Arvystas M, Shprintzen R J
J Craniofac Genet Dev Biol. 1984;4(1):39-45.
The velo -cardio-facial syndrome is a recently delineated congenital malformation syndrome, probably of autosomal dominant inheritance. Previous reports have concentrated on facial, oropharyngeal, cardiac, speech, language, and psychological features of this fairly common syndrome. To date, no radiographic data have been presented which might help to further delineate the syndrome, nor has there been an explanation of the characteristic facial appearance seen in this syndrome. This current study reports on cephalometric evidence of platybasia (obtuse angulation of the cranial base) in the velo -cardio-facial syndrome. The finding of platybasia adds one more phenotypic feature to the syndrome and also may help to explain the facial features of retrognathia, malar flatness, and prominence of the nasal root.
腭心面综合征是一种最近才被描述的先天性畸形综合征,可能为常染色体显性遗传。既往报道主要集中在这种相当常见综合征的面部、口咽、心脏、言语、语言及心理特征方面。迄今为止,尚未有有助于进一步明确该综合征的影像学资料,也没有对该综合征中特征性面部外观作出解释。本项研究报告了腭心面综合征中颅底扁平(颅底钝角)的头影测量证据。颅底扁平这一发现为该综合征增添了又一表型特征,也可能有助于解释下颌后缩、颧骨扁平及鼻根突出等面部特征。