Stavropoulos Dimitrios, Tarnow Peter, Mohlin Bengt, Kahnberg Karl-Erik, Hagberg Catharina
Department of Orthodontics, Institute of Odontology, Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden.
Swed Dent J. 2012;36(1):25-34.
Cranio-maxillofacial malformations, as seen in Crouzon and Apert syndromes, may impose an immense distress on both function and aesthetics of the person affected. The aims of this study were to describe and compare the main facial and intraoral features of patients with Apert and Crouzon syndromes, the clinical manifestations that may be present, additionally to the main syndromic traits, as well as the cranio-maxillofacial surgical treatment protocols followed.Twenty-three patients with Apert syndrome (6 males, 17 females), and 28 patients with Crouzon syndrome (20 males, 8 females) were evaluated for general medical aspects, craniofacial characteristics, dentoalveolar traits before and after the final orthognathic surgery, and types and timing of cranio-maxillofacial operations. Mental retardation, associated additional malformations, cleft palate, and extensive lateral palatal soft tissue swellings were more common in children with Apert syndrome. In both syndromes, clinical findings included concave profile, negative overjet, posterior crossbites, anterior openbite, and dental midline deviation, which were corrected in almost all cases with the final orthognathic surgery, with the exception of the lateral crossbites, including more than one tooth pair, which were persisting in about half of the cases. Cranial vault decompression and/or reshaping, midfacial and orbital advancement procedures, often in conjunction with a mandibular setback, were the most frequent cranio-maxillofacial operations performed. In conclusion, Apert syndrome is more asymmetric in nature and a more severe clinical entity than Crouzon syndrome. The syndromic dentofacial features of both conditions could be significantly improved after a series of surgical procedures in almost all cases with the exception of the posterior crossbites, with haIf of them persisting post-surgically.
颅颌面畸形，如在克鲁宗综合征和阿佩尔综合征中所见，可能会给受影响者的功能和美观带来极大困扰。本研究的目的是描述和比较阿佩尔综合征和克鲁宗综合征患者的主要面部和口腔内特征、除主要综合征特征外可能出现的临床表现，以及所遵循的颅颌面外科治疗方案。对23例阿佩尔综合征患者（6例男性，17例女性）和28例克鲁宗综合征患者（20例男性，8例女性）进行了评估，内容包括一般医学方面、颅面部特征、最终正颌手术前后的牙牙槽特征以及颅颌面手术的类型和时间。智力发育迟缓、相关的其他畸形、腭裂和广泛的腭侧软组织肿胀在阿佩尔综合征患儿中更为常见。在这两种综合征中，临床表现包括面部轮廓凹陷、覆盖为负、后牙反合、前牙开合和牙中线偏差，几乎所有病例在最终正颌手术后这些问题都得到了纠正，但包括不止一对牙齿的侧方反合在约一半的病例中仍然存在。颅骨减压和/或重塑、面中部和眼眶前移手术，通常与下颌后缩联合进行，是最常进行的颅颌面手术。总之，阿佩尔综合征在本质上比克鲁宗综合征更不对称，是一种更严重的临床病症。在几乎所有病例中，经过一系列手术，这两种病症的综合征性牙颌面特征都能得到显著改善，但后牙反合除外，其中一半在术后仍然存在。
