[Plagiocephaly. Craniofacial asymmetry with variable curling-up]

Abstract

The plagiocephaly syndrome is an asymmetrical cranial deformity due to premature synostosis of a coronal hemi-suture. The predominant alteration is at the spheno-frontal suture. The classification is attempted between the isolated plagiocephaly (P) an plagiocephaly with hypertelorism, on one side, that are to be distinguished from complex asymmetrical cranio-facial deformities. The frontal P. are differentiated from occipital P (pachycephaly) and from mixed hemi-cranial P. The deformity affects the fronto-orbital region situated anterior to the synostosis. The coronal suture incurves around a pivot formed by the lateral orbital pillar and the pterion, giving rise to a set of facial and cranial deformities, variable according to the precocity and the topography of the synostosis. Investigating the syndrome is clinical and radiological. The deformity reveals itself in 88% of cases, the affects are mainly orbital and ophthalmologic. The authors insist on the necessity of a bilateral orbito-fronto-nasal osteotomy, as a therapeutic act that gives better harmony on short and long term; on the other hand, they draw the attention to the importance of abolishing the synostocic lock by the resection of the lesser wing of the sphenoid extended till the superior orbital fissure and the resection of the outher periosteal layer of the dura matter which allows a remodeling effect the growing brain. The results and complications are evaluated on 44 operated children.