Gougoutas Alexander J, Singh Davinder J, Low David W, Bartlett Scott P
Philadelphia, Pa.; and Phoenix, Ariz. From the Division of Plastic Surgery, Department of Surgery, University of Pennsylvania; Children's Hospital of Philadelphia; Edwin and Fannie Gray Hall Center for Human Appearance; and Barrow Craniofacial Center, Barrow Neurological Institute.
Plast Reconstr Surg. 2007 Dec;120(7):112e-113e. doi: 10.1097/01.prs.0000287383.35963.5e.
After reviewing this article, the participant should be able to: 1. Describe the fundamental malformations defining hemifacial microsomia. 2. Distinguish hemifacial microsomia from other congenital craniofacial anomalies sharing similar features. 3. Understand the variety of systems developed to clinically classify the features of this disorder. 4. Describe the format of the OMENS clinical classification system and appreciate its possible advantages and limitations.
The clinical manifestations of hemifacial microsomia comprise a spectrum that is both broad and complex. The fundamental features include unilateral hypoplasia of the craniofacial skeleton and its overlying soft tissue. Numerous schemes have been developed to classify this spectrum. One of the most recent classification systems, the OMENS system, scores five clinical manifestations of hemifacial microsomia according to dysmorphic severity on a scale from 0 to 3: orbital asymmetry, mandibular hypoplasia, ear deformity, nerve dysfunction, and soft-tissue deficiency.
The authors describe the diverse features of hemifacial microsomia and the numerous attempts at its clinical classification, with particular emphasis on the OMENS system.
With the possible exception of the OMENS scheme, the various systems developed to classify the clinical features of hemifacial microsomia fail to possess the flexibility and versatility needed to categorize all potential phenotypes of this complex disorder.
The OMENS system represents the most comprehensive, versatile, objective, and easily adaptable attempt at clinical classification of hemifacial microsomia to date. The authors propose a concise clinical evaluation form using a modified version of the system to promote the use of the OMENS system, to aid in the evaluation of hemifacial microsomia patients, and to assist in data sharing among academic institutions.
阅读本文后，读者应能够：1. 描述定义半侧颜面短小畸形的基本畸形。2. 将半侧颜面短小畸形与具有相似特征的其他先天性颅面畸形相区分。3. 了解为临床分类该疾病特征而开发的各种系统。4. 描述OMENS临床分类系统的形式，并认识到其可能的优点和局限性。
半侧颜面短小畸形的临床表现范围广泛且复杂。其基本特征包括颅面骨骼及其上方软组织的单侧发育不全。已经开发了许多方案来对这一范围进行分类。最新的分类系统之一，即OMENS系统，根据畸形严重程度将半侧颜面短小畸形的五种临床表现从0到3进行评分：眼眶不对称、下颌发育不全、耳部畸形、神经功能障碍和软组织缺损。
作者描述了半侧颜面短小畸形的多样特征以及对其进行临床分类的众多尝试，特别强调了OMENS系统。
除了OMENS方案外，为分类半侧颜面短小畸形临床特征而开发的各种系统都缺乏对这种复杂疾病所有潜在表型进行分类所需的灵活性和通用性。
OMENS系统是迄今为止对半侧颜面短小畸形进行临床分类的最全面、通用、客观且易于应用的尝试。作者提出了一种简洁的临床评估表，使用该系统的修改版本来促进OMENS系统的应用，辅助对半侧颜面短小畸形患者的评估，并协助学术机构之间的数据共享。
