Management of Retroperitoneal Fibrosis with Endovascular Aneurysm Repair in Patients Refractory to Medical Management

Abstract

Early diagnosis and treatment of under-recognized retroperitoneal fibrosis (RPF) are essential before reaching the poorly responsive fibrotic stage. Although most patients respond to medical therapy, relapses are common. Open surgery in medically resistant patients is associated with major adverse clinical events. Therefore, we aim to compare the outcomes of endovascular repair in patients with RPF who were refractory to the optimal medical management. This is a single-center longitudinal study of optimal medical therapy (OMT) vs endovascular aneurysm repair (EVAR) in patients with RPF to our tertiary referral vascular center. Seventeen patients were referred to us with peri-aortic RPF over the study period. Eleven received OMT only, whereas six underwent EVAR after the failure of OMT. Eighty-two percent (n = 14) were male, and the mean follow-up was 74.40 ± 58.40 months. Nine (52%) had immunoglobulin G4-related disease (4 OMT vs 5 EVAR). EVAR patients had 100% technical success without perioperative mortality. Furthermore, all the EVAR patients were symptom-free following the intervention. Pre-operative aortic RPF index (maximum peri-aortic soft tissue diameter/maximum aortic diameter) was higher in the EVAR than in the OMT group. However, there was a significant decrement in the aortic RPF index following EVAR (P = .04). EVAR in RPF medical-resistant patients is a valuable option in the armamentarium of nephrologists and rheumatologists. Our study shows that an endovascular intervention provides a promising outcome in RPF/IgG4-RD periaortitis, which is refractory to medical therapy. We recommend further studies to investigate and establish the long-term effectiveness of endovascular repair in RPF.