Disappearing multiple visceral aneurysms in Vascular Ehlers-Danlos syndrome.

ABSTRACT

OBJECTIVE: To assess the complex management of arterial anomalies in Vascular Ehlers-Danlos Syndrome (vEDS). METHODS: We report the case of a 34-year-old male, diagnosed with vEDS, who presented with acute intraperitoneal hemorrhage caused by the rupture of a splenic artery aneurysm, treated in emergency with coil embolization and splenectomy. Computed Tomography (CT) scan showed the concomitant presence of right renal artery (RRA) and common hepatic artery (CHA) aneurysms. RESULTS: Both aneurysms were conservatively managed and the patient went through serial CT imaging. After 3 months, rapid regression of the vascular abnormalities led to complete disappearing of RRA and CHA aneurysms, confirmed at 24-month imaging follow-up. In the same time span, two pseudoaneurysms developed in other sites used for transarterial access, requiring two secondary interventions. The present case emphasizes the unpredictability of disease's evolution and arterial complications in vEDS. Conservative management of complex lesions such as visceral artery aneurysms, which in this case resulted to be the best strategy, avoided the risks associated with surgical intervention in such fragile tissues. The reported complications underline that operative indications should be carefully weighed in these patients.