Fathoming the scientific paradox of intangibles: protocol reappraisal for optimizing cognitive outcomes in faciocraniosynostosis-an institutional experience.

BACKGROUND

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.

OBJECTIVE

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.

METHODS

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.

SURGICAL PROTOCOL

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.

RESULTS

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.

CONCLUSION

This comprehensive strategy addresses both cranial and midface issues and the challenge of optimizing cognitive development within the critical developmental window.