A Rare Case of Idiopathic Retroperitoneal Fibrosis Involving Obstruction of the Mesenteric Arteries, Duodenum, Common Bile Duct, and Inferior Vena Cava

Abstract

Idiopathic retroperitoneal fibrosis (IRF), usually affects the ureter, although the biliary tree, duodenum and vasculature may also be susceptible. This case report describes a 64-year-old man with IRF, who presented painless watery diarrhea, radiological features of obstructive jaundice and duodenal obstruction, and ultimately an obstruction of the inferior vena cava. We employed tamoxifen for his treatment, but the disease progressed and the patient died of multiple organ failure two years after the onset. While the cause of IRF in this patient was obscure, we suspected his painless watery diarrhea indicated chronic ischemia of the small bowel, and the findings of an abdominal CT scan were extremely valuable in indicating IRF.
(Internal Medicine 42: 812-817, 2003)

Full text

CASE REPORT
ARare Case of Idiopathic Retroperitoneal Fibrosis
Involving Obstruction of the Mesenteric Arteries,
Duodenum, CommonBile Duct, and Inferior Vena Cava
Satoru TAMURA*, Yuichi YOKOYAMA**,Kazuo NAKAJO**,
Tomoko MORITA**, Kayoko WADA**and Saburo ONISHI*' **
Abstract
Idiopathic
retroperitoneal
fibrosis
(IRF),
usually af-
fects the ureter, although the biliary tree, duodenum and
vasculature mayalso be susceptible. This case report de-
scribes a 64-year-old man with IRF, who presented pain-
less watery diarrhea, radiological features of obstructive
jaundice and duodenal obstruction, and ultimately an ob-
struction of
the inferior
vena
cava.
We employed
tamoxifen
for
his
treatment,
but
the
disease progressed
and the patient died of multiple organ failure two years
after the onset. While the cause of IRF in this patient was
obscure, we suspected his painless watery diarrhea indi-
cated chronic ischemia of the small bowel, and the find-
ings of an abdominal CTscan were extremely valuable in
indicating IRF.
(Internal Medicine 42: 812-817, 2003)
Key words: idiopathic retroperitoneal fibrosis, duodenal ob-
struction, tamoxifen, painless diarrhea
Introduction
Idiopathic
retroperitoneal
fibrosis
(IRF),
a
rare finding,
was first described by Albarran in 1905 (1) as an inflamma-
tory retroperitoneal process compressing the ureters. A mat
of fibrous tissue develops in the retroperitoneal wall without
obvious cause. The ureter is the most vulnerable organ, al-
though the biliary tree, duodenum and vasculature mayalso
be affected. The ureteral encasement leads to its obstruction
and
consequent
hydronephrosis.
The
distal
part
of
the com-
mon
bile
duct
can
also
be
affected
and
may
lead to
cholestasis
(2,
3).
Duodenal
obstruction
due
to
IRF
is a
rarely reported clinical entity and the symptomsof nausea,
vomiting, and body weight loss are usually presented several
months after the initial diagnosis (4). IRF association with
vascular
obstruction
also
has
been
reported
rarely
(5), and
chronic small bowel ischemia has primarily manifested itself
by abdominal pain, diarrhea and body weight loss occurring
in close relation to meals (6).
Surgery and /or drugs have been employed for the treat-
ment of IRF. Corticosteroids have mainly been used and
many cases of IRF have been treated (7). Furthermore, some
cases of benign fibrotic tumors and IRF were treated suc-
cessfully
with
the
estrogen
receptor
antagonist, tamoxifen
(8).
Here, we describe a patient with IRF, who presented with
painless watery diarrhea, radiological features of obstructive
jaundice and duodenal obstruction, with the ultimate obstruc-
tion of the inferior vena cava.
Case Report
A 64-year-old man was referred to our hospital with a
one-year history of body weight loss (12 kg) and a painless
watery diarrhea (8-9 times a day) occurring after meals. He
was admitted in January 1999. No previous history of special
drug ingestion (e.g. methysergide) was elicited. His mother
had died of gastric cancer and his grandfather had died of
pancreatic cancer. His abdomenwassoft and flat with nor-
mal bowel sound. Abruit of the abdominal artery was noted
by auscultation. Superficial lymph nodes were not palpable.
All
other
physical
examinations
were
unremarkable. His
fecal occult blood test was negative and urinalysis was nor-
mal with a specific gravity of 1.017. The complete blood cell
count
showed
normochromic
normocytic
anemia
with a
hematocrit 32% (normal=40.5-51.5%). The blood urea nitro-
gen
was
23
mg/dl
(normal=8-20
mg/dl),
and
the serum
From *the Department of Endoscopy and **the First Department of Internal Medicine, Kochi Medical School, Nankoku
Received for publication February 26, 2003; Accepted for publication May 1, 2003
Reprint requests should be addressed to Dr. Satoru Tamura, the Department of Endoscopy, Kochi Medical School, Kohasu, Okoh-cho, Nankoku, Kochi
783-8505
812
Internal Medicine Vol. 42, No. 9 (September 2003)

Idiopathic Retroperitoneal Fibrosis
creatinine was 0.8 mg/dl (normal=0.4-1.0 mg/dl). Other bio-
chemical screening tests and electrolytes were all within nor-
mal limits. Both the carcinoembryonic antigen (CEA) and
CA19-9level were within normal ranges.
Abdominal
ultrasonography,
gastrointestinal endoscopy,
and colonoscopy showed no remarkable findings. CTscan of
the abdomen demonstrated evidence of periarterial (superior
mesenteric arteries) soft tissue suspect of retroperitoneal fi-
brosis, but no sign of hydronephrosis (Fig. 1A). Abdominal
angiography
was
subsequently
performed,
and
revealed a
narrowing of the origin of the superior mesenteric arteries
and
its
branches
(Fig.
2A),
and
slight
extrinsic compression
of the origin of the celiac axis (Fig. 2B). Inferior mesenteric
arteries were normal. These changes in the artery were local-
ized at its origin from the aorta, and its peripheral parts were
smooth. Collateral flow to the superior mesenteric arteries
was not derived from the celiac artery or the inferior mesen-
teric arteries. Weascribed the symptomsto small bowel
ischemia.
The patient started taking tamoxifen, 10 mg twice a day,
in March 1999. In July 1999, his diarrhea improved (4-5
times a day) but the periarterial soft tissue was not changed
(Fig. IB). In August 1999, an abdominal sonographic exami-
nation
detected
the
dilatation
of intrahepatic
bile
ducts and
commonbile duct (CBD). Endoscopic retrograde cholangio-
pancreatography
(ERCP)
showed
a
narrowing
of
the com-
mon
bile
duct
with
a
small
stone.
A
few
days
after the
ERCP, he was readmitted with severe nausea and vomiting.
Upper gastrointestinal series showedalmost total obstruction
of the third portion of the duodenumwith proximal dilatation
(Fig. 3). To assess the lesion, abdominal angiography was
reperformed, which revealed the aggravated narrowing of the
origin of the superior mesenteric arteries and its branches
(Fig. 2C), as well as the aggravated narrowing of the origin
of
the
celiac
axis
(Fig.
2D).
He
discontinued taking
tamoxifen.
After eight weeks of hospitalization, laparotomy revealed
a
diffuse,
poorly
delineated,
focally
dense fibrotic
retroperitoneum,
which
encircled
the
third
and
fourth por-
tions
of
the
duodenum
and
abdominal
aorta. Gastro-
jejunostomy was performed in November 1999. Histological
assessment
of
the
tissue
from
three
retroperitoneal sites
showed fibrous tissue and fat with no evidence of malig-
nancy
(Fig.
4).
This
operative intervention
improved his
symptomsof nausea and vomiting, and the patient resumed
eating.
However, the bile duct dilatation did not improve, along
with
the
following: alkaline
phosphatase
698
IU/Z (normal
70-220);
gamma-glutamyl
transpeptidase
246
IU/Z (normal
5-50);
aspartate
aminotransferase
59
IU/Z
(normal 9-27);
alanine
aminotransferase
147
IU/Z
(normal
5-37); total
bilirubin 0.8 mg/dl (normal 0.3-1.1). ERCPdemonstrated a
stenosis
of
the
CBD and
endoscopic
sphincterotomy (Fig.
5A) and subsequent transpapillary biliary drainage was per-
formed in January 2000 (Fig. 5B).
The patient started taking prednisolone (PSL), 60 mg a
day, in January 2000. In March 2000, PSL was tapered to 20
mg, but narrowing of CBDwas not improved. To makemat-
ters
worse,
a
liver
abscess
concurred
(Fig.
1C)
and severe
edema in both legs appeared. Angiography showed a narrow-
ing of the inferior vena cava with collateral flow (Fig. 6).
PSL
was
tapered
off
and
liver
abscess
was improved.
Unfortunately renal dysfunction, abnormality of electrolytes,
arrhythmia and pleural effusion appeared in late June 2000.
The patient died of multiple organ failure in July 2000.
Discussion
Retroperitoneal fibrosis is a relatively uncommondisease,
with its etiology unable to be identified in two-thirds of pa-
tients.
Drug-induced
retroperitoneal
fibrosis
occurs
in about
12%, and malignant disease has been associated with
Figure 1. CTscan of the abdomenwith intravenous contrast. A. CTscan prior to treatment demonstrate periarterial (supe-
rior mesenteric arteries) soft tissue (arrow). B. CTscan performed four months after treatment shows no reduction in the size
of the periarterial soft tissue (arrow). C. CTscan shows a low-density lesion of the liver which is compatible with the abscess.
Internal Medicine Vol. 42, No. 9 (September 2003)
813

Tamuraet al
Figure 2. A. Abdominal angiography in January 1999 reveals a narrowing of the origin of the supe-
rior mesenteric arteries (black arrows) and its branches (white arrows). B. Abdominal angiography
in January 1999 reveals slight extrinsic compression of the origin of the celiac axis. C. Abdominal
angiography in October 1999 reveals the aggravated narrowing of the origin of the superior mesen-
teric arteries (black arrows) and its branches (white arrows). D. Abdominal angiography in October
1999 reveals the aggravated narrowing of the origin of the celiac axis.
retroperitoneal fibrosis in about 8% of patients (2). The clini-
cal features reflect the extent of fibrous tissue proliferation.
Usually the fibrous tissue extends from the brim of the pelvis
upward to the renal pedicle and ureter; however, occasion-
ally it extends beyond them into the pelvis or through the
crura of the diaphragm. The typical patient with IRF is a man
in his late 50s (9) and a urinary tract obstruction is the most
commonclinical manifestation. Reviewof the literature re-
vealed a few reported cases of retroperitoneal vessel (5, 6,
9-12), duodenal (4) and CBD involvement (3, 13-15). As
case reports accumulate, however, it has becomemoreevi-
dent that the location and extent of the fibrous tissue may
vary
widely
with
the
involvement
of
any retroperitoneal
structure. However, a case which coincidently involves ves-
sel, biliary
and
duodenal
obstruction
is
very
rare (16).
Furthermore, to the best of our knowledge, no previous case
has been reported which coincidently involves the mesen-
teric artery, biliary, duodenum and inferior vena cava.
The
cause
of
IRF
in
this
patient
was
obscure, and
methysergide,
ergotamine
and
other drugs (analgesics,
antihypertensives, etc) were not elicited. Intraabdominal in-
flammatory
conditions
or
systemic
fibrosing
disorders or
immunological
disease
were
also
not
implicated.
We sus-
pected
his
painless
watery
diarrhea
to
be
due
to chronic
ischemia of the small bowel, and the findings of abdominal
CT
scanning
were
extremely
valuable
in
indicating a
814
Internal Medicine Vol. 42, No. 9 (September 2003)

Idiopathic Retroperitoneal Fibrosis
Figure
3.
Upper
gastrointestinal
series
shows
almost
total ob-
struction (arrow) of the third portion of the duodenumwith
proximal dilatation.
Figure 4. Histology from three retroperitoneal sites reveals fi-
brous tissue, fat and patchy lymphocytic infiltrates with no evi-
dence of malignancy (HE stain, x50).
suspicion
of
IRF.
Following
a
previous
report
of chronic
small
bowel
ischemia,
clinical
descriptions
of the condition
have
emphasized
the
universal
presence
of food-related
epigastric pain of intestinal angina. The case described here,
however,
strongly
suggested
that
chronic ischemia
of the
small
bowel
can
occur
in
the
absence
of
typical
pain, and
that this condition should be considered in the differential di-
agnosis of painless water diarrhea in the context of weight
loss(5, 6, 10-12). We estimated that the mass surrounding
the
superior
mesenteric
artery of
CT
scan
was
due to
retroperitoneal
fibrosis,
and
that
this
was
the
cause
of the
small bowel ischemia which induced the painless watery di-
arrhea of our patient. The narrowing of the superior mesen-
teric arteries of abdominal angiography wascompatible with
the CT scan findings.
Duodenal
obstruction
produces
vomiting
as
one
of the
presenting symptoms, though this is a rare clinical entity (4,
16). The third portion may be the most vulnerable portion of
the duodenum in patients with IRF.
Retroperitoneal
fibrosis
with
cholestatic
jaundice
due to
an affection of the distal part of the commonbile duct is also
a rare condition and difficult to distinguish from cholangio-
carcinomas (2, 3, 13, 14), or pancreatic cancer (15). Wetter
et al reported that 31%of Klatskin tumors (30/98) were ulti-
mately
diagnosed
as
other
than
cholangiocarcinomas, and
that 3 cases of IRF were included in Klatskin tumors (14).
The cholangiogram of this case showedsmooth stenotic
change,
and
the
cytology
of
bile
from
the transpapillary
biliary drainage tube showed no malignancy; these findings
indicated that the obstructive jaundice of this case originated
in
the
retroperitoneal fibrosis.
Severe edema of both legs due to a narrowing of the infe-
rior vena cava may occur in the late stage of IRF (9), as with
our case. Angiography of our case showed a collateral flow,
but caput Medusaor other obvious signs of the development
of a venous collateral circulation of the surface of body were
not noted.
The lack of ureteral involvement may be attributed to the
site of the fibrosis in this case because fibrous change usu-
ally occurs and extends from the brim of the pelvis upward
to the renal pedicle and ureter in almost all cases of IRF.
The
treatment
of
IRF has
not
been
well established.
Operative
intervention followed
by
steroid
therapy
is se-
lected in most cases of ureteral or alimentary tract obstruc-
tion
(3,
4,
7,
17,
18).
However,
there
is
still disagreement
concerning the efficacy of corticosteroids (19). Patients with
retroperitoneal
fibrosis
may
be
divided into
two defined
groups (7): the first group is those patients whoare the most
seriously ill and have a poor response both to surgery and
corticosteroids;
the
second group
consists
of
the
less seri-
ously ill patients whoare presumably in the early stages of
the disease. The response to corticosteroids maydepend on
the
pathologic
stage
of
the
disease
condition.
On
the other
hand,
success
with
the
estrogen
receptor antagonist,
tamoxifen,
in
the
treatment
of
retroperitoneal
fibrosis has
been reported (8). Unfortunately, our patient did not respond
to
either
corticosteroids
or
tamoxifen
and
died nineteen
months
following
the
first admission.
We
considered
that the
response
to
treatment
for IRF
mainly depends on the stage of disease, and the patients in
the most advanced stage may fail to respond favorably.
Internal Medicine Vol. 42, No. 9 (September 2003)
815

Tamuraet al
Figure
5. A:
ERCP
demonstrate
a
tapered
stenosis
of
the
common
bile
duct (arrow).
B:
Cholangiography
using a
transpapillary biliary drainage tube shows almost complete obstruction of commonbile duct and dilated intrahepatic bile
ducts.
Figure 6. Angiography shows a tapered narrowing of the infe-
rior vena cava with collateral flow.
Acknowledgements:
The
following
individuals
are
acknowledged
for their
contribution to this case: Yukiko Nagayoshi, M.D., Chiaki Watanabe, M.D.,
Kaori Morimoto, M.D., Isao Nishimori, M.D., Ph.D., Shinji Iwasaki, M.D.,
Ph.D. (First Department of Internal Medicine, Kochi Medical School),
Yasuhisa Matsumoto, M.D., Yoshinobu Ohmori, M.D. (Second Department
of Surgery, Kochi Medical School).
References
1)
Albarran
J.
Retention
renale
per
perureterite;
liberation
externe de
l'uretere. Asso Franc Urol 9: 511, 1905.
2) Chutaputti A, Burrell MI, Boyer JL. Pseudotumor of the pancreas asso-
ciated
with retroperitoneal
fibrosis:
A
dramatic
response
to corti-
costeroid therapy. Am J Gastroenterol 90: 1 155-1 158, 1995.
3) Dejaco C, Ferenci P, Schober E, et al. Stenosis of the common bile duct
due to Ormond's disease: case report and review of the literature. J
Hepatol
31:
156-159, 1999.
4)
Siegel
GJ,
Hall
JM,
Welling
RE.
Idiopathic
retroperitoneal fibrosis
with functional duodenal obstruction. South Med J 73: 946-948, 1980.
5) Snow N, Kursh E, DePalma RG, Hubay CA. Peripheral ischemia due
to retroperitoneal fibrosis. AmJ Surg 133: 640-642, 1977.
6) Jones DEJ, Barton J, Cobden I. Painless small bowel ischemia present-
ing with diarrhea and weight loss. AmJ Gastroenterol 93: 653-655,
1998.
7) Higgins PM, Bennett-Jones DN, Naish PF, Aber GM. Non-operative
management of retroperitoneal fibrosis. Br J Surg 75: 573-577, 1988.
816
Internal Medicine Vol. 42, No. 9 (September 2003)

Idiopathic Retroperitoneal Fibrosis
8) Clark CP, Vanderpool D, Preskitt JT. The response of retroperitoneal
fibrosis
to
tamoxifen.
Surgery
109:
502-506, 1991.
9) Hackett E. Idiopathic retroperitoneal fibrosis. A condition involving the
ureters, the aorta, and the inferior vena cava. Br J Surg 46: 3-9, 1958.
10) Parums DV. The spectrum of chronic periaortitis. Histopathlogy 16:
423-431, 1990.
1 1) Martina FB, Niiesch R, Gasser TC. Retroperitoneal fibrosis and chronic
periaortitis: A new hypothesis. Eur Urol 23: 371-374, 1993.
12) Wicks IP, Robertson MR, Murnaghan GF, Bertouch JV. Idiopathic
retroperitoneal
fibrosis
presenting with
back
pain.
J
Rheumatol 15:
1572-1574, 1988.
13)
Renner
IG,
Ponto
GC,
Savage
WT
III,
Boswell
WD. Idiopathic
retroperitoneal
fibrosis
producing
common
bile
duct
and pancreatic
duct obstruction. Gastroenterology 79: 348-351, 1980.
14) Wetter LA, Ring EJ, Pellegrini CA, Way LW. Differential diagnosis of
sclerosing cholangiocarcinomas of the commonhepatic duct (Klatskin
tumors). Am J Surg 161: 57-63, 1991.
15) Lundstrom B. Idiopathic retroperitoneal fibrosis and common bile duct
stenosis. A case report. Acta Chir Scand 149: 713-715, 1983.
16) Schneider CF. Idiopathic retroperitoneal fibrosis producing vena caval,
biliary,
ureteral
and
duodenal
obstructions.
Ann
Surg
159: 316-320,
1964.
17) Cogan E, Fastrez R. Azathioprine. An alternative treatment for recur-
rent idiopathic retroperitoneal fibrosis. Arch Intern Med 145: 753-755,
1985.
18) Baker LR, Mallinson WJ, Gregory MC, et al. Idiopathic retroperitoneal
fibrosis. A retrospective analysis of 60 cases. Br J Urol 60: 497-503,
1988.
19)
Robertson
RH,
Mcdowell
HA
Jr,
Jander
HP, Groarke
JF. Toxic
magacolondue to ischemic enterocolitis associated with retroperitoneal
fibrosis. Gastroenterology 78: 585-591, 1980.
Internal Medicine Vol. 42, No. 9 (September 2003)
817