The Turkish Journal of Gastroenterology
2008, Volume 19, No 1, Page(s) 49-53
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|Secondary aortoenteric fistula in Behçet's disease
|Hüseyin ALKIM1, Erkan PARLAK1, Nurgül ŞAŞMAZ1, Meral AKDOĞAN1, Sedef Ö. KURAN1,
Murat BAYAZIT2, Canbek SEVEN3, Gönül GÜRKAYNAK1
|Departments of 1Gastroenterology, 2Cardiovascular Surgery and 3Gastroenterology Surgery, Türkiye Yüksek İhtisas Hospital,
|Keywords: Behçet's disease, aneurysm, pseudo-aneurysm,
Vascular manifestations of Behçet's disease include venous and
arterial occlusions, arterial aneurysm and pseudo-aneurysm
formation. The main problem of the surgical treatment of vascular
lesions in Behçet's disease is the high incidence of complications
such as recurrent aneurysms, thrombosis and fistulization
to the adjacent organs. Here we present a case of Behçet's
disease with multiple complications after aortic reconstructive
surgery, including perigraft infection, abscess distal to the
graft, occlusion of arteries of the lower extremities, aortoenteric
fistula and distal anastomotic site aneurysm rupture.
Aortoenteric fistula (AEF) is a rare but deadly medical
emergency. Primary AEFs occur between the
native aorta and the intestinal tract. Secondary
AEFs occur between an aortic graft and the gastrointestinal
tract. Patients often present with a "herald
bleed", followed by massive gastrointestinal hemorrhage
or with sepsis and abdominal pain (1-3).
In 1937, the Turkish dermatologist Hulusi Behçet
described the triad of relapsing iridocyclitis and
orogenital ulcerations that now bears his name
(4). In addition, Behçet's disease commonly involves
cardiovascular, pulmonary, neurologic, articular
and gastrointestinal systems (5-7). Vascular
manifestations include venous and arterial occlusions,
arterial aneurysm and pseudoaneurysm formation.
The main problem in surgical treatment
of vascular lesions in Behçet's disease is the high
incidence of complications such as recurrent aneurysms,
thrombosis and fistulization to the adjacent
Here we present a case of Behçet's disease with
multiple complications after aortic reconstructive
A Turkish male patient was first diagnosed as having
Behçet's disease at the age of 35 years in
1995. At that time he had the classical triad of the
disease. In March 1997, he was admitted to our
hospital gastroenterology clinic, for the first time,
with a complaint of recurrent, dull epigastric pain.
Based on clinical and laboratory findings, he was
considered as having chronic alcoholic pancreatitis.
In March 1998, he was readmitted with newly
onset very severe right lumbar and inguinal pain.
A diagnosis of ruptured abdominal aortic pseudoaneurysm
was made and a tubular hemo-shield
graft was inserted with aneurysmorrhaphy. An
enlarged, rock hard pancreatic gland was seen during
the operation. A possible relation between the pancreatitis and Behçet's disease was suggested
and the case was reported as the ninth pancreatitis
case in Behçet's disease (10).
In November 1998, nine months after the operation,
he was readmitted to our gastroenterology clinic
with a history of back and left leg pain for one
and a half months and increasing fever with chills
for the last 15 days. There was a tender and hyperemic
swelling over the gastrocnemius muscle of
his left leg. Bleeding history was negative. The
white blood cell count (WBC) was 32000/gl, hemoglobin
7.7 g/dl, hematocrit 24%, platelets 389,000/gl,
erythrocyte sedimentation rate (ESR) 70 mm/h, fibrinogen
8.1 g/L, C-reactive protein (CRP) 18.5
mg/dl, albumin 2.6 g/dl, serum iron 26 g/dl, serum
iron binding capacity 298 g/dl, transferrin saturation
9%, ferritin 234 ng/ml, and haptoglobin 4.6
g/L. Fecal occult blood was negative. On Doppler
ultrasonographic imaging of the aorta, the graft
was reported as patent, but there was a tortuosity
just distal to the renal arteries (Figure 1). The maximal
diameter of the aorta was measured as 18
mm. Vena cava inferior and lower extremity arteries
and veins were reported as normal sonographically.
Sonographic evaluation of the swelling in the
left leg revealed a mass with heterogeneous and
mobile echo pattern and not well-demarcated borders
that were reported as compatible with abscess
or infected hematoma. The mass lesion was incised
and a purulent discharge was noted. Both the cultures
of the blood and abscess yielded enterococcus.
Upper gastrointestinal endoscopic examination
was reported as normal. The patient's complaints
resolved with drainage, antibiotic therapy of four
weeks and other supportive therapies. His anemia was corrected with transfusion of four units packed
red blood cells. He was consulted with cardiovascular
surgeons for a possible graft complication, especially
for perigraft infection, but they believed that
the graft was functioning well and that the existing
findings were inadequate for reoperation of a patient
with Behçet's disease. He was discharged with
colchicine, ciprofloxacin, Coumadin, famotidine
and vitamin B complexes.
In March 1999, he was readmitted in poor condition
with very severe back and leg pain, increasing
fever with chills, fatigue and weight loss. He was
well for the first 15 days after discharge while taking
ciprofloxacin. After discontinuing the drug,
his pain and fever reappeared with a gradual increase
in severity. Again there was no bleeding in
his history. The laboratory values were as follows:
hemoglobin 8.6 g/dl, hematocrit 26.5%, platelets
256000/gl, WBC count 10300/gl, ESR 76 mm/h, fibrinogen
7.8 g/L and CRP 17.25 mg/dl. Fecal occult
blood was negative again. Both urinary and blood
cultures yielded enterococcus. Abdominal ultrasonography
demonstrated that the abdominal aortic
graft was bending anteriorly together with a minimal
splenomegaly and peripancreatic lymph-adenomegaly.
On the abdominal computed tomography,
the presence of a saccular aneurysmal dilation
between the renal arteries and the iliac bifurcation,
5 cm in length, with a maximal diameter of
32 mm was reported (Figure 2). Peripheric angiography demonstrated the occlusion of posterior and
anterior tibial and peroneal arteries of both legs
with collateral circulation. The patient experienced
an upper gastrointestinal bleeding during hospitalization.
Emergency upper endoscopy showed
diffuse mucosal erosions at the antrum, bulbus
and descending duodenum. He was reconsulted
with cardiovascular surgeons with a suggestion of
perigraft infection. The surgeons wished to confirm
the suspicion with indium-labeled leukocytes
scintigraphy, which was reported as normal. Despite
the presence of aneurysm on the tomography,
the negative scintigraphy result encouraged the
surgeons to follow the patient without operation
and the patient was discharged on 15 April 1999.
On 8 June 1999, he was readmitted with a history
of massive upper gastrointestinal bleeding that required
10 units of packed red blood cells transfusions.
On the sonography, there was an anterior
bending of the aortic graft that seemed to have
migrated into the duodenum. On the upper endoscopy,
a pulsatile foreign body (graft), with a mesh
appearance filling all the bowel lumen, was seen
between the second and third parts of the duodenum
(Figure 3). He was taken for emergency surgery.
A duodenal defect 10 cm in length and 2 cm
in width was seen and closed primarily with Prolene
sutures. The graft was excised and a new tubular
hemo-shield graft was inserted with omentoplasty.
The cultures taken from the graft and perigraft
tissue yielded enterococcus and Escherichia
coli. Postoperative recovery was uneventful and
he was discharged with omeprazole, colchicine,
Coumadin and vitamin B complexes.
During the routine follow-up on 6 September
1999, an aneurysmal dilation at the distal end of
the graft just proximal to the iliac bifurcation was
seen on sonography. He had no complaints. While
waiting for the operation, a hypotensive crisis developed.
He was reoperated on an emergency basis
with distal anastomotic site aneurysm rupture
and an axillobifemoral extra-anatomic bypass
with total graft excision and aortic closure was done.
He was well at the last follow-up examination
in September 2005.
Perigraft infection without fistulization occurs in
2-6% of patients, while AEF is a complication occurring
in 0.6-2% of aortic graft operations. Bacterial
infection of a prosthetic graft is caused by intraoperative
contamination from bowel injury or the environment, postoperative pressure necrosis
of the bowel or transient bacteremia from a satellite
source. AEFs occur as two basic types: a fistula
from the prosthetic graft suture line to the intestine,
which commonly develops as a consequence of
suture line pseudoaneurysm eroding the bowel
wall (graft enteric fistula), and a paraprosthetic
enteric fistula in which the body of the graft erodes
into the adjacent bowel (midgraft enteric erosion).
The third and fourth portions of the duodenum are
most frequently involved. The incidence increases
when the initial surgery was necessary to manage
aortic rupture (1-3). The case presented here had a
paraprosthetic type enteric fistula that eroded the
third portion of the duodenum.
Paraprosthetic enteric fistulas form when the mechanical
pulsations or infection of an aortic graft
erodes the bowel wall. Unexplained fever and malaise
frequently develop. Septic emboli that cause
abscesses on the lower extremities, septic arthritis,
multicentric osteomyelitis and hypertrophic osteoarthropathy
have been described (1-3). There was
an abscess over the left gastrocnemius muscle, increasing fever with chills, leg and back pain,
unexplained anemia and sepsis during the first
hospitalization of the case. All these can be explained
by the presence of perigraft infection and/or
paraprosthetic AEF. In fact, all his findings were
"heralding" the presence of perigraft infection
and/or AEF. However, this could not be proven until
the demonstration of the direct involvement of
the duodenum with endoscopy.
The incidence of vascular involvement in Behçet's
disease is 10-80% in the literature. Ten percent of
vascular lesions are severe or life-threatening. Approximately
two- thirds of the arterial lesions are
aneurysms that develop most commonly in the
aorta. They are most often saccular, punched-out
pseudoaneurysms that are prone to infection, rapid
enlargement and rupture. Obliterative endarteritis
of the vasa vasorum is the etiological factor
thought to be responsible for aneurysm formation.
Since the wall of the artery is weakened by inflammation,
surgical treatment of aneurysms is often
complicated (up to 25%) with recurrent aneurysms
(especially anastomotic), thrombosis, infection and
fistulization to adjacent structures such as vein
and intestine (5-9). Surgical results are not satisfactory
in Behçet's disease, because of progressive
graft thrombosis and formation of new aneurysms
at the anastomotic sites (10, 11). In a recent study
from our unit, the 10-year complication-free survival
rate of patients operated for arterial manifestations
of Behçet's disease was found as low as
13%, and the five-year reoperation-free survival
rate was only 26% (12).
The first vascular manifestation of Behçet's disease
in our case was a ruptured abdominal aortic
pseudoaneurysm that had been treated with in situ
graft placement and aneurysmorrhaphy. Infection
of the prosthetic material, fistulization to the
intestine and occlusion of the arteries of the lower
extremities were all seen after the first operation.
After the second operation, in which in situ replacement
of the infected graft with omentoplasty was done, an aneurysm at the distal suture line
developed within three months. In case of inflammatory
aneurysms, extraanatomic bypass technique
is preferred by most authors (1-3, 14).
Tomography is the technique of choice for examining
patients with possible aortic graft infection,
but its sensitivity may be as low as 33%. Air within
the graft, presence of perigraft soft tissue or fluid,
new aneurysm formation and persistent separation
of the graft and native aortic wrap are the findings
compatible with infection. Tomographic manifestations
of AEF are similar to those of perigraft
infection. In addition, extravasation of contrast
materials used may be seen rarely. Some authors
report high sensitivity for indium labeled leukocytes
scintigraphy in the diagnosis of graft infection,
but others have found tomography to be more sensitive
(15-17). In our case, the failure of the scintigraphy
was apparent; however, tomography reported
that there was an aneurysm at the level of the
aortic graft. In fact, when we reviewed the tomography
films, we saw that the graft was displaced
anteriorly and migrated into the duodenum (Figure
2). In addition, the sonographic findings (tortuosity
and anterior bending of the graft), which were
seen in our case, may be important clues compatible
with graft complications. It must be mentioned
that these ultrasonographic findings were present
since his first admission after the first operation.
Tomography and ultrasonography were indeed giving
valuable clues about the graft complications
in this case, but the correct identification of these
findings is not always possible.
In summary, when a patient with known previous
prosthetic aortic reconstruction presents with pain,
anemia or sepsis, a high index of suspicion must
be maintained in order to not miss an underlying
graft complication. From the gastroenterologists'
standpoint, an upper endoscopy up to the third or
if possible fourth portion of the duodenum must be
performed in order to demonstrate different clinical
presentations of graft complications.
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