Endovascular Management of Vascular Complications in Ehlers–Danlos Syndrome Type IV

Abstract

(1) Background: The vascular type of Ehlers–Danlos syndrome (vEDS) is a rare genetic connective tissue disorder caused by pathogenic variants in the COL3A1 gene that result in arterial and organ fragility and premature death. We present five cases of vEDS that highlight the diagnosis and treatment challenges encountered by clinicians with these patients. (2) Case presentations: we present the cases of five patients with vascular complications of vEDS who were successfully managed using endovascular interventions or hybrid techniques at our institution from 2005 to 2022. (3) Conclusions: These data emphasize that a multidisciplinary approach is needed for vEDS patients and that when endovascular or hybrid treatment is performed in a timely manner by a skilled team of interventional radiologists, good results can be achieved. Our report also demonstrates that the prognosis of vEDS patients has improved over the past 20 years with a new prevention program including celiprolol therapy, physical activity adaptation and limitation, and scheduled monitoring by expert clinicians.

Case Report: Hybrid approach as a Rescue Treatment in a patient with vascular Ehlers–Danlos Syndrome

“…Additionally, percutaneous arterial access in vEDS can precipitate multiple bleeding complications such as femoral artery rupture or pseudoaneurysms, especially when large devices are used ( 13 , 14 ). Special attention has already been emphasized to reduce the risk of device-induced or procedure-related vessel damage ( 15 ). In the first operation, as mentioned in literature, felt pledgets were used to anastomose the right femoral artery with the graft following the failure of percutaneous closing device.…”