Early radical treatment of pancraniofacial synostosis

Premature fusion of all major cranial and facial sutures, termed pancraniofacial synostosis by us, was first described as "Kleeblattschädel deformity," but can also be found in anomalies such as Crouzon's disease, Apert's syndrome, Pfeiffer's syndrome, and severe forms of plagiocephaly. This rare craniofacial anomaly often presents an immediate threat to life right after birth. Early subtotal craniectomy frequently combined with frontoorbitofacial advancement must be performed to decrease intracranial pressure, preserve vision, and open up the blocked upper airway. Concomitant hydrocephaly is shunted preoperatively for internal decompression. Eleven patients with pancraniofacial synostosis underwent craniofacial surgery in infancy or early childhood. Only shunting of the hydrocephalus was done in 1 child. Two children died in the course of an emergency procedure and 2 children died after secondary operations for miniplate removal and revision of a valve shunting the hydrocephalus several months after the initial successful craniofacial surgery. The remaining 8 children have developed satisfactorily so far. Further corrective surgeries had to be performed in 2 patients. Good functional and aesthetic improvement could be obtained. In view of the high mortality especially after emergency procedures, we believe that early total craniofacial mobilization should be reserved for severe cases where the natural course is dismal without immediate intervention and temporary procedures such as shunting and tracheostomy are not sufficient. Elective surgeries after the age of 3 months can be performed safely in milder cases.