AJR 2000; 175:1283-1285
© American Roentgen Ray Society
Percutaneous Stent Treatment for Arterial Occlusion Caused by Retroperitoneal Fibrosis
Glenn Kerwin1,
Mark Silverstein and
Curtis Lewis
1
All authors: Department of Radiology, Division of Vascular and Interventional
Radiology, Emory University Hospitals, 1364 Clifton Rd., N.E., Atlanta, GA
30322.
Received June 30, 1999;
accepted after revision April 14, 2000.
Address correspondence to G. Kerwin.
Introduction
Retroperitoneal fibrosis is a rare disorder first described in 1948 by
Ormond [1]. Signs and symptoms
of this disease are related to compression of the ureters, inferior vena cava,
aorta, and aortic branches. Affected patients most often develop
hydronephrosis probably because the low-pressure ureter conduits offer the
least resistance to compression. Inferior vena cava compression may lead to
lower extremity edema or venous thrombosis. Rarely, the aorta and common iliac
arteries may become affected and result in ischemia of the lower extremities.
This type of arterial involvement has been discussed in a case report by
Mathew et al. [2] in which a
54-year-old man with retroperitoneal fibrosis developed claudication, rest
pain, and, finally, gangrene of his third right toe as a result of occlusion
of the infrarenal aorta. We present a case of distal aorta and common iliac
artery occlusion caused by retroperitoneal fibrosis that was managed by
percutaneous stent placement.
Subject and Methods
A 35-year-old woman with history of cocaine abuse complained of throbbing
back, buttocks, and right leg pain of 2 months' duration. Faintly palpable
common femoral arterial pulses were felt at the groins. Neither lower
extremity edema nor swelling was identified. Laboratory results revealed an
elevated level of creatinine of 2.0 mg/dL, and sonographic findings depicted
bilateral hydronephrosis. A retrograde pyelogram revealed medial deviation of
the ureters. Unenhanced CT was performed because of the elevated creatinine
level. CT did not clearly show the masslike infiltrate typical of
retroperitoneal fibrosis or a well-defined mass to explain the medial
deviation of the ureters (Fig.
1A). Although no tissue sample was obtained for pathologic
diagnosis, the typical retrograde pyelogram findings and history of drug abuse
supported a presumptive diagnosis of retroperitoneal fibrosis. Bilateral
double-J Percuflex ureteral stents (Microvasive/Boston Scientific, Watertown,
MA) were placed in the ureters by a urologist to relieve the obstruction
(Fig. 1B).

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Fig. 1B. 35-year-old woman with retroperitoneal fibrosis. Unenhanced
radiograph of abdomen shows bilateral double-J stents within medially deviated
ureters at L4-L5. Note mild-to-moderate hydronephrosis.
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Because of the patient's symptoms, a diagnostic angiogram was obtained.
This study showed a mid abdominal aortic dissection and occlusion of the
distal aorta and common iliac arteries bilaterally
(Fig. 1C). The internal and
external iliac arteries were reconstituted via collaterals. The common
femoral, superficial femoral, and popliteal arteries were patent bilaterally
with three-vessel runoff to the feet.

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Fig. 1C. 35-year-old woman with retroperitoneal fibrosis. Diagnostic
angiogram via left common femoral artery approach reveals occlusion of distal
aorta and common iliac arteries bilaterally. Note limited mid abdominal aortic
dissection (arrow).
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We decided against surgical treatment options including aortobiiliac,
aortobifemoral, and axillarybifemoral bypass grafting. Percutaneous
aortoiliac angioplasty with stent placement was considered a possible
alternative. The left iliac lesion was crossed with an angled catheter
(Glidewire; Boston Scientific Vascular, Watertown, MA) and a 5-French catheter
(Straight; Cook, Bloomington, IN), and an initial diagnostic arteriogram was
obtained. The patient was anticoagulated with 5000 U of heparin. A 10 mm
x 3 cm stent (Palmaz balloon expandable stent; Johnson & Johnson
Interventional Systems, New York, NY) was advanced, via the left common
femoral approach, and inflated to 10 mm in the distal aorta. With a similar
technique, the right iliac occlusion was traversed. Then, 10 x 94 mm
stents (Wallstents; Schneider, Minneapolis, MN) were deployed across the
distal aorta, common iliac artery, and proximal external iliac artery
bilaterally. The Wallstent was chosen because of the extensive length of the
retroperitoneal fibrosis and the occlusive, rather than stenotic, nature of
the lesions. In each Wallstent, 5 mm x 10 cm balloons were inflated. In
residual stenotic areas, 6 mm x 4 cm balloons were inflated
(Fig. 1D). The patient
experienced significant pain with inflation to 6 mm, and we decided to not
proceed with larger balloons. Although a residual systolic resting gradient of
25 mm Hg across both stents was present, we thought this gradient might
decrease with time given the self-expansile property of the Wallstent. The
patient was started on a 6-month course of Coumadin ([warfarin sodium] DuPont,
Wilmington, DE) to prevent early stent thrombosis. When this procedure was
performed, antiplatelet medication was not commonly used by the interventional
staff at our institution. The patient's symptoms improved almost immediately
after the procedure.

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Fig. 1D. 35-year-old woman with retroperitoneal fibrosis. Fluoroscopic
spot film shows 10 mm x 3 cm stent in distal aorta and 10x94 mm
stents in distal aorta extending into proximal external iliac arteries
bilaterally. We used kissing-balloon technique to inflate 5 mm x 10 cm
angioplasty balloons.
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To check the patency of the stents and because there was no literature
about the follow-up of this procedure, an angiogram was obtained 15 months
later. This study showed the aortic Palmaz stent and the left iliac Wallstent
to be widely patent. The proximal right common iliac artery was mildly
stenotic as a result of presumed intimal hyperplasia, but because the patient
was asymptomatic at this time, angioplasty was not performed. The pelvic and
lower extremity vessels remained widely patent.
The patient returned for angiographic examination 1 year 6 months after
stent placement because of progressive right lower extremity claudication.
Although the previously stented infrarenal abdominal aorta and left iliac
arteries remained widely patent, the mild stenosis of the proximal right
common iliac artery had progressed to 50-60% stenosis
(Fig. 1E). Of note, the
bilateral ureter obstruction had resolved (both internalized ureter stents
were removed) after the patient stopped using cocaine and started taking
steroids. Because of the bilateral common iliac artery stents, we decided to
perform angioplasty using the kissing-balloon technique. An 8 mm x 4 cm
angioplasty balloon (Diamond; Medi-tech/Boston Scientific, Watertown, MA) was
inflated within each common iliac artery. The patient tolerated inflation to 8
mm during this procedure without significant discomfort unlike 1.5 years
earlier when she experienced pain during dilatation to 6 mm. After
angioplasty, only a 10 mm Hg systolic pressure gradient was present across the
right common iliac artery stenosis, and a 6 mm Hg gradient was present across
the left common iliac artery. Although the proximal right common iliac artery
stenosis was most likely caused by intimal hyperplasia, which is often
resistant to angioplasty alone, we took the more conservative approach of not
placing an additional stent because of the satisfactory pressure gradient and
our lack of experience with treating this disease. This angiogram was the last
one obtained in the patient; she remains clinically asymptomatic more than 6
years after the first intervention.

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Fig. 1E. 35-year-old woman with retroperitoneal fibrosis. Digitally
subtracted angiogram, obtained 1 year 6 months after stent placement after
patient presented with recurrent right lower extremity claudication, shows
50-60% stenosis of proximal right common iliac artery (arrow).
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Discussion
Retroperitoneal fibrosis is manifested by the formation of a fibrous plaque
over the anterior surface of the lower lumbar vertebra. This plaque may encase
and compress the ureters, inferior vena cava, and aorta and characteristically
deviates the middle third of the ureters medially
[3]. This condition is often
diagnosed by the characteristic radiographic and clinical findings but can be
confirmed by pathologic analysis. Approximately two thirds of cases are
considered idiopathic (primary retroperitoneal fibrosis or Ormond's disease).
The remaining one third of cases results from an inciting process (secondary
retroperitoneal fibrosis). Secondary retroperitoneal fibrosis has multiple
causes including malignancy, inflammatory aneurysm, radiation, connective
tissue diseases, prior surgery, and certain medications. Secondary
retroperitoneal fibrosis is also caused by illicit drugs (amphetamines,
lysergic acid diethylamide, and cocaine)
[4], as shown in our
patient.
Aortography and venography may show smooth extrinsic narrowing of the
aorta, iliac vessels, and inferior vena cava. With extensive plaques, stenosis
of the renal arteries may result in renovascular hypertension, and occlusion
of the superior and inferior mesenteric arteries may lead to bowel
ischemia.
Retroperitoneal fibrosis that compresses the aorta and is unresponsive to
conservative measures, such as cessation of the offending agent and
immunosuppression, may be surgically treated with axillarybifemoral
bypass [5]. This procedure
obviates surgical dissection of an inflammatory mass and prevents damage to
extensive venous collaterals that develop in response to inferior vena cava
occlusion [2]. Retroperitoneal
fibrosis that occludes the aorta has been previously treated with transluminal
balloon angioplasty [6].
However, despite an extensive literature review, we found no cases in which
stents were used to treat aorta and iliac artery occlusion that resulted from
retroperitoneal fibrosis. Although an isolated case, we have described the
long-term efficacy of percutaneous stents for the treatment of arterial
occlusion caused by retroperitoneal fibrosis. Percutaneous therapy, including
covered stents in the future, may provide a beneficial alternative for those
patients who acquire this rare disease.
References
-
Ormond JK. Bilateral ureteral obstruction due to envelopment and
compression by an inflammatory retroperitoneal process. J
Urol 1948;59:1072
-1079
-
Mathew CV, Shanabo A, Zyka I, Eklof B. Retroperitoneal fibrosis
with large-vessel obstruction. Acta Chir Scand
1985;151:475
-480[Medline]
-
Gilkeson GS, Allen NB. Retroperitoneal fibrosis: a true connective
tissue disease. Rheum Dis Clin North Am
1996;22:23
-38[Medline]
-
Kottra JJ, Dunnick NR. Retroperitoneal fibrosis. Radiol
Clin North Am 1996;34:1259
-1275[Medline]
-
Amis ES Jr. Retroperitoneal fibrosis. AJR
1991;157:321
-329[Abstract/Free Full Text]
-
Haynes IG, Simon J, Hamer JD. Idiopathic retroperitoneal fibrosis
with occlusion of the abdominal aorta treated by transluminal angioplasty.
Br J Surg
1982;69:432
-433[Medline]

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