Retroperitoneal fibrosis masquerading as pelvi-ureteric junction obstruction – Laparoscopic management. (January 2022)

• Record Type: Journal Article
• Authors: Chawda, Vishal; Joshi, Abhijit; Shetty, Prakashchandra
• Is Part Of: International journal of surgery case reports. Volume 90(2022)
• Publication Date: 2022-01
• Subjects: Retroperitoneal fibrosis -- Obstructive uropathy -- Pelvi - ureteric junction obstruction -- Computed tomography -- Magnetic resonance imaging -- Surveillance; Surgery -- Periodicals; Surgical Procedures, Operative -- Periodicals; Surgery; Electronic journals; Periodicals; 617.005
• DOI: 10.1016/j.ijscr.2021.106652
• Languages: English
• ISSNs: 2210-2612

Abstract

Abstract: Introduction: Retroperitoneal fibrosis (RPF) is a rare fibro-inflammatory condition which is characterized by development of extensive fibrosis throughout the retroperitoneum. It is classically centred over the anterior surface of the fourth and fifth lumbar vertebrae. It results in entrapment and extrinsic compression of retroperitoneal structures. Presentation of the case: We present the case of a 69 years old man who was reported to have right pelvi - ureteric junction obstruction on computed tomography, but turned out to have RPF. Discussion: Retroperitoneal fibrosis commonly causes obstructive uropathy (either unilateral, bilateral or progressing from unilateral to bilateral) and if untreated, renal failure. It has high response/remission rates to glucocorticoid therapy. However, relapse rates are also high. Hence, close surveillance with serial laboratory and imaging investigations, after achieving remission, is key to long term disease control. Conclusion: Although classical imaging findings, supportive laboratory markers and suggestive/diagnostic histopathology appearances for RPF are well documented, its accurate preoperative diagnosis is not always an assured certainty. Highlights: This case is unique in that the original plan to perform a laparoscopic pyeloplasty, purportedly for right pelvi-ureteric junction obstruction; was aborted 'on table' and a laparoscopic ureterolysis performed, instead. Though ureteral involvement is bilateral in most cases of retroperitoneal fibrosis, this case is rare and unique in that the patient had unilateral involvement of his ureter (right sided). Periaortic fibro-inflammatory mass at the level of the lumbar vertebrae is the hallmark of primary / idiopathic retroperitoneal fibrosis. However in this case, no such imaging finding was noted. Instead, the patient had just obvious unilateral (ride sided) disease on pre-operative imaging.