| The Nuss technique for Jeune asphyxiating thoracic dystrophy repair in siblings. | |
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MedLine Citation:
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PMID: 20606585 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Jeune syndrome, or asphyxiating thoracic dysplasia, is an autosomal recessive osteochondrodysplasia. Four forms of Jeune syndrome have been proposed: lethal, severe, mild, and latent. In the severe form, respiratory failure leads to death in early infancy. We present 2 cases of a mild variant of Jeune syndrome in a 14-year-old girl and her 9-year-old brother, who were referred to us because of characteristic concave deformities of bilateral middle-lower chest walls without cardiopulmonary distress or renal failure. In addition, both showed short statue (-2.1 and -2.5 SD), progressive retinal dystrophy, and metaphyseal dysplasia (cone-shaped metaphysis and metacarpal brachydactyly). The chest wall deformity was treated at the age of 9 years in the sister and at the age of 7 years in the brother. According to the Nuss procedure, 2 bent bars were inserted into the thoracic cavity from each lateral intercostal space at the midaxillary line and pulled out over the lower end of the sternum. The ends of inserted bars were fixed to soft tissue over the sternum with nonabsorbable sutures. Conjoined costal cartilage around the sternum restricts the number of bars that can be positioned. A single bar for deformity of each side could not achieve complete reconstruction, but the patients and their parents were satisfied with the results cosmetically. |
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Authors:
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Noriaki Kikuchi; Hideo Kashiwa; Toshihoko Ogino; Mituhiro Kato; Kiyoshi Hayasaka |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Annals of plastic surgery Volume: 65 ISSN: 1536-3708 ISO Abbreviation: Ann Plast Surg Publication Date: 2010 Aug |
Date Detail:
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Created Date: 2010-08-02 Completed Date: 2010-12-08 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 7805336 Medline TA: Ann Plast Surg Country: United States |
Other Details:
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Languages: eng Pagination: 214-8 Citation Subset: IM |
Affiliation:
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Yamagata University Hospital, Japan. nkikuchi@med.id.yamagata-u.ac.jp |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Abnormalities, Multiple Asphyxia / pathology Child Female Humans Male Reconstructive Surgical Procedures / methods* Siblings Syndrome Thoracic Wall / abnormalities*, surgery* |
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