Two-Stage Strategy: FOA and Le Fort III
摘要
Apert syndrome is a rare craniosynostosis syndrome caused by fibroblast growth factor receptor 2 (FGFR2) mutations and characterized by complex craniofacial anomalies, including turribrachycephaly, midface hypoplasia, and airway obstruction. This chapter outlines a multidisciplinary, staged surgical protocol for managing craniofacial abnormalities in patients with Apert syndrome, focusing on NYU’s institutional approach to midface advancement. Treatment begins in infancy with interventions to relieve intracranial pressure and protect the eyes, including posterior vault distraction osteogenesis (PVDO) and fronto-orbital advancement (FOA). Midface hypoplasia is addressed during mixed dentition, typically between ages 7 and 9, using subcranial Le Fort III distraction osteogenesis. A detailed surgical technique for Le Fort III osteotomy and distraction is provided, emphasizing anatomical considerations, vector planning, and hardware placement. This chapter highlights the importance of individualized care informed by functional needs such as airway compromise or ocular protection. Advantages of subcranial Le Fort III over monobloc advancement include improved safety and aesthetic outcomes. Overcorrection is routinely pursued to accommodate facial growth and prevent phenotypic relapse. A collaborative team including neurosurgery, otolaryngology, ophthalmology, orthodontics, and psychology is critical to optimizing outcomes across the patient’s lifespan. The described algorithm balances surgical precision with long-term functional and aesthetic goals for comprehensive care of patients with Apert syndrome.