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Systemic Multiple Aneurysms Caused by Vascular Ehlers-Danlos Syndrome
Xinyu Gui1, Fangda Li1, Lingeer Wu1
1Department of Vascular Surgery, Peking Union Medical College Hospital, Beijing, China.
Vascular and Endovascular Surgery
|May 22, 2016
Summary
Systemic multiple aneurysms are rare, often linked to connective tissue disorders. This case highlights acute hemorrhage in vascular Ehlers-Danlos syndrome, emphasizing arterial fragility.
Area of Science:
- Vascular Surgery
- Genetics
- Cardiology
Background:
- Systemic multiple aneurysms are uncommon, frequently associated with connective tissue disorders like Ehlers-Danlos syndrome (EDS) and Marfan syndrome.
- Vascular EDS, a subtype of EDS, is characterized by arterial and organ rupture due to connective tissue abnormalities.
Observation:
- A 39-year-old male presented with systemic multiple aneurysms and acute intraperitoneal hemorrhage.
- Clinical diagnosis was vascular Ehlers-Danlos syndrome, indicating extreme arterial fragility.
Findings:
- Coil embolization of the distal common hepatic artery was successfully performed to manage the hemorrhage.
- The intervention effectively resolved the patient's acute symptoms.
Implications:
- This case underscores the importance of recognizing vascular EDS in patients presenting with multiple aneurysms and bleeding.
- Increased clinical awareness can lead to timely diagnosis and management, potentially preventing life-threatening complications associated with arterial fragility.
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