Dambrain R, Freund M, Verellen G, Pellerin P, Francke J P, Dhem A
Unité d'Anatomie humaine, Université Catholique de Louvain, Bruxelles, Belgique.
J Craniofac Genet Dev Biol. 1987;7(4):387-401.
The study of four cloverleaf skulls (two fetuses, one infant, and a young adult) concerns two Pfeiffer syndromes, a thanatophoric dysplasia and an isolated case. Clinical and radiologic examinations showed malformations at the level of the calvarium, the base, orbital cavities, and, sometimes, limb abnormalities. Correlations between these findings and the microradiographic analysis of nondemineralized sections elucidate this trilobular appearance of the skull. Premature temporoparietal suture closure terminates at a constricted surface of the lateral sides of the skull during the fetal life. Extension of the synostosis to coronal and frontal sutures and thickening of the occipital bone squama block lengthening of the skull. The consecutive reduction in skull volume is compensated by the maintenance of the permeability during the neonatal period of the sagittal and lambdoid sutures. Microradiographic examination shows that this anomaly may be of vascular origin and associated with abnormal osteoclastic resorption.
对四个三叶草形颅骨（两个胎儿、一个婴儿和一个青年成人）的研究涉及两种 Pfeiffer 综合征、一种致死性骨发育不全和一个孤立病例。临床和放射学检查显示在颅盖、颅底、眶腔水平存在畸形，有时还伴有肢体异常。这些发现与未脱钙切片的显微放射学分析之间的相关性阐明了颅骨的这种三叶形外观。颞顶缝过早闭合在胎儿期终止于颅骨两侧的狭窄表面。骨缝融合扩展至冠状缝和额缝以及枕骨鳞部增厚阻碍了颅骨的延长。颅骨体积的持续减小通过矢状缝和人字缝在新生儿期保持通透性得到补偿。显微放射学检查表明这种异常可能起源于血管，并与异常的破骨细胞吸收有关。
