Mooney M P, Smith T D, Burrows A M, Langdon H L, Stone C E, Losken H W, Caruso K, Siegel M I
Department of Anatomy and Histology, University of Pittsburgh, PA 15261, USA.
Cleft Palate Craniofac J. 1996 Sep;33(5):369-78. doi: 10.1597/1545-1569_1996_033_0369_cspasp_2.3.co_2.
The purpose of the present study was to describe coronal suture pathology and cross sectional synostotic progression in an inbred strain of rabbits with congenital craniosynostosis. Calvaria from 102 perinatal rabbits (39 unaffected; 63 bilateral or unilateral synostosis) were collected at fetal days 21 (n = 12), 25 (n = 20), 27 (n = 22), 30 (term) (n = 32), and 3 days post-term (n = 16) for gross morphologic and histologic examination. Synostotic foci, the extent of relative bony bridging, and suture morphology were evaluated qualitatively and quantitatively. Of the 204 coronal sutures examined, 91 sutures were synostosed, and 113 were patent. All synostosed sutures showed similar foci by day 25, which originated as bony bridges in the middle of each suture on the ectocortic surface. Bony bridging width increased significantly (p < .001) from day 25 through 3 days post-term, and was best described by a linear regression equation. Osteogenic front areas of synostosed sutures were up to 2.5 times greater than patent sutures in term fetuses. Findings demonstrate that coronal suture synostosis in the congenital rabbit model (1) begins early during suture morphogenesis (before 25 days of gestation); (2) consistently radiates from a single focus corresponding to a normal interdigitating region (i.e., a high-tension environment); (3) varies in onset and rate as evidenced by low R2 value between age and extent of bony bridging; and (4) is the result of early hyperostosis of the osteogenic fronts and sutural agenesis. A number of possible pathogenetic mechanisms are discussed.
本研究的目的是描述患有先天性颅缝早闭的近交系兔子的冠状缝病理及横断面融合进展情况。在胎儿期第21天（n = 12）、25天（n = 20）、27天（n = 22）、30天（足月）（n = 32）和足月后3天（n = 16）收集102只围产期兔子的颅骨（39只未受影响；63只双侧或单侧融合），进行大体形态学和组织学检查。对融合灶、相对骨桥接范围和缝形态进行定性和定量评估。在检查的204条冠状缝中，91条缝发生融合，113条缝未融合。到第25天时，所有融合的缝均显示出相似的病灶，这些病灶起源于外板表面每条缝中间的骨桥。从第25天到足月后3天，骨桥接宽度显著增加（p <.001），用线性回归方程描述最佳。足月胎儿中，融合缝的成骨前沿面积比未融合缝大2.5倍。研究结果表明，先天性兔子模型中的冠状缝融合（1）在缝形态发生早期（妊娠25天前）开始；（2）始终从对应于正常交错区域（即高张力环境）的单个病灶放射状发展；（3）起始和速率存在差异，这可通过年龄与骨桥接范围之间的低R2值证明；（4）是成骨前沿早期骨质增生和缝发育不全的结果。文中讨论了一些可能的发病机制。
