D16. An Evaluation of Soft-tissue Procedures in the Long-term Management of Patients with Syndromic Craniosynostosis

PURPOSE: Patients with syndromic craniosynostosis often undergo a spectrum of secondary soft-tissue procedures (STP) to normalize facial appearance. This study characterizes the secondary STPs required for patients with different syndromic craniosynostoses. METHODS: A retrospective review was conducted of patients with syndromic craniosynostosis who received care at a tertiary children's hospital between 2004-2023. Descriptive statistics, chi-squared, and independent samples t-tests were conducted to analyze differences in STPs amongst different craniofacial syndromes. RESULTS: One-hundred and three syndromic craniosynostosis patients were included. Diagnoses included Apert (n=39, 37.9%), Crouzon (n=32, 31.1%), Pfieffer (n=16, 15.5%), Saethre-Chotzen (n=10, 9.7%), Muenke (n=5, 4.9%), and Carpenter syndromes (n=1, 1.0%). Fifty-five patients (53.4%) underwent at least one STP, averaging 2.7±2.0 procedures per patient. Apert syndrome patients underwent more lateral canthopexies (48.7%) compared to Crouzon (25.0%, p=0.041), Saethre-Chotzen (20.0%, p=0.102), and Pfeiffer syndrome (12.5%, p=0.012). Crouzon syndrome patients demonstrated higher rates of septorhinoplasty (21.9%) compared to Pfeiffer (9.1%; p=0.347) and Apert syndrome (5.1%; p=0.035). Two medial canthopexies were performed, both in Crouzon syndrome patients. CONCLUSION: Patients with Apert, Crouzon, Saethre Chotzen, and Pfeiffer syndrome demonstrated high rates of STPs. Many patients with syndromic craniosynostosis deteriorate in adulthood despite successful initial correction, possibly due to premature aging. To ensure adequate correction, STPs are a common and valuable adjunct to bony reconstruction in patients with syndromic craniosynostosis.