A new technique for neonatal Jeune syndrome: External thoracic expansion

Uğur Temel

doi:10.5606/tgkdc.dergisi.2021.19731

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

Viewed : 1846

Downloaded : 687