CTICasesEhlers-Danlos Syndrome Type IV
Imaging findings
CT chest shows a massive right-sided extrapleural hemothorax/hematoma. CT angiography shows large aneurysms of the right brachiocephalic and mesenteric arteries.
Key takeaways
Vascular Ehlers-Danlos syndrome (type IV) is a rare, life-threatening genetic disorder of collagen synthesis. It predisposes to spontaneous arterial dissection, aneurysm formation, and rupture. Surgical or interventional procedures are avoided due to extreme tissue and vascular fragility.
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