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.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.12816/0002627 | DOI Listing |
Cureus
November 2024
Department of Internal Medicine/Rheumatology, Texas Tech University Health Sciences Center Paul L. Foster School of Medicine, El Paso, USA.
Retroperitoneal fibrosis (RPF) is a rare disease with a nonspecific presentation. RPF can be classified into Idiopathic, the most common, or secondary due to malignancy and various medications resulting in chronic inflammation and fibrosis in the retroperitoneum. The complications arise due to the compression of structures in the retroperitoneum.
View Article and Find Full Text PDFRadiol Case Rep
February 2025
Department of Urology Surgery, Hanoi Medical University Hospital, No1, Ton That Tung, Dong Da, Hanoi, Vietnam.
Postoperative chylous ascites is a rare complication following retroperitoneal surgeries, presenting significant challenges in diagnosis and management. Retroperitoneal cyst surgery resulting in chylous leaks is an uncommon complication that has not been previously reported in the literature. Therefore, we report a clinical case of postoperative chylous ascites following retroperitoneal cyst removal with underlying idiopathic thoracic duct obstruction.
View Article and Find Full Text PDFRheumatology (Oxford)
December 2024
Department of Rheumatology and Clinical Immunology, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), The Ministry of Education Key Laboratory, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Objectives: Idiopathic retroperitoneal fibrosis (IRF) is a rare autoimmune-mediated condition characterized by fibro-inflammatory tissue development around the abdominal aorta and iliac arteries. Ureteral entrapment and hydronephrosis are the most common manifestations and acute renal failure or chronic renal insufficiency may occur as the consequence. Glucocorticoids and immunosuppressants may be effective but the therapeutic response and outcome are heterogeneous and hard to predict.
View Article and Find Full Text PDFBackground: Idiopathic retroperitoneal fibrosis is characterized by the development of inflammatory infiltrates with marked fibrosis along the large retroperitoneal vessels. Rituximab in combination with glucocorticoids constitute an effective therapy, but the responses are not long-lasting. In other similar situations, addition of cyclophosphamide to the combination achieved longer and deeper responses.
View Article and Find Full Text PDFWorld J Urol
November 2024
Department of Urology, University of Leipzig, Leipzig, Germany.
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!