Smith T D, Mooney M P, Burrows A M, Losken H W, Siegel M I
Department of Anthropology, University of Pittsburgh, PA 15261, USA.
J Craniofac Genet Dev Biol. 1996 Apr-Jun;16(2):107-17.
Cranial base abnormalities are a common correlate of human craniosynostosis, although controversy exists as to whether they are primary malformations or secondary deformities. The current study assesses longitudinal cranial base growth and shape changes in rabbits with congenital coronal suture (CS) synostosis. Data were collected from 96 New Zealand White rabbits: 21 with complete bilateral or unilateral CS synostosis; 33 with partial CS synostosis; and 42 unaffected, "control" litter mates. Serial radiographs were taken at 1.5, 6, 12, and 18 weeks of age. Linear, angular, and shape measurements of the cranial base were compared among the three groups. Results revealed that at 1.5 weeks of age, rabbits with complete synostosis had significantly (P < 0.05) shortened anterior cranial bases, orthocephalic cranial base angeles, and acute, dysmorphic cribriform plate triangular shapes compared to the two other groups. Some significant differences persisted through 18 weeks of age. No significant differences were noted between partially synostosed and normal rabbits at any time period. If partial synostosis represents a continuum of the craniosynostotic phenotype, then the craniosynostosis may be seen as the primary deformity in this model and the cranial base abnormalaties as secondary, deformational changes. However, fetal data are still needed to more clearly delineate the role of the cranial base in this congenital rabbit model. Results reiterate the appropriateness and continued development of these rabbits for modeling cases of familial, human non-syndromic coronal suture synostoses.
颅底异常是人类颅缝早闭的常见相关因素，尽管对于它们是原发性畸形还是继发性畸形存在争议。本研究评估了先天性冠状缝（CS）早闭家兔的颅底纵向生长和形状变化。收集了96只新西兰白兔的数据：21只患有完全性双侧或单侧CS早闭；33只患有部分CS早闭；42只未受影响的“对照”同窝仔兔。在1.5、6、12和18周龄时拍摄系列X线片。比较了三组之间颅底的线性、角度和形状测量值。结果显示，在1.5周龄时，与其他两组相比，完全性早闭的家兔前颅底明显缩短（P<0.05），颅底角度呈正头型，筛板三角形形状尖锐且形态异常。一些显著差异一直持续到18周龄。在任何时间段，部分早闭家兔和正常家兔之间均未发现显著差异。如果部分早闭代表颅缝早闭表型的一个连续过程，那么在这个模型中，颅缝早闭可能被视为原发性畸形，而颅底异常则被视为继发性的变形改变。然而，仍需要胎儿数据来更清楚地界定颅底在这个先天性家兔模型中的作用。结果再次强调了这些家兔在模拟家族性、人类非综合征性冠状缝早闭病例方面的适用性和持续发展性。
