[Craniosynostosis and faciocraniosynostosis]

Abstract

The authors present a review of the aetiopathogenesis and treatment, based on a series of 1321 craniostenoses operated by the Enfants Malades team. After briefly reviewing the embryology of craniofacial growth, the authors describe the morphological classification of craniostenoses and their morphological and functional consequences. The main neurosurgical problems related to craniofacial surgery are described. The surgical techniques currently used by the unit are described for each type of craniostenosis, according to age: H or flap transposition craniectomies for scaphocephaly, unilateral advancement of a bilateral head-band for plagiocephaly, anterior transposition for oxycephaly, and fronto-orbital adbancement for brachycephaly. The results are presented with a follow-up of several years. The principles of one-stage or two-stage surgical treatment for the main types of faciocraniostenosis are recalled: initial fronto-orbital advancement then secondary treatment of maxillary recession. The prospects of one-stage combined treatment with early maxillary distraction are proposed. Surgical indications are described. The complications, morbidity and mortality are indicated for the series. It must be remembered that craniostenosis surgery is a form of plastic surgery with neurosurgical complications. To obtain optimal results with the lowest risk, craniofacial must be performed by multidisciplinary teams in specialized centres.