Retroperitoneal fibrosis masquerading as pelvi-ureteric junction obstruction - Laparoscopic management

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.

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.

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.

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.