Department of Pediatric Neurosurgery, Hôpital Femme Mère Enfant, INSERM 1033, Université de Lyon, Centre de Reference Malformations Craniofaciales, Lyon, France.
Department of Neurosurgery, Boston Children's Hospital/Harvard Medical School, 300 Longwood Ave, Boston, MA, 02459, USA.
Childs Nerv Syst. 2023 Oct;39(10):2779-2787. doi: 10.1007/s00381-023-06113-w. Epub 2023 Aug 16.
Very few clinical entities have undergone so many different treatment approaches over such a short period of time as craniosynostosis. Surgical treatments for this condition have ranged from simple linear craniectomies, accounting for the specific role of cranial sutures in assuring the normal growth of the skull, to more complex cranial vault reconstructions, based on the perceived role of the skull base in affecting the growth of the skull. While a great deal of evolution has occurred, there remains controversy regarding the ideal treatment including the best surgical technique, the optimal age for surgery, and the long-term morphological and neurodevelopmental outcomes. The evolution of the surgical management of craniosynostosis in the last 50 years has been affected by several factors. This includes the awareness of needing to operate on affected children during infancy to achieve the best results, the use of multistage operations, the availability of more sophisticated surgical tools, and improved perioperative care. In some forms of craniosynostosis, the operations can be carried out at a very young age with low morbidity, and with the postoperative use of a molding helmet, springs, or distractors, these operations prove to be as effective as traditional larger cranial reconstructions performed in older children. As a consequence, complex surgical operations have become progressively less utilized. A second relevant advance was the more recent advent of a molecular diagnosis, which allowed us to understand the pathogenesis of some associated malformations and neurodevelopmental issues that were observed in some children despite appropriate surgical treatment. Future research should focus on improving the analysis of longer-term outcomes and understanding the natural history of craniofacial conditions, including what issues persist despite optimal surgical correction. Progress in molecular investigations concerning the normal and pathological development of cranial sutures could be a further significant step in the management of craniosynostosis, possibly favoring a "medical" treatment in the near future. Artificial intelligence will likely have a role in establishing the diagnosis with less reliance on radiographic studies and in assisting with surgical planning. Overall, much progress has been made, but there remains much to do.
在如此短的时间内，很少有临床实体像颅缝早闭那样经历了如此多不同的治疗方法。这种情况的手术治疗方法范围从简单的线性颅骨切开术，解释颅缝在确保颅骨正常生长中的特定作用，到更复杂的颅盖重建，基于颅底在影响颅骨生长中的作用的感知。虽然已经发生了很大的演变，但对于理想的治疗方法仍存在争议，包括最佳手术技术、手术的最佳年龄以及长期的形态和神经发育结果。过去 50 年来，颅缝早闭的手术治疗方法的演变受到了几个因素的影响。这包括需要在婴儿期对受影响的儿童进行手术以获得最佳结果的意识、使用多阶段手术、更复杂的手术工具的可用性以及围手术期护理的改善。在某些形式的颅缝早闭中，可以在非常年幼的年龄进行手术，并且具有较低的发病率，并且在手术后使用塑形头盔、弹簧或牵引器，这些手术被证明与在年龄较大的儿童中进行的传统更大的颅骨重建一样有效。因此，复杂的手术操作已逐渐减少。第二个相关的进展是最近出现了分子诊断，这使我们能够理解一些相关畸形和神经发育问题的发病机制，尽管进行了适当的手术治疗，但这些问题仍在一些儿童中观察到。未来的研究应侧重于改善对长期结果的分析并了解颅面状况的自然史，包括尽管进行了最佳手术矫正但仍存在的问题。关于颅骨缝正常和病理发育的分子研究的进展可能是颅缝早闭管理的又一重要步骤，可能在不久的将来有利于“医学”治疗。人工智能可能在建立诊断方面发挥作用，减少对放射学研究的依赖，并协助手术计划。总的来说，已经取得了很大的进展，但仍有很多工作要做。
