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Syndrome de poland: à propos d’un cas et revue de la litterature

Poland syndrome is a rare congenital malformation associated with various degrees of thoracic and homolateral upper limb abnormalities. We report the case of a 7-year old girl who underwent exploration for depression of the left hemithorax associated with homolateral subclavicular mass. CT scan show...

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Autores principales: Benzalim, Meriam, Berghalout, Laila, Elfakir, Sophia, Jalal, Hicham
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The African Field Epidemiology Network 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5398224/
https://www.ncbi.nlm.nih.gov/pubmed/28450991
http://dx.doi.org/10.11604/pamj.2017.26.12.11222
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author Benzalim, Meriam
Berghalout, Laila
Elfakir, Sophia
Jalal, Hicham
author_facet Benzalim, Meriam
Berghalout, Laila
Elfakir, Sophia
Jalal, Hicham
author_sort Benzalim, Meriam
collection PubMed
description Poland syndrome is a rare congenital malformation associated with various degrees of thoracic and homolateral upper limb abnormalities. We report the case of a 7-year old girl who underwent exploration for depression of the left hemithorax associated with homolateral subclavicular mass. CT scan showed that the deformation of the thoracic wall was related to the absence of left pectoralis major muscle sterno-costal heads insertion associated with agenesis of the pectoralis minor muscle and hypoplasia of the anterior arches of the first six corresponding ribs. These abnormalities were related to Poland syndrome. General assessment was based on abdominal ultrasound, X-rays of both hands and did not show any associated malformation. Poland syndrome results from a deficit of blood flow to musculoskeletal elements of the chest wall during fetal life. There are many variants of Poland syndrome that can be best detected by CT scan and which must be performed whenever necessary, without omitting the role of general radiology in the detection of associated malformations. This syndrome is characterized by the agenesis of the sternocostal bundles of the pectoralis major muscle. Its etiology remains unknown and discussed. It can be caused by a vascular anomaly, without the primum movens of this vascular anomaly being known.
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spelling pubmed-53982242017-04-27 Syndrome de poland: à propos d’un cas et revue de la litterature Benzalim, Meriam Berghalout, Laila Elfakir, Sophia Jalal, Hicham Pan Afr Med J Case Report Poland syndrome is a rare congenital malformation associated with various degrees of thoracic and homolateral upper limb abnormalities. We report the case of a 7-year old girl who underwent exploration for depression of the left hemithorax associated with homolateral subclavicular mass. CT scan showed that the deformation of the thoracic wall was related to the absence of left pectoralis major muscle sterno-costal heads insertion associated with agenesis of the pectoralis minor muscle and hypoplasia of the anterior arches of the first six corresponding ribs. These abnormalities were related to Poland syndrome. General assessment was based on abdominal ultrasound, X-rays of both hands and did not show any associated malformation. Poland syndrome results from a deficit of blood flow to musculoskeletal elements of the chest wall during fetal life. There are many variants of Poland syndrome that can be best detected by CT scan and which must be performed whenever necessary, without omitting the role of general radiology in the detection of associated malformations. This syndrome is characterized by the agenesis of the sternocostal bundles of the pectoralis major muscle. Its etiology remains unknown and discussed. It can be caused by a vascular anomaly, without the primum movens of this vascular anomaly being known. The African Field Epidemiology Network 2017-01-05 /pmc/articles/PMC5398224/ /pubmed/28450991 http://dx.doi.org/10.11604/pamj.2017.26.12.11222 Text en © Meriam Benzalim et al. http://creativecommons.org/licenses/by/2.0/ The Pan African Medical Journal - ISSN 1937-8688. This is an Open Access article distributed under the terms of 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
Benzalim, Meriam
Berghalout, Laila
Elfakir, Sophia
Jalal, Hicham
Syndrome de poland: à propos d’un cas et revue de la litterature
title Syndrome de poland: à propos d’un cas et revue de la litterature
title_full Syndrome de poland: à propos d’un cas et revue de la litterature
title_fullStr Syndrome de poland: à propos d’un cas et revue de la litterature
title_full_unstemmed Syndrome de poland: à propos d’un cas et revue de la litterature
title_short Syndrome de poland: à propos d’un cas et revue de la litterature
title_sort syndrome de poland: à propos d’un cas et revue de la litterature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5398224/
https://www.ncbi.nlm.nih.gov/pubmed/28450991
http://dx.doi.org/10.11604/pamj.2017.26.12.11222
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