Document Detail

Posterior vault distraction with midface distraction without osteotomy as a first stage for syndromic craniosynostosis.
MedLine Citation:
PMID:  23851785     Owner:  NLM     Status:  In-Data-Review    
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.
Jesse A Goldstein; James Thomas Paliga; Robert L Bailey; Gregory G Heuer; Jesse A Taylor
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The Journal of craniofacial surgery     Volume:  24     ISSN:  1536-3732     ISO Abbreviation:  J Craniofac Surg     Publication Date:  2013 Jul 
Date Detail:
Created Date:  2013-07-15     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9010410     Medline TA:  J Craniofac Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1263-7     Citation Subset:  D    
From the *Department of Plastic Surgery, Children's Hospital of Philadelphia, †Department of Neurosurgery, The Perelman School of Medicine at the University of Pennsylvania, and ‡The Children's Hospital of Philadelphia, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
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