Prophylactic craniofacial surgery

One-stage radical reconstructive surgery for the common craniofacial deformities has become standard procedure in a few medical centers. With increasing experience and expertise, young children and adolescents, as well as a few adults have been greatly benefited. It has long been recognized that both the cranial and facial deformities of Couzon's disease and Apert's syndrome are progressive, having their inception either before birth or in the neonatal period, and evolving to the full blown syndrome during the first years of life. The authors believe that the progressive nature of these anomalies is secondary to a combination of permaturely fused sutures of the calvarium and cranial base (coronal, sphenozygomatic, frontoethmoidal and frontosphenoidal). In an effort to arrest and correct both the cranial and facial deformities as well as obviate the need for future radical surgery the authors have treated several children with Crouzon's disease and Apert's syndrome with suture craniectomy along the base of the skull. Following this procedure, deformity has regressed and ultimate cosmetic improvement has been dramatic. It is unlikely that radical craniofacial surgery will be necessary in this group of patients at any future time. On the basis of this experience, it is believed that the pendulum is now swinging and that in the future, stripping of the appropriate sutures along the base of the skull will limit deformity and make additional surgery unnecessary.