Peripheral ischemia due to retroperitoneal fibrosis

Peripheral lschemia Due to Retroperitoneal Fibrosis Norman Snow, MD, Cleveland, Ohio Elroy Kursh, MD, Cleveland, Ohio Ralph G. DePalma, MD, Cleveland, Ohio Charles A. Hubay, MD, Cleveland, Ohio

In 1948 Ormond [I] first described the entity of retroperitoneal fibrosis in English literature. Since that time there has been an increased understanding of its pathophysiology with emphasis on the process as an obstructive uropathy since the ureter is the retroperitoneal structure most vulnerable to compression. While there have been reports of its association with vascular obstruction, recognition and treatment of significant peripheral ischemia caused by this entity has not yet been emphasized. We describe two patients with symptomatic aortoiliac occlusive disease secondary to retroperitoneal fibrosis to stress detection and therapy of peripheral ischemia caused by this process. Case Reports Case I. EG, a forty-eight year old white female, presented to her physicians with low, dull, constant back and pelvic pain of one month’s duration. The pain was exacerbated by hip extension, for example, standing or lying in the supine position. Intermittent claudication in both calves was noted three weeks prior to examination. She had been taking methysergide maleate sporadically for migraine headaches. Blood pressure was normal and abdominal examination revealed no irregularities. Femoral pulses were diminished bilaterally and became even weaker when the patient extended her nhips and lumbar spine in the standing or supine position. Intravenous urography showed bilateral hydronephrosis, medial deviation, and extrinsic compression of both ureters. Aortography demonstrated thinning of the contrast medium in the distal aorta suggesting compression in the anteroposterior plane. The common and external iliac arteries were also narrowed, and beading with segmental narrowing was noted in the left renal and hepatic arteries. Blood urea nitrogen was 24 mg/lOO ml, creatinine was 1.6

From the Departments of General Surgery and Urology, University Hospitals of Cleveland and Case Western Reserve University School of Medicine, Cleveland, Ohio. Reprint requests should be addressed to Ralph G. DePalma, MD, University Hospitals, 2065 Adelbert Road, Cleveland, Ohio 44106.

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mg/lOO ml, erythrocyte sedimentation rate (ESR) was normal, and the serum haptoglobin level was reduced. Other laboratory findings were normal. On April 23, 1975, bilateral ureterolysis and aortolysis was accomplished through a midline abdominal incision. On exploration, the only major vascular abnormalities were diminished distal aortic pulsations. Ureterolysis was performed by reflecting the ascending and descending colon, dissecting the ureters from their fibrous encasement and transposing them to an intraperitoneal position. Aortolysis was required to divide a thick white band of fibrous tissue that compressed the aorta against the sacral promontory and compromised the lumen of the distal aorta as well as both common iliac arteries. (Figure 1.) The fibrotic mass was sharply incised to the vascular adventitia anteriorly from the level of the renal veins to the bifurcation of the common iliac arteries, freeing the vessels from their encasement and compression. The dissection was difficult due to a poorly defined plane between the vessels and the fibrous mass. To completely free the aorta, the left renal vein was ligated and divided. Brisk bleeding from the friable thin adventitia of the aorta required several merseline sutures bolstered by Teflon@ pledgets. Ten units of blood were transfused. The method of dissection and completed arteriolysis is shown in Figures 2 and 3, respectively. Microscopic examination of the fibrotic mass showed chronic inflammation and dense fibrous tissue consistent with the operative findings of retroperitoneal fibrosis. Recovery was uncomplicated and at ten months the patient was asymptomatic and peripheral pulses were normal. Methysergide maleate has been discontinued. Case II. GP, a forty-nine year old white male, was referred because of crampy leg pain suggestive of claudication. He had a three year history of impotence and drug dependence. He required large quantities of analgesics to relieve what were thought to be “histamine headaches.” He also took methysergide maleate regularly for the headaches. The femoral pulses were palpable but reduced; no popliteal or pedal pulses were palpable. Blood urea nitrogen, creatinine, ESR, and intravenous urography were normal, but the creatinine clearance was reduced to 67 cc/min. Translumbar aortography showed minimal atherosclerotic disease in the aorta but normal

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Retroperitoneal Fibrosis

femoral vessels. Because of the history of methysergide ingestion, surgery was undertaken with a preoperative diagnosis of atherosclerotic disease or retroperitoneal fibrosis. On November 7,1973, exploration revealed a plaque of condensed connective tissue that encased the distal abdominal aorta and iliac arteries. No definable plane between the fibrosis and the adventitia of the vessels existed. There were poor pulsations in the external iliac arteries until sharp arteriolysis of the aorta and common iliac arteries was completed. This tedious dissection was completed without incident or need for transfusion. Postoperatively, the pedal pulses were bounding. At twenty-one months there were excellent pedal pulses and no physical signs of peripheral vascular insufficiency. Impotence remained and the patient continues to complain of migraine headaches, poorly controlled with propranalol and propoxyphene.

Comments

The etiology of retroperitoneal fibrosis is unknown. It has been suggested that the cause is due to an occult infectious process with lymphangitic spread and retroperitoneal periadenitis [2]. It may coexist with other fibrotic diseases such as Reidel’s Struma, fibrosing mediastinitis, sclerosing cholangitis, and Peyronie’s disease, thus implicating genetic and systemic factors [3]. Ergot compounds, especially methysergide, clearly play an important role in the etiology of this process [3], perhaps by acting as a hapten with a resulting autoimmune reaction [4]. Our first patient had a reduced serum haptoglobin level. Despite the often extensive retroperitoneal involvement by the disease which may extend from the renal pedicle deep into the pelvis, presenting symptoms of lower extremity ischemia, as seen in our two patients, are unusual. Although the aortoiliac vessels are frequently encased in the fibrotic mass, encroachment on the vessel lumen has previously been considered distinctly unusual [5-71. Arteriographic demonstration of lumenal narrowing caused by retroperitoneal fibrosis is difficult to detect with conventional uniplanar angiography. The arteriograms of both our patients, which were obtained in the anteroposterior view, were not diagnostic but did demonstrate a decreased flow of contrast medium in the involved vessels. Associated urographic abnormalities and a history of methysergide ingestion provided important clues to the cause of the vascular obstruction. Lateral views of the aortoiliac system should be employed in the future if retroperitoneal fibrosis is suspected. The pathology often resembles an arteritis. Indeed, the first patient

Volume 133, May 1977

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Retroperitoneal fibrous

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Figure 1. Extent of re@peritoneal fibrosis In case I; note compression at sacral promontory.

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Figure 2. Method of dissection of retroperitoneal involvement in intimate relation to aortlc adventitia; note use of right angle clamp.

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Ureter

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Figure 3. Completed dissection; note ligation of inferior mesenteric artery and completed arteriolysis and ureterolysis.

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displayed angiographic changes in the renal and hepatic arteries consistent with such a diagnosis. The therapeutic approach to the vascular compromise associated with retroperitoneal fibrosis has varied in the few reports of this complication. Iliac artery occlusion has been treated by arteriolysis [6] and resection with end-to-end anastomosis [8]. Aortic compression has been managed by aortolysis [3,5], radiotherapy [9], steroid administration [3], and occasionally, by observation only [lO,ll]. The most direct approach and the best long-term solution to the vascular obstruction caused by retroperitoneal fibrosis is complete arteriolysis. The cleavage plane between the fibrotic mass and the aorta was relatively simple to establish in previously reported cases of vascular compression since the vessels were encased but not invaded by the inflammatory process. In the past, it was felt that the fibrous tissue enveloped but did not invade the retroperitoneal structures. Recent reports have emphasized that invasion of the psoas muscles and ureters by the fibrous process may occur [12,13]. McMillan, Swarts, and Knudtson [2] suggested that the dissection plane between the plaque and the aorta was not easy to define. We found that the aorta was invaded by the fibrotic plaque in both of the reported cases making aortolysis extremely difficult to perform. The difficulty of the dissection is illustrated by the profuse blood loss noted in case I. It is apparent that an intimate adherence of the fibrotic process and the vessel wall may exist and the surgeon must anticipate the possibility of a tedious dissection requiring considerable blood replacement when performing arteriolysis for retroperitoneal fibrosis. Summary

Two patients with retroperitoneal fibrosis demonstrated symptoms of peripheral vascular ischemia. Arteriolysis to free the distal aorta and iliac vessels was successful in both patients. Aortography in the

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posteroanterior view was equivocal, but the accompanying urologic abnormalities and a history of methysergide ingestion helped establish the etiology of the ischemia. The most direct approach to the treatment of the vascular obstruction caused by retroperitoneal fibrosis is complete arteriolysis. Most previous reports indicate that it is relatively easy to establish a dissection plane between the fibrotic plaque and the vessel wall. We found that the fibrotic process invaded the vessel wall. Accordingly, the surgeon must anticipate a difficult, tedious dissection when performing arteriolysis for the treatment of vascular compression secondary to retroperitoneal fibrosis. References 1. Ormond JK: Bilateral ureteral obstruction due to envelopment and compression by an inflammatory retroperitoneal process. J Ural 59: 1072, 1948. 2. McMillan JC, Swarts CJ, Knudtson KP: Idiopathic fibrous retroperitonitis. Northwest Med 63: 605, 1964. 3. Utz DC, Rooke ED, Spittel JA Jr, Bartholomew LG: Retroperi, toneal fibrosis in patients taking methysergide. JAMA 191: 983, 1965. 4. Stecker JF, Rawls HP, Devine CJ Jr, Devine PC: Retroperitoneal fibrosis and ergot derivatives. J Ural 112: 30, 1974. 5. Larson RE. Ross JV Jr: Intermittent claudication secondary to idiopathic fibrous retroperitonitis. Circulation 28: 1145, 1963. 6. Perlow S: Obstruction of the iliac artery caused by retroperitoneal fibrosis. Am J Surg 105: 285, 1963. 7. Hache L, Utz DC, Woolner LB: Idiopathic fibrous retroperitonitis. Surg Gynecol Obstet 115: 737, 1962. 8. Clouse ME: Bilateral ureteral obstruction. JAMA 188: 299, 1964. 9. Hackett E: Idiopathic retroperitoneal fibrosis. A condition involving the ureters, the aorta, and the inferior vena cava. Br J Surg 46: 3, 1958. 10. Dineen i Asch T, Pierce JM: Retroperitoneal fibrosis. Radio/logy 75: 380, 1960. 11. Furlong JH Jr, Connerty HV: Compression of the aorta and ureters by a retroperitoneal inflammatory mass. De/ Med J 30: 63, 1958. 12. Persky L, Huus JC: Atypical manifestations of retroperitoneal fibrosis. J Ural 111: 340, 1974. 13. Skeel DA, Shols GW, Sullivan MJ, Witherrington R: Retroperitoneal fibrosis with intrinsic ureteral involvement. J Ural 113: 166,1975.

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