20-YEAR EXPERIENCE WITH EARLY SURGERY FOR CRANIOSYNOSTOSIS .2. THE CRANIOFACIAL SYNOSTOSIS SYNDROMES AND PANSYNOSTOSIS - RESULTS AND UNSOLVED PROBLEMS

As the second of a two-part series, 76 patients with pansynostosis and craniofacial synostosis syndromes were retrospectively analyzed. Diag noses included pansynostosis (7), craniofrontonasal dysplasia (8), and Apert (24), Crouzon (15), and Pfeiffer (15) syndromes. All patients u nderwent primary fronto-orbital advancement-calvarial vault remodeling procedures at less than 18 months of age (mean 6.1 months). Twenty-ei ght patients (36.8 percent) required a secondary cranial vault operati on (mean age 28.4 months). Additionally, a major tertiary procedure wa s necessary in 5 patients to deal with persistent unacceptable craniof acial form. To address the associated finding of midface hypoplasia, 6 4.8 percent (n = 35) of patients underwent Le Fort III midface advance ment or had that procedure recommended for them. The remainder were aw aiting appropriate age for this reconstruction. The more extensive pat hologic involvement of the pansynostosis and craniofacial syndrome gro up is illustrated. As compared with the isolated craniofacial synostos is group previously reported, the incidence of major secondary procedu res (36.8 versus 13.5 percent), perioperative complications (11.3 vers us 5.0 percent), follow-up complications (44.7 versus 7.7 percent), hy drocephalus (42.1 versus 3.9 percent), shunt placement (22.4 versus 1. 0 percent), and seizures (11.8 versus 2.9 percent) was significantly i ncreased. Complex problems including those of increased intracranial p ressure, airway obstruction, and recurrent turricephaly or cranial vau lt maldevelopment are repeatedly encountered. In addition, that early frontoorbital advancement-cranial vault remodeling failed to promote m idface development and hypoplasia of this region is almost a consisten t finding in the craniofacial syndromic group. The average length of p ostoperative follow-up was 6 years. According to the classification of Whitaker et al., which assesses surgical results, 73.7 percent of pat ients were considered to have at least satisfactory craniofacial form (category I-II) at latest evaluation. An algorithmic approach to the t reatment of all patients with craniosynostosis is presented utilizing early surgical intervention as the key element.