Postnatal Development of Craniofacial Malformations
Postnatal Development of Craniofacial Malformations
复制标题
颅面畸形的产后发育
DOI：
10.1177/00220345680470066101
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发表时间：
1968
影响因子：
7.6
通讯作者：
S. Pruzansky
中科院分区：
文献类型：
--
作者：
S. Pruzansky
本文旨在强调需要更严格的分析下颌骨畸形的性质及其出生后的生长模式。为了区分下颌骨，研究者面临两个问题：第一，必须使用精确的人体测量来描述异常; X线头影测量在本研究中是成功的。其次，一个偏离下颌骨必须与其他异常下颌骨的大小，形状，以及与头骨和面部轮廓的关系进行比较。在特定的综合征中，表现性的变化需要对受累器官的受累程度进行定量测量。对性状的描述越完整，检测杂合子的能力就越强。这种分析还应该包括时间的第四维，即出生后的生长潜力。有些下颌骨发育不全，其恢复是相当可观的。其他人保持同样的畸形，在少数情况变得更糟。对未来生长潜力的预测提出了关于缺陷发病机制的重要问题。皮埃尔·罗宾综合征这种综合征的病理生理特征通常归因于下颌骨后缩或小下颌骨，从而产生舌下垂。目前，X线头影测量数据超过50例（C。T. PAVLICK和S. PRUZANSKY，未发表的数据）。在考虑这种综合征的起源时，认为主要缺陷是下颌骨的宫内发育迟缓。舌在闭合的关键阶段保持在腭架之间，防止融合。这表明小颌畸形而不是腭裂是其遗传标记。下颌后缩会随着时间的推移逐渐减少，直到与正常变异相融合，因此遗传标记似乎消失了。更仔细的检查表明，
This paper is intended to stress the need for more rigid analysis of the nature of mandibular deformities and their postnatal growth patterns. To differentiate between mandibles, the investigator faces two problems: First, a precise anthropometric measure to delineate abnormality must be used; roentgencephalometry was successful in this study. Second, a deviate mandible must be compared with other abnormal mandibles in size, shape, and relation to the skull and facial profile. In a given syndrome, variation in expressivity demands a quantitative measure of the degree of involvement of affected organs. The more complete the description of the trait, the greater capacity to detect the heterozygote. Such analyzes should also include the fourth dimension of time; ie, the postnatal growth potential. In some hypoplastic mandibles recuperation is considerable. Others retain the same deformity, and in a few the condition becomes worse. The prognostication of future growth potential raises important questions regarding the pathogenesis of the defect. PIERRE ROBIN'S SYNDROME.-The pathophysiologic feature of this syndrome is generally ascribed to the retrognathic or micrognathic mandible, which produces glossoptosis. At present, roentgencephalometric data are available on more than 50 cases (C. T. PAVLICK and S. PRUZANSKY, unpublished data). In considering the origin of this syndrome, it was reasoned that the primary defect was in the intrauterine growth retardation of the mandible. The tongue was maintained between the palatal shelves at the critical stage of closure, preventing fusion. It is implied that the mandibular micrognathia, rather than the cleft palate, is the genetic marker. The retrognathia diminishes in time to the point of merging with normal variation, thus the genetic marker seemed to vanish. Closer examination showed that the