History

Since antiquity, various cultures have focused on individuals with abnormal cranial contour. Allusions to aberrant skull shape have been noted in writings connected with the ancient Chinese gods of good fortune and long life (Fukurokuju and Shou Lao), by Homer in "The Iliad" and by Hippocrates and Galen [1] and others. Nearly 200 years ago, Sommerring reported the first scientific investigation of cranial deformities [2]. He discussed cranial sutures, recognized their primary importance in skull growth and asserted that premature suture fusion produced cranial deformity. In

1830, Otto postulated that premature suture closure gives rise to cranial deformity, with compensatory expansion elsewhere [3]. In an extension of Otto's observations, Virchow published his seminal work on skull deformity that provided the basis for future scientific study of cranial deformity and craniosynostosis [4]. Although not the first to make these observations, Virchow noted that cranial growth occurs along suture lines and premature fusion arrested growth perpendicular to the fusion, producing skull deformity .

Over the next century, understanding of cran-iosynostosis burgeoned. Reports of craniosynostosis were increasingly disseminated [1] and ophthalmological perspectives were introduced. Authors also described craniosynostosis in association with other anomalies and provided the impetus for future classification of syndromic craniosynostosis. Apert [5] and Crouzon [6], among others, described those syndromes that continue to bear their names. In the late nineteenth century, Lane [7] and Lannelongue [8] reported the first modern surgical corrections of skull deformity resulting from premature suture closure. From these original pioneering experiences, subsequent advances in treatment have continued into contemporary times.

Virchow's hypotheses concerning craniosyn-ostosis remained the standard for nearly a century. However, in the mid-twentieth century, van der Klaauw and Moss questioned the primacy of the calvarial sutures as the antecedent mediator of skull deformities [9, 10]. Based on his original ideas, subsequent work and the efforts of others, Moss proposed that the primary anomaly in craniosynostosis arose in the cranial base. He hypothesized that the primary abnormality arose in the cranial base, and this resulted in the secondary fusion of the cranial vault suture(s). His arguments were fourfold [10-12]: (1) on occasion, suture patency was found at surgery, despite pre-oper-ative suspicion of premature suture fusion and characteristic skull configuration [13]; (2) characteristic anomalies of the cranial base were associated with specific calvarial suture closures; (3) experimental removal of normal cranial vault sutures resulted in no significant change in overall skull shape; and (4) cranial base development and maturation precede those of the cranial vault. Additionally, Moss believed that the primary force driving the

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