Case Report: A rare presentation of vascular Ehlers-Danlos syndrome with a massive hemothorax and a chest wall hematoma
In this case, the patient presented with a massive right-sided hemothorax and a large chest wall hematoma without any obvious precipitating factors, in the absence of other typical clinical manifestations of vEDS, such as gastrointestinal perforation, which increased the diagnostic challenge. Possible pathogenic mechanisms include type III collagen abnormalities leading to increased fragility of the subpleural vessels, triggering vascular rupture. Clinically, young patients with recurrent hemothorax or multiple arterial lesions should be kept on high alert for early genetic testing to clarify the diagnosis and optimize management. This case helps to raise awareness of the heterogeneous clinical manifestations of vEDS and to avoid misdiagnosis and underdiagnosis.
Authors
- Xiaoyu Lu (ORCID: https://orcid.org/0000-0002-1330-5925)
- Li Su (ORCID: https://orcid.org/0000-0001-6789-8618)
- Beisi Han
- Heng Zhang (ORCID: https://orcid.org/0000-0001-9287-2428)
- Y J Ye
Institutions
- Jinan University (CN)
- Southern University of Science and Technology (CN)
- Southern Medical University Shenzhen Hospital (CN)
- Shenzhen Second People's Hospital (CN)
Publication Details
- Journal
- Frontiers in Medicine
- Published
- 2025-08-08
- DOI
- https://doi.org/10.3389/fmed.2025.1648439
- Primary Topic
- Connective tissue disorders research
- Type
- article
- Field-Weighted Citation Impact
- 0.00