March 3, 2026Open Access

Vascular Ehlers Danlos Syndrome with Ruptured Thoracic Aortic Aneurysm Complicated by Vascular Graft and Endograft Infection and Aorto-Bronchial Fistula

Puntos clave

Recurrent pseudoaneurysms followed the initial treatment for ruptured aortic aneurysm, leading to a fatal outcome.
Genetic testing identified a pathogenic COL3A1 mutation, confirming a diagnosis of vascular Ehlers-Danlos syndrome.
This observational analysis details a case managed with TEVAR, open interventions, and prolonged antibiotic therapy.
Combining vascular graft infection with aortic hereditary disease poses significant treatment challenges and risks.

Resumen

ABSTRACT The combination of vascular graft or endograft infection (VGEI) and aortic hereditary disease (AHD) is extremely rare and just as challenging. We report a 40-year-old man presenting with a ruptured saccular aortic isthmus aneurysm treated with TEVAR, complicated by VGEI with an aorto-bronchial fistula, and multiple pseudoaneurysms. Despite complete graft explantation, prolonged antibiotic therapy, and additional endovascular and open interventions, he developed recurrent pseudoaneurysms with ultimately a fatal outcome. Genetic testing revealed a pathogenic COL3A1 mutation consistent with vascular Ehlers–Danlos syndrome. This case illustrates the diagnostic and therapeutic dilemma at the intersection of infection and AHD, underscoring the need for multidisciplinary management in complex aortic pathology.

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Cite This Study

Riahi et al. (Sun,) studied this question.

synapsesocial.com/papers/69a76828badf0bb9e87e3ca4 https://doi.org/https://doi.org/10.1016/j.jvscit.2026.102169

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