Celiac artery compression syndrome managed by laparoscopy.

Abstract

Celiac artery compression syndrome (CACS) is an unusual condition caused by abnormally low insertion of the median fibrous arcuate ligament and muscular diaphragmatic fiber resulting in luminal narrowing of the celiac trunk. Surgical treatment is the release of the extrinsic compression by division of the median arcuate ligament overlying the celiac axis and skeletonization of the aorta and celiac trunk. The laparoscopic approach has been recently reported for single cases. Percutaneous transluminal angioplasty (PTA) and stenting of the CA alone, before or after the surgical relief of external compression to the celiac axis, has also been used. We report our 7-year experience with the laparoscopic management of CACS caused by the median arcuate ligament.
Between July 2001 and May 2008, 16 patients (5 men; mean age, 52 years) were treated. Diagnosis was made by duplex ultrasound scan and angiogram (computed tomography [CT] or magnetic resonance). The mean body mass index of the patients was 21.2 kg/m(2). One patient underwent laparoscopic surgery after failure of PTA and stenting of the CA, and two patients after a stenting attempt failed.
All procedural steps were laparoscopically completed, and the celiac trunk was skeletonized. The laparoscopic procedures lasted a mean of 90 minutes. Two cases were converted to open surgery for bleeding at the end of the operation when high energies were used. The postoperative course was uneventful. Mean postoperative hospital stay was 3 days. On follow-up, 14 patients remained asymptomatic, with postoperative CT angiogram showing no residual stenosis of the celiac trunk. One patient had restenosis and underwent aortoceliac artery bypass grafting after 3 months. Another patient had PTA and stenting 2 months after laparoscopic operation. All patients reported complete resolution of symptoms at a mean follow-up of 28.3 months.
The laparoscopic approach to CACS appears to be feasible, safe, and successful, if performed by experienced laparoscopic surgeons. PTA and stenting resulted in a valid complementary procedure only when performed after the release of the extrinsic compression on the CA. Additional patients with longer follow-up are needed.

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Celiac artery compression syndrome managed by
laparoscopy
Paolo Baccari, MD,aEfrem Civilini, MD,bLaura Dordoni, MD,bGermano Melissano, MD,b
Roberto Nicoletti, MD,cand Roberto Chiesa, MD,bMilan, Italy
Objective: Celiac artery compression syndrome (CACS) is an unusual condition caused by abnormally low insertion of the
median fibrous arcuate ligament and muscular diaphragmatic fiber resulting in luminal narrowing of the celiac trunk.
Surgical treatment is the release of the extrinsic compression by division of the median arcuate ligament overlying the
celiac axis and skeletonization of the aorta and celiac trunk. The laparoscopic approach has been recently reported for
single cases. Percutaneous transluminal angioplasty (PTA) and stenting of the CA alone, before or after the surgical relief
of external compression to the celiac axis, has also been used. We report our 7-year experience with the laparoscopic
management of CACS caused by the median arcuate ligament.
Methods: Between July 2001 and May 2008, 16 patients (5 men; mean age, 52 years) were treated. Diagnosis was made by
duplex ultrasound scan and angiogram (computed tomography [CT] or magnetic resonance). The mean body mass index
of the patients was 21.2 kg/m2. One patient underwent laparoscopic surgery after failure of PTA and stenting of the CA,
and two patients after a stenting attempt failed.
Results: All procedural steps were laparoscopically completed, and the celiac trunk was skeletonized. The laparoscopic
procedureslastedameanof90minutes.Twocaseswereconvertedtoopensurgeryforbleedingattheendoftheoperation
when high energies were used. The postoperative course was uneventful. Mean postoperative hospital stay was 3 days. On
follow-up, 14 patients remained asymptomatic, with postoperative CT angiogram showing no residual stenosis of the
celiac trunk. One patient had restenosis and underwent aortoceliac artery bypass grafting after 3 months. Another patient
had PTA and stenting 2 months after laparoscopic operation. All patients reported complete resolution of symptoms at
a mean follow-up of 28.3 months.
Conclusions: The laparoscopic approach to CACS appears to be feasible, safe, and successful, if performed by experienced
laparoscopic surgeons. PTA and stenting resulted in a valid complementary procedure only when performed after the
release of the extrinsic compression on the CA. Additional patients with longer follow-up are needed. (J Vasc Surg 2009;
50:134-9.)
Celiac artery compression syndrome (CACS) is an un-
usual disease associated with chronic postprandial abdom-
inal pain, epigastric bruit, and weight loss. The syndrome
occurs when the median arcuate ligament (MAL) of the
diaphragm,afibrousarchcrossingtheaortacephaladtothe
proximal portion of the celiac artery that has a relatively low
insertion, causes extrinsic compression and narrowing of
the CA. Controversies still exist about several aspects of the
syndrome, including the pathophysiologic origin of the
symptoms, diagnosis, and the optimal treatment and man-
agement.
Traditional surgical therapy is the division, through a
midline laparotomy, of the anomalous fibrous diaphrag-
matic bands overlying the CA, along with the celiac plexus
and lymphatic tissues. Arterial bypass surgery is another
treatment option. Percutaneous transluminal angioplasty
(PTA) and stenting of the CA alone, before or after the
surgical relief of external compression to the celiac axis, has
also been used.1
In recent years, a successful laparoscopic approach to
the treatment of this disorder by laparoscopic division of
the MAL has been reported in single cases. Here we de-
scribe the clinical manifestations and minimally invasive
treatment of 16 consecutive patients with CACS operated
on by laparoscopic technique at our hospital. To our
knowledge, this is the first report of a group of consecutive
patients with CACS treated with a laparoscopic approach.
PATIENTS AND METHODS
From 2002 to 2008, 53 patients with a suspected
chronic mesenteric ischemia came up to our attention; of
whom 16 (11 women, 5 men) were diagnosed with CACS
and were prospectively assigned to laparoscopic treatment.
These patients had a mean age of 54 years (range, 29-75
years) and had mean body mass index of 21.1 kg/m2
(range, 17.8-27.0 kg/m2). Detailed clinical characteristics
of the patients are summarized in the Table. All the patients
had a history of symptoms, including abdominal pain,
weight loss, bloating, and vomiting, variously combined
and sometimes associated with an abdominal bruit. The
average duration of complaints before diagnosis was 26
months (range, 3-120 months).
As part of the search for more common causes of upper
abdominal pain, investigations such as upper gastrointesti-
nal series, upper endoscopy, or abdominal ultrasound (US)
imaging were performed in most patients, without conclu-
From the Department of General Surgery,aVascular Surgery,band Radi-
ology,cScientific Institute San Raffaele University Hospital.
Competition of interest: none.
Reprint requests: Paolo Baccari, MD, Scientific Institute San Raffaele Uni-
versity Hospital, Via Olgettina 60, 20132 Milan, Italy (e-mail: paolo.
baccari@hsr.it).
0741-5214/$36.00
Copyright © 2009 by the Society for Vascular Surgery.
doi:10.1016/j.jvs.2008.11.124
134

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Author's personal copy
sive results. All patients underwent a diagnostic work-up
that included basal and dynamic tests (inspiratory and
expiratory) with duplex US (DUS)2scanning associated
with computed tomography angiography (CTA). At the
beginning of our experience, three patients were also stud-
ied with angiography together with magnetic resonance
angiography (MRA).
Diagnosis of CACS and indication to treatment
was made in the presence of two or more of the above-
mentioned abdominal symptoms and signs, together with
radiologic findings of an eccentric stenosis of the celiac
trunk at basal scan, varying with respiration acts. A close
relation of the celiac trunk with the diaphragmatic crux was
also evident at a sagittal view in all cases.
A predischarge evaluation with dynamic DUS imaging
was performed in all patients. Our follow-up protocol
included clinical evaluation together with inspiration and
expiration abdominal DUS scanning and CTA at 2 months
after the procedure, then DUS imaging alone at 6 months
and yearly thereafter.
Operative technique. All the laparoscopic procedures
were performed by the same author (P. B.), and a vascular
team was always readily available. The patients were placed
under general anesthesia in the supine position, in steep
reverse Trendelenburg with the legs apart. A nasogastric
tube was placed. For 11 operations, four ports were re-
quired (Fig 1); in five procedures, a fifth port was inserted.
The first step of the operation was a thorough exam-
ination of the peritoneal cavity, always excluding other
intra-abdominal diseases. The avascular region of the
gastrohepatic omentum was then divided, and the right
crus was isolated inferiorly to the cardia. A fifth port can
be used for a grasper to retract to the left the stomach to
gain better exposure of the aortoceliac region. In six
patients, the left gastric artery was looped and retracted
inferiorly to the left.
The dissection was conducted posteriorly to the esoph-
agus, and the crural decussation was identified and isolated.
The muscular fibers of the crural decussation were divided,
and the anterior surface of the aorta was exposed. The CA
Table. Characteristics of patients with celiac artery compression syndrome caused by the median arcuate ligament
PatientSex
Age
(y)Symptoms
Complaints
duration
(mon)
Associated pathologies
Vascular anomaliesBMI
FU
(mon)
Conversion
(open surg)
Associated
procedures
1F45Postprandial pain
Exercise-induced pain
Weight loss (10 kg)
Postprandial pain
Weight loss (3 kg)
Vomiting
Postprandial pain
Epigastric pain
Epigastric bruit
Postprandial pain
Postprandial pain
Weight loss (7 kg)
Postprandial pain
Weight loss (14 kg)
120No18.5 83 No No
2M 30 12 Crohn disease
Visceral art aneurysm
20.250 NoNo
3
4
M
F
64
54
12
24
Aortic atherosclerosis
Gastritis
26.3
19.4
47
45
No
No
No
No
5
6
M
F
75
61
24
24
Cardiovascular history
Anxious syndrome
MV insufficiency
Chronic HCV hepatitis
Hypertension
Cerebral aneurysm
Susp. celiac disease
26.4
20.3
43
38
No
No
Pre-op CA stent
No
7M 632027.0 28 NoPre-op CA stent
attempt
8F 45 Postprandial pain
Weight loss (10 kg)
Epigastric bruit
Postprandial pain
Weight loss (4 kg)
Epigastric pain
Weight loss (4 kg)
Epigastric bruit
Postprandial pain
Bloating
Postprandial pain
Postprandial pain
Weight loss (12 kg)
Epigastric bruit
Nausea
Weight loss (5 kg)
Epigastric bruit
Postprandial pain
Postprandial pain
Weight loss (7 kg)
2419.225 NoNo
9F 2918 No18.5 25 YesNo
10F 643 Hysterectomy18.0 19No Post-op CA
stent
11F5112Anxious syndrome
GE reflux
21.215Yes No
12
13
M
F
54
49
10
60
23.2
17.8
14
12
No
No
No
NoAnxious syndrome
14F 7024 Hypertension
Osteoporosis
Hysterectomy
19.64 No Pre-op CA stent
attempt
15
16
F
F
49
60
24
12
20.1
22.6
3
2
No
No
No
NoAnxious syndrome
Hysterectomy
CA, Celiac artery; FU, follow-up; GE, gastroesophageal; HCV, hepatitis C virus; MV, mitral valve.
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was compressed on its superior aspect at its origin from the
aorta by a band of fibers forming the MAL. The celiac
trifurcation was always clearly seen because of its postste-
notic dilatation and pulsations. The CA was completely
isolated. The left gastric, common hepatic, and splenic
artery were identified. Gentle inferior traction of this trifur-
cation was of great help to expose the MAL. The ligament
was cut with the coagulating hook along with the nervous
celiac plexus and any fibrous and lymphatic tissue (Fig 2).
The procedure ended when the origin of the CA was
completely exposed and freed by any external stricture. A
drain was then placed near the CA for the first 24 hours,
and the wounds were closed in a routine fashion. All
patients were extubated at the end of the procedure and
discharged to the surgical ward.
RESULTS
Mesenteric DUS imaging revealed a stenosis of the CA
?70% during deep expiration in all patients, and a stenosis
?70% was also present during inspiration in 12 patients. In
all patients the anatomy of the superior and inferior mes-
enteric artery was normal at the preoperative diagnostic
workup.
Themeanlengthofthelaparoscopicprocedureswas90
minutes (range, 35-180 minutes). The mean intraoperative
blood loss was 50 mL (range, 0-300 mL). The average
postoperative hospital length of stay was 3 days (range, 2-5
days).
The laparoscopic operations in two patients were con-
verted to an open approach, at the last step of the proce-
dure, to obtain hemostasis for bleeding at the proximal part
of the CA. At this level, the arterial wall was damaged by
the Harmonic scalpel (Ethicon Endo-Surgery, Cincinnati,
Ohio)inthefirstpatientandbythecoagulatinghookinthe
second. In both patients, the damaged artery was readily
controlled by the vascular surgeon through a xiphoumbili-
cal midline incision. After laparotomy, the lesser omentum
and the diaphragmatic crus were already divided, and the
bleeding from CA was promptly controlled with 6-0
pledgeted suture, without major bleeding. No bleeding-
related hypotension was recorded, and no patient required
abloodtransfusion.Thepostoperativecoursewasunevent-
ful in all the patients, without complications.
In two patients the laparoscopic surgical division of the
MAL was not followed by a complete and persistent relief
of the clinical symptoms of CACS. DUS imaging and CTA
showed a recurrent, fixed stenosis of the CA in both pa-
tients, even in the absence of extrinsic compression. Patient
5 had undergone laparoscopic division of the MAL for
restenosis after PTA and stenting of the CA. The minimally
invasive procedure apparently resulted in the release of the
extrinsic CA compression as diagnosed by postoperative
DUS imaging, but was followed by an early restenosis 3
months later. Complete relief of symptoms was achieved
with aortoceliac artery bypass grafting. In patient 10, early
restenosis occurred 2 months after the laparoscopic proce-
dure. The complete resolution of the clinical syndrome was
obtained by PTA and stenting.
At a mean follow-up of 28.3 months (range, 2-83
months), symptoms were completely resolved in all 16
patients. Postoperative imaging studies demonstrated no
residual CA stenosis.
DISCUSSION
Chronic abdominal symptoms caused by respiration-
varying eccentric stenoses of the CA represent the classic
manifestation of CACS: a rare entity first described by
Harjola3in 1963 and by Dunbar et al4in 1965. Other
reported symptoms are weight loss, nausea, vomiting, and
bloating associated with a midepigastric bruit. CACS has
been attributed to compression of the CA by the MAL as a
result of the too-high emergence of the CA from the aorta
or of the too-low insertion of the crus.
Controversies exist regarding the clinical features,
pathophysiology, diagnosis, and treatment of CACS, and
the observation of CA compression in asymptomatic pa-
tients even leads to questions about the real existence of the
syndrome.5Some authors5-8suggested that pain is caused
by ischemia secondary to the reduction of blood flow
through the stenotic CA. Others9,10claimed that pain
comes from direct compression of celiac ganglias. Both
mechanisms may be involved.
We found that symptoms are variable. The classic triad
of postprandial abdominal pain, weight loss, and midepi-
gastric bruit was found in only three patients, the most
frequent symptom being abdominal epigastric or epi-
mesogastric pain, mainly related to meals. Weight loss was
the second most frequent symptom, with patients report-
ing a mean weight loss of 7 kg (range, 3-14 kg).
In three patients if our series, CACS was not recog-
nized because of negative results after abdominal investiga-
tions; a psychosomatic disease was initially diagnosed, and
antidepressive therapy was started. After a mean of 32
Fig 1. Position of the surgeon during the laparoscopic proce-
dure. The camera is inserted through an access above the umbili-
cus. Inset, The positions of the four ports are shown:A, camera;B,
operating ports; C, retractor. When a fifth port was needed, it was
inserted in the left flank.
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months (range 12-60 months), the persisting pain resulted
in CACS being diagnosed after abdominal dynamic DUS
scanning and CTA.
Diagnosis. In the past, CACS, when suspected, was
detected during conventional angiography. Lateral projec-
tion of aortography was considered the first choice to find
the CA stenosis, showing a characteristic superior indenta-
tion along the proximal CA, usually about 5 mm from its
origin at the abdominal aorta and during expiration, fre-
quently associated with poststenotic dilatation. Severe
compression persists during inspiration; however, the dia-
phragmcannotbeseenanddiaphragmaticcompressioncan
be easily missed. This technique was used only at the very
beginning of our experience and then was substituted with
the less invasive CTA.
In our experience, abdominal visceral DUS imaging is
thefirstdiagnosticapproachwhenCAstenosisissuspected.
DUS imaging during maximum inspiration and expiration
can easily demonstrate the existence and the exact config-
uration of a dynamic CA stenosis.11DUS scanning can also
show reversal of flow in the common hepatic artery, which
in these patients comes preferentially from the gastroduo-
denal, pancreaticoduodenal, and superior mesenteric artery
rather than from the stenotic CA.12
New, thin-section multidetector CT scanners, along
with three-dimensional (3D) reconstruction, have greatly
improved the ability to obtain high-resolution images of
the aorta and its branches. In CACS, the sagittal plane and
3D imaging are optimal to visualize the characteristic focal
narrowing in the proximal CA. CT is typically performed
during inspiration; therefore, an expiratory scan is also
acquired when CACS is suspected to demonstrate the
variable stenosis. We have found that CTA adds supple-
mentary, useful information about the local anatomy and
the presence of calcifications when an atherosclerotic etiol-
ogy is suspected (Fig 3). Postoperative CT is useful to
demonstrate the absence of residual stenosis of the CA after
decompression (Fig 4), but DUS imaging was adequate in
detecting early restenosis and was adopted as the noninva-
sive diagnostic tool in healthy patients at follow-up.
MRA with 3D reconstruction has improved the ability
to study the aorta and its branches. Even if sagittal plane
and 3D reconstruction showed the focal narrowing in the
CA in patients with CACS, the images were less accurate
and arterial calcifications were missed.
Gastric exercise tonometry has recently been suggested
as the “key investigation” in patients with suspected
CACS13because it can detect actual gastrointestinal isch-
emia, thereby selecting patients who are most likely to
benefit from treatment. This diagnostic technique has been
proposed only recently by Mensink et al13to approach
CACS, and we are planning to include this interesting
method in our protocol.
Therapy. Given the rarity of CACS, what constitutes
standard therapy is a matter of considerable debate. Pa-
Fig 3. A three-dimensional reconstruction of the aorta and its
main branches in patient 8 shows the extrinsic compression to the
celiac trunk during expiration (arrow).
Fig 2. A, The median arcuate ligament is divided by coagulating hook (arrow). B, The diaphragmatic aspect of the
aorta is completely exposed.
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tients who exhibit symptoms should undergo operative
treatment. Some patients are asymptomatic or have suffi-
ciently minimal symptoms that they don’t warrant treat-
ment. Surgical division of the MAL and diaphragmatic
pillars by open surgery has been so far the mainstay treat-
ment, which can remove the extrinsic compression on the
CA. Takach et al14in 1996 reported patients asymptomatic
after 15 years with the open surgical approach.
After laparoscopic surgery became more widespread, a
small number of patients with CACS have been reported
treated with the minimally invasive approach. Roayaie et
al15in 2000 described a patient in whom laparoscopy
provided a less invasive but equally effective method for
decompressing the CA; moreover, laparoscopic intraoper-
ative US scanning demonstrated markedly improved flow
in the CA. Other single cases of laparoscopic management
of CACS were subsequently reported by Carbonell et al,16
Baldassarre at al,17and by our group.18Laparoscopy al-
lowed surgeons to skeletonize the CA without a laparot-
omy; however, adequate laparoscopic experience is re-
quired because this is not a simple procedure.
No postoperative complications occurred in our series.
Despite a spacious release of the CA (Fig 2) from the
anomalous MAL, often involving the diaphragmatic crus,
at follow-up we recorded no symptoms of postoperative
gastric reflux or evidence of hiatal hernia. Less blood loss
and postoperative pain, faster return to normal diet, shorter
hospital stay, and better cosmetic effect were the obtained
benefits.
After the first successful approach, we decided to use
laparoscopyastheinterventionfortheotherCACSpatients
subsequently observed at our institution or transferred
from other centers. To our knowledge, this is the first
report of a series of consecutive patients with CACS treated
byaminimallyinvasiveapproach.Ofthe16patientstreated
by us by laparoscopy, 14 had uneventful operations and
postoperative courses. Patients 9 and 11 of the series were
converted to open surgery because of bleeding from the
takeoff of the CA from the aorta. This was a small lesion to
the proximal part of a CA with a thin wall, caused by
Harmonic Scalpel in one patient and by the coagulating
hook in the other patient. Both lesions occurred at the end
of the procedure during an attempt to improve the aorto-
celiacskeletonizationbyremovingtheabundantganglionic
tissue encasing the CA. We believe that, during the last
stepsoftheprocedure,avoidinghighenergiesandreducing
the coagulating force can be advisable.
A successful robotic-assisted laparoscopic approach
to CACS using the da Vinci Surgical System (Intuitive
Surgical, Sunnyvale, Calif) was recently reported.19Ro-
botic assistance is a significant technologic advancement in
minimally invasive surgery. Benefits of robotic surgery
could be the superior stereoscopic view of the surgical field
and the improved surgical precision of movement due to
the wrist-like articulations of the instruments. Disadvan-
tages are the higher cost and longer time requirements for
theprocedureandforequipmentsetup.Operativetimewas
168minutesinthereportedcase,incontrastwithameanof
90 minutes of our series. In the future, however, robotics
could have a role in reconstructive surgery of visceral arter-
ies.
Endovascular treatment. PTA and stenting in the
treatment of CACS is also controversial. The successful
endovascular treatment with balloon angioplasty and stent-
ing for celiac and mesenteric atherosclerotic stenoses20
suggested the extension of PTA also to patients with
CACS. However, the endovascular procedure is not con-
sidered adequate as the sole mode of treatment before the
surgical release of the extrinsic compression on the CA
because the compression on the CA often does not allow
adequate expansion of the stent,21as confirmed by our
series. Patients 7 and 14 had undergone a previous attempt
of CA stenting that failed as a result of technical difficulties,
and no stent was implanted.
A recent report pointed at the partial, short-lived relief
of symptoms and early CA restenosis after repeat PTA and
stenting in the presence of high-grade CA compression due
to CACS.22The patient in that report recovered after open
surgical division of the ligament and reconstruction of the
CA, which exactly matches with our experience. In patient
5, early restenosis of CA occurred after the endovascular
procedure before undergoing laparoscopic surgical division
of the ligament and release of the extrinsic compression on
the CA. The laparoscopic procedure, however, was also
followed by only temporary relief of symptoms and early
CA restenosis. Three months later, this patient therefore
underwent open surgical reconstruction of the CA with a
side-to-end aortoceliac interposition graft, followed by re-
lief of the symptoms linked to CACS. We speculated that
Fig 4. A three-dimensional aortic reconstruction of patient 8
(Fig 3), after laparoscopic release of the median arcuate liga-
ment, shows good patency of the celiac trunk (arrow) at the
1-month follow-up.
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failures of stenting in the presence of CACS are usually due
to crushing of the stent, which later would not be reversed
by extrinsic decompression alone.
In the presence of CACS, we therefore strongly advise
against using endovascular treatment of the lesion; that
should be limited to the management of residual fixed
disease after extrinsic decompression either by laparoscopic
or open means. As detailed above, patient 10 from our
series may summarize this statement.
CONCLUSION
Laparoscopic release of CA compression by CACS is
technically feasible and an appealing option to an open
approach in centers with large experience in vascular sur-
gery and in major laparoscopic operations. Given its less
invasivenature,itcanprovideanimportantapproach(ther-
apeutic or even diagnostic) in younger patients where the
risk of surgery and cosmetic impairment are significant.
PTA and stenting in the context of CACS should be
avoided. Balloon dilatation alone may be used as a tempo-
rizing modality but is likely to fail quickly. The role of
endovascular therapy should be limited to the management
of residual fixed disease after extrinsic decompression.
The laparoscopic technique for treatment of CACS is a
promising alternative to open surgery, but additional cases
with longer follow-up are needed to confirm the validity of
the minimally invasive approach.
AUTHOR CONTRIBUTIONS
Conception and design: PB, RC
Analysis and interpretation: PB, LD, GM
Data collection: LD
Writing the article: PB, EC, RN
Critical revision of the article: PB, EC, GM
Final approval of the article: PB
Statistical analysis: Not applicable
Obtained funding: Not applicable
Overall responsibility: RC
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Submitted Sep 10, 2008; accepted Nov 17, 2008.
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