Idiopathic retroperitoneal fibrosis presenting as recurrent scrotal edema : report of a case and review of the literature

Primary or idiopathic retroperitoneal fibrosis, first described by Ormond in 1948, is a rare and elusive diagnosis, requiring a high level of suspicion. Patients usually present with entrapment of retroperitoneal organs, the most common being the ureters, causing hydronephrosis and acute kidney injury. Here, we present the case of a 56-year-old male presenting for recurrent and intermittent scrotal edema. Upon routine laboratory workup, he was found to have an elevated creatinine level. Imaging showed encasement of bilateral ureters. Bilateral ureteral stents were placed with relief of his obstructive uropathy, followed by normalization of creatinine. The patient later underwent laparoscopic release of retroperitoneal adhesions. Biopsies taken from the operative site showed fibroblast proliferation and elements of acute and chronic inflammation. With further workup of etiologies being negative, he was diagnosed with idiopathic retroperitoneal fibrosis. Primary or idiopathic retroperitoneal fibrosis, first described by Ormond in 1948, is a rare and elusive diagnosis, requiring a high level of suspicion. Patients usually present with entrapment of retroperitoneal organs, the most common being the ureters, causing hydronephrosis and acute kidney injury. Here, we present the case of a 56-year-old male presenting for recurrent and intermittent scrotal edema. Upon routine laboratory workup, he was found to have an elevated creatinine level. Imaging showed encasement of bilateral ureters. Bilateral ureteral stents were placed with relief of his obstructive uropathy, followed by normalization of creatinine. The patient later underwent laparoscopic release of retroperitoneal adhesions. Biopsies taken from the operative site showed fibroblast proliferation and elements of acute and chronic inflammation. With further workup of etiologies being negative, he was diagnosed with idiopathic retroperitoneal fibrosis.