽度、骨性鼻咽的高度和深度以及鼻咽气道的大小均明显减小。下颌骨大小和形状基本正常，但向后倾斜。头部姿势相对于颈椎呈伸展状态。面部总高度增加，而上面部高度明显减小。切牙咬合显示下颌前突和开牙合。然后将Apert综合征患者与一组Crouzon综合征患者进行比较。在几乎所有颅面部区域，两种综合征之间均发现明显且显著的差异，并且Apert综合征患者的颅面畸形通常更严重。
