A Case of Marfan Syndrome Presenting as Tension Pneumothorax in A Tertiary Care Setup
Marfan Syndrome is a rare connective tissue condition that affects several systems, including musculoskeletal, cardiovascular and ocular systems. Although less frequent, pulmonary involvement can nevertheless lead to emphysema, bullae, apical blebs, and a higher risk of spontaneous pneumothorax. Ano...
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Bahria Univerisy Health Sciences, Campus Karachi
2024-01-01
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| Series: | Journal of Bahria University Medical and Dental College |
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| Online Access: | https://jbumdc.bahria.edu.pk/index.php/ojs/article/view/1357 |
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| author | Tehreem Ahmad Hina Asghar Areeba Hasan |
| author_facet | Tehreem Ahmad Hina Asghar Areeba Hasan |
| author_sort | Tehreem Ahmad |
| collection | DOAJ |
| description | Marfan Syndrome is a rare connective tissue condition that affects several systems, including musculoskeletal, cardiovascular and ocular systems. Although less frequent, pulmonary involvement can nevertheless lead to emphysema, bullae, apical blebs, and a higher risk of spontaneous pneumothorax. Another option is pectus excavatum, carinatum, or scoliosis-related restrictive lung disease. We will discuss a case of 18 year old girl, with marfanoid habitus, non-smoker with history of ATT taken on radiological grounds, with complains of shortness of breath on and off and presenting with sudden onset worsening of shortness of breath and dry cough for 3 weeks. Chest x-ray showed tension pneumothorax. After emergency management echo was done and it showed MVP with MR. Patient was diagnosed as a case of Marfan Syndrome following Ghent criteria. The case indicates that pneumothorax though rare can be first presentation of Marfan Syndrome |
| format | Article |
| id | doaj-art-bc812336405d420aa671f496a7ec4ff8 |
| institution | DOAJ |
| issn | 2220-7562 2617-9482 |
| language | English |
| publishDate | 2024-01-01 |
| publisher | Bahria Univerisy Health Sciences, Campus Karachi |
| record_format | Article |
| series | Journal of Bahria University Medical and Dental College |
| spelling | doaj-art-bc812336405d420aa671f496a7ec4ff82025-08-20T03:10:57ZengBahria Univerisy Health Sciences, Campus KarachiJournal of Bahria University Medical and Dental College2220-75622617-94822024-01-011401909210.51985/JBUMDC20232081357A Case of Marfan Syndrome Presenting as Tension Pneumothorax in A Tertiary Care SetupTehreem AhmadHina AsgharAreeba HasanMarfan Syndrome is a rare connective tissue condition that affects several systems, including musculoskeletal, cardiovascular and ocular systems. Although less frequent, pulmonary involvement can nevertheless lead to emphysema, bullae, apical blebs, and a higher risk of spontaneous pneumothorax. Another option is pectus excavatum, carinatum, or scoliosis-related restrictive lung disease. We will discuss a case of 18 year old girl, with marfanoid habitus, non-smoker with history of ATT taken on radiological grounds, with complains of shortness of breath on and off and presenting with sudden onset worsening of shortness of breath and dry cough for 3 weeks. Chest x-ray showed tension pneumothorax. After emergency management echo was done and it showed MVP with MR. Patient was diagnosed as a case of Marfan Syndrome following Ghent criteria. The case indicates that pneumothorax though rare can be first presentation of Marfan Syndromehttps://jbumdc.bahria.edu.pk/index.php/ojs/article/view/1357marfan, pneumothorax, mitral valve prolapse, mitral regurgitation |
| spellingShingle | Tehreem Ahmad Hina Asghar Areeba Hasan A Case of Marfan Syndrome Presenting as Tension Pneumothorax in A Tertiary Care Setup Journal of Bahria University Medical and Dental College marfan, pneumothorax, mitral valve prolapse, mitral regurgitation |
| title | A Case of Marfan Syndrome Presenting as Tension Pneumothorax in A Tertiary Care Setup |
| title_full | A Case of Marfan Syndrome Presenting as Tension Pneumothorax in A Tertiary Care Setup |
| title_fullStr | A Case of Marfan Syndrome Presenting as Tension Pneumothorax in A Tertiary Care Setup |
| title_full_unstemmed | A Case of Marfan Syndrome Presenting as Tension Pneumothorax in A Tertiary Care Setup |
| title_short | A Case of Marfan Syndrome Presenting as Tension Pneumothorax in A Tertiary Care Setup |
| title_sort | case of marfan syndrome presenting as tension pneumothorax in a tertiary care setup |
| topic | marfan, pneumothorax, mitral valve prolapse, mitral regurgitation |
| url | https://jbumdc.bahria.edu.pk/index.php/ojs/article/view/1357 |
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