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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...

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Autores principales: Mundama, Meni, Ayong, Serge, Rossillon, Renaud
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2018
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.
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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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