Mooney M P, Cooper G M, Burrows A M, Wigginton W, Smith T D, Dechant J, Mitchell R, Losken H W, Siegel M I
Department of Oral Medicine and Pathology, University of Pittsburgh, Pittsburgh, Pennsylvania 15261, USA.
Anat Rec. 2000 Nov 1;260(3):238-51. doi: 10.1002/1097-0185(20001101)260:3<238::AID-AR40>3.0.CO;2-Q.
Previous studies from our laboratory have characterized the craniofacial morphology and growth patterns of an inbred strain of rabbits with autosomal dominant coronal suture synostosis. A number of rabbit perinates from this colony have been collected sporadically over a 5-year period with premature interfrontal suture synostosis. The present study describes the very early onset of craniofacial dysmorphology of these rabbits and compares them to similar-aged normal control rabbits. A total of 40 perinatal New Zealand White rabbits were used in the present study. Twenty-one comprised the sample with interfrontal suture synostosis and ranged in age from 27 to 38 days postconception (term = 31 days) with a mean age of 33.53 days (+/-2.84 days). Nineteen rabbits served as age-matched, normal controls (mean age = 33.05 days +/-2.79 days). Lateral and dorsoventral radiographs were collected from each rabbit. The radiographs were traced, computer digitized, and 12 craniofacial measurements, angles, and indices were obtained. Mean measures were compared using an unpaired Student's t-test. All synostosed rabbits were stillborn or died shortly after birth. Grossly, these rabbits exhibited extreme frontal bossing, trigonocephaly with sagittal keeling, and midfacial shortening. No somatic anomalies were noted. Radiographically, rabbits with interfrontal suture synostosis had significantly (P < 0.05) narrower bifrontal widths, shorter cranial vault lengths, kyphotic cranial base angles, and different cranial vault indices (shapes) compared to controls. Results reveal severe and early pathological and compensatory cranial vault changes associated with premature interfrontal suture synostosis in this rabbit model. The 100% mortality rate noted in this condition may be related to the inheritance of a lethal genetic mutation or to neural compression from reduced intracranial volume. Results are discussed in light of current pathogenic hypotheses for human infants with premature metopic suture synostosis.
我们实验室之前的研究已经对患有常染色体显性冠状缝早闭的近交系兔子的颅面形态和生长模式进行了特征描述。在5年时间里，我们偶尔从这个兔群中收集了一些患有额缝过早闭合的新生兔。本研究描述了这些兔子颅面畸形的极早期发病情况，并将它们与同龄的正常对照兔子进行比较。本研究共使用了40只围产期新西兰白兔。其中21只为额缝早闭样本，受孕后年龄在27至38天之间（足月为31天），平均年龄为33.53天（±2.84天）。19只兔子作为年龄匹配的正常对照（平均年龄 = 33.05天±2.79天）。从每只兔子身上采集了侧位和背腹位X线片。对X线片进行描图、计算机数字化处理，并获得了12项颅面测量值、角度和指数。使用未配对的学生t检验比较平均测量值。所有早闭的兔子均为死产或出生后不久死亡。大体上，这些兔子表现出极度的额部隆起、伴有矢状嵴的三角头畸形和中面部缩短。未发现躯体异常。影像学上，与对照组相比，额缝早闭的兔子双额宽度明显变窄（P < 0.05）、颅顶长度缩短、颅底呈驼背样角度以及颅顶指数（形状）不同。结果显示，在这个兔子模型中，与额缝过早闭合相关的颅顶存在严重且早期的病理和代偿性变化。这种情况下观察到的100%死亡率可能与致死基因突变的遗传或颅内体积减小导致的神经受压有关。我们根据目前关于人类婴儿额缝过早闭合的致病假说对结果进行了讨论。
