From the Division of Plastic and Reconstructive Surgery, Yale School of Medicine; Division of Plastic and Reconstructive Surgery, Mayo Clinic Florida; Department of Plastic and Reconstructive Surgery, Erasmus Medical Center; Division of Plastic and Reconstructive Surgery, New York Medical College; Division of Plastic Surgery, University of Connecticut School of Medicine; and Department of Plastic Surgery, University of São Paulo.
Plast Reconstr Surg. 2021 Jul 1;148(1):145-156. doi: 10.1097/PRS.0000000000008101.
Little is known about the detailed growth of the cranial fossae, even though they provide an important structural connection between the cranial vault and the facial skeleton. This study details the morphologic development of isolated cranial vault synostosis and associated syndromes on cranial fossa development.
A total of 125 computed tomographic scans were included (nonsyndromic bicoronal synostosis, n = 36; Apert syndrome associated with bicoronal synostosis, n = 24; Crouzon syndrome associated with bicoronal synostosis, n = 11; and controls, n = 54). Three-dimensional analyses were produced using Materialise software.
The regional anterior and middle cranial fossae volumes of nonsyndromic bicoronal synostosis are characterized by significant increases of 43 percent (p < 0.001) and 60 percent (p < 0.001), respectively, and normal posterior cranial fossa volume. The cranial fossae depths of nonsyndromic bicoronal synostosis were increased, by 37, 42, and 21 percent (all p < 0.001) for anterior, middle, and posterior cranial fossae, respectively, accompanying the shortened cranial fossae lengths. The volume and morphology of all cranial fossae in Apert syndrome nearly paralleled nonsyndromic bicoronal synostosis. However, Crouzon syndrome had reduced depths of cranial fossae, and more restricted fossa volumes than both Apert syndrome and nonsyndromic bicoronal synostosis.
Cranial vault suture synostosis is likely to be more influential on cranial fossae development than other associated influences (genetic, morphologic) in Apert and Crouzon syndromes. Isolated Apert syndrome pathogenesis is associated with an elongation of the anterior cranial fossa length in infants, whereas in Crouzon syndrome, there is a tendency to reduce cranial fossa depth, suggesting individual adaptability in cranial fossae development related to vault synostosis.
尽管颅腔为颅顶和面颅骨之间提供了重要的结构连接，但对于颅腔的详细生长过程却知之甚少。本研究详细描述了孤立性颅盖骨缝早闭及其相关综合征对颅腔发育的影响。
共纳入 125 例头颅 CT 扫描（非综合征性矢状缝早闭 36 例，Apert 综合征合并矢状缝早闭 24 例，Crouzon 综合征合并矢状缝早闭 11 例，对照组 54 例）。使用 Materialise 软件进行三维分析。
非综合征性矢状缝早闭患者的颅前窝和颅中窝的区域性体积分别显著增加 43%（p<0.001）和 60%（p<0.001），而颅后窝体积正常。非综合征性矢状缝早闭患者的颅窝深度增加，颅前窝、颅中窝和颅后窝分别增加 37%、42%和 21%（均 p<0.001），同时颅窝长度缩短。Apert 综合征患者所有颅窝的体积和形态与非综合征性矢状缝早闭患者相似。然而，Crouzon 综合征患者颅窝深度减小，颅窝容积较 Apert 综合征和非综合征性矢状缝早闭患者更受限制。
在 Apert 和 Crouzon 综合征中，颅盖骨缝早闭对颅腔发育的影响可能大于其他相关影响（遗传、形态）。孤立性 Apert 综合征的发病机制与婴儿期颅前窝长度的延长有关，而在 Crouzon 综合征中，颅窝深度有减小的趋势，这表明与颅盖骨缝早闭相关的颅腔发育存在个体适应性。
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