Udayakumaran Suhas, Nerurkar Shibani, V Vinanthi P, Krishnadas Arjun, Subash Pramod
Division of Paediatric Neurosurgery and Craniofacial Surgery, Amrita Institute of Medical Sciences and Research Centre, Amrita Vishwa Vidyapeetham, Kochi-41, Kochi, Kerala, India.
Department of Craniomaxillofacial Surgery, Amrita Institute of Medical Sciences and Research Centre, Amrita Vishwa Vidyapeetham, Kochi-41, Kochi, Kerala, India.
Childs Nerv Syst. 2025 May 13;41(1):178. doi: 10.1007/s00381-025-06832-2.
The critical nature of normal intracranial pressure (ICP) and proper airway function in early brain development, particularly in the first 5 years, are well established. These elements are compromised in faciocraniosynostoses and potentially affect neurological growth. The impact of variations in ICP and airway on cognitive development remains underappreciated and challenging to quantify.
The primary aim of this study was to assess the efficacy and safety of a comprehensive early surgical intervention institutional protocol in children (0-5 years) with syndromic faciocraniosynostosis.
We retrospectively analyzed our comprehensive surgical protocol in children who consecutively underwent cranial vault and midface procedures for syndromic craniosynostosis between July 2015 and January 2024, focusing on those who underwent both calvarial and midface surgeries before the age of 5 years.
Our treatment algorithm prioritized posterior calvarial distraction (PCVD) as the initial intervention for children with faciocraniosynostoses. Frontal orbital advancement and remodeling (FOAR) is preferred to address aesthetic concerns and ICP in older children. Midface distraction was performed for clinically significant airway compromise.
The cohort included 31 children with a mean age of 18.8 months at the initial surgery. The primary procedures included PCVD (n = 16), robotic-assisted frontofacial advancement (RAFFA) (n = 5), robotic-assisted midface distraction (RAMD) (n = 6), and FOAR (n = 4). The second procedure (n = 25) included RAFFA (n = 5), RAMD (n = 13), and FOAR (n = 7). The third procedure (n = 11) included RAMD (n = 1), FOAR (n = 9), and repeat PCVD (n = 1). The mean age of the patients at the final surgery was 57.5 months.
This comprehensive strategy addresses both cranial and midface issues and the challenge of optimizing cognitive development within the critical developmental window.
正常颅内压（ICP）和正常气道功能在早期脑发育中，尤其是在最初5年的关键性质已得到充分证实。这些因素在面颅缝早闭中会受到损害，并可能影响神经发育。ICP和气道变化对认知发育的影响仍未得到充分认识，且难以量化。
本研究的主要目的是评估一种针对患有综合征性面颅缝早闭的儿童（0至5岁）的综合性早期手术干预机构方案的有效性和安全性。
我们回顾性分析了2015年7月至2024年1月期间连续接受颅骨穹窿和中面部手术治疗综合征性颅缝早闭的儿童的综合手术方案，重点关注5岁前接受颅骨和中面部手术的儿童。
我们的治疗算法将后颅骨牵张术（PCVD）作为面颅缝早闭儿童的初始干预措施。对于年龄较大的儿童，为解决美观问题和ICP问题，更倾向于采用额眶前移和重塑术（FOAR）。当中面部出现严重气道受损时，则进行中面部牵张术。
该队列包括31名儿童，初次手术时的平均年龄为18.8个月。主要手术包括PCVD（n = 16）、机器人辅助额面部前移术（RAFFA）（n = 5）、机器人辅助中面部牵张术（RAMD）（n = 6）和FOAR（n = 4）。二次手术（n = 25）包括RAFFA（n = 5）、RAMD（n = 13）和FOAR（n = 7）。三次手术（n = 11）包括RAMD（n = 1）、FOAR（n = 9）和重复PCVD（n = 1）。最后一次手术时患者的平均年龄为57.5个月。
这种综合策略解决了颅骨和中面部问题，以及在关键发育窗口期内优化认知发育的挑战。
