Early radical treatment of pancraniofacial synostosis.

Premature fusion of all major cranial and facial sutures, termed pancraniofacial synostosis by us, was first described as «Kleeblattschbdel 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