Format

Send to

Choose Destination
J Craniofac Surg. 2014 May;25(3):e287-9. doi: 10.1097/SCS.0000000000000717.

Freeman-Sheldon syndrome and respiratory obstruction: a novel use of distraction osteogenesis.

Author information

1
From the *Division of Aesthetic and Plastic Surgery, University of California, Irvine; Divisions of †Pulmonology, ‡Otolaryngology, and §Plastic Surgery, Children's Hospital Los Angeles, Los Angeles, California.

Abstract

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.

PMID:
24777016
DOI:
10.1097/SCS.0000000000000717
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Wolters Kluwer
Loading ...
Support Center