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Anterior tibial artery rupture treated using covered stent in a patient with vascular Ehlers-Danlos syndrome
Takashi Miwa1, Satoshi Ikeda2, Takahiro Muroya1,3
1Department of Cardiology, Ureshino Medical Center, Saga, Japan.
Insights
Vascular Ehlers-Danlos syndrome (vEDS) causes arterial rupture. Endovascular repair with covered stents offers a promising treatment for rare lower limb artery ruptures in vEDS patients.
Area of Science:
- Vascular Surgery
- Genetics
- Connective Tissue Diseases
Background:
- Vascular Ehlers-Danlos syndrome (vEDS) is a rare genetic disorder caused by COL3A1 mutations.
- It leads to systemic vascular fragility and a high risk of arterial rupture, a major cause of mortality.
- Treatment for vascular complications in vEDS remains challenging due to persistent vessel fragility.
Observation:
- Rupture or pseudoaneurysm of infrapopliteal arteries is exceptionally rare in vEDS patients.
- A case of a 38-year-old woman with vEDS presenting with widespread anterior tibial artery rupture is described.
- The patient experienced sudden, extensive rupture of the anterior tibial artery.
Findings:
- The patient was successfully treated with endovascular reconstruction using covered stents.
- She remained free of vascular events for two years post-surgery with an uneventful recovery.
- Endovascular repair with covered stents demonstrated efficacy in this rare presentation.
Implications:
- Covered stent repair may be a viable alternative for managing ruptured arteries in the lower extremities of vEDS patients.
- This approach might avoid disrupting lower extremity blood flow compared to traditional methods.
- Further research is warranted to establish definitive treatment guidelines for infrapopliteal artery complications in vEDS.
Abstract:
Vascular Ehlers-Danlos syndrome (vEDS) is a rare degenerative connective tissue disease caused by a mutation of the COL3A1 gene that results in systemic vascular fragility. Arterial rupture is a potentially fatal serious complication that is the most commonly reported cause of death among patients with this disease, as ruptured vessels remain fragile even after surgical or endovascular reconstruction. Therefore, treatment for vascular complications in patients with vEDS remains controversial. Rupture or pseudoaneurysm of the infrapopliteal artery is extremely rare. We describe a 38-year-old woman with vEDS who presented with sudden widespread rupture of the anterior tibial artery. She was treated by endovascular reconstruction using covered stents. She has remained free of vascular events for two years after surgery, and the course has been uneventful. Endovascular reconstruction using covered stents might offer an alternative for relatively small ruptured arteries and avoid disturbing blood flow in the lower extremities of patients with vEDS. <Learning objective: Vascular Ehlers-Danlos syndrome (vEDS) causes systemic vascular fragility that results in arterial rupture. However, rupture of the arteries in the lower extremities is rare, and definitive treatment has not been established. Endovascular reconstruction with covered stents might offer an alternative treatment for ruptured arteries in the lower extremities of patients with vEDS.>.
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