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Formation of a traumatic air cyst and ensuing hemopneumothorax during CT angiography in a patient with Ehlers-Danlos syndrome

Ehlers-Danlos syndrome (EDS) refers to a rare group of genetic disorders that makeup part of the connective tissue disorders consortium. It is characterized by clinical features such as skin hyperextensibility, joint hypermobility, and tissue fragility. A vascular subtype (EDS IV) exists, that predi...

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Autores principales: Michell, Hans, Chopra, Prajna, Bhave, Anant, Ali, Naiim, Parkinson, William, Shields, Joseph, Scriver, Geoffrey, Morris, Christopher
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The British Institute of Radiology. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7709068/
https://www.ncbi.nlm.nih.gov/pubmed/33299598
http://dx.doi.org/10.1259/bjrcr.20200082
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author Michell, Hans
Chopra, Prajna
Bhave, Anant
Ali, Naiim
Parkinson, William
Shields, Joseph
Scriver, Geoffrey
Morris, Christopher
author_facet Michell, Hans
Chopra, Prajna
Bhave, Anant
Ali, Naiim
Parkinson, William
Shields, Joseph
Scriver, Geoffrey
Morris, Christopher
author_sort Michell, Hans
collection PubMed
description Ehlers-Danlos syndrome (EDS) refers to a rare group of genetic disorders that makeup part of the connective tissue disorders consortium. It is characterized by clinical features such as skin hyperextensibility, joint hypermobility, and tissue fragility. A vascular subtype (EDS IV) exists, that predisposes affected patients to vascular injury and is well-known and documented. However, other manifestations of EDS IV are less commonly understood and reported. Though spontaneous pneumothorax has been described in several cases, formation of traumatic air cysts/pneumatoceles with little to no inciting factors has not. This can eventually lead to pulmonary hemorrhage or hemopneumothorax. We present a case of spontaneous formation of a traumatic air cyst with ensuing large-volume hemopneumothorax occurring in a time period of under 3 minutes, between pre- and post-contrast-media administration during CT angiography of the chest.
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spelling pubmed-77090682020-12-08 Formation of a traumatic air cyst and ensuing hemopneumothorax during CT angiography in a patient with Ehlers-Danlos syndrome Michell, Hans Chopra, Prajna Bhave, Anant Ali, Naiim Parkinson, William Shields, Joseph Scriver, Geoffrey Morris, Christopher BJR Case Rep Case Report Ehlers-Danlos syndrome (EDS) refers to a rare group of genetic disorders that makeup part of the connective tissue disorders consortium. It is characterized by clinical features such as skin hyperextensibility, joint hypermobility, and tissue fragility. A vascular subtype (EDS IV) exists, that predisposes affected patients to vascular injury and is well-known and documented. However, other manifestations of EDS IV are less commonly understood and reported. Though spontaneous pneumothorax has been described in several cases, formation of traumatic air cysts/pneumatoceles with little to no inciting factors has not. This can eventually lead to pulmonary hemorrhage or hemopneumothorax. We present a case of spontaneous formation of a traumatic air cyst with ensuing large-volume hemopneumothorax occurring in a time period of under 3 minutes, between pre- and post-contrast-media administration during CT angiography of the chest. The British Institute of Radiology. 2020-07-29 /pmc/articles/PMC7709068/ /pubmed/33299598 http://dx.doi.org/10.1259/bjrcr.20200082 Text en © 2020 The Authors. Published by the British Institute of Radiology This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International License (https://creativecommons.org/licenses/by/4.0/) , which permits unrestricted use, distribution and reproduction in any medium, provided the original author and source are credited.
spellingShingle Case Report
Michell, Hans
Chopra, Prajna
Bhave, Anant
Ali, Naiim
Parkinson, William
Shields, Joseph
Scriver, Geoffrey
Morris, Christopher
Formation of a traumatic air cyst and ensuing hemopneumothorax during CT angiography in a patient with Ehlers-Danlos syndrome
title Formation of a traumatic air cyst and ensuing hemopneumothorax during CT angiography in a patient with Ehlers-Danlos syndrome
title_full Formation of a traumatic air cyst and ensuing hemopneumothorax during CT angiography in a patient with Ehlers-Danlos syndrome
title_fullStr Formation of a traumatic air cyst and ensuing hemopneumothorax during CT angiography in a patient with Ehlers-Danlos syndrome
title_full_unstemmed Formation of a traumatic air cyst and ensuing hemopneumothorax during CT angiography in a patient with Ehlers-Danlos syndrome
title_short Formation of a traumatic air cyst and ensuing hemopneumothorax during CT angiography in a patient with Ehlers-Danlos syndrome
title_sort formation of a traumatic air cyst and ensuing hemopneumothorax during ct angiography in a patient with ehlers-danlos syndrome
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7709068/
https://www.ncbi.nlm.nih.gov/pubmed/33299598
http://dx.doi.org/10.1259/bjrcr.20200082
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