Mooney M P, Siegel M I, Burrows A M, Smith T D, Losken H W, Dechant J, Cooper G, Kapucu M R
Department of Anatomy and Histology, University of Pittsburgh, PA 15261, USA.
Childs Nerv Syst. 1998 Jun;14(6):236-46. doi: 10.1007/s003810050219.
Poswillo has stated, "The more severe anomalies of the calvaria, such as plagiocephaly, Crouzon [syndrome], and Apert syndrome still defy explanation, in the absence of an appropriate animal system to study" (p. 207). This two-part study reviews data from a recently developed colony of New Zealand white rabbits with familial, nonsyndromic unilateral coronal suture synostosis. Part 1 presents pathological findings and compensatory sutural growth data from 109 normal rabbits and 82 craniosynostotic rabbits from this colony. Synostotic foci, onset, and progression were described in the calvariae from 102 staged (fetal days 21, 25, 27, 33; term = 30 days) fetuses (39 normal, 63 synostosed). Calvarial suture growth patterns from 10 to 126 days of age were assessed from serial radiographs obtained from 89 rabbits (70 normal rabbits and 19 rabbits with unicoronal suture synostosis) with amalgam bone marker implants. Perinatal results revealed that by fetal day 25 the synostotic focal point in synostotic rabbits consistently originated from the endocortical surface of the calvaria in the middle of the coronal suture at a presumed high-tension, interdigitating zone. Histological analysis revealed hyperostotic osteogenic fronts on the affected side compared with the unaffected side. Postnatal sutural growth data revealed a predictable pattern of plagiocephaly (contralateral coronal sutures growing more than ipsilateral sutures and ipsilateral frontonasal and anterior lambdoidal sutures growing more than contralateral sutures), which resulted in early cranial vault deformities and a double "S" shape torquing towards the affected side. The advantages and disadvantages of these rabbits as a model for human familial, nonsyndromic unicoronal suture synostosis are discussed, especially in light of recent cytokine and genetic findings from human craniosynostotic studies.
“在缺乏合适的动物研究系统的情况下，颅骨的一些较为严重的异常情况，如斜头畸形、克鲁宗[综合征]和阿佩尔综合征，仍然无法得到解释”（第207页）。这项分为两部分的研究回顾了来自一个最近培育的新西兰白兔群体的数据，这些兔子患有家族性、非综合征性单侧冠状缝早闭。第一部分介绍了来自该群体的109只正常兔子和82只患有颅缝早闭的兔子的病理结果及代偿性缝线生长数据。描述了102只分期（胎儿第21、25、27、33天；足月为30天）胎儿（39只正常，63只发生缝早闭）颅骨中的缝早闭病灶、发病情况和进展。通过对89只植入汞合金骨标记物的兔子（70只正常兔子和19只患有单侧冠状缝早闭的兔子）拍摄的系列X光片，评估了10至126日龄颅骨缝线的生长模式。围产期结果显示，到胎儿第25天时，患有缝早闭的兔子的缝早闭病灶始终起源于冠状缝中部颅骨的内皮质表面，处于一个假定的高张力、相互交错区域。组织学分析显示，与未受影响的一侧相比，受影响一侧有骨增生的成骨前沿。出生后缝线生长数据显示出一种可预测的斜头畸形模式（对侧冠状缝比同侧缝线生长更多，同侧额鼻缝和前人字缝比另一侧缝线生长更多），这导致了早期颅穹窿畸形以及向受影响一侧扭转的双“S”形。讨论了这些兔子作为人类家族性、非综合征性单侧冠状缝早闭模型的优缺点，特别是鉴于最近人类颅缝早闭研究中的细胞因子和基因研究结果。
