Open AccessA new technique for neonatal Jeune syndrome: External thoracic expansion
Author(s) -
Uğur Temel,
Aslı Gül Akgül
Publication year - 2021
Publication title -
türk göğüs kalp damar cerrahisi dergisi :/türk göğüs kalp damar cerrahisi dergisi
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.149
H-Index - 10
eISSN - 2149-8156
pISSN - 1301-5680
DOI - 10.5606/tgkdc.dergisi.2021.19731
Subject(s) - medicine , rib cage , flail chest , pectus excavatum , distraction , dysplasia , external fixator , surgery , distraction osteogenesis , anatomy , neuroscience , biology
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.