Volume 26, Issue 3 (Avicenna Journal of Clinical Medicine-Autumn 2019)                   Avicenna J Clin Med 2019, 26(3): 188-191 | Back to browse issues page

XML Persian Abstract Print

Download citation:
BibTeX | RIS | EndNote | Medlars | ProCite | Reference Manager | RefWorks
Send citation to:

Kahramfar Z, Aloosh O. Spontaneous Hemothorax in a Patient with Neurofibromatosis Type 1: A Case Report. Avicenna J Clin Med 2019; 26 (3) :188-191
URL: http://sjh.umsha.ac.ir/article-1-1947-en.html
1- , kahram_z@yahoo.com
Abstract:   (5520 Views)
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
Full-Text [PDF 671 kb]   (904 Downloads)    
Type of Study: Case Report | Subject: Lung Diseases

1. Pulivarthi S, Simmons B, Shearen J, Gurram MK. Spontaneous hemothorax associated with neurofibromatosis type I: A review of the literature. J Neurosci Rural Pract. 2014;5(3):269-71. PMID: 25002768 DOI: 10.4103/0976-3147.133592
2. Teitelbaum GP, Hurvitz RJ, Esrig BC. Hemothorax in type I neurofibromatosis. Ann Thorac Surg. 1998;66(2):569-71. PMID: 9725414 DOI: 10.1016/s0003-4975(98)00513-x
3. Kabir T, Asmat A, Aneez DB. Massive spontaneous hemothorax in neurofibromatosis type 1. Chest. 2016;150(4):63A. DOI: 10.1016/j.chest.2016.08.070
4. Conlon NP, Redmond KC, Celi LA. Spontaneous hemothorax in a patient with neurofibromatosis type 1 and undiagnosed pheochromocytoma. Ann Thorac Surg. 2007;84(3):1021-3. PMID: 17720427 DOI: 10.1016/j.athoracsur.2007.04.024
5. Miyamoto E, Hamakawa H, Sakanoue I, Takahashi Y. Surgical intervention for spontaneous hemothorax associated with neurofibromatosis type I. Eur Respiratory J. 2013;42:4350.
6. Miyazaki T, Tsuchiya T, Tagawa T, Yamasaki N, Nagayasu T. Spontaneous hemothorax associated with von Recklinghausen’s disease: report of a case. Ann Thorac Cardiovasc Surg. 2011;17(3):301-3. PMID: 21697796 DOI: 10.5761/atcs.cr.09.01517

Add your comments about this article : Your username or Email:

Send email to the article author

Rights and permissions
Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

© 2024 CC BY-NC 4.0 | Avicenna Journal of Clinical Medicine

Designed & Developed by : Yektaweb