Kokich V G, Moffett B C, Cohen M M
Cleft Palate J. 1982 Apr;19(2):89-99.
Two neonatal human specimens exhibiting the cloverleaf skull anomaly were studied in detail using gross, radiographic and histologic techniques. Both specimens exhibited premature fusion of the right and left coronal sutures, right and left lambdoidal sutures, and midsagittal sutures. Although the specimens grossly exhibited a similar trilobed appearance of the craniofacial complex, the interrelationship and spatial orientation of their respective articular and skeletal components differed markedly. The development of the cranial base in one specimen appeared normal, while in the other specimen, the synchondroses between the supraoccipital, exoccipital, basioccipital, basisphenoid, and presphenoid bones were prematurely obliterated histologically, resulting in a substantial decrease in the length of the cranial floor. The latter specimen also exhibited micromelic shortening of the limbs with abnormal chondrocyte proliferation and maturation at the epiphyseal growth plates. The alterations in the size and shape of the calvarial bones subsequent to premature sutural synostosis were more severe in the specimen with premature closure of the cranial base synchondroses. The striking differences observed in the anatomic and histologic analysis of the specimens demonstrated tha the cloverleaf skull malformation must be etiologically and pathogenetically heterogeneous.
使用大体观察、影像学和组织学技术对两例表现为三叶形颅骨异常的新生儿人体标本进行了详细研究。两例标本均表现出左右冠状缝、左右人字缝和矢状缝的过早融合。尽管标本在大体上呈现出类似的颅面复合体三叶形外观,但其各自关节和骨骼成分的相互关系及空间取向明显不同。其中一例标本的颅底发育看起来正常,而另一例标本在组织学上枕骨上部、枕骨外侧、枕骨基部、蝶骨基部和蝶骨前部之间的软骨结合过早消失,导致颅底长度大幅缩短。后一例标本还表现出四肢短肢性缩短,骨骺生长板处软骨细胞增殖和成熟异常。在颅底软骨结合过早闭合的标本中,过早缝合性骨性连接后颅骨大小和形状的改变更为严重。在标本的解剖学和组织学分析中观察到的显著差异表明,三叶形颅骨畸形在病因和发病机制上必然是异质性的。