CTICasesHughes-Stovin Syndrome
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Imaging findings
The patient exhibits a very large pulmonary artery aneurysm, accompanied by other smaller pulmonary artery aneurysms and areas of thrombus formation. Notably, there is thrombosis of the right inferior pulmonary vein and extensive iliac and deep venous thrombosis. Associated regions of hemorrhage or edema are present, likely secondary to venous outflow obstruction.
Key takeaways
Hughes-Stovin Syndrome is a rare clinical entity characterized by the triad of multiple pulmonary artery aneurysms, pulmonary venous thrombosis, and systemic deep venous thrombosis (often involving the inferior vena cava, but not exclusively). It is considered a variant of Behçet's disease without the classic mucocutaneous manifestations. Patients are at high risk for aneurysm rupture.
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