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/10.12816/0002627DOI Listing

Publication Analysis

Top Keywords

idiopathic retroperitoneal
12
retroperitoneal fibrosis
12
presenting recurrent
8
scrotal edema
8
fibrosis presenting
4
recurrent scrotal
4
edema report
4
report case
4
case review
4
review literature
4

Similar Publications

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 PDF

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 PDF

Factors associated with early clinical remission in patients with idiopathic retroperitoneal fibrosis.

Rheumatology (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 PDF

Background: 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 PDF
Article Synopsis
  • - The study reviews the effectiveness of the omentum in robotic-assisted reconstructive urology surgeries, highlighting its biological benefits and potential applications in complex procedures.
  • - A systematic search of three databases yielded 13 relevant studies, covering various repairs, such as vesicovaginal and rectourethral fistulas, with recurrence rates between 0% and 6.7% and low complication rates.
  • - The authors conclude that using omentum in robotic surgeries could be a safe and efficient option for complicated urological issues, but emphasize the need for more extensive studies to solidify its routine use in the field.
View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!