Woon K C, Kokich V G, Clarren S K, Cohen M M
Teratology. 1980 Aug;22(1):23-35. doi: 10.1002/tera.1420220105.
A set of like-sexed male twins with the same malformation syndrome and exhibiting identical anomalous alterations of the craniofacial complex were studied in detail. The right and left coronal and left lamboidal sutures were prematurely synostosed in both specimens. The cranial base exhibited a mediolateral asymmetry, with the right half larger than the left. The occipital bone demonstrated the greatest abnormality, including bilateral clefting of the basioccipital component and alterations in the size and shape of the exoccipital bone. In lateral view, the cranial base was flattened and the ventral flexure occurred between the two components of the basioccipital. The odontoid process of the second cervical vertebra was rotated anteroinferiorly and formed part of the cranial floor. Histologically, the bilateral clefts of the basioccipital bone were united by fibrous connective tissue, and the central area of union contained chondroid-like bone. The cartilaginous spheno-occipital synchondrosis and the epiphyseal cartilage of the finger demonstrated similar dystrophic alterations. The brain was normal but exhibited external cerebral deformation secondary to aberrant skull shape.
对一组患有相同畸形综合征且颅面复合体表现出相同异常改变的同性男性双胞胎进行了详细研究。在两个标本中，左右冠状缝和左人字缝均过早闭合。颅底表现出内外侧不对称，右侧比左侧大。枕骨表现出最严重的异常，包括基枕部双侧裂开以及枕外骨大小和形状的改变。在侧视图中，颅底变平，基枕部的两个部分之间出现腹侧弯曲。第二颈椎齿突向前下旋转并构成颅底的一部分。组织学上，基枕骨的双侧裂隙由纤维结缔组织连接，连接的中央区域含有类软骨样骨。蝶枕软骨结合和手指的骨骺软骨表现出类似的营养不良性改变。大脑正常，但由于颅骨形状异常而出现脑外部变形。
