RESUMO
Premature synostosis of all major cranial sutures represents a rare craniofacial anomaly often leading to serious consequences. It is regularly associated with Kleeblattschädel-syndrome (clover-leaf skull) but can also be observed in Crouzon's craniofacial dysostosis, Apert's acrocephalosyndactyly, Pfeiffer's-syndrome and severe plagiocephaly. Since the disease not only affects the cranial vault but also the facial skeleton, we simply termed it pancraniofacial synostosis (PCS). Radical surgical therapy is frequently necessary after birth for vital indication. This study presents results and complications of 12 children with PCS. Eleven children were operated on by subtotal craniectomy and advancement of the fronto-orbito-facial skeleton. Concomitant hydrocephalus was shunted preoperatively. Four children with impending loss of vision and intracranial hypertension had to be operated on under emergency conditions only a few weeks after birth. One child with normal intracranial pressure has been closely followed for over ten years without surgery. Lethal complications occurred in three of four children that had been operated on under emergency conditions. Craniofacial surgery significantly improved intracranial hypertension, exorbitism, nasopharyngeal obstruction, and aesthetic appearance in all of the remaining children. Total craniofacial correction in PCS can safely be achieved at an age of three to twelve months. Emergency surgery carries a much higher risk. Simultaneous mid-face advancement should be avoided in those cases if at all possible.
