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

Institutions

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
Controls
|||
ALL TIME
JAN
FEB
MAR
APR
MAY
JUN
JUL
AUG
SEP
OCT
article

Case Report: A rare presentation of vascular Ehlers-Danlos syndrome with a massive hemothorax and a chest wall hematoma

Xiaoyu Lu, Li Su, Beisi Han, Heng Zhang et al.
Frontiers in Medicine
Connective tissue disorders research
article

Case Report: A rare presentation of vascular Ehlers-Danlos syndrome with a massive hemothorax and a chest wall hematoma

Xiaoyu Lu, Li Su, Beisi Han, Heng Zhang, Y J Ye
article en

Abstract

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.

Frontiers in MedicineVol. 12
Jinan University (CN), Southern University of Science and Technology (CN), Southern Medical University Shenzhen Hospital (CN), Shenzhen Second People's Hospital (CN)
Good health and well-being
Openalex Percentile: Top 77%
Connective tissue disorders research
AI Navigator

Ask Laika to Summarize, Analyze, and Connect papers live on the map.

Summarize Papers & Methodologies

Extract key findings, datasets, and comparative methods across publications.

Benchmark Rankings & Visual Analytics

Rank top research institutions, authors, funders, topics, and journals by Field-Weighted Citation Impact (FWCI) and paper volume with instant charts.

Connect Distant Disciplines

Bridge topological clusters on the map to find hidden collaborative intersections.