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Mechanical loading of cranial joints minimizes the craniofacial phenotype in Crouzon syndrome
bioRxiv - Biophysics Pub Date : 2022-05-30 , DOI: 10.1101/2021.08.31.458360
Mehran Moazen , Mahbubeh Hejazi , Dawn Savery , Dominic Jones , Arsalan Marghoub , Ali Alazmani , Erwin Pauws

Children with syndromic forms of craniosynostosis undergo a plethora of surgical interventions to resolve the clinical features caused by the premature fusion of cranial sutures. While surgical correction is reliable, the need for repeated rounds of invasive treatment puts a heavy burden on the child and their family. This study explores a non-surgical alternative using mechanical loading of the cranial joints to prevent or delay craniofacial phenotypes associated with Crouzon syndrome. We treated Crouzon syndrome mice before the onset of craniosynostosis by cyclical mechanical loading of cranial joints using a custom designed set-up. Cranial loading applied to the frontal bone partially restores normal skull morphology, significantly reducing the typical brachycephalic appearance. This is underpinned by the delayed closure of the coronal suture and of the intersphenoidal synchondrosis. This study provides a novel treatment alternative for syndromic craniosynostosis which has the potential to be an important step towards replacing, reducing or refining the surgical treatment of all craniosynostosis patients.

中文翻译:

颅关节的机械负荷使 Crouzon 综合征的颅面表型最小化

患有综合征型颅缝早闭的儿童接受了大量的手术干预,以解决颅缝过早融合引起的临床特征。虽然手术矫正是可靠的,但需要反复进行几轮侵入性治疗,这给孩子及其家人带来了沉重的负担。本研究探索了一种非手术替代方法,使用颅关节的机械负荷来预防或延迟与 Crouzon 综合征相关的颅面表型。我们使用定制设计的装置通过颅关节的周期性机械负荷在颅缝早闭发作前治疗 Crouzon 综合征小鼠。施加于额骨的颅负荷部分恢复了正常的颅骨形态,显着减少了典型的短头颅外观。冠状缝和蝶骨间软骨的延迟闭合支持了这一点。这项研究为综合征性颅缝早闭提供了一种新的治疗选择,它有可能成为替代、减少或改进所有颅缝早闭患者的手术治疗的重要一步。
更新日期:2022-06-01
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