A new technique for neonatal Jeune syndrome: External thoracic expansion

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.

Neonatal Jeune sendromu için yeni bir teknik: Eksternal torasik ekspansiyon

Jeune sendromu nadir görülen bir iskelet displazisi olup, dar, çan şeklinde toraks (göğüs kafesi) ve falanks ve pelvis kemiklerinde tipik deformiteler ile karakterizedir. Yenidoğan döneminde dar ve yatay yerleşimli kaburgalar nedeni ile akciğer ekspansiyonu sağlanamaz; bu da, birçok olguda alveolar hipoventilasyon ve nihayetinde yenidoğan ve bebek ölümlerine neden olur. Sternoplasti ile eksternal distraksiyon, ulnar uzatma için tasarlanan kaydırılan parmak fiksatör ile ilk kez ekibimiz tarafından bir yenidoğanda kullanılan, Jeune sendromu tedavisinde yeni bir tekniktir. Bu yaklaşımın geliştirilip yaygınlaştırılarak uygulanması ile yenidoğanlarda hayat kurtarıcı olabileceği kanısındayız.

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