- Email: [email protected]
Hybrid Laparoscopic and Endovascular Treatment for Median Arcuate Ligament Syndrome: Case Report and Review of Literature
Journal Pre-proof Hybrid Laparoscopic and Endovascular Treatment for Median Arcuate Ligament Syndrome (MALS): Case Report and Review of Literature Luca Garriboli, MD, Tommaso Miccoli, MD, Isacco Damoli, MD, Roberto Rossini, MD, Carlo Alberto Sartori, MD, Giacomo Ruffo, MD, Antonio Maria Jannello, MD PII:
S0890-5096(19)30747-2
DOI:
https://doi.org/10.1016/j.avsg.2019.08.077
Reference:
AVSG 4610
To appear in:
Annals of Vascular Surgery
Received Date: 22 May 2019 Revised Date:
30 July 2019
Accepted Date: 4 August 2019
Please cite this article as: Garriboli L, Miccoli T, Damoli I, Rossini R, Sartori CA, Ruffo G, Jannello AM, Hybrid Laparoscopic and Endovascular Treatment for Median Arcuate Ligament Syndrome (MALS): Case Report and Review of Literature, Annals of Vascular Surgery (2019), doi: https://doi.org/10.1016/ j.avsg.2019.08.077. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2019 Published by Elsevier Inc.
1
Hybrid Laparoscopic and Endovascular Treatment for Median Arcuate
2
Ligament Syndrome (MALS): Case Report and Review of Literature
3 4
Authors: Luca Garriboli MD, Tommaso Miccoli MD, Isacco Damoli MD, Roberto Rossini* MD,
5
Carlo Alberto Sartori* MD, Giacomo Ruffo* MD, Antonio Maria Jannello MD.
6
Affiliations: Department of Vascular Surgery, IRCCS Sacro Cuore-Don Calabria, Negrar (Vr),
7
Italy, *Department of General Surgery, IRCCS Sacro Cuore-Don Calabria, Negrar (Vr), Italy
8
Sources of financial support: XX
9
Prior Presentations: XX
10
Acknowledgements: XX
11 12
Corresponding author:
13
Luca Garriboli
14
Department: U.O. Vascular Surgery
15
Institution, IRCCS SacroCuore-Don Calabria
16
Address: Via Don SempreBoni 5
17
Zip Code, Town: 37024 Negrar (Verona)
18
Country: Italy
19
E-mail: [email protected]
20 21
ABSTRACT
22
Median Arcuate Ligament Syndrome (MALS) is a rare cause of chronic gastrointestinal ischemia
23
caused by compression of median arcuate ligament (MAL) on celiac trunk (CT). A 38 year-old
24
male presented at our Institution with unspecific crampy abdominal pain. After several diagnostic
25
exams, he firstly underwent arcuate ligament resection by laparoscopic approach and two months
26
later percutaneous transluminal angioplasty (PTA) with stenting of the stenotic vessel. Post-
27
operatory and follow-up controls showed regular patency of the artery with fully relief of abdominal
28
symptoms. We propose a revision of Literature on this uncommon condition describing different
29
surgical approaches.
30
Keywords: Stenosis; Median Arcuate Ligament; Stenting; Laparoscopic treatment; Celiac Trunk
31
32
INTRODUCTION
33
Median Arcuate Ligament Syndrome (MALS) is a rare cause of chronic gastrointestinal ischemia
34
(1). Anatomically, median arcuate ligament is a muscular-fibrous structure which connects the left
35
and right diaphragmatic crux on both sides of the aortic hiatus (2). Occasionally, this ligament may
36
have an intimate relationship with the celiac trunk and may cause a focal indentation. This
37
syndrome occurs typically in young slim woman (20 to 40 years old). The celiac stenosis may
38
present as an incidental finding or may be symptomatic, causing nausea, vomit, weight loss, post-
39
prandial pain in the epigastrium or after exercise (3). The gold standard for diagnosis is catheter
40
angiography performed during inspiration and expiration, although nowadays this procedure is
41
currently replaced by multi-detector computed tomography (MDCT) and CT-angiography.
42
Typically, a “hook like” appearance of the celiac artery is seen. Controversies still exist about
43
several aspects of the syndrome, including pathophysiologic origin of the symptoms, diagnosis and
44
optimal surgical strategies (4). Median Arcuate Ligament Syndrome requires treatment only in
45
symptomatic patients. Decompression of the celiac trunk can be achieved by cleavage of the median
46
arcuate ligament with a laparoscopic approach; alternative methods, such as percutaneous
47
transluminal angioplasty and stenting of the celiac trunk, can also be performed.
48 49
CASE REPORT
50
A 38 year-old male without any prior significant surgical history (except for laparoscopic
51
cholecystectomy) presented with unspecific crampy abdominal pain that occurred 30 minutes to 1
52
hour after eating and usually located in the right mid-abdomen. Pain had a severity of 8/10 with no
53
relieving factors. The patient experienced 6-8 kilograms weight loss in the last three months. All
54
laboratory testing results were normal as well as abdominal ultrasonography. He firstly underwent
55
esophagus-stomach X-Ray exam that was normal and then esophagus-gastro-duodenoscopy that
56
highlighted the presence of a pair of small erosions in the distal part of the esophagus, without
57
lesions in the gastric mucosa and in the first part of duodenum. He was discharged after 2 days of
58
hospitalization with the diagnosis of reflux esophagitis. After 2 months he presented at the
59
Emergency Department of our Institution with persistent abdominal pain and weigh loss of other 3
60
kilograms. He was hospitalized and underwent Angio-Computed Tomography (CTA) of abdomen
61
which revealed antral and duodenal bulb thickening and a focal stenosis of the origin of the celiac
62
axis, with the vessel demonstrating a hook like appearance on the sagittal view with a 80% stenosis
63
arising 5 mm from the origin of celiac trunk and extending 8 mm in length due to an extrinsic
64
compression induced by median arcuate ligament (Fig.1). The patient underwent a standard work-
65
up, which involved evaluation by a multidisciplinary team including vascular surgery, general
66
surgery and gastroenterologist. In order to remove the extrinsic compression of the CA, the patient
67
was submitted to arcuate ligament resection and a laparoscopic approach was preferred. He was
68
positioned supine on the operating table with lower extremities abducted. The table was in an anti-
69
Trendelenburg position and carbon dioxide was insufflated into the peritoneal cavity to a pressure
70
of 12 mmHg. The first 10 mm Trocar for the camera was introduced in the midline about 12 cm
71
below the xiphoid process of the sternum. After a thorough inspection of the whole peritoneal
72
cavity under the control of the vision, the remaining trocars were inserted: 5 mm in the right
73
hypochondrium, 5 mm in left hypochondrium, 5 mm in the left side and 5 mm under xiphoidal
74
process. After inspection throughout the peritoneal cavity, the pars flaccida of the hepato-gastric
75
ligament was cut, revealing the left crus of the diaphragm. The CT and compressing ligament were
76
exposed. The MAL was cut with a harmonic scalpel, which resulted in immediate filling with blood
77
and increased diameter of the vessel. Patient was discharged on third postoperative day in good
78
conditions. Two months later he continued referring not fully relief of symptoms, especially post-
79
prandial abdominal pain, which have been decreased after laparoscopic release of the MAL but was
80
still present and was associated to a stab pain in epigastrium during deep breathing. The patient was
81
hospitalized for the third time, a CT scan showed a partial improvement of the stenosis though still
82
present (Fig.2). A selective catheter angiography of the abdominal aorta and visceral vessels both
83
during inspiration and expiration was performed. A left brachial artery approach was used and a 6
84
Fr introducer sheath was placed according to the Seldinger method. A Terumo guidewire was
85
advanced first in the descending thoracic aorta and a 5 Fr diagnostic Pigtail catheter was used to
86
perform angiography of the abdominal aorta. The exam confirmed the presence of a residual severe
87
stenosis 5 mm after the origin of the CT with an extension of 8 mm and the presence of a post-
88
stenotic dilatation of the vessel, with regular patency of the superior and inferior mesenteric artery.
89
After selective cannulation of the CT with a 4 Fr Bernstein catheter, exchange of the guidewire with
90
a stiff Rosen one and pre-dilatation of the vessel with a 6x20 mm semi-compliant angioplasty
91
balloon (Powerflex, Cordis), a 7x19 mm bare-metal stent (Express, Boston Scientific) post-dilatated
92
with 7x20 mm angioplasty balloon (Powerflex, Cordis) was deployed at the point of the stenosis.
93
Final control angiography showed regular patency of the vessel and of its main branches (Fig.3).
94
The punctured artery was manual compressed. During the procedure, 5000 UI of unfractionated
95
heparin were administered. He was discharged after three days of hospitalization under single
96
antiplatelet therapy with acetilsalicilic acid 100 mg/die. Post-operatory and 3-months Doppler
97
ultrasound controls showed regular patency of the vessel without any significant variation of flow
98
velocities during inspiration and deep expiration, confirming the correct deployment and patency of
99
the stent.
100
DISCUSSION
101
Celiac trunk stenosis has been reported in older autoptic studies with an incidence ranging from
102
12.5-24% (5). Main etiological factors consist of median arcuate ligament compression, followed
103
by arteriosclerosis, pancreatitis, tumoral invasion or congenital abnormalities. According to
104
Sakorafas et al. causes can be classified into three different groups: extrinsic (mediate arcuate
105
ligament syndrome, compression induced by the celiac ganglion or surrounding fibrotic
106
transformations), intrinsic (due to arteriosclerosis) and others (including congenital malformations,
107
acute or chronic diseases, malignant invasion or compression due to chronic pancreatitis) (6; 7).
108
Another important fact which influences the presence of symptomatic disease is the existence of a
109
collateral circulation via the superior mesenteric artery. It is estimated that patients presenting celiac
110
artery stenosis will develop collateral circulation via the superior mesenteric artery (8). In patients
111
presenting chronic celiac artery stenosis, adequate collateral circulation develops in up to 80% of
112
patients (9). Only a very small proportion of patients with stenosis of CA develop symptoms
113
indicative of MALS, such as abdominal discomfort or even pain in the first two hours after food
114
intake, weight loss, or diarrhea [10]. The proposed pathophysiological mechanisms are different and
115
include: (a) postprandial hypoperfusion of the stomach, (b) expiratory hypoperfusion due to
116
repeated compression of CA by MAL during respiration, (c) progressive stenosis of the CA caused
117
by accelerated atherosclerosis due to fibrosis of the arterial wall or (d) irritation of the celiac
118
ganglion causing visceral pain and even reactive vasoconstriction [11;14].
119
In a Mayo Clinic study (12), symptoms of MALS included abdominal pain (94%), postprandial
120
abdominal pain (80%), weight loss (50%), bloating (39%), nausea and vomiting (55.6%), and
121
abdominal pain triggered by exercise (8%). However, because symptoms closely mimic those of
122
other abdominal disorders, it is commonly considered a diagnosis of exclusion.
123
Abdominal visceral ultrasound imaging (DUS) is the first diagnostic approach when CA stenosis is
124
suspected. DUS scanning can also show reversal flow in the common hepatic artery, which in these
125
patients comes preferentially from the gastroduodenal, pancreatico-duodenal and superior
126
mesenteric artery rather than from the stenotic CA (13). Thin-section multidetector CT scanners,
127
along with three-dimensional (3D) reconstruction, have greatly improved the ability to obtain high-
128
resolution images of the aorta and its branches. In MALS, the sagittal plane and 3D imaging are
129
optimal to visualize the characteristic focal narrowing in the proximal CA.
130
Gastric exercise tonometry has been suggested as another useful exam that can be performed in
131
patients with suspected MALS because it can detect actual gastrointestinal ischemia, thereby
132
selecting patients who are most likely to benefit from treatment. This diagnostic technique has been
133
proposed by Mensink et al., who identified 29 patients with celiac artery compression using gastric
134
exercise tonometry in a prospective cohort study (14).
135
Regarding treatment choices, surgical division of MAL and diaphragmatic pillars by open surgery
136
has been so far the mainstay treatment. After laparoscopic surgery became more widespread, a
137
bigger number of patients have been reported treated with minimally invasive approach (15), even
138
using a robotic-assisted approach (16). Laparoscopic surgical treatment might be followed in certain
139
cases by the persistence of the celiac artery syndrome; therefore, it can represent the first step of a
140
hybrid strategy of treatment combined with percutaneous angioplasty and stenting of the CA.
141
Baccari et. al. (17) treated 16 patients by laparoscopic transection of the MAL and only in 4 cases
142
needed to complementary angioplasty of the CA. Although PTA and stenting of TC has proved to
143
be useful as an adjunctive therapy to prior surgical MAL division, when used as the sole therapy
144
without MAL division, patient outcomes have been poor. These results may be due to the sustained
145
extrinsic pressure that the intact MAL exerts on the celiac artery, causing a chronic process of
146
intimal hyperplasia and intraluminal narrowing. Multiple case reports have demonstrated that
147
endovascular angioplasty alone is unsuccessful at achieving long-term resolution of the symptoms
148
(18-21).
149
In conclusion, laparoscopic release of CT compression by MALS is technically feasible and an
150
appealing option in centers with large experience in major laparoscopic operations. PTA and
151
stenting alone of the CT in the context of MALS should be avoided. In our experience, hybrid
152
combined laparoscopic and endovascular procedures seems to guarantee good early- and long-term
153
results.
154 155
REFERENCES
156
1. Lainez RA, Richardson WS. Median Arcuate Ligament Syndrome: A Case Report. Ochsner J
157
2013;13:561-4.
158
2. A-Cienfuegos J, Rotellar F, Valentí V, Arredondo J, Pedano N, Bueno A, et al. The celiac axis
159
compression syndrome (CACS). Critical review in the laparoscopic era. Rev Esp Enferm Dig
160
2010;102:193-201.
161
3. Kotarać M, Radovanović N, Lekić N, Ražnatović Z, Djordjević V, Lekć D, et al. Surgical
162
treatment of median arcuate ligament syndrome: case report and review of literature. Srp Arh Celok
163
Lek 2015;143:74-8.
164
4. J. M. Weber, M. Boules, et al. Median Arcuate Ligament Syndrome Is Not a Vascular Disease.
165
Annals of Vascular Surgery, Vol. 30, p22-27.
166
5. Reuter SR and Olin T: Stenosis of the celiac artery. Radiology 85: 616-627, 1965.
167
6. Sakorafas GH, Sarr MG and Peros G: Celiac artery stenosis: an underappreciated and unpleasant
168
surprise in patients undergoing pancreaticoduodenectomy. J Am Coll Surg 206: 349-356, 2008.
169
7. Serkan Burç Deşer, Savas Yuruker, Mustafa Kemal Demirag. Treatment of the Median Arcuate
170
Ligament Syndrome in a Patient with Behcet's Disease. Annals of Vascular Surgery, Vol. 51,
171
p325.e9-325.e11.
172
8. Berney T, Pretre R, Chassot G and Morel P: The role of revascularization in celiac occlusion and
173
pancreatoduodenectomy. Am J Surg 176: 352-356, 1998.
174
9. A. J. Doyle, A. Chandra. Chronic Mesenteric Ischemia in a 26-Year-Old Man: Multivessel
175
Median Arcuate Ligament Compression Syndrome. Annals of Vascular Surgery, Vol. 26, Issue 1,
176
p108.e5-108.e9
177
10. Park CM, Chung JW, Kim HB, et al. Celiac axis stenosis: incidence and etiologies in
178
asymptomatic individuals. Korean J Radiol. 2001;2:8–13.11. Desmond CP, Roberts SK. Exercise-
179
related abdominal pain as a manifestation of the median arcuate ligament syndrome. Scand J
180
Gastroenterol. 2004;39: 1310–1313.
181
12. Cusati DA, Noel AA, Gloviczki P, et al. Median arcuate ligament syndrome: a 20-year
182
experience of surgical treatment. Presented at: 60th Annual Meeting of the Society for Vascular
183
Surgery; June 1-4, 2006; Philadelphia, PA.
184
13. Scholbach T. Celiac artery compression syndrome in children, adolescents, and young adults:
185
clinical and color duplex sonographic features in a series of 59 cases. J Ultrasound Med.
186
2006;25(3):299-305.
187
14. Mensink PBF, van Petersen AS, Kolkman J, Otte JA, Huisman AB, Geelkerken RH. Gastric
188
exercise tonometry: the key investigation in patients with suspected celiac artery compression
189
syndrome. J Vasc Surg 2006;44:277-81.
190
15. Roayaie S, Jossart G, Gitlitz D, Lamparello P, Hollier L, Gagner M. Laparoscopic release of
191
celiac artery compression syndrome facilitated by laparoscopic ultrasound scanning to confirm
192
restoration of flow. J Vasc Surg 2000;32:814-7.
193
16. Jaik NP, Stawicki SP, Weger NS, Lukaszczyk JJ. Celiac artery compression syndrome:
194
successful utilization of robotic-assisted laparoscopic approach. J Gastrointest Liver Dis
195
2007;16:93-6.
196
17. Baccari, P., Civilini, E., Dordoni, L., Melissano, G., Nicoletti, R., & Chiesa, R. (2009). Celiac
197
artery compression syndrome managed by laparoscopy. Journal of Vascular Surgery, 50(1), 134–
198
139. http://doi.org/10.1016/j.jvs.2008.11.124
199
18. Wang X, Impeduglia T, Dubin Z, Dardik H. Celiac revascularization as a requisite for treating
200
the median arcuate ligament syndrome. Ann Vasc Surg. 2008;22(4):571-574.
201
19. Cinà CS, Safar H. Successful treatment of recurrent celiac axis compression syndrome: a case
202
report. Panminerva Med. 2002;44(1):69-72.
203
20. Delis KT, Gloviczki P, Altuwaijri M, McKusick MA. Median arcuate ligament syndrome: open
204
celiac artery reconstruction and ligament division after endovascular failure. J Vasc Surg.
205
2007;46(4): 799-802.
206
21. Matsumoto AH, Tegtmeyer CJ, Fitzcharles EK, et al. Percutaneous transluminal angioplasty of
207
visceral arterial stenoses: results and long-term clinical follow-up. J Vasc Interv Radiol. 1995;6(2):
208
165-174.
209 210 211 212
213
Fig. 1: Angio-Computed Tomography (CTA) of abdominal aorta demonstrating the presence of a focal stenosis at the origin of the celiac axis due to an extrinsic compression induced by median arcuate ligament. Fig. 2: Focal stenosis at the origin of the celiac axis before and after resection of arcuate ligament by laparoscopic approach. To note the partial improvement in size of the celiac axis after pillar section. Fig. 3: Selective catheter angiography of the celiac artery demonstrating the persistence of the stenosis due to extrinsic compression with its fully resolution after balloon angioplasty and stenting of the origin of the vessel.
S0890-5096(19)30747-2
DOI:
https://doi.org/10.1016/j.avsg.2019.08.077
Reference:
AVSG 4610
To appear in:
Annals of Vascular Surgery
Received Date: 22 May 2019 Revised Date:
30 July 2019
Accepted Date: 4 August 2019
Please cite this article as: Garriboli L, Miccoli T, Damoli I, Rossini R, Sartori CA, Ruffo G, Jannello AM, Hybrid Laparoscopic and Endovascular Treatment for Median Arcuate Ligament Syndrome (MALS): Case Report and Review of Literature, Annals of Vascular Surgery (2019), doi: https://doi.org/10.1016/ j.avsg.2019.08.077. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2019 Published by Elsevier Inc.
1
Hybrid Laparoscopic and Endovascular Treatment for Median Arcuate
2
Ligament Syndrome (MALS): Case Report and Review of Literature
3 4
Authors: Luca Garriboli MD, Tommaso Miccoli MD, Isacco Damoli MD, Roberto Rossini* MD,
5
Carlo Alberto Sartori* MD, Giacomo Ruffo* MD, Antonio Maria Jannello MD.
6
Affiliations: Department of Vascular Surgery, IRCCS Sacro Cuore-Don Calabria, Negrar (Vr),
7
Italy, *Department of General Surgery, IRCCS Sacro Cuore-Don Calabria, Negrar (Vr), Italy
8
Sources of financial support: XX
9
Prior Presentations: XX
10
Acknowledgements: XX
11 12
Corresponding author:
13
Luca Garriboli
14
Department: U.O. Vascular Surgery
15
Institution, IRCCS SacroCuore-Don Calabria
16
Address: Via Don SempreBoni 5
17
Zip Code, Town: 37024 Negrar (Verona)
18
Country: Italy
19
E-mail: [email protected]
20 21
ABSTRACT
22
Median Arcuate Ligament Syndrome (MALS) is a rare cause of chronic gastrointestinal ischemia
23
caused by compression of median arcuate ligament (MAL) on celiac trunk (CT). A 38 year-old
24
male presented at our Institution with unspecific crampy abdominal pain. After several diagnostic
25
exams, he firstly underwent arcuate ligament resection by laparoscopic approach and two months
26
later percutaneous transluminal angioplasty (PTA) with stenting of the stenotic vessel. Post-
27
operatory and follow-up controls showed regular patency of the artery with fully relief of abdominal
28
symptoms. We propose a revision of Literature on this uncommon condition describing different
29
surgical approaches.
30
Keywords: Stenosis; Median Arcuate Ligament; Stenting; Laparoscopic treatment; Celiac Trunk
31
32
INTRODUCTION
33
Median Arcuate Ligament Syndrome (MALS) is a rare cause of chronic gastrointestinal ischemia
34
(1). Anatomically, median arcuate ligament is a muscular-fibrous structure which connects the left
35
and right diaphragmatic crux on both sides of the aortic hiatus (2). Occasionally, this ligament may
36
have an intimate relationship with the celiac trunk and may cause a focal indentation. This
37
syndrome occurs typically in young slim woman (20 to 40 years old). The celiac stenosis may
38
present as an incidental finding or may be symptomatic, causing nausea, vomit, weight loss, post-
39
prandial pain in the epigastrium or after exercise (3). The gold standard for diagnosis is catheter
40
angiography performed during inspiration and expiration, although nowadays this procedure is
41
currently replaced by multi-detector computed tomography (MDCT) and CT-angiography.
42
Typically, a “hook like” appearance of the celiac artery is seen. Controversies still exist about
43
several aspects of the syndrome, including pathophysiologic origin of the symptoms, diagnosis and
44
optimal surgical strategies (4). Median Arcuate Ligament Syndrome requires treatment only in
45
symptomatic patients. Decompression of the celiac trunk can be achieved by cleavage of the median
46
arcuate ligament with a laparoscopic approach; alternative methods, such as percutaneous
47
transluminal angioplasty and stenting of the celiac trunk, can also be performed.
48 49
CASE REPORT
50
A 38 year-old male without any prior significant surgical history (except for laparoscopic
51
cholecystectomy) presented with unspecific crampy abdominal pain that occurred 30 minutes to 1
52
hour after eating and usually located in the right mid-abdomen. Pain had a severity of 8/10 with no
53
relieving factors. The patient experienced 6-8 kilograms weight loss in the last three months. All
54
laboratory testing results were normal as well as abdominal ultrasonography. He firstly underwent
55
esophagus-stomach X-Ray exam that was normal and then esophagus-gastro-duodenoscopy that
56
highlighted the presence of a pair of small erosions in the distal part of the esophagus, without
57
lesions in the gastric mucosa and in the first part of duodenum. He was discharged after 2 days of
58
hospitalization with the diagnosis of reflux esophagitis. After 2 months he presented at the
59
Emergency Department of our Institution with persistent abdominal pain and weigh loss of other 3
60
kilograms. He was hospitalized and underwent Angio-Computed Tomography (CTA) of abdomen
61
which revealed antral and duodenal bulb thickening and a focal stenosis of the origin of the celiac
62
axis, with the vessel demonstrating a hook like appearance on the sagittal view with a 80% stenosis
63
arising 5 mm from the origin of celiac trunk and extending 8 mm in length due to an extrinsic
64
compression induced by median arcuate ligament (Fig.1). The patient underwent a standard work-
65
up, which involved evaluation by a multidisciplinary team including vascular surgery, general
66
surgery and gastroenterologist. In order to remove the extrinsic compression of the CA, the patient
67
was submitted to arcuate ligament resection and a laparoscopic approach was preferred. He was
68
positioned supine on the operating table with lower extremities abducted. The table was in an anti-
69
Trendelenburg position and carbon dioxide was insufflated into the peritoneal cavity to a pressure
70
of 12 mmHg. The first 10 mm Trocar for the camera was introduced in the midline about 12 cm
71
below the xiphoid process of the sternum. After a thorough inspection of the whole peritoneal
72
cavity under the control of the vision, the remaining trocars were inserted: 5 mm in the right
73
hypochondrium, 5 mm in left hypochondrium, 5 mm in the left side and 5 mm under xiphoidal
74
process. After inspection throughout the peritoneal cavity, the pars flaccida of the hepato-gastric
75
ligament was cut, revealing the left crus of the diaphragm. The CT and compressing ligament were
76
exposed. The MAL was cut with a harmonic scalpel, which resulted in immediate filling with blood
77
and increased diameter of the vessel. Patient was discharged on third postoperative day in good
78
conditions. Two months later he continued referring not fully relief of symptoms, especially post-
79
prandial abdominal pain, which have been decreased after laparoscopic release of the MAL but was
80
still present and was associated to a stab pain in epigastrium during deep breathing. The patient was
81
hospitalized for the third time, a CT scan showed a partial improvement of the stenosis though still
82
present (Fig.2). A selective catheter angiography of the abdominal aorta and visceral vessels both
83
during inspiration and expiration was performed. A left brachial artery approach was used and a 6
84
Fr introducer sheath was placed according to the Seldinger method. A Terumo guidewire was
85
advanced first in the descending thoracic aorta and a 5 Fr diagnostic Pigtail catheter was used to
86
perform angiography of the abdominal aorta. The exam confirmed the presence of a residual severe
87
stenosis 5 mm after the origin of the CT with an extension of 8 mm and the presence of a post-
88
stenotic dilatation of the vessel, with regular patency of the superior and inferior mesenteric artery.
89
After selective cannulation of the CT with a 4 Fr Bernstein catheter, exchange of the guidewire with
90
a stiff Rosen one and pre-dilatation of the vessel with a 6x20 mm semi-compliant angioplasty
91
balloon (Powerflex, Cordis), a 7x19 mm bare-metal stent (Express, Boston Scientific) post-dilatated
92
with 7x20 mm angioplasty balloon (Powerflex, Cordis) was deployed at the point of the stenosis.
93
Final control angiography showed regular patency of the vessel and of its main branches (Fig.3).
94
The punctured artery was manual compressed. During the procedure, 5000 UI of unfractionated
95
heparin were administered. He was discharged after three days of hospitalization under single
96
antiplatelet therapy with acetilsalicilic acid 100 mg/die. Post-operatory and 3-months Doppler
97
ultrasound controls showed regular patency of the vessel without any significant variation of flow
98
velocities during inspiration and deep expiration, confirming the correct deployment and patency of
99
the stent.
100
DISCUSSION
101
Celiac trunk stenosis has been reported in older autoptic studies with an incidence ranging from
102
12.5-24% (5). Main etiological factors consist of median arcuate ligament compression, followed
103
by arteriosclerosis, pancreatitis, tumoral invasion or congenital abnormalities. According to
104
Sakorafas et al. causes can be classified into three different groups: extrinsic (mediate arcuate
105
ligament syndrome, compression induced by the celiac ganglion or surrounding fibrotic
106
transformations), intrinsic (due to arteriosclerosis) and others (including congenital malformations,
107
acute or chronic diseases, malignant invasion or compression due to chronic pancreatitis) (6; 7).
108
Another important fact which influences the presence of symptomatic disease is the existence of a
109
collateral circulation via the superior mesenteric artery. It is estimated that patients presenting celiac
110
artery stenosis will develop collateral circulation via the superior mesenteric artery (8). In patients
111
presenting chronic celiac artery stenosis, adequate collateral circulation develops in up to 80% of
112
patients (9). Only a very small proportion of patients with stenosis of CA develop symptoms
113
indicative of MALS, such as abdominal discomfort or even pain in the first two hours after food
114
intake, weight loss, or diarrhea [10]. The proposed pathophysiological mechanisms are different and
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include: (a) postprandial hypoperfusion of the stomach, (b) expiratory hypoperfusion due to
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repeated compression of CA by MAL during respiration, (c) progressive stenosis of the CA caused
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by accelerated atherosclerosis due to fibrosis of the arterial wall or (d) irritation of the celiac
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ganglion causing visceral pain and even reactive vasoconstriction [11;14].
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In a Mayo Clinic study (12), symptoms of MALS included abdominal pain (94%), postprandial
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abdominal pain (80%), weight loss (50%), bloating (39%), nausea and vomiting (55.6%), and
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abdominal pain triggered by exercise (8%). However, because symptoms closely mimic those of
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other abdominal disorders, it is commonly considered a diagnosis of exclusion.
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Abdominal visceral ultrasound imaging (DUS) is the first diagnostic approach when CA stenosis is
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suspected. DUS scanning can also show reversal flow in the common hepatic artery, which in these
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patients comes preferentially from the gastroduodenal, pancreatico-duodenal and superior
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mesenteric artery rather than from the stenotic CA (13). Thin-section multidetector CT scanners,
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along with three-dimensional (3D) reconstruction, have greatly improved the ability to obtain high-
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resolution images of the aorta and its branches. In MALS, the sagittal plane and 3D imaging are
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optimal to visualize the characteristic focal narrowing in the proximal CA.
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Gastric exercise tonometry has been suggested as another useful exam that can be performed in
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patients with suspected MALS because it can detect actual gastrointestinal ischemia, thereby
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selecting patients who are most likely to benefit from treatment. This diagnostic technique has been
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proposed by Mensink et al., who identified 29 patients with celiac artery compression using gastric
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exercise tonometry in a prospective cohort study (14).
135
Regarding treatment choices, surgical division of MAL and diaphragmatic pillars by open surgery
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has been so far the mainstay treatment. After laparoscopic surgery became more widespread, a
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bigger number of patients have been reported treated with minimally invasive approach (15), even
138
using a robotic-assisted approach (16). Laparoscopic surgical treatment might be followed in certain
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cases by the persistence of the celiac artery syndrome; therefore, it can represent the first step of a
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hybrid strategy of treatment combined with percutaneous angioplasty and stenting of the CA.
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Baccari et. al. (17) treated 16 patients by laparoscopic transection of the MAL and only in 4 cases
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needed to complementary angioplasty of the CA. Although PTA and stenting of TC has proved to
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be useful as an adjunctive therapy to prior surgical MAL division, when used as the sole therapy
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without MAL division, patient outcomes have been poor. These results may be due to the sustained
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extrinsic pressure that the intact MAL exerts on the celiac artery, causing a chronic process of
146
intimal hyperplasia and intraluminal narrowing. Multiple case reports have demonstrated that
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endovascular angioplasty alone is unsuccessful at achieving long-term resolution of the symptoms
148
(18-21).
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In conclusion, laparoscopic release of CT compression by MALS is technically feasible and an
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appealing option in centers with large experience in major laparoscopic operations. PTA and
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stenting alone of the CT in the context of MALS should be avoided. In our experience, hybrid
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combined laparoscopic and endovascular procedures seems to guarantee good early- and long-term
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results.
154 155
REFERENCES
156
1. Lainez RA, Richardson WS. Median Arcuate Ligament Syndrome: A Case Report. Ochsner J
157
2013;13:561-4.
158
2. A-Cienfuegos J, Rotellar F, Valentí V, Arredondo J, Pedano N, Bueno A, et al. The celiac axis
159
compression syndrome (CACS). Critical review in the laparoscopic era. Rev Esp Enferm Dig
160
2010;102:193-201.
161
3. Kotarać M, Radovanović N, Lekić N, Ražnatović Z, Djordjević V, Lekć D, et al. Surgical
162
treatment of median arcuate ligament syndrome: case report and review of literature. Srp Arh Celok
163
Lek 2015;143:74-8.
164
4. J. M. Weber, M. Boules, et al. Median Arcuate Ligament Syndrome Is Not a Vascular Disease.
165
Annals of Vascular Surgery, Vol. 30, p22-27.
166
5. Reuter SR and Olin T: Stenosis of the celiac artery. Radiology 85: 616-627, 1965.
167
6. Sakorafas GH, Sarr MG and Peros G: Celiac artery stenosis: an underappreciated and unpleasant
168
surprise in patients undergoing pancreaticoduodenectomy. J Am Coll Surg 206: 349-356, 2008.
169
7. Serkan Burç Deşer, Savas Yuruker, Mustafa Kemal Demirag. Treatment of the Median Arcuate
170
Ligament Syndrome in a Patient with Behcet's Disease. Annals of Vascular Surgery, Vol. 51,
171
p325.e9-325.e11.
172
8. Berney T, Pretre R, Chassot G and Morel P: The role of revascularization in celiac occlusion and
173
pancreatoduodenectomy. Am J Surg 176: 352-356, 1998.
174
9. A. J. Doyle, A. Chandra. Chronic Mesenteric Ischemia in a 26-Year-Old Man: Multivessel
175
Median Arcuate Ligament Compression Syndrome. Annals of Vascular Surgery, Vol. 26, Issue 1,
176
p108.e5-108.e9
177
10. Park CM, Chung JW, Kim HB, et al. Celiac axis stenosis: incidence and etiologies in
178
asymptomatic individuals. Korean J Radiol. 2001;2:8–13.11. Desmond CP, Roberts SK. Exercise-
179
related abdominal pain as a manifestation of the median arcuate ligament syndrome. Scand J
180
Gastroenterol. 2004;39: 1310–1313.
181
12. Cusati DA, Noel AA, Gloviczki P, et al. Median arcuate ligament syndrome: a 20-year
182
experience of surgical treatment. Presented at: 60th Annual Meeting of the Society for Vascular
183
Surgery; June 1-4, 2006; Philadelphia, PA.
184
13. Scholbach T. Celiac artery compression syndrome in children, adolescents, and young adults:
185
clinical and color duplex sonographic features in a series of 59 cases. J Ultrasound Med.
186
2006;25(3):299-305.
187
14. Mensink PBF, van Petersen AS, Kolkman J, Otte JA, Huisman AB, Geelkerken RH. Gastric
188
exercise tonometry: the key investigation in patients with suspected celiac artery compression
189
syndrome. J Vasc Surg 2006;44:277-81.
190
15. Roayaie S, Jossart G, Gitlitz D, Lamparello P, Hollier L, Gagner M. Laparoscopic release of
191
celiac artery compression syndrome facilitated by laparoscopic ultrasound scanning to confirm
192
restoration of flow. J Vasc Surg 2000;32:814-7.
193
16. Jaik NP, Stawicki SP, Weger NS, Lukaszczyk JJ. Celiac artery compression syndrome:
194
successful utilization of robotic-assisted laparoscopic approach. J Gastrointest Liver Dis
195
2007;16:93-6.
196
17. Baccari, P., Civilini, E., Dordoni, L., Melissano, G., Nicoletti, R., & Chiesa, R. (2009). Celiac
197
artery compression syndrome managed by laparoscopy. Journal of Vascular Surgery, 50(1), 134–
198
139. http://doi.org/10.1016/j.jvs.2008.11.124
199
18. Wang X, Impeduglia T, Dubin Z, Dardik H. Celiac revascularization as a requisite for treating
200
the median arcuate ligament syndrome. Ann Vasc Surg. 2008;22(4):571-574.
201
19. Cinà CS, Safar H. Successful treatment of recurrent celiac axis compression syndrome: a case
202
report. Panminerva Med. 2002;44(1):69-72.
203
20. Delis KT, Gloviczki P, Altuwaijri M, McKusick MA. Median arcuate ligament syndrome: open
204
celiac artery reconstruction and ligament division after endovascular failure. J Vasc Surg.
205
2007;46(4): 799-802.
206
21. Matsumoto AH, Tegtmeyer CJ, Fitzcharles EK, et al. Percutaneous transluminal angioplasty of
207
visceral arterial stenoses: results and long-term clinical follow-up. J Vasc Interv Radiol. 1995;6(2):
208
165-174.
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Fig. 1: Angio-Computed Tomography (CTA) of abdominal aorta demonstrating the presence of a focal stenosis at the origin of the celiac axis due to an extrinsic compression induced by median arcuate ligament. Fig. 2: Focal stenosis at the origin of the celiac axis before and after resection of arcuate ligament by laparoscopic approach. To note the partial improvement in size of the celiac axis after pillar section. Fig. 3: Selective catheter angiography of the celiac artery demonstrating the persistence of the stenosis due to extrinsic compression with its fully resolution after balloon angioplasty and stenting of the origin of the vessel.