Mazzola Riccardo F, Mazzola Isabella C
From the *Departments of Clinical Sciences and Community Health, Fondazione Ospedale Maggiore Policlinico IRCCS, Milan, Italy.
J Craniofac Surg. 2014 Jan;25(1):26-34. doi: 10.1097/SCS.0b013e3182a2ea94.
Most craniofacial malformations are identified by their appearance. The majority of the classification systems are mainly clinical or anatomical, not related to the different levels of development of the malformation, and underlying pathology is usually not taken into consideration. In 1976, Tessier first emphasized the relationship between soft tissues and the underlying bone stating that "a fissure of the soft tissue corresponds, as a general rule, with a cleft of the bony structure". He introduced a cleft numbering system around the orbit from 0 to 14 depending on its relationship to the zero line (ie, the vertical midline cleft of the face). The classification, easy to understand, became widely accepted because the recording of the malformations was simple and communication between observers facilitated. It represented a great breakthrough in identifying craniofacial malformations, named clefts by him. In the present paper, the embryological-based classification of craniofacial malformations, proposed in 1983 and in 1990 by us, has been revisited. Its aim was to clarify some unanswered questions regarding apparently atypical or bizarre anomalies and to establish as much as possible the moment when this event occurred. In our opinion, this classification system may well integrate the one proposed by Tessier and tries at the same time to find a correlation between clinical observation and morphogenesis.Terminology is important. The overused term cleft should be reserved to true clefts only, developed from disturbances in the union of the embryonic facial processes, between the lateronasal and maxillary process (or oro-naso-ocular cleft); between the medionasal and maxillary process (or cleft of the lip); between the maxillary processes (or cleft of the palate); and between the maxillary and mandibular process (or macrostomia).For the other types of defects, derived from alteration of bone production centers, the word dysplasia should be used instead. Facial dysplasias have been ranged in a helix form and named after the site of the developmental arrest. Thus, an internasal, nasal, nasomaxillary, maxillary and malar dysplasia, depending on the involved area, have been identified.The classification may provide a useful guide in better understanding the morphogenesis of rare craniofacial malformations.
大多数颅面畸形是通过其外观来识别的。大多数分类系统主要是临床或解剖学的，与畸形发育的不同阶段无关，并且通常不考虑潜在的病理学因素。1976年，泰西埃首次强调了软组织与下方骨骼之间的关系，称“软组织的裂隙通常与骨结构的裂隙相对应”。他引入了一种围绕眼眶的裂隙编号系统，从0到14，这取决于其与零线（即面部垂直中线裂隙）的关系。这种分类易于理解，被广泛接受，因为畸形的记录简单，便于观察者之间的交流。它在识别颅面畸形方面是一个重大突破，他将其命名为裂隙。在本文中，我们重新审视了1983年和1990年提出的基于胚胎学的颅面畸形分类。其目的是澄清一些关于明显非典型或奇异异常的未解决问题，并尽可能确定该事件发生的时间。我们认为，这种分类系统可以很好地整合泰西埃提出的分类系统，同时试图找到临床观察与形态发生之间的相关性。术语很重要。过度使用的“裂隙”一词应仅保留用于真正的裂隙，这些裂隙是由胚胎面部突起联合过程中的干扰形成的，如外侧鼻突与上颌突之间（或口鼻眼裂隙）；中鼻突与上颌突之间（或唇裂）；上颌突之间（或腭裂）；以及上颌突与下颌突之间（或巨口症）。对于其他类型的缺陷，由骨生成中心改变引起的，应使用“发育异常”一词。面部发育异常已按螺旋形式排列，并根据发育停滞的部位命名。因此，根据受累区域，已确定了鼻内、鼻、鼻上颌、上颌和颧骨发育异常。这种分类可为更好地理解罕见颅面畸形的形态发生提供有用的指导。
