[Retroperitoneal fibrosis].

Abstract

B ureteral ob truction due to a n inAammatory retroperitoneal process involving both ureter and t he intervening great vessel. ha been reported by a number of ob ervers in the pa -t 12 year. Ormond, in 1948, reported two pat ients with "sudden, complete anuria due to com pre ion of both ureter by a dense, grayi h-white mat of fibrou connective ti ue." (1) ince this original report , 21 additional instances have been carefully documented in the literature, and a ll of these seem to have imilar clinical manife tation and pathologicfinding '. Raper reported t he larue ·t group of patient in 1956, and gave the process the term "retroperitoneal fibrosi '." (2) Hutch et at. published a compr hensi\'e review of the :ubject and a follow-up tudy on a ll of the pre\"iou Iyreported c-ase , adding two of their own, to bring thc total number reported to 23 (3). The e authors preferred to call t he di 'ea:e " perirenal fasc-iiti s," ince a ll of the inAammatory reaction appeared to be c-onfined " 'ithin the perirenal (Gerota's) fa cia. The ymptom of thi ' condition are ml'iable a nd may con ist of oliguria complete anu ria, or symptom of renal in::;uffic-iency, 'uch a nau 'ea, vomiting weaknes ', or fatigue. The most commonly-ob en 'ed symptom reported is pain in the back or lower abdomen. ince the . ymptomatology is .0 non-specific , the diagno:is is quite diffic-ult to make, and many of these patient ha \'e been fir t in ve tigated by the medical , neurologic-ai , orthopedic or 'urgi c-a l en'ic ' before the urologic a pects became e\'ident. The diagno i is not usually : uspected until bilateral uretera l obstruction is seen on the x-ray of the urinary tract, and later confirmed by biop y of the fibrou retroperitoneal plaque. Pathologically, biop y of this ti ' ue re\"ea l it to be predominantly fibrou in nature with varying amounts of fat and inflammatory cell ; usually lymphocyte,