Complete resolution of papilledema in syndromic craniosynostosis with posterior cranial vault distraction

We report a case of surgical management of Crouzon syndrome with multisuture craniosynostosis presenting with increased intracranial pressure (ICP) manifesting with chronic papilledema without ventriculomegaly. A 12-month-old boy had complete resolution of papilledema after posterior cranial vault distraction followed by staged fronto-orbital advancement. Expansion of the cranial vault with posterior distraction osteogenesis posed an elegant treatment, obviating ventriculoperitoneal shunt placement for cerebrospinal fluid (CSF) diversion. Strategies for the management of elevated ICP without ventriculomegaly in craniosynostosis include CSF shunting and cranial vault expansion. Posterior calvarial vault distraction associated with resolved papilledema has not been previously reported. Addressing the craniocephalic disproportion for this child with chronic papilledema, without ventriculomegaly, allowed the possibility of shunt freedom.