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Spontaneous Diaphragmatic Rupture in Hypermobile Type Ehlers-Danlos Syndrome

Ehlers-Danlos Syndrome refers to a spectrum of connective tissue disorders that have a variety of clinical manifestations. In this case, we present a spontaneous diaphragmatic rupture in a patient with type III Ehlers-Danlos Syndrome. The patient presented with worsening shortness of breath after fa...

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Detalles Bibliográficos
Autores principales: Amin, Ruchi, Waibel, Brett H.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5530413/
https://www.ncbi.nlm.nih.gov/pubmed/28785503
http://dx.doi.org/10.1155/2017/2081725
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author Amin, Ruchi
Waibel, Brett H.
author_facet Amin, Ruchi
Waibel, Brett H.
author_sort Amin, Ruchi
collection PubMed
description Ehlers-Danlos Syndrome refers to a spectrum of connective tissue disorders that have a variety of clinical manifestations. In this case, we present a spontaneous diaphragmatic rupture in a patient with type III Ehlers-Danlos Syndrome. The patient presented with worsening shortness of breath after failure of medical therapy for a presumed pneumonia. A CT scan was obtained which showed diaphragmatic rupture with splenic herniation which was repaired in the operating room via thoracotomy. It is important to include diaphragmatic rupture in the differential diagnosis for patients with connective tissue disease and acute onset tachypnea and pain, as this complication has the potential for significant morbidity without prompt surgical intervention.
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spelling pubmed-55304132017-08-07 Spontaneous Diaphragmatic Rupture in Hypermobile Type Ehlers-Danlos Syndrome Amin, Ruchi Waibel, Brett H. Case Rep Surg Case Report Ehlers-Danlos Syndrome refers to a spectrum of connective tissue disorders that have a variety of clinical manifestations. In this case, we present a spontaneous diaphragmatic rupture in a patient with type III Ehlers-Danlos Syndrome. The patient presented with worsening shortness of breath after failure of medical therapy for a presumed pneumonia. A CT scan was obtained which showed diaphragmatic rupture with splenic herniation which was repaired in the operating room via thoracotomy. It is important to include diaphragmatic rupture in the differential diagnosis for patients with connective tissue disease and acute onset tachypnea and pain, as this complication has the potential for significant morbidity without prompt surgical intervention. Hindawi 2017 2017-07-13 /pmc/articles/PMC5530413/ /pubmed/28785503 http://dx.doi.org/10.1155/2017/2081725 Text en Copyright © 2017 Ruchi Amin and Brett H. Waibel. https://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
Amin, Ruchi
Waibel, Brett H.
Spontaneous Diaphragmatic Rupture in Hypermobile Type Ehlers-Danlos Syndrome
title Spontaneous Diaphragmatic Rupture in Hypermobile Type Ehlers-Danlos Syndrome
title_full Spontaneous Diaphragmatic Rupture in Hypermobile Type Ehlers-Danlos Syndrome
title_fullStr Spontaneous Diaphragmatic Rupture in Hypermobile Type Ehlers-Danlos Syndrome
title_full_unstemmed Spontaneous Diaphragmatic Rupture in Hypermobile Type Ehlers-Danlos Syndrome
title_short Spontaneous Diaphragmatic Rupture in Hypermobile Type Ehlers-Danlos Syndrome
title_sort spontaneous diaphragmatic rupture in hypermobile type ehlers-danlos syndrome
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5530413/
https://www.ncbi.nlm.nih.gov/pubmed/28785503
http://dx.doi.org/10.1155/2017/2081725
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