Posterior vault distraction with midface distraction without osteotomy as a first stage for syndromic...

Syndromic craniosynostosis (SC) may cause intracranial hypertension, exorbitism, midfacial hypoplasia associated with airway obstruction, and class III malocclusion. Current treatment strategies for SC involve expansion of the cranial vault and orbits, and midfacial advancement to relieve symptoms and sequelae of obstructive sleep apnea. We present a case of a 12-week-old female patient with Saethre-Chotzen syndrome who presented with bicoronal craniosynostosis, severe turribrachycephaly, midface hypoplasia, and a history of obstructive sleep apnea (apnea-hypopnea index = 14). Posterior vault distraction and simultaneous transfacial pin placement under 3D CT surgical navigation was planned to concurrently relieve intracranial pressure and address airway obstruction by distraction of the midface without osteotomy. An uncomplicated surgery was completed in 290 minutes with an estimated blood loss of 340 mL. Midface distraction was initiated on postoperative day 1 at a rate of 1 mm per day for 21 days, which translated to 13 mm of midfacial advancement. Posterior vault distraction was initiated on postoperative day 5 at a rate of 1 mm per day for 25 days with a resultant 25 mm of posterior vault expansion. Transfacial pin and external distractors were removed after 6 weeks, and posterior vault distractors were removed after 8 weeks of consolidation. Craniometric comparison of 3D-CT scan demonstrated an increase in cranial vault volume of 47%, and the patient was able to be weaned from her home oxygen requirement. This is the first report of simultaneous posterior vault distraction with midfacial advancement across open facial sutures using surgical navigation as an initial stage in treating syndromic craniosynostosis.