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Staged Strategy in Craniofrontonasal Dysplasia: Endoscopic Fronto-Orbital Distraction to Preserve Planes for Future
Allison C Hu1, Philip D Tolley, Jordan W Swanson
1Division of Plastic, Reconstructive, and Oral Surgery, Children's Hospital of Philadelphia, Pennsylvania, PA.
Abstract:
Craniofrontonasal dysplasia (CFND) is a rare X-linked craniofacial malformation that manifests with midline defects such as hypertelorism, coronal craniosynostosis, and extracranial skeletal anomalies. The standard timeline for surgical management typically includes early cranial vault remodeling and later orbital hypertelorism correction, most commonly through orbital box osteotomies at mixed dentition. For infants presenting with unicoronal craniosynostosis (UCS), fronto-orbital advancement (FOAR) is often used to correct asymmetry and allow for cranial expansion. However, in patients with known or expected need for future hypertelorism correction, the authors argue that traditional FOAR imposes a significant scar burden and disruption to surgical planes that may complicate future reconstruction. As an alternative, the authors propose endoscopic-assisted fronto-orbital distraction osteogenesis for those patients presenting with UCS and CFND.
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