Case report: A simple and reliable approach for progressive internal distraction of the sternum for Jeune syndrome (asphyxiating thoracic dystrophy): preliminary experience and literature review of surgical techniques
BackgroundDescribed for the first time in 1954, Jeune syndrome (JS), often called asphyxiating thoracic dystrophy, is a congenital musculoskeletal disease characterized by short ribs, a narrow thorax, and small limbs. In this study, we analyzed and presented our preliminary experience with a device...
| Principais autores: | , , , , , , , |
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| Formato: | Artigo |
| Idioma: | English |
| Publicado em: |
Frontiers Media S.A.
2023-09-01
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| coleção: | Frontiers in Pediatrics |
| Assuntos: | |
| Acesso em linha: | https://www.frontiersin.org/articles/10.3389/fped.2023.1253383/full |
| _version_ | 1827805960678146048 |
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| author | Alessandro Inserra Alessandro Inserra Angelo Zarfati Angelo Zarfati Valerio Pardi Arianna Bertocchini Antonella Accinni Ivan Pietro Aloi Cristina Martucci Simone Frediani |
| author_facet | Alessandro Inserra Alessandro Inserra Angelo Zarfati Angelo Zarfati Valerio Pardi Arianna Bertocchini Antonella Accinni Ivan Pietro Aloi Cristina Martucci Simone Frediani |
| author_sort | Alessandro Inserra |
| collection | DOAJ |
| description | BackgroundDescribed for the first time in 1954, Jeune syndrome (JS), often called asphyxiating thoracic dystrophy, is a congenital musculoskeletal disease characterized by short ribs, a narrow thorax, and small limbs. In this study, we analyzed and presented our preliminary experience with a device for progressive internal distraction of the sternum (PIDS) in patients with symptomatic JS. In addition, we reviewed the contemporary English literature on existing surgical techniques for treating children with congenital JS.Material and methodsA retrospective analysis of pediatric patients (<18 years old) treated for symptomatic JS at our tertiary center between 2017 and 2023 was performed.ResultsWe presented two patients with JS who underwent surgery using an internal sternal distractor, a Zurich II Micro Zurich Modular Distractor, placed at the corpus of the sternum among the divided halves.ConclusionsWe obtained promising results regarding the safety and effectiveness of this less-invasive device for PIDS in patients with symptomatic JS. Further studies on long-term outcomes are needed to validate these findings. |
| first_indexed | 2024-03-11T21:33:39Z |
| format | Article |
| id | doaj.art-347c006bb0db428fafa33d5915cd79e9 |
| institution | Directory Open Access Journal |
| issn | 2296-2360 |
| language | English |
| last_indexed | 2024-03-11T21:33:39Z |
| publishDate | 2023-09-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Pediatrics |
| spelling | doaj.art-347c006bb0db428fafa33d5915cd79e92023-09-27T05:15:27ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602023-09-011110.3389/fped.2023.12533831253383Case report: A simple and reliable approach for progressive internal distraction of the sternum for Jeune syndrome (asphyxiating thoracic dystrophy): preliminary experience and literature review of surgical techniquesAlessandro Inserra0Alessandro Inserra1Angelo Zarfati2Angelo Zarfati3Valerio Pardi4Arianna Bertocchini5Antonella Accinni6Ivan Pietro Aloi7Cristina Martucci8Simone Frediani9General and Thoracic Pediatric Surgery Unit, Bambino Gesù Children’s Hospital, IRCCS, Rome, ItalyUniversity of “Tor Vergata”, Rome, ItalyGeneral and Thoracic Pediatric Surgery Unit, Bambino Gesù Children’s Hospital, IRCCS, Rome, ItalyUniversity of “Tor Vergata”, Rome, ItalyGeneral and Thoracic Pediatric Surgery Unit, Bambino Gesù Children’s Hospital, IRCCS, Rome, ItalyGeneral and Thoracic Pediatric Surgery Unit, Bambino Gesù Children’s Hospital, IRCCS, Rome, ItalyGeneral and Thoracic Pediatric Surgery Unit, Bambino Gesù Children’s Hospital, IRCCS, Rome, ItalyGeneral and Thoracic Pediatric Surgery Unit, Bambino Gesù Children’s Hospital, IRCCS, Rome, ItalyGeneral and Thoracic Pediatric Surgery Unit, Bambino Gesù Children’s Hospital, IRCCS, Rome, ItalyGeneral and Thoracic Pediatric Surgery Unit, Bambino Gesù Children’s Hospital, IRCCS, Rome, ItalyBackgroundDescribed for the first time in 1954, Jeune syndrome (JS), often called asphyxiating thoracic dystrophy, is a congenital musculoskeletal disease characterized by short ribs, a narrow thorax, and small limbs. In this study, we analyzed and presented our preliminary experience with a device for progressive internal distraction of the sternum (PIDS) in patients with symptomatic JS. In addition, we reviewed the contemporary English literature on existing surgical techniques for treating children with congenital JS.Material and methodsA retrospective analysis of pediatric patients (<18 years old) treated for symptomatic JS at our tertiary center between 2017 and 2023 was performed.ResultsWe presented two patients with JS who underwent surgery using an internal sternal distractor, a Zurich II Micro Zurich Modular Distractor, placed at the corpus of the sternum among the divided halves.ConclusionsWe obtained promising results regarding the safety and effectiveness of this less-invasive device for PIDS in patients with symptomatic JS. Further studies on long-term outcomes are needed to validate these findings.https://www.frontiersin.org/articles/10.3389/fped.2023.1253383/fullJeune syndromesternal distractorthoracic dystrophythoracic insufficiency syndrome (TIS)children |
| spellingShingle | Alessandro Inserra Alessandro Inserra Angelo Zarfati Angelo Zarfati Valerio Pardi Arianna Bertocchini Antonella Accinni Ivan Pietro Aloi Cristina Martucci Simone Frediani Case report: A simple and reliable approach for progressive internal distraction of the sternum for Jeune syndrome (asphyxiating thoracic dystrophy): preliminary experience and literature review of surgical techniques Frontiers in Pediatrics Jeune syndrome sternal distractor thoracic dystrophy thoracic insufficiency syndrome (TIS) children |
| title | Case report: A simple and reliable approach for progressive internal distraction of the sternum for Jeune syndrome (asphyxiating thoracic dystrophy): preliminary experience and literature review of surgical techniques |
| title_full | Case report: A simple and reliable approach for progressive internal distraction of the sternum for Jeune syndrome (asphyxiating thoracic dystrophy): preliminary experience and literature review of surgical techniques |
| title_fullStr | Case report: A simple and reliable approach for progressive internal distraction of the sternum for Jeune syndrome (asphyxiating thoracic dystrophy): preliminary experience and literature review of surgical techniques |
| title_full_unstemmed | Case report: A simple and reliable approach for progressive internal distraction of the sternum for Jeune syndrome (asphyxiating thoracic dystrophy): preliminary experience and literature review of surgical techniques |
| title_short | Case report: A simple and reliable approach for progressive internal distraction of the sternum for Jeune syndrome (asphyxiating thoracic dystrophy): preliminary experience and literature review of surgical techniques |
| title_sort | case report a simple and reliable approach for progressive internal distraction of the sternum for jeune syndrome asphyxiating thoracic dystrophy preliminary experience and literature review of surgical techniques |
| topic | Jeune syndrome sternal distractor thoracic dystrophy thoracic insufficiency syndrome (TIS) children |
| url | https://www.frontiersin.org/articles/10.3389/fped.2023.1253383/full |
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