Document Detail


Freeman-Sheldon Syndrome and Respiratory Obstruction: A Novel Use of Distraction Osteogenesis.
MedLine Citation:
PMID:  24777016     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Freeman-Sheldon syndrome, or distal arthrogryposis type IIA (DA 2A), is a rare and severe multiple congenital contracture syndrome that is associated with upper airway obstruction. This obstruction has been clinically significant enough to warrant tracheostomy and has been associated with mortality. We describe a patient who presented to us as a neonate and the novel management of her respiratory obstruction in the setting of DA 2A. Bilateral mandibular osteotomies were performed and bilateral internal mandibular distracters were placed. She was distracted a total of 3 cm over 15 days without event and successfully extubated on the postoperative day 16. Preoperative polysomnogram demonstrated an obstructive apnea hypopnea index of 43.7, but a repeat polysomnogram demonstrated an apnea hypopnea index of 8.1. In this study, we report the first use of distraction osteogenesis in the setting of severe obstructive sleep apnea syndrome secondary to DA 2A.
Authors:
Jason D Toranto; Sally Davidson Ward; Aaron Lin; Mark M Urata
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-4-25
Journal Detail:
Title:  The Journal of craniofacial surgery     Volume:  -     ISSN:  1536-3732     ISO Abbreviation:  J Craniofac Surg     Publication Date:  2014 Apr 
Date Detail:
Created Date:  2014-4-29     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9010410     Medline TA:  J Craniofac Surg     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
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