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Early Posterior Vault Distraction Osteogenesis Changes the Syndromic Craniosynostosis Treatment Paradigm: Impact on Secondary Frontal Procedures and Syndrome-Specific Patterns
Meagan Wu
*, Sarah Barnett, Benjamin Massenburg, Jinggang Ng, Dominic Romeo, Jesse A. Taylor, Scott Bartlett, Jordan W. Swanson
Plastic and Reconstructive Surgery, Children's Hospital of Philadelphia, Philadelphia, PA
Background: Early surgical management of syndromic craniosynostosis varies widely between centers. This study evaluates the long-term impact of early posterior vault distraction osteogenesis (PVDO) versus conventional treatment paradigms on the number and timing of subsequent craniofacial procedures.
Methods: We retrospectively analyzed the operative patterns of patients with syndromic craniosynostosis treated from 2000 to 2023 with greater than five years of follow-up. Outcomes of patients who underwent early PVDO versus conventional vault reconstruction were compared.
Results: Fifty-five patients met inclusion criteria (30 PVDO, 25 conventional). Age at initial vault surgery was similar between the PVDO and conventional cohorts (7.6 vs. 8.8 months), as were baseline craniometrics (
p>0.05). The conventional protocol relied almost exclusively on fronto-orbital advancement (FOA) for initial vault expansion, and half of these patients required secondary or tertiary FOA due to relapse or pseudorelapse. Multiple FOA procedures were performed in only 1/30 (3%) PVDO-treated patient versus 12/25 (48%) conventionally treated patients (
p<0.001). Twelve (40%) PVDO-treated patients did not undergo FOA at all.
Among patients with Apert and Crouzon syndromes, fewer PVDO-treated patients required FOA prior to midface/frontofacial surgery (33% vs. 92%,
p=0.004) or repeat FOA (6% vs. 50%,
p=0.005) compared to conventionally treated patients. Among patients with Muenke and Saethre-Chotzen syndromes, a similar number of patients required FOA in the PVDO and conventional cohorts (91% vs. 100%,
p=0.353), though no PVDO-treated patients required repeat FOA (0% vs. 44%,
p=0.018).
Conclusion: Early PVDO is associated with a reduction in the high burden of revisionary frontal procedures commonly seen in conventionally treated syndromic patients. Importantly, frontal surgery is increasingly performed on an as-needed basis, and can be combined with midface surgery at an older age in many cases as a single-stage frontofacial advancement.
Syndrome-specific operative courses in patients treated with a conventional protocol (top) and PVDO-based protocol (bottom), with node size and flow width representing the relative proportion of patients undergoing that particular care pathway. PVDO, posterior vault distraction osteogenesis; FOA, fronto-orbital advancement; MB, monobloc advancement; LFIII, Le Fort III advancement.
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