DOI:10.2214/AJR.06.0315
AJR 2007; 188:W506-W511
© American Roentgen Ray Society
Spontaneous Isolated Dissection of the Celiac Artery: CT Findings in Adults
Nicholas D'Ambrosio1,
Barak Friedman1,
David Siegel1,
Douglas Katz2,
Amit Newatia1 and
John Hines1
1 Department of Radiology, Long Island Jewish Medical Center, 270-05 76th Ave.,
New Hyde Park, NY 11040.
2 Department of Radiology, Winthrop-University Hospital, Mineola, NY.
Received March 2, 2006;
accepted after revision July 31, 2006.
Address correspondence to N. D'Ambrosio
(dambrosn{at}hotmail.com).
WEB
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Abstract
OBJECTIVE. Our objective was to describe the CT features of
spontaneous isolated celiac artery dissection in a series of six otherwise
healthy patients with acute abdominal pain.
CONCLUSION. Although once believed rare, isolated spontaneous celiac
artery dissection should be considered in the diagnosis of acute abdominal
pain, especially in middle-aged adults.
Keywords: abdominal imaging • cardiovascular imaging • celiac artery • CT angiography
Introduction
Arterial dissection is defined as cleavage of the arterial wall by an
intramural hematoma between two elastic layers
[1]. Isolated arterial
dissection, which occurs without aortic dissection, has been reported in
carotid and renal arteries but rarely in visceral arteries
[2]. Spontaneous dissection of
a visceral artery is an uncommon occurrence that is usually diagnosed after
fatal hemorrhage or ischemia
[1]. Only 11 cases of isolated
spontaneous celiac artery dissection have been reported, dating to 1959. We
report six cases of isolated spontaneous celiac artery dissection. To our
knowledge, this is the largest case series of isolated spontaneous celiac
artery dissection.
Isolated dissection of visceral arteries is rare and historically has
afforded a poor prognosis. The natural history is unpredictable, but
spontaneous resolution, definitive occlusion of a visceral artery, aneurysm
formation, or rupture can occur. The prognosis depends on the extent of
involvement of sub-segmental branches. Acute signs of bleeding or liver
ischemia are poor prognostic features
[3,
4]. Risk factors include
atherosclerotic disease, hypertension, fibromuscular dysplasia, cystic medial
necrosis, trauma, pregnancy, and connective tissue disorders
[5]. In most cases, however, no
cause is found. Epigastric pain and weight loss are cardinal symptoms, but the
lesions can be asymptomatic or manifest as obstructive jaundice, pancreatitis,
and intestinal angina [1].
Spontaneous celiac artery dissection is rare but may have been
underdiagnosed in the past. Although also considered rare, spontaneous
dissection of the superior mesenteric artery (SMA) is the most frequently
reported type of visceral artery dissection. Dissection of the SMA is
symptomatic more often than celiac artery dissection, contributing to the
difference in reported incidence. Fewer than 50 cases of spontaneous SMA
dissection have been reported to our knowledge. Patients with SMA dissection
are predominantly men (88% of cases) with an average age of 55 years (range,
45–87 years) [6]. These
sex and age predilections are nearly identical to those for spontaneous celiac
artery dissection, as reported in the literature and found in this series.
Materials and Methods
Within the span of less than 1 year (July 2004 through June 2005), six
patients arrived at our medical center with spontaneous dissection of the
celiac artery. All six patients were men, and the average age was 59 years
(range, 45–89 years). The most common symptom was abdominal pain. In one
case, abdominal pain was accompanied by lower gastrointestinal bleeding and in
another case by syncope. One patient had no symptoms. No relevant elements of
medical history were elicited from any patient. Two of the patients had
hypertension during the hospital admission. The absence of other vascular
lesions at initial presentation and a lack of risk factors for arterial
dissection suggested the occurrence of spontaneous dissection
[2]. Extensive evaluation of
all patients revealed no serologic evidence to support the presence of
vasculitis or an inflammatory disorder.
All six patients underwent 4- or 16-MDCT. In all cases, the diagnosis was
made by identifying an intimal flap on contrast-enhanced CT images (Fig.
1A,
1B,
1C). In two of the six
patients, this diagnosis was confirmed on selective angiography, which was
performed for preoperative planning for endovascular or surgical repair.
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Fig. 1A —45-year-old man with abdominal pain. Axial contrast-enhanced
CT scan through upper abdomen shows intimal flap (arrow) in celiac
trunk. Finding is consistent with celiac artery dissection.
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Fig. 1B —45-year-old man with abdominal pain. Coronal reformatted CT
scan shows celiac artery dissection (arrow) and normal caliber,
widely patent superior mesenteric artery (chevron) below it.
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Fig. 1C —45-year-old man with abdominal pain. Lateral projection from
selective celiac angiogram 7 days after A shows irregular filling
defect (arrow) corresponding to thrombosed false lumen not seen on
initial CT.
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Results
Complications
We found a spectrum of CT features of spontaneous dissection of the celiac
artery (Table 1). Four of the
six patients had vascular complications, none of which was life-threatening.
In three of the six patients, dissection extended into adjacent splenic and
proximal hepatic arteries (Fig.
2A,
2B). Viscera were affected in
two of six patients. One patient had splenic infarction 6 days after initial
presentation (Fig. 3A,
3B).
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Fig. 2A —56-year-old man with bilateral lower-quadrant pain. Initial
diagnosis was uncomplicated isolated celiac artery dissection. Follow-up CT
scan 5 days after initial diagnosis shows extension of dissection from celiac
trunk into proximal hepatic artery (arrow).
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Fig. 2B —56-year-old man with bilateral lower-quadrant pain. Initial
diagnosis was uncomplicated isolated celiac artery dissection. Axial
maximum-intensity-projection image shows extension of dissection from celiac
trunk into proximal hepatic artery, which is markedly attenuated by thrombosed
false lumen (star).
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Fig. 3A —45-year-old man with vague abdominal pain and isolated celiac
artery dissection. Coronal oblique CT multiplanar reformatted image 6 days
after initial study shows extension of dissection into proximal splenic artery
(arrow).
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Fig. 3B —45-year-old man with vague abdominal pain and isolated celiac
artery dissection. Axial CT image from same examination as A shows
splenic infarct (chevron) due to dissection into proximal splenic
artery (arrow).
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A second patient experienced bilateral renal infarction 5 days after
initial presentation (Fig. 4A,
4B). Although the aorta and
main renal arteries were patent, there was evidence of new dissection
involving the distal left renal artery and of probable distal small-vessel
dissection on the right. The creatinine level was not compromised.
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Fig. 4A —56-year-old man with initial diagnosis of isolated celiac
artery dissection. Initial CT showed normal homogeneous enhancement of both
kidneys. CT scan obtained 5 days after initial CT examination shows new distal
left renal artery dissection (arrow).
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Fig. 4B —56-year-old man with initial diagnosis of isolated celiac
artery dissection. Initial CT showed normal homogeneous enhancement of both
kidneys. CT scan from same examination as A shows renal infarct
(arrow). Normal widely patent aorta shows no evidence of dissection
or thrombus.
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The final complication, encountered in one of the six patients in this
series, was development of a new left hepatic artery aneurysm, which measured
1 cm in diameter. This vascular abnormality developed within 2 months after
initial presentation (Fig. 5).
Initial CT had shown isolated celiac dissection without evidence of extension
into adjacent vessels. The cause of this finding is unclear, but it may
represent a genetic predisposition toward arterial wall weakening.
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Fig. 5 —56-year-old man with initial diagnosis of isolated celiac
dissection without evidence of extension into adjacent vessels. Axial
contrast-enhanced CT scan shows 1-cm aneurysm (arrow) of left hepatic
artery not found on initial CT scans 2 months earlier.
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Associated Findings
A number of associated imaging findings were present at initial
presentation. The first imaging finding in two of the six patients was
infiltration of the fat surrounding the celiac axis artery. Increased
attenuation of the surrounding fat has been described in isolated dissection
of the SMA [7]. This finding
was visualized in a 56-year-old man who arrived in the emergency department
with abdominal pain. Unenhanced CT was performed to rule out renal stone. The
findings were essentially normal except for nonspecific inflammatory
infiltration of the fat surrounding the celiac axis (Fig.
6A,
6B). This nonspecific finding
along with patient's unexplained abdominal pain warranted hospital admission.
Contrast-enhanced CT performed the next day showed dissection of the celiac
trunk.
Another significant associated finding in three of the six patients was
celiac artery aneurysm, itself a rare entity. Celiac artery aneurysms are
usually caused by atherosclerosis. Infection and trauma also are reported
causes. In rare instances, the aneurysm is a complication of isolated
dissection of the celiac artery
[8]. Patients may have
abdominal pain, as in this case, or the finding can be incidental. In most
cases, surgery is the desired treatment for avoiding rupture and thrombosis
[9].
Only one of three patients with an associated celiac artery aneurysm had CT
evidence of atherosclerosis elsewhere. There was no history of trauma or
infection. The first patient was a 56-year-old man with a patellar fracture
who presented with an episode of syncope followed by back and abdominal pain.
CT pulmonary angiography performed in the emergency department showed no
evidence of pulmonary embolus. However, focal dissection of the celiac artery
and an associated celiac artery aneurysm measuring 1.4 cm were identified
(Fig. 7A,
7B,
7C). In this case, celiac
dissection was associated with occlusion of the splenic artery. However,
splenic perfusion was normal, presumably through collateral vessels.
Therefore, the celiac artery dissection likely preceded the splenic artery
occlusion and was probably chronic. It is likely that discovery of chronic
asymptomatic forms of celiac artery dissection will be increasingly frequent
with the growing use of noninvasive imaging
[3].
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Fig. 7A —56-year-old man with patellar fracture and syncope followed
by back and abdominal pain. Contrast-enhanced axial CT pulmonary angiogram
shows focal celiac artery dissection and associated celiac artery aneurysm
measuring 1.4 cm.
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Fig. 7B —56-year-old man with patellar fracture and syncope followed
by back and abdominal pain. Three-dimensional volume-rendered reformatted
image shows narrowing of proximal celiac trunk (arrow), which
represents true lumen.
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Only one case of isolated dissection of the celiac artery was found
incidentally, and it was presumed asymptomatic. The patient was a 62-year-old
man undergoing CT to rule out metastatic disease. Also found was an associated
celiac artery aneurysm measuring 1.4 cm. Associated celiac artery aneurysm
also was found in an 89-year-old man with abdominal pain and lower
gastrointestinal bleeding. The celiac artery dissection and aneurysm were the
only significant abnormalities found during the examination. The aneurysm
measured 1.5 cm in maximum diameter (Fig.
8A,
8B).
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Fig. 8A —89-year-old man with abdominal pain and lower
gastrointestinal bleeding. Contrast-enhanced axial (A) and sagittal
reformatted (B) CT images show celiac artery aneurysm (arrow)
measuring 1.5 cm. Portion of intimal flap of celiac dissection is evident
immediately proximal to aneurysm in celiac trunk.
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Fig. 8B —89-year-old man with abdominal pain and lower
gastrointestinal bleeding. Contrast-enhanced axial (A) and sagittal
reformatted (B) CT images show celiac artery aneurysm (arrow)
measuring 1.5 cm. Portion of intimal flap of celiac dissection is evident
immediately proximal to aneurysm in celiac trunk.
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Management
Given the small number of cases reported in the literature, predicting the
course of celiac artery dissection and prognosis is difficult. Five of six
patients in this series were admitted to the hospital for vascular surgery and
interventional radiology consultation. One patient was treated conservatively
as an outpatient. None of the patients had clinical or laboratory evidence of
bowel ischemia, and all were treated conservatively without surgical or
endovascular intervention. Extensive evaluation of all patients to determine
the cause was inconclusive. All patients admitted were discharged without
symptoms after medical therapy. The average duration of the hospital stay was
7 days. Short-term follow-up imaging was performed for all but one patient,
who at 89 years was the oldest patient in the series. For the five middle-aged
patients, initial follow-up CT was performed an average of 1 week after
hospital discharge. The second follow-up CT examination was performed
2–6 months after the first. Four of the five patients who underwent
follow-up CT had no interval change compared with the last CT findings before
discharge. One of the five patients underwent follow-up at an outside
facility. The average length of imaging follow-up in this series was 9 months
(range, 7–14 months).
Medical treatment included antihypertensive drugs, antiinflammatory drugs,
steroids, and anticoagulants. Treatment options previously reported in the
literature included emergency surgical repair and endovascular repair. The
surgical repair was resection and anastomosis in one case and prosthetic
bypass to the hepatic artery in two cases
[3]. In addition, a case of
iatrogenic celiac artery dissection that occurred during transcatheter
arterial chemoembolization was immediately successfully recanalized with
balloon fenestration of the intimal flap
[10]. Endovascular treatment
with a stent is an attractive option for patients at high surgical risk,
although data on its efficacy compared with surgery are not conclusive
[11].
Although isolated SMA dissection is a rare event, there is more information
in the medical literature regarding this entity and its management than there
is on isolated celiac artery dissection. Of the approximately 50 cases
reported, most were managed surgically or the patient died before surgery.
Percutaneous stent placement has been described in the literature as a safe
and feasible therapeutic alternative in the management of isolated spontaneous
dissection of the SMA [12].
Studies with larger groups of patients and long-term follow-up are needed to
validate this procedure. It is likely that the significantly increased need
for surgical intervention in patients with SMA dissection as opposed to celiac
artery dissection lies in the more essential role of the SMA in bowel
perfusion.
According to Chaillou et al.
[1], surgical reconstruction is
mandatory for symptomatic forms of celiac artery dissection to manage the
lesion and obtain a definitive histologic diagnosis. However, all six of our
patients were treated conservatively with medical therapy and close
surveillance.
Discussion
Isolated spontaneous celiac artery dissection is a rare entity. We found
only 11 cases in the literature. To our knowledge, ours is the largest case
series of isolated spontaneous celiac artery dissection to date. The initial
manifestation is usually abdominal pain. The average age of patients is
approximately 55 years, and men outnumber women in a ratio of 5:1. The
reported risk factors include hypertension, cystic medial necrosis, abdominal
aortic aneurysm, fibromuscular dysplasia, trauma, pregnancy, and connective
tissue disorders. Most of the patients in our series, however, were generally
healthy with no underlying disease
[12].
Contrast-enhanced CT is considered the primary technique for diagnosing
celiac artery dissection; however, MR angiography, sonography, and
conventional angiography also can be used
[3]. Celiac artery dissection
can be accompanied by celiac artery aneurysm
[2]. Diagnostic imaging
findings on CT include an intimal flap, which was found in all patients in
this series and is pathognomonic, or eccentric mural thrombus in the celiac
lumen, which should raise suspicion for dissection. Because the intimal flap
is not always visible, mural thrombus may be the only clue to the presence of
dissection. In such instances, misdiagnosis of dissection as thromboembolic
occlusion can lead to unnecessary pharmacologic thrombolysis
[12].
We found several vascular complications, including propagation of
dissection into adjacent vessels leading to splenic and renal infarcts and, in
one patient, development of a hepatic artery aneurysm. Why this aneurysm
formed is unclear, but it may represent a genetic predisposition toward
arterial wall weakening.
In two of the six patients, we found infiltration of the fat surrounding
the celiac axis as a secondary sign of acute spontaneous celiac artery
dissection. The dissection in both patients was complicated by propagation and
visceral infarction. Although a small cohort of patients is described, this
finding may be predictive of the acuity of dissection and predisposition
toward extension of dissection into adjacent vessels. In four patients without
infiltration of the periceliac fat, it was difficult to determine the age of
the dissection. Dissection was suspected of being acute in three of four
patients because they reported acute abdominal pain, and no alternative
clinical or imaging diagnosis was established.
Although the risk of bowel ischemia associated with celiac artery
dissection is less than that associated with SMA dissection, surgical repair
may still be considered for patients with celiac artery dissection. Surgery
and endovascular procedures are indicated when a patient in hemodynamically
unstable condition has persistent abdominal pain, when medical therapy fails
to control blood pressure, and when dissection is progressing
[11]. Most patients described
in the literature underwent surgical repair, which limits us in drawing
conclusions about the natural course of celiac artery dissection
[3]. Some patients with
uncomplicated asymptomatic lesions may be eligible for medical treatment and
regular imaging surveillance
[3], as was found in our
series. Although our patients underwent follow-up for only 1 year, all
continued to be symptom free without endovascular or surgical repair.
To prevent thromboembolic complications, therapy with anticoagulant or
antiplatelet agents for 3–6 months with a target international
normalized ratio of 2.0–3.0 with strict blood pressure control
[11,
13] has been suggested.
Modification of cardiac risk factors limits propagation of the dissection and
reduces the risk of rupture.
Clinicians must be aware of the possibility of spontaneous isolated
dissection of the celiac artery in patients with postprandial abdominal pain.
Vascular surgery and interventional radiology consultations are warranted as a
safeguard for potential surgical or endovascular intervention
[4]. This lesion is a rare
cause of abdominal pain, usually found in middle-aged men. In most cases the
cause of dissection is unknown. CT has become the imaging technique of choice
for establishing the diagnosis. The optimal treatment has not been established
but may involve surveillance and surgical or endovascular repair, depending on
the clinical features. Surgical or endovascular treatment may be reserved for
patients with persistent signs of ischemia despite adequate anticoagulation or
those with uncontrolled hypertension or progression of dissection
[11].
Spontaneous celiac artery dissection is rare, but the incidence may have
been underestimated in the past. Continued improvement of cross-sectional
imaging has facilitated the diagnosis of this lesion, which should be
considered in the differential diagnosis of abdominal pain in middle-aged
men.
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Abstract
Introduction
