Craniofacial Dysostosis: Staging of Reconstruction and Management of the Midface Deformity

Abstract

Crouzon's and Apert's syndromes are the most common of the craniofacial dysostosis syndromes. A team approach is required to achieve effective patient management. The team evaluation begins shortly after birth and follows the patient through infancy, childhood, and adolescence into early adulthood. The role of each team member varies according to the patient's age and individual circumstances. For example, in infancy and early childhood, constant combined reassessment by the pediatrician, neurosurgeon, ophthalmologist, neuroradiologist, and craniofacial surgeon is essential. Later in adolescence, the role of the ophthalmologist, neuroradiologist, and neurosurgeon becomes less important, whereas that of the orthodontist, speech pathologist, maxillofacial surgeon, and psychosocial team becomes more dominant. Major craniofacial centers should be encouraged to develop protocols for patient management and to follow a consistent prospective process of collecting data. Meeting these objectives allows us to learn from the past. During the past decade since the introduction of the modern era of craniofacial surgery by Tessier in 1967, craniomaxillofacial surgery has advanced in many ways: through the use of autogenous cranial bone grafts for onlay or interpositional use; refinements in bone stabilization techniques that include miniplate and microplate and screw fixation rather than direct wires; the reintroduction of creative osteotomies for the management of midface deficiency (monobloc and monobloc bipartition); the development of CT scanning techniques applied to the craniofacial skeleton for both qualitative and quantitative measurement; and the presence of the dedicated craniofacial anesthetist whose meticulous monitoring, airway management, and fluid replacement allows for the safe execution of complex total midface osteotomies. The recognition of the need for a staged surgical approach to the correction of the deformities caused by Apert's and Crouzon's syndromes has clarified reconstructive goals and allowed the surgeon to take advantage of differential craniofacial growth patterns similar to those used to help the patient with congenital cleft lip and palate defects. By continuing to define our rationale for the timing of surgical intervention--for example, using cranial vault surgery in infancy to relieve increased intracranial pressure and papilledema; total midface advancement in childhood to further increase intracranial and orbital volume, improve nasal airflow, occlusion, and body image; and orthognathic surgery in adolescence to improve occlusion, speech, and aesthetics--we can avoid unproductive surgery and select the optimal timing for surgical intervention to maximize long-term functional and aesthetic results.