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Management of Patella Dislocation in Say-Barber-Biesecker-Young-Simpson's Syndrome: A Report of Two Cases
Say-Barber-Biesecker-Young-Simpson's syndrome is one of the Ohdo-like syndromes. It is a very rare congenital condition that is commonly defined by its main clinical features that are blepharophimosis, ptosis, mental retardation, and delayed motor development. They are often associated with ske...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Hindawi
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5903308/ https://www.ncbi.nlm.nih.gov/pubmed/29850329 http://dx.doi.org/10.1155/2018/6415713 |
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author | Mundama, Meni Ayong, Serge Rossillon, Renaud |
author_facet | Mundama, Meni Ayong, Serge Rossillon, Renaud |
author_sort | Mundama, Meni |
collection | PubMed |
description | Say-Barber-Biesecker-Young-Simpson's syndrome is one of the Ohdo-like syndromes. It is a very rare congenital condition that is commonly defined by its main clinical features that are blepharophimosis, ptosis, mental retardation, and delayed motor development. They are often associated with skeletal manifestations that are joint laxity, long thumbs and toes, and hypoplastic and/or dislocated patellae. To our knowledge, the available literature does not report any case where attention is drawn to management of skeletal aspect of this specific syndrome, especially surgically. We report 2 cases of SBBYS syndrome with patellar dislocation that we followed for 11 years. One case (with bilateral dislocation) was managed conservatively, and the other (with unilateral dislocation) underwent conservative and surgical treatment. Both had good functional outcome at follow-up. This experience shows that patellar abnormality in this condition can be efficiently addressed conservatively and/or surgically with satisfying results. |
format | Online Article Text |
id | pubmed-5903308 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | Hindawi |
record_format | MEDLINE/PubMed |
spelling | pubmed-59033082018-05-30 Management of Patella Dislocation in Say-Barber-Biesecker-Young-Simpson's Syndrome: A Report of Two Cases Mundama, Meni Ayong, Serge Rossillon, Renaud Case Rep Orthop Case Report Say-Barber-Biesecker-Young-Simpson's syndrome is one of the Ohdo-like syndromes. It is a very rare congenital condition that is commonly defined by its main clinical features that are blepharophimosis, ptosis, mental retardation, and delayed motor development. They are often associated with skeletal manifestations that are joint laxity, long thumbs and toes, and hypoplastic and/or dislocated patellae. To our knowledge, the available literature does not report any case where attention is drawn to management of skeletal aspect of this specific syndrome, especially surgically. We report 2 cases of SBBYS syndrome with patellar dislocation that we followed for 11 years. One case (with bilateral dislocation) was managed conservatively, and the other (with unilateral dislocation) underwent conservative and surgical treatment. Both had good functional outcome at follow-up. This experience shows that patellar abnormality in this condition can be efficiently addressed conservatively and/or surgically with satisfying results. Hindawi 2018-04-03 /pmc/articles/PMC5903308/ /pubmed/29850329 http://dx.doi.org/10.1155/2018/6415713 Text en Copyright © 2018 Meni Mundama et al. http://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Mundama, Meni Ayong, Serge Rossillon, Renaud Management of Patella Dislocation in Say-Barber-Biesecker-Young-Simpson's Syndrome: A Report of Two Cases |
title | Management of Patella Dislocation in Say-Barber-Biesecker-Young-Simpson's Syndrome: A Report of Two Cases |
title_full | Management of Patella Dislocation in Say-Barber-Biesecker-Young-Simpson's Syndrome: A Report of Two Cases |
title_fullStr | Management of Patella Dislocation in Say-Barber-Biesecker-Young-Simpson's Syndrome: A Report of Two Cases |
title_full_unstemmed | Management of Patella Dislocation in Say-Barber-Biesecker-Young-Simpson's Syndrome: A Report of Two Cases |
title_short | Management of Patella Dislocation in Say-Barber-Biesecker-Young-Simpson's Syndrome: A Report of Two Cases |
title_sort | management of patella dislocation in say-barber-biesecker-young-simpson's syndrome: a report of two cases |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5903308/ https://www.ncbi.nlm.nih.gov/pubmed/29850329 http://dx.doi.org/10.1155/2018/6415713 |
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