Management of The Craniofacial Dysostosis Syndromes: Analysis of 45 Consecutive Cases

摘要

Introduction: Management of the craniofa-cial dysostosis CFD syndromes is surgical but effectiveness of reconstruction and the optimal timing of CFD repair have not been well evaluated. Methods: A retrospective study of 256 consecutive patients aged 24 days-9.1 years old (mean 11 months) at surgery with craniosynos-tosis was reviewed between 1979 and 2006. Forty-five CFD were operated on by the same team (sixty surgeries) Results: Crouzon's syndrome was the most common CFD in 13 cases (28.8%). The fronto-orbital advancement was performed in 25 cases (55.5 %) and monobloc advancement with internal distraction in three patients since 2003. Progressive hydrocephalus was observed in 5 patients. Three patients were submitted to posterior fossa decompression (Chiari I malformation). Conclusions: Multiple surgeries may be required for each deformity in different steps. A multidisciplinary approach provides the best outcome for children with CFD.