What’s New in Syndromic Craniosynostosis Surgery?

Abstract

Crouzon, Apert, Pfeiffer, Muenke, and Saethre-Chotzen syndromes are the five most common forms of syndromic craniosynostosis. Although each has different genetic underpinnings and associated anomalies, their hallmark finding is turribrachycephaly most often associated with bicoronal craniosynostosis. The role of prenatal screening and counseling is growing, with caregivers becoming involved before birth. Multidisciplinary care from birth onward involves craniofacial plastic surgeons, neurosurgeons, otolaryngologists, ophthalmologists, orthodontists, anesthesiologists, psychologists, speech therapists, and geneticists. Early partial, or regional, craniectomy may be urgently indicated in multisuture cases with signs of increased intracranial pressure. Others may be managed successfully with posterior cranial vault distraction, middle vault expansion, or fronto-orbital advancement. Some authors have advocated early monobloc advancement for those patients who require acute airway intervention and globe protection, although the risks of these procedures are high. Many patients will require midfacial advancement with a Le Fort III, Le Fort II plus zygomatic repositioning, monobloc, or facial bipartition. The indications, risks, and benefits for each midfacial procedure must be considered, as this step in the treatment algorithm may carry the greatest functional and aesthetic benefits but also the potential for the greatest morbidity. At the culmination of facial growth, it is not uncommon for patients to require conventional orthognathic surgery and other bony contouring and soft-tissue procedures. Finally, an understanding of the psychological aspects of craniofacial difference, both in affected individuals and in their families, is essential to a successful, holistic approach.