Thromboembolic renal infarction due to a renal artery aneurysm in a patient with Ehlers-Danlos syndrome type IV.

Type IV Ehlers-Danlos syndrome (EDS) is the only lethal form of EDS, mostly due to vascular complications. We describe a patient suffering from EDS type IV who presented with right thromboembolic renal infarctions due to a right renal artery aneurysm. We preferred observation to anticoagulation therapy because the thrombus in the renal artery aneurysm sealed the pending rupture of the arterial wall. This unusual embolic presentation of EDS exemplifies problematic aspects in the management of these patients.