Background:
Ehlers-Danlos type IV primarily affects collagen synthesis in the vasculature, increasing the risk of these patients to have dissection and pseudoaneurysm formation. Due to friable vessels, antiplatelet or anticoagulation has been the treatment of choice. However, newer intravascular surgical devices may be promising for future management.
Case description:
A 24-year-old man with a history of Ehlers-Danlos type IV with multiple vascular and bleeding complications presented after recurrent, unprovoked presyncopal episodes. Patient was found to have dissection of bilateral internal carotid arteries (ICA) and right vertebral artery. Left ICA pseudoaneurysm was found in the proximal cervical segment. Patient was stabilized as an inpatient and discharged with outpatient follow-up with neurointerventional surgery. Follow-up imaging showed growth of the left ICA aneurysm. Patient elected to have pipeline stenting of the left ICA pseudoaneurysm. The procedure was performed without complication. Patient was discharged on dual antiplatelet therapy. At 7-month follow-up appointment, patient noted no neurological deficits. Follow-up digital subtraction angiogram at 7 months documented near-complete resolution of the pseudoaneurysm secondary to pipeline stenting.
Conclusion:
Pipeline stent implantation may be a viable corrective surgical option for patients with connective tissue disorders (specifically Ehlers-Danlos type IV) who present with pseudoaneurysm formation.