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A Compressed Celiac Artery May Lead to High-Risk

Surgery
Allison J. Porter, MD; Robert B. Yates, MD; Joshua M. Mourot, MD; Brant K. Oelschlager, MD

September 11, 2015

Case Presentation

A 44-year-old woman presented with chronic abdominal pain, which was epigastric. In terms of intensity,
it varied from a constant dull pain that the patient rated as 7 out of 10 on a visual analogue scale to a
sharp pain after meals that she rated as 10 out of 10. She was not taking narcotic pain medications.

Over the course of 1 year, the patient experienced an involuntary 23-lb weight loss. She had undergone
upper endoscopy, colonoscopy, hepatobiliary (HIDA) scan, abdominal ultrasound, and upper
gastrointestinal fluoroscopy, all of which were normal except for mild nonspecific gastric erythema and a
fatty liver. A mesenteric duplex demonstrated a peak celiac expiratory velocity of 371 cm/sec and a peak
inspiratory velocity of 283 cm/sec. CT angiography of the abdomen was normal, with the exception of
extrinsic narrowing of the celiac axis (Figure 1).
Figure 1. Narrowing of the celiac axis, shown in the sagittal view.

High Prevalence, but Also Rare

Celiac artery compression syndrome, also referred to as "median arcuate ligament syndrome" and
"Dunbar syndrome," is a clinical condition in which extrinsic compression of the celiac artery and celiac
plexus is associated with abdominal symptoms, such as epigastric pain, nausea, emesis, and weight
loss.

The most common cause of celiac artery compression is the median arcuate ligament, the band of tissue
that connects the left and right crus of the diaphragm posterior to the esophageal hiatus. Normally, the
celiac artery originates below the median arcuate ligament. However, variability in the anatomy of the
celiac artery and the median arcuate ligament has been reported.[1] Compression of the celiac artery can
result from either a low-lying median arcuate ligament or a celiac artery that exits more cephalad on the
aorta. In the general population, celiac artery compression has been found in 10%-24% of patients on CT
analysis[2] and in up to 33% of individuals in an autopsy study.[3]

Despite the relatively high prevalence of celiac artery compression, clinically relevant celiac artery
compression syndrome is rare. Discordance between the presence of anatomical compression and
clinically significant symptoms has led some to question whether the clinical syndrome actually exists.
[4]
 In addition, symptoms of celiac artery compression syndrome are typically chronic and nonspecific,
including vague upper abdominal pain, nausea, and emesis. In patients with long-standing symptoms,
weight loss may occur owing to decreased oral intake in an attempt to mitigate postprandial symptoms.

Physical examination is generally unremarkable. Careful auscultation may identify an epigastric bruit, but
focal abdominal tenderness and peritoneal signs are absent. Patients with celiac artery compression
syndrome frequently undergo multiple extensive clinical evaluations before an astute clinician considers
this diagnosis.

An Unclear Pathophysiology

The pathophysiology of celiac artery compression syndrome is not fully understood. There may be either
a vascular or a neurogenic component to this disease, or both. Historically, the vascular component has
been described as compression of the celiac artery by the median arcuate ligament that results in
decreased blood flow through the celiac artery. Owing to extensive collateralization of the splanchnic
vasculature, however, symptomatic chronic mesenteric ischemia typically only develops after two of the
three mesenteric vessels are completely occluded or severely stenotic. In almost all patients with this
disease, the superior and inferior mesenteric arteries are widely patent.

Nevertheless, there is evidence that gastric ischemia can occur in patients with isolated celiac artery
compression.[5,6] Mensink and colleagues[7] advocate for performing gastric exercise tonometry as part of
the evaluation of patients with suspected celiac artery compression syndrome.

If mesenteric ischemia was the only cause of this syndrome, revascularization should result in complete
symptom resolution. However, celiac revascularization alone rarely results in clinical improvement in
symptoms, which suggests a concomitant nonvascular mechanism to this disease.

The second underlying mechanism is thought to be pathologic stimulation of the celiac ganglion and
neural plexus by a low-lying median arcuate ligament. The celiac plexus contains inhibitory motor fibers
to the stomach and pain fibers. These neural structures are intimately related to the celiac artery at its
exit from the aorta, and their compression has been implicated in chronic pain due to locally advanced
pancreatic cancer.[8] Celiac ganglion block has similarly been shown to improve pain, with some authors
suggesting it be performed to identify patients who may benefit from further management.[9]

Until recently, management of celiac artery compression syndrome has been achieved through open
surgical release of the median arcuate ligament. This technique restores normal vascular flow through
the celiac artery and relieves compression of the associated neural structures. In some rare cases when
median arcuate ligament release fails to relieve vascular stenosis, concomitant celiac artery
revascularization may be necessary.

Recently, the use of minimally invasive surgical and endovascular techniques has been reported.
[2,10]
 Despite these advances in operative technique, clinical improvement in symptoms is highly variable
and difficult to predict.

In this review, we elaborate on the clinical diagnosis and surgical management of celiac artery
compression syndrome, with emphasis on our operative technique of laparoscopic median arcuate
ligament release.
The operation to treat median arcuate ligament syndrome is high risk, and the symptoms of this condition
(eg, abdominal pain, nausea) are very nonspecific. Therefore, it is of paramount importance to rule out
more common disorders before considering this diagnosis as the source of these symptoms.

Celiac artery ultrasound can evaluate the flow through the celiac axis and therefore provide an estimation
of the degree of narrowing of this vessel.

CT angiography is also helpful but exposes the patient to both radiation and contrast. It can provide a
better estimation of anatomy in patients with a compromised [difficult-to-interpret] ultrasound, which may
be a result of such anatomical factors as body habitus or bowel gas.

Arteriography may be used but exposes the patient to radiation, contrast, and the risks of an invasive
procedure, and therefore is not a typical first step.

Diagnostic laparoscopy is not helpful in the diagnosis of this syndrome.

Patient Selection and Preoperative Evaluation

Evaluation of a patient with suspected median arcuate ligament syndrome is carried out jointly between a
vascular and general surgeon.

Although saying that there is a "typical" patient with this syndrome is misleading, it is true that patients
often present with epigastric or diffuse abdominal pain that worsens after eating, in addition to nausea,
vomiting, and weight loss.

Age at diagnosis is variable, but patients tend to present with symptoms similar to those of mesenteric
ischemia, albeit at a younger age than the typical ischemic patient.

Owing to the difficulty in diagnosis, an extensive workup, including endoscopy, ultrasound, CT, and MRI,
and operations, such as diagnostic laparoscopy, lysis of adhesions, or cholecystectomy, have sometimes
been performed in an attempt to address the symptoms. In one series looking at persons with median
arcuate ligament syndrome, 10 out of 15 patients had undergone prior abdominal operations without
symptom resolution.[11] Although weight loss may not be found on history in every presentation, patients
tend to be thin, with a flat, nontender abdomen.

Once celiac artery compression syndrome is suspected, celiac artery ultrasound may be helpful for
evaluation of baseline, peak inspiratory, and peak expiratory velocities. Should these velocities be
elevated, subsequent evaluation with magnetic resonance angiography or CT angiography can be
pursued.

Alternatively, the diagnosis may be arrived at without ultrasound, on the basis of symptoms in
conjunction with a CT angiogram suggesting significant narrowing at the celiac origin. However, it is
important to remember that reports looking at healthy individuals undergoing CT for other reasons
indicate that anywhere from 10% to 60% of patients without any symptoms may show stenosis at the
celiac origin.[11]

The diagnosis is ultimately one of exclusion, based on both clinical findings and evidence gleaned from
various imaging modalities, such as CT angiography, magnetic resonance angiography, and duplex or
formal arteriography.

Accordingly, the patient in this case had undergone an extensive workup, with all other possible
etiologies considered for her abdominal symptoms. Because her findings were essentially normal, and
her CT angiogram and abdominal vascular duplex were consistent with celiac artery compression
syndrome, celiac artery compression syndrome was diagnosed and operative management was
pursued.
Patients with celiac artery compression syndrome typically have involuntary weight loss, a baseline
celiac velocity greater than 200 cm/m2, and stenosis of the celiac artery on CT angiography.

Gray stool and hepatosteatosis are not typical of this syndrome. When a HIDA scan is performed, it is
frequently normal.

Celiac artery narrowing may be incidentally seen on imaging in 10%-60% of asymptomatic individuals
and is not an indication for laparoscopic median arcuate ligament release.

Operative Technique

Video Clip: Laparoscopic median arcuate ligament release for celiac artery compression syndrome.

This feature requires the newest version of Flash. You can download it here.

The consequences of error in performing laparoscopic median arcuate ligament release can be
catastrophic; therefore, we recommend that a vascular surgeon be involved or immediately available.

During surgery, the patient is placed in either a low lithotomy or a split-leg position with both arms tucked.
A Foley catheter and adequate intravenous access are obtained, again with the knowledge that if injury
to the aorta or its branches were to occur, it would require a significant, immediate need for
administration of fluid or blood.

Deep venous thromboprophylaxis and preoperative antibiotics are administered. Four ports are placed in
the upper abdomen to accommodate for the right and left hands of the surgeon, a camera, and the right
hand of the assistant. A retractor is placed to elevate the left lateral segment of the liver, exposing the
caudate lobe.

The gastrohepatic ligament is divided, and the right crus of the diaphragm is exposed. The coronary vein
is identified and elevated to facilitate identification of the left gastric artery. Once this vessel has been
exposed, a loop is placed around it for retraction. The surrounding tissue is dissected to expose the
trifurcation of the celiac artery, and dissection proceeds along the celiac artery to expose the median
arcuate ligament. In the case demonstrated in the video, a vessel loop is placed around the common
hepatic artery, because retraction held here flattens the plane of the celiac axis to facilitate its dissection.

The first fibers of the median arcuate ligament are divided and may be somewhat firm, requiring some
force to be applied for division. Cautery is generally avoided throughout the case when working
immediately adjacent to the wall of any artery. When cautery is necessary, the tissue to be divided is
retracted as far from the artery as possible, and very small fibers are divided sequentially. Fibers are
divided in a superficial-to-deep and inferior-to-superior fashion. Intraoperative ultrasound is not used.

All fibers overlying the celiac artery are completely removed using careful technique. This includes the
fibers of the celiac plexus, which may contribute to the pathophysiology of this disease and, as a result,
the relief of symptoms after the procedure. The trifurcation of the celiac artery is seen. Post-stenotic
dilation can be seen on the video and is indicated by a yellow star. Vessel loops are removed at the
completion of the case.

Although this approach begins with identification of the branches of the celiac axis and the dissection
proceeds proximally along the vessels, it is also possible to approach the aorta first with dissection
distally.[11] To do so, the fibers of the right crura are divided longitudinally to expose the abdominal aorta.
The assistant retracts these fibers to the patient's left and the surgeon to the patient's right. The
dissection continues distally on the aorta to expose the junction of the aorta and celiac trunk. The fibers
overlying the celiac artery are then divided, ideally in a circumferential fashion when feasible. When the
celiac artery has been fully released and the neurovascular bundles divided as confirmed by visual
inspection, the operation is considered to be complete (Figure 2).

Figure 2. The complete dissection demonstrates the aorta (Ao), celiac artery (CA), common hepatic
artery (CHA), left gastric artery (LGA), and splenic artery (SplA).

Postoperative Care

The patient is admitted to the hospital postoperatively for overnight monitoring and may resume a regular
diet immediately. In the absence of complications and adequate oral intake, the patient may be
discharged from the hospital the next day.

Typically, patients are seen once in the clinic for follow-up to assess healing of incisions and effect on
symptoms. Some surgeons, in particular those who believe the pathophysiology of this disease stems
from a vascular rather than neurologic etiology, may choose to perform repeat abdominal vascular
duplex studies; however, this is not our standard of practice. Approximately 75% of patients experience
relief of symptoms.[11,12]

The patient in this case underwent the operation, as described above, without complication. She was
admitted to the hospital postoperatively, her diet was advanced, and she was discharged the following
day. At her postoperative follow-up clinic appointment, she reported complete resolution of her
abdominal pain.

Summary

Celiac artery compression syndrome is a complex condition without a concrete pathophysiology and
remains a diagnosis of exclusion in patients with chronic abdominal pain, nausea, emesis, and weight
loss. However, with proper patient selection, symptoms may dramatically improve postoperatively, with
significant increase in quality of life.

Laparoscopic median arcuate ligament release is a very high-risk operation and should only be
performed by a surgeon with substantial experience with laparoscopic approaches to the diaphragmatic
hiatus, and with immediate access to a vascular surgeon.

Editor's Recommendations

 Laparoscopic Release of Celiac Artery Compression Syndrome

 Giant Celiac Artery Aneurysm with Associated Visceral Occlusive
Disease

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