DOI:10.2214/AJR.05.0628
AJR 2007; 188:W400-W402
© American Roentgen Ray Society
Endovascular Treatment for Rupture of Intrahepatic Artery Aneurysm in a Patient with Behçet's Syndrome
Na Young Jung1,
Seung Kwon Kim1,
Eun Chul Chung1,
Haewon Park1 and
Yong Kyun Cho2
1 Department of Radiology, Kangbuk Samsung Hospital, Sungkyunkwan University
School of Medicine, 180, Pyung-Dong, Jongro-Ku, Seoul 110-746, South
Korea.
2 Department of Internal Medicine, Kangbuk Samsung Hospital, Sungkyunkwan
University School of Medicine, Seoul 110-746, South Korea.
Received April 12, 2005;
accepted after revision June 24, 2005.
Address correspondence to S. K. Kim
(radi{at}lycos.co.kr).
WEB This is a Web exclusive article.
Keywords: Behçet's syndrome • digital subtraction angiography • interventional radiology • intrahepatic artery • liver
Introduction
Behçet's syndrome is a multisystem disorder characterized by
recurrent aphthous stomatitis, genital ulceration, and relapsing uveitis. It
most often affects men between 20 and 40 years old. The syndrome is most
prevalent in the Mediterranean region, Middle East, and Far East. The cause of
Behçet's syndrome is unclear
[1,
2]. Cardiovascular involvement
appears in only 7-29% of patients
[1,
2]. Vascular involvement of
Behçet's syndrome manifests as arterial occlusion, aneurysm, venous
occlusion, and varices [1,
2].
To our knowledge, spontaneous rupture of an intrahepatic artery aneurysm
has not been reported in patients with Behçet's syndrome. We describe a
case of spontaneous rupture of an intrahepatic artery aneurysm in a patient
with Behçet's syndrome that we successfully treated by performing
transcatheter arterial coil embolization.
Case Report
A 33-year-old man with Behçet's syndrome presented with right upper
abdominal pain that he had experienced for 1 day. He was admitted to our
hospital. There was no history of trauma. He was diagnosed with
Behçet's syndrome 3 years before at a different hospital, and the
diagnosis was confirmed by symptoms of recurrent oral and genital ulcerations
and relapsing uveitis. The patient did not have fever or chills. The initial
hemoglobin was 11.3 g/dL and the hematocrit was 34.1%.
Unenhanced and contrast-enhanced abdominal CT was then performed. The CT
images showed a large subcapsular hematoma of mixed attenuation at the lateral
and posterior aspects of the right lobe of the liver
(Fig. 1A). As a result, the
liver was compressed by the hematoma. There was a small high-attenuating focus
within the hematoma, compatible with extravasation of the contrast material
and active bleeding (Fig.
1B).
View larger version (116K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1A —33-year-old man with Behçet's syndrome. Contrast-enhanced
abdominal CT images show large hematoma (arrows) of mixed attenuation
at lateral and posterior aspects of right lobe of liver along with compression
of adjacent liver. Small high-attenuating focus (arrowhead, B)
is noted within hematoma, suggesting extravasation of contrast material and
active bleeding.
|
|
View larger version (120K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1B —33-year-old man with Behçet's syndrome. Contrast-enhanced
abdominal CT images show large hematoma (arrows) of mixed attenuation
at lateral and posterior aspects of right lobe of liver along with compression
of adjacent liver. Small high-attenuating focus (arrowhead, B)
is noted within hematoma, suggesting extravasation of contrast material and
active bleeding.
|
|
Angiography and embolization of the aneurysm were then planned. A 5-French
Yashiro type catheter (Glidecath, Terumo) was placed via the femoral artery
approach at the superior mesenteric artery (SMA). The SMA angiogram showed a
small aneurysm in the segment 6 branch of the right hepatic artery arising
from the SMA, and there was also extravasation of the contrast material
(Fig. 1C). The selective
hepatic angiogram that was obtained using a 2.4-French microcatheter
(Progreat, Terumo) clearly showed an approximately 7.8 x 3.0 mm
aneurysm. The 2.4-French microcatheter was replaced with a 2-French
microcatheter (Progreat) for the small diameter of the segment 6 branch of the
right hepatic artery and placed at the segment 6 branch of the right hepatic
artery. Incidentally, one microcoil migrated to the segment 7 branch of the
right hepatic artery during embolization, which may have been due to
inadequate advancement of the microcatheter to the segment 6 branch of the
right hepatic artery.
View larger version (159K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1C —33-year-old man with Behçet's syndrome. Superior mesenteric
artery (SMA) angiogram shows approximately 7.8 x 3.0 mm aneurysm
(arrow) in segment 6 branch of right hepatic artery arising from SMA,
and there is extravasation of contrast material (arrowhead).
|
|
After further advancement of the microcatheter to the segment 6 branch of
the right hepatic artery, the aneurysm was successfully embolized using two
2-mm microcoils (Hilal Coil, Cook). The hepatic and SMA angiograms obtained
after embolization of the segment 6 branch of the right hepatic artery with
microcoils showed that the aneurysm was completely occluded, and the
extravasation of contrast material was no longer seen. The incidentally
migrated coil was noted in the segment 7 branch of the right hepatic artery
(Fig. 1D).
View larger version (131K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1D —33-year-old man with Behçet's syndrome. SMA angiogram
obtained after embolization of segment 6 branch of right hepatic artery with
microcoils (arrow) shows that aneurysm is completely occluded, and
extravasation of contrast material is no longer seen. Incidentally migrated
coil (arrowhead) is seen in segment 7 branch of right hepatic
artery.
|
|
After 1 week, abdominal CT images showed a decreased amount of subcapsular
hematoma in the right liver, and there was no evidence of active bleeding. The
patient was discharged 1 day later. After 2 months, abdominal CT was performed
again. The CT images showed the greatly decreased size of the subcapsular
hematoma of the liver (Fig.
1E). Clinical follow-up at 1 year showed normal findings.
View larger version (130K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1E —33-year-old man with Behçet's syndrome. Contrast-enhanced
abdominal CT image obtained 2 months later shows that hepatic subcapsular
hematoma (arrow) is markedly decreased, and embolized microcoils
(arrowhead) are seen in segment 6 of liver.
|
|
Discussion
Behçet's syndrome is now recognized as a systemic disorder with
mucocutaneous, ophthalmic, neurologic, cardiovascular, pulmonary,
gastrointestinal, urogenital, and muscu-loskeletal involvement
[1,
2]. Any artery or vein of the
body may be affected, and the involvement of the vascular tree manifests
itself pathologically as arterial occlusion, aneurysm, venous occlusion, and
varices [1,
2]. In vascular involvement,
arterial lesions are less frequent than venous lesions, and the arterial
lesions account for only 12% of vascular complications in Behçet's
syndrome. The arterial lesions usually develop in the aorta and pulmonary
artery and in their major branches. An aneurysm is present in 65% of patients
with an arterial lesion and an occlusion in 35%
[1,
2].
Histologically, the vascular manifestation is a vasculitis of the vasa
vasorum of the large arteries and veins that causes wall compromise,
thrombosis, obstruction, and aneurysm formation
[3]. Perforation of the
arterial wall due to obliterative endarteritis of the vasa vasorum may result
in aneurysm formation or rupture
[3].
The most common site of aneurysm formation is the abdominal aorta followed
by the pulmonary, femoral, subclavian, popliteal, common carotid, coronary,
brachial, ulnar, common iliac, external iliac, tibial, renal, cerebral,
axillary, and splenic arteries
[1,
2]. Aneurysm of the visceral
arteries has been rarely reported
[4,
5]. There is one report of a
common hepatic artery aneurysm having a fistulous communication with the
superior mesenteric vein in a patient with Behçet's syndrome
[5]. However, an intrahepatic
artery aneurysm has not been reported in patients with Behçet's
syndrome.
Hepatic artery aneurysms constitute 20% of all visceral artery aneurysms
and approximately 20% of hepatic aneurysms are intrahepatic. They are caused
by atherosclerosis, vasculitis, septic emboli, iatrogenic injury, or trauma
[6]. In this case, the patient
had no history of recent or remote trauma. Therefore, vasculitis from
underlying Behçet's syndrome is the most likely cause of the hepatic
artery aneurysm.
Spontaneous rupture of an arterial aneu-rysm is the most common cause of
mortality in patients with Behçet's syndrome
[1,
2], and fast, aggressive
treatment for this malady is absolutely necessary. However, the walls of the
involved vessels or aneurysms in Behçet's syndrome are too fragile to
repair. Therefore, surgical repair of these aneurysms is often unsuccessful
and may result in new aneurysm formation and graft occlusion
[7].
Endovascular treatment is a reasonable alternative to prevent complications
or recurrences after surgical repair. Endovascular treatments such as
stent-graft or transcatheter arterial embolization have recently been reported
and they are considered to be safe and effective for the aortic and arterial
aneurysms seen in Behçet's syndrome
[8,
9].
The migration of one microcoil may have been due to inadequate advancement
of microcatheter to the segment 6 branch of the right hepatic artery. This
migration could have been prevented by using a guiding catheter to obtain more
secure purchase and advancement of the 5-French catheter into the SMA. No
sequelae from the migrated microcoil were seen on the follow-up CT scan, which
may have been due to adequate collateral arteries.
We report the successful treatment of an intrahepatic artery aneurysm with
transcatheter arterial coil embolization in a patient with Behçet's
syndrome.
References
- Koc Y, Gullu I, Akpek G, et al. Vascular involvement in Behcet's
disease. J Rheumatol 1992;19
: 402-410[Medline]
- Park JH, Han MC, Bettmann MA. Arterial manifestations of Behcet's
disease. AJR 1984;143
: 821-825[Abstract/Free Full Text]
- Matsumoto T, Uekusa T, Fukuda Y. Vasculo-Behcet's disease: a
pathologic study of eight cases. Hum Pathol1991; 22:45
-51[CrossRef][Medline]
- Men S, Ozmen MN, Balkanci F, Boyacigil S, Akbari H. Superior
mesenteric artery aneurysm in Behcet's disease. Abdom
Imaging 1994; 19:333
-334[Medline]
- Cekirge S, Gulsun M, Oto A, Dogan R, Balkanci F, Besim A.
Endovascular treatment of an unusual arterioportal fistula caused by the
rupture of a giant hepatic artery aneurysm into the superior mesenteric vein
in Behcet disease. J Vasc Interv Radiol2000; 11:465
-467[CrossRef][Medline]
- O'Driscoll D, Olliff SP, Olliff JF. Hepatic artery aneurysm.
Br J Radiol 1999;72
: 1018-1025[Abstract]
- Sasaki S, Yasuda K, Takigami K, Shiiya N, Matsui Y, Sakuma M.
Surgical experiences with peripheral arterial aneurysms due to
vasculo-Behcet's disease. J Cardiovasc Surg (Torino)1998; 39:147
-150[Medline]
- Kasirajan K, Marek JM, Langsfeld M. Behcet's disease: endovascular
management of a ruptured peripheral arterial aneurysm. J Vasc
Surg 2001; 34:1127
-1129[CrossRef][Medline]
- Park JH, Chung JW, Joh JH, et al. Aortic and arterial aneurysms in
Behcet disease: management with stent-grafts—initial experience.
Radiology 2001;220
: 745-750[Abstract/Free Full Text]
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
Top
Introduction
Case Report
