Twenty-year experience with early surgery for craniosynostosis: I. Isolated craniofacial synostosis--results and unsolved problems.

Early surgery for isolated craniosynostosis is designed to improve morphology, to prevent functional disturbances, and equally important, to enhance the psychosocial development of the child. As the first of a two-part series, 104 patients with isolated craniofacial synostosis were retrospectively analyzed. Diagnoses included bilateral coronal (10), unilateral coronal (57), metopic (29), and sagittal synostosis (8). All patients underwent primary fronto-orbital advancement-calvarial vault remodeling procedures at less than 18 months of age (mean 8.1 months). Thirteen percent of patients (14) required a secondary cranial vault operation (mean age 22.6 months) to address residual deficits in craniofacial form. Perioperative complications were minimal (5.0 percent), and there was no mortality. Average length of postoperative follow-up was 46.0 months. By the classification of Whitaker et al., which assesses surgical results, 87.5 percent of patients were considered to have at least satisfactory craniofacial form (category I-II) at latest evaluation. Overall rates of hydrocephalus, shunt placement, and seizures (3.8, 1.0, and 2.9 percent, respectively) were low. Among the isolated craniosynostoses, unilateral coronal synostosis/plagiocephaly poses the most complex problems, including vertical orbital dystopia, nasal tip deviation, and residual craniofacial asymmetry; there is also a wide spectrum of findings and growth patterns in this subgroup.