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Swyer-James-MacLeod syndrome presenting as spontaneous pneumothorax in an adult: Case report and review of literature
Swyer-James-MacLeod Syndrome (SJMS) is an uncommon, emphysematous disease characterized by obliteration of the small bronchioles, hypoplasia/or absence of pulmonary artery and peripheral vascular bed. It is most commonly diagnosed in childhood. Patients are often asymptomatic or they could suffer fr...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7943970/ https://www.ncbi.nlm.nih.gov/pubmed/33732407 http://dx.doi.org/10.1016/j.radcr.2021.02.047 |
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author | Conti, Luigi Palmieri, Gerardo Delfanti, Rocco Grassi, Carmine Daccò, Maria Diletta Capelli, Patrizio |
author_facet | Conti, Luigi Palmieri, Gerardo Delfanti, Rocco Grassi, Carmine Daccò, Maria Diletta Capelli, Patrizio |
author_sort | Conti, Luigi |
collection | PubMed |
description | Swyer-James-MacLeod Syndrome (SJMS) is an uncommon, emphysematous disease characterized by obliteration of the small bronchioles, hypoplasia/or absence of pulmonary artery and peripheral vascular bed. It is most commonly diagnosed in childhood. Patients are often asymptomatic or they could suffer from symptoms of recurrent pulmonary infections. Spontaneous pneumothorax is a rare presentation of this syndrome. We report a case of a 42-year old female patient presented at our Emergency Department with complaints of dyspnea and pleuritic chest pain with a diagnosis of spontaneous pneumothorax in emphysematous disease. The diagnosis of SJMS is usually based on imaging and clinical findings rather than on the results of pathologic examination; indeed, asymptomatic adult patients with SJMS are often diagnosed after a chest radiograph obtained for another reason. High-resolution computed tomography (HRCT) seems to be the most appropriate technique for the diagnosis. SJMS can be associated with spontaneous pneumothorax which represent an emergency condition due to the underlying pathological disease. The surgical treatment of the affected lung should be considered when conservative approach is ineffective. |
format | Online Article Text |
id | pubmed-7943970 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Elsevier |
record_format | MEDLINE/PubMed |
spelling | pubmed-79439702021-03-16 Swyer-James-MacLeod syndrome presenting as spontaneous pneumothorax in an adult: Case report and review of literature Conti, Luigi Palmieri, Gerardo Delfanti, Rocco Grassi, Carmine Daccò, Maria Diletta Capelli, Patrizio Radiol Case Rep Case Report Swyer-James-MacLeod Syndrome (SJMS) is an uncommon, emphysematous disease characterized by obliteration of the small bronchioles, hypoplasia/or absence of pulmonary artery and peripheral vascular bed. It is most commonly diagnosed in childhood. Patients are often asymptomatic or they could suffer from symptoms of recurrent pulmonary infections. Spontaneous pneumothorax is a rare presentation of this syndrome. We report a case of a 42-year old female patient presented at our Emergency Department with complaints of dyspnea and pleuritic chest pain with a diagnosis of spontaneous pneumothorax in emphysematous disease. The diagnosis of SJMS is usually based on imaging and clinical findings rather than on the results of pathologic examination; indeed, asymptomatic adult patients with SJMS are often diagnosed after a chest radiograph obtained for another reason. High-resolution computed tomography (HRCT) seems to be the most appropriate technique for the diagnosis. SJMS can be associated with spontaneous pneumothorax which represent an emergency condition due to the underlying pathological disease. The surgical treatment of the affected lung should be considered when conservative approach is ineffective. Elsevier 2021-03-08 /pmc/articles/PMC7943970/ /pubmed/33732407 http://dx.doi.org/10.1016/j.radcr.2021.02.047 Text en © 2021 The Authors. Published by Elsevier Inc. on behalf of University of Washington. http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). |
spellingShingle | Case Report Conti, Luigi Palmieri, Gerardo Delfanti, Rocco Grassi, Carmine Daccò, Maria Diletta Capelli, Patrizio Swyer-James-MacLeod syndrome presenting as spontaneous pneumothorax in an adult: Case report and review of literature |
title | Swyer-James-MacLeod syndrome presenting as spontaneous pneumothorax in an adult: Case report and review of literature |
title_full | Swyer-James-MacLeod syndrome presenting as spontaneous pneumothorax in an adult: Case report and review of literature |
title_fullStr | Swyer-James-MacLeod syndrome presenting as spontaneous pneumothorax in an adult: Case report and review of literature |
title_full_unstemmed | Swyer-James-MacLeod syndrome presenting as spontaneous pneumothorax in an adult: Case report and review of literature |
title_short | Swyer-James-MacLeod syndrome presenting as spontaneous pneumothorax in an adult: Case report and review of literature |
title_sort | swyer-james-macleod syndrome presenting as spontaneous pneumothorax in an adult: case report and review of literature |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7943970/ https://www.ncbi.nlm.nih.gov/pubmed/33732407 http://dx.doi.org/10.1016/j.radcr.2021.02.047 |
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