Spontaneous Hemothorax in a Patient with Neurofibromatosis Type 1: A Case Report
Background: Neurofibromatosis type1 (NF-1) is a hereditary autosomal dominant disease that is accompanied by complications, such as benign and malignant tumors and vascular involvement, including pulmonary hypertension, artery stenosis, and pulmonary artery aneurysm. Spontaneous hemothorax is a rare...
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| Format: | Article |
| Language: | fas |
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Hamadan University of Medical Sciences
2019-12-01
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| Series: | پزشکی بالینی ابن سینا |
| Subjects: | |
| Online Access: | http://sjh.umsha.ac.ir/article-1-1947-en.html |
| _version_ | 1818192547109928960 |
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| author | Zohreh Kahramfar Oldooz Aloosh |
| author_facet | Zohreh Kahramfar Oldooz Aloosh |
| author_sort | Zohreh Kahramfar |
| collection | DOAJ |
| description | Background: Neurofibromatosis type1 (NF-1) is a hereditary autosomal dominant disease that is accompanied by complications, such as benign and malignant tumors and vascular involvement, including pulmonary hypertension, artery stenosis, and pulmonary artery aneurysm. Spontaneous hemothorax is a rare and lethal complication of NF-1 due to vasculopathy as stenosis or aneurysmal modifications of large intrathoracic vessels, and dysplastic alterations of small vessels in highly vascularized mesenchymal tumors. Most commonly, tumor-related hemothorax is due to neurofibroma, and the most frequently involved artery is the intercostal artery.
Case Presentation: A 48-year-old male referred to our hospital with a chief complaint of dyspnea from a week ago. His symptoms began with pleuritic chest pain followed by dyspnea. The physical examination showed many neurofibromatosis lesions and café-au-lait macules on the skin. Thoracotomy was performed during the hemothorax examination. Moreover, inflammation of the pleura and fibrothorax were reported without any site of bleeding.
Conclusion: Vascular complications should be regarded in NF-1. Any patient presents with hemodynamic disorder or pleural effusion should be examined and treated promptly |
| first_indexed | 2024-12-12T00:32:14Z |
| format | Article |
| id | doaj.art-aabba3566e00407d8f2f69a007cd90a1 |
| institution | Directory Open Access Journal |
| issn | 2588-722X 2588-7238 |
| language | fas |
| last_indexed | 2024-12-12T00:32:14Z |
| publishDate | 2019-12-01 |
| publisher | Hamadan University of Medical Sciences |
| record_format | Article |
| series | پزشکی بالینی ابن سینا |
| spelling | doaj.art-aabba3566e00407d8f2f69a007cd90a12022-12-22T00:44:27ZfasHamadan University of Medical Sciencesپزشکی بالینی ابن سینا2588-722X2588-72382019-12-01263188191Spontaneous Hemothorax in a Patient with Neurofibromatosis Type 1: A Case ReportZohreh Kahramfar0Oldooz Aloosh1 Background: Neurofibromatosis type1 (NF-1) is a hereditary autosomal dominant disease that is accompanied by complications, such as benign and malignant tumors and vascular involvement, including pulmonary hypertension, artery stenosis, and pulmonary artery aneurysm. Spontaneous hemothorax is a rare and lethal complication of NF-1 due to vasculopathy as stenosis or aneurysmal modifications of large intrathoracic vessels, and dysplastic alterations of small vessels in highly vascularized mesenchymal tumors. Most commonly, tumor-related hemothorax is due to neurofibroma, and the most frequently involved artery is the intercostal artery. Case Presentation: A 48-year-old male referred to our hospital with a chief complaint of dyspnea from a week ago. His symptoms began with pleuritic chest pain followed by dyspnea. The physical examination showed many neurofibromatosis lesions and café-au-lait macules on the skin. Thoracotomy was performed during the hemothorax examination. Moreover, inflammation of the pleura and fibrothorax were reported without any site of bleeding. Conclusion: Vascular complications should be regarded in NF-1. Any patient presents with hemodynamic disorder or pleural effusion should be examined and treated promptlyhttp://sjh.umsha.ac.ir/article-1-1947-en.htmlhemothoraxneurofibromatosis type 1 |
| spellingShingle | Zohreh Kahramfar Oldooz Aloosh Spontaneous Hemothorax in a Patient with Neurofibromatosis Type 1: A Case Report پزشکی بالینی ابن سینا hemothorax neurofibromatosis type 1 |
| title | Spontaneous Hemothorax in a Patient with Neurofibromatosis Type 1: A Case Report |
| title_full | Spontaneous Hemothorax in a Patient with Neurofibromatosis Type 1: A Case Report |
| title_fullStr | Spontaneous Hemothorax in a Patient with Neurofibromatosis Type 1: A Case Report |
| title_full_unstemmed | Spontaneous Hemothorax in a Patient with Neurofibromatosis Type 1: A Case Report |
| title_short | Spontaneous Hemothorax in a Patient with Neurofibromatosis Type 1: A Case Report |
| title_sort | spontaneous hemothorax in a patient with neurofibromatosis type 1 a case report |
| topic | hemothorax neurofibromatosis type 1 |
| url | http://sjh.umsha.ac.ir/article-1-1947-en.html |
| work_keys_str_mv | AT zohrehkahramfar spontaneoushemothoraxinapatientwithneurofibromatosistype1acasereport AT oldoozaloosh spontaneoushemothoraxinapatientwithneurofibromatosistype1acasereport |