[Early treatment of facial-craniostenosis (Crouzon-Apert) (author's transl)].
The possibilities of treatment of the facial deformities related to Apert and Crouzon syndromes were transformed by the total facial advancement osteotomies described by Paul Tessier. These facial osteotomies are possible in children, and there is enough follow-up nom to assure that facial advancement osteotomies in children do not impair significantly the remaining growth possibilities. There are however severe psychological sequalaes in patients operated as children or youngsters, resulting from a deformed appearance during early childhood. We therefore try to give these children an acceptable appearance before entering school, around 3 or 4 years of age. Now in fact, we are trying not only to correct, but to prevent these deformities by early age release of the synostoses. The synostoses are usually involving the coronal suture and the cranial base. The cranial base being out of safe reach, we have started in 1976 to perform a 2 cm advancement of the forehead, including the supra-orbital rim. At first, we were using a lateral tenon-mortise fixation. We have tried the extended coronal craniectomy advocated by McCarthy and Coll., but since July 1977, we are using the original floating forehead principle: the advanced forehead is wired back only to the face, malar bones and root of the nose, leaving behind and extended craniectomy. Thus, the rapidly expanding brain of the infants is able to project anteriorly the forehead and upper part of the face. We have operated on 26 infants with faciocraniostenosis and severe brachycephaly according to these early release principle, the last 11 with the floating forehead approach. The results are very encouraging, the appearance being normal or subnormal after a few months. Even if an upper maxillary osteotomy is necessary later, we feel that an early improvement of these children is of high benefit to them. The improvement can also be functional, the psychological tests and the intracranial pressure measures showing that an early release is benefitial even in the absence of obvious intracranial hypertension.
D002675:Child, Preschool; D003394:Craniofacial Dysostosis / Q000601:surgery*; D003398:Craniosynostoses / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008722:Methods; D010027:Osteotomy; D012886:Skull / Q000601:surgery
Traitement précoce des facio-craniosténoses (Crouzon-Apert).