CASE REPORTS

IDIOPATHIC RETROPERITONEAL FIBROSIS INFILTRATING URETERAL WALL JOHN E. HELLER, M.D. JOHN TEGGATZ, M.D.

From the Division of Urology, Department of Surgery, Hennepin County Medical Center, Minneapolis, Minnesota

ABSTRACT- We describe the eighth case of idiopathic retroperitoneal fibrosis infiltrating the ureter to came intrinsic obstruction. Discovery of this situation intraoperatively necessitates a change in treatment plan, as ureterolysis is not suffcient.

Ormond first described retroperitoneal fibrosis in the English-language literature in 1948.’ Classically, this condition is described as a fibrous mass or plaque enveloping the great vessels at the level of the lower aorta, sometimes extending as high as the renal hilus and as low as the true pelvis. The process does not extend far laterally but, as it progresses, the ureters are drawn medially toward the aorta and become encased and obstructed extrinsically.2 In only 7 previous cases has fibrotic infiltration of the ureteral wall been described.3-5 We herein report the eighth such case, which caused significant intrinsic obstruction.

from 40.9 percent seven months earlier), a white blood cell count of 5,20O/mL, a normal serum acid phosphatase of 0.2 IUIL, and no occult blood in three stool specimens. Results of a tuberculin test were negative. Renal ultrasound showed bilateral hydroureteronephrosis, and retrograde pyelograms demonstrated a 3-cm narrowing on the right at L5-Sl and 2-cm narrowings on the left at L4-L5 and at the level of the mid sacroiliac joint, with dilatation of the ureter and renal pelvis above the stenosis. There was a suggestion of the presence of unseen duplicate upper-pole collecting systems bilaterally (Fig. 1). Only two ureteral orifices were visible cystoscopically. Attempts to insert ureteral stents failed. Renal scans demonstrated poor blood flow and function bilaterally, much worse on the left than on the right. A right percutaneous nephrostomy tube was inserted with a stent passing easily through the stenosis down into the bladder. This tube drained almost all of the urine output. Cytologic examination of urine from the nephrostomy tube and the bladder was negative for malignancy. A computed tomography scan of the abdomen and pelvis revealed a mass encircling the lower abdominal aorta and inferior vena cava and extending inferiorly to the level of the aortic bifurcation consistent with retroperitoneal fibrosis. There were no hepatic, renal, or adrenal masses and no aortic aneurysm. At operation the patient was found to have ureteral duplication bilaterally. A fibrous

Case Report A sixty-eight-year-old man was admitted after a routine chemistry profile showed a serum creatinine of 3.0 mg/dL, increased from 0.6 mg/dL seven months earlier. He denied flank pain. His medical history was significant for chronic obstructive pulmonary disease, previous alcohol abuse, organic brain syndrome with cerebral atrophy and ataxia, and mild Parkinson disease not requiring medical therapy. He had previously undergone right and left inguinal hernia repairs and a hemorrhoidectomy. His only medications were oral theophylline and an albuterol inhaler. He had never taken methysergide. The physical examination was unremarkable. Laboratory studies included an acellular urinalysis, a hematocrit of 33.1 percent (down

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FIGURE 1. (A) Right retrograde pyelogram; (B) left retrograde pyelogram.

plaque surrounded the great vessels, and both ureters on each side deviated medially into the lateral margin of the plaque. Right ureterolysis was easily accomplished, and the right ureters were placed lateral to the ascending colon in an intraperitoneal position. On the left, even after dissection from the fibrous plaque, both the upper- and lower-pole ureters remained stenotic over a 2-cm segment at about L4-L5, and the lower pole ureter became disrupted at this level. Because the ureters were too short for tension-free anastomoses after lateral displace-

ment, and because the kidney had so little function, left nephrectomy was performed. A frozen section of the stenotic ureter had revealed no evidence of malignancy. A biopsy specimen of the retroperitoneal plaque on the right revealed proliferating fibrous tissue and chronic inflammation extending into adipose tissue. On the left, an inflammatory, fibrotic process extended into the wall of the ureter with compression of the lumen eccentrically (Fig. 2). The left kidney specimen contained duplicated dilated renal pelves and

FIGURES. (A)Section of right ureter shows proliferating fibrous tissue extending into wall of ureter as far as mucosa, with eccentrically located, severely compromised lumen. (B) Higher power view demonstrates inflammatory fibrotic process with chronic inflammatory infiltrate of lymphocytes and plasma cells. (Hematoxylin and eosin, reduced from x 40 and x 400, respectively.)

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decreased parenchyma focally infiltrated with chronic inflammatory cells. Postoperatively, this patient’s nephrostomy tube was removed. Excretory urography three months later showed lateral displacement of the two right ureters with no evidence of hydronephrosis. The serum creatinine concentration eleven months postoperatively was 1.4 mg/dL. Comment A computer-assisted review of the literature revealed only 7 previous cases of idiopathic retroperitoneal fibrosis with ureteral invasion. Three of the 7 cases of retroperitoneal fibrosis reported by Raper in 19563 were associated with ureteral “invasion” or ureteral “obliteration,” and a photograph of the histologic section of one of these cases documents fibrous tissue infiltrating the muscular fibers of the ureter. The histologic findings in 1 of the other 2 cases are described as “similar.” In 1975, Skeel and coworkers4 reported 2 cases of idiopathic retroperitoneal fibrosis with intrinsic ureteral involvement well documented histologically, and in 1982, Frens, Dardenne, and Van Cangh5 reported what they believed to be the third and fourth cases of idiopathic retroperitoneal fibrosis infiltrating the ureter. In their 1977 review of 491 cases of retroperitoneal fibrosis, 333 (67.8%) o f w h ic h were considered idiopathic, Koep and Zuidemae made no mention of fibrotic infiltration of the ureter or intrinsic obstruction, further evidence of its rarity. Idiopathic retroperitoneal fibrosis is, as its name implies, poorly understood, and one can only speculate about the reasons for infiltration of the ureters by the fibrotic process. Ormond in 1960’ stated that the few instances of degeneration of the ureteral wall could be explained by pressure and ischemia as opposed to invasion. Similarly, cases such as ours, in which a ureter can be dissected free of the fibrosis but remains stenotic, may be caused by ureteral inflammation and fibrosis secondary to extrinsic pressure or ischemia rather than by direct invasion by a benign plaque. Unfortunately, no drugs are routinely effective in controlling retroperitoneal fibrosis, so the urologist often is called on to relieve urinary obstruction. Ureteral stenting (which was impossible in our patient) or, more often, nephros-

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tomy tube placement may be useful temporarily, but generally it will be necessary to free the ureters from the plaque. The urologist should be prepared to alter his original operative plans if intrinsic ureteral involvement is found, since simple ureterolysis will not be effective in these cases. Treatment may involve partial ureterectomy with ureteroureterostomy or ileal replacement. Renal autotransplantation may sometimes be necessary. Transureteroureterostomy will seldom be appropriate because of the presence of the fibrotic mass, and cutaneous ureterostomy or bowel diversion, although effective, seems unnecessarily drastic. In our patient, the involved kidney had minimal function, and there was not enough ureteral length to create tension-free ureteroureterostomies and still reposition the duplicated ureters away from the fibrosis. Because contralateral renal function was satisfactory, and because the patient’s many medical problems militated against an extensive reconstructive procedure, nephrectomy appeared the best treatment. Obviously, it would be desirable to identify fibrotic ureteral invasion preoperatively. Theoretically, this might be possible by ureteroscopy. However, in patients with urinary obstruction caused by retroperitoneal fibrosis, the ureters often are too distorted by the plaque to permit passage of the examining instrument. This was the case in our patient.

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Department of Urology St. Paul-Ramsey Medical Center 640 Jackson Street St. Paul, Minnesota 55101 (DR. HELLER) References 1. Ormond JK: Bilateral ureteral obstruction due to envelopment and compression by one inflammatory process, J Urol 59: 1072 (1948). 2. Mitchison MJ: The pathology of idiopathic retroperitoneal fibrosis, J Clin Path01 23: 681 (1970). 3. Raper FP: Idiopathic retroperitoneal fibrosis involving the ureters, Br J Urol 28: 436 (1956). 4. Skeel DA, Shols CW, Sullivan MJ, and Witherington R: Retroperitoneal fibrosis with intrinsic ureteral involvement, J Urol 113: 166 (1975). 5. Frens PH, Dardenne AN, and Van Cangh PJ: Retroperitoneal fibrosis infiltrating ureter, Urology 19: 366 (1982). 6. Koep L, and Zuidema GD: The clinical significance of retroperitoneal fibrosis, Surgery 81: 256 (1977). 7. Ormond JK: Idiopathic retroperitoneal fibrosis, JAMA 174: 1561 (1966).

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Idiopathic retroperitoneal fibrosis infiltrating ureteral wall.

We describe the eight case of idiopathic retroperitoneal fibrosis infiltrating the ureter to cause intrinsic obstruction. Discovery of this situation ...
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