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-...

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Published inTürk göğüs kalp damar cerrahisi dergisi Vol. 29; no. 2; pp. 279 - 282
Main Authors Temel, Uğur, Gül Akgül, Aslı
Format Journal Article
LanguageEnglish
Published Bayçınar Medical Publishing 26.04.2021
Subjects
Case Report
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Abstract 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.
AbstractList 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.
Author Temel, Uğur
Gül Akgül, Aslı
AuthorAffiliation 1 Department of Thoracic Surgery, University of Medical Sciences, Şişli Etfal Training and Research Hospital, Istanbul, Turkey
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Cites_doi 10.1055/s-2008-1038987
10.1007/s00431-009-0991-3
10.1111/crj.12620
10.1136/adc.44.233.11
10.1016/S0022-3468(86)80073-2
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Copyright Copyright © 2021, Turkish Society of Cardiovascular Surgery 2021 Turkish Society of Cardiovascular Surgery
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Title A new technique for neonatal Jeune syndrome: External thoracic expansion
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