ISSN : 1301-5680
e-ISSN : 2149-8156
Turkish Journal of Thoracic and Cardiovascular Surgery     
A new technique for neonatal Jeune syndrome: External thoracic expansion
Uğur Temel1, Aslı Gül Akgül1
1Department of Thoracic Surgery, University of Medical Sciences, Şişli Etfal Training and Research Hospital, Istanbul, Turkey
DOI : 10.5606/tgkdc.dergisi.2021.19731
Jeune syndrome is a rare form of skeletal dysplasia characterized by a narrow, bell-shaped chest (thoracic cage), and typical phalangeal and pelvic bone deformities. Chest expansion is impaired by the short, horizontally positioned ribs, resulting in alveolar hypoventilation and eventually neonatal-infantile death in most cases. External distraction with sternoplasty is a new technique for the treatment of Jeune syndrome, which was firstly used by our team on a newborn by placing a sliding finger fixator which was designed for ulnar lengthening. We believe that this approach can be life-saving in neonates with improved and widespread usage.
Keywords : Jeune syndrome, sternoplasty, thoracic expansion
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