SURGEON AT WORK
Laparoscopic Repair of Median Arcuate Ligament Syndrome: A New Approach Simone Fajer,
MD,
Randall Cornateanu,
BsC,
Ronen Ghinea,
MD,
Roye Inbar,
MD,
Shmuel Avital,
MD
pain was most pronounced after meals and occasionally woke her up at night. There was a 3-kg weight loss during this time period. On examination, her abdomen was mildly tender, with no peritoneal signs. Her laboratory tests were normal, with no evidence of metabolic acidosis. Before her admission she underwent gastroscopy, which showed no indication of upper gastrointestinal pathology, including peptic disease. A breath test for Helicobacter pylori was negative. Continued investigation with abdominal ultrasound demonstrated severe stenosis of the celiac artery with post-stenotic dilatation. The superior mesenteric artery was patent and normal. Computed tomography angiography demonstrated severe stenosis with post-stenotic dilatation of the celiac artery distal to the origin (Fig. 1). Additionally, there were several small intestinal loops near the right colon, which suggested a possible internal hernia, but with no signs of obstruction. Diagnostic laparoscopy was decided on to rule out the presence of an internal hernia, and it was normal. Due to these results, we considered treatment for MALS and decided on a laparoscopic treatment. We approached the celiac artery from 2 directions: supraceliac aortic dissection from proximal to distal down the aorta and dissection from distal to proximal toward the celiac trunk via the hepatic artery. The steps were as follows:
Median arcuate ligament syndrome (MALS), also known as celiac artery compression syndrome, is a rare disorder characterized by postprandial epigastric pain and nausea, weight loss, and an epigastric bruit. The median arcuate ligament is a fibrous band that crosses over the aorta and connects the right and left crura of the diaphragm. Symptoms are related to an extrinsic compression and result in a narrowing of the celiac artery due to a low insertion of the median arcuate ligament or high origin of the celiac artery.1 This syndrome was first described by Harjola2 in 1963, and the first surgical treatment was reported 2 years later by Dunbar and colleagues.3 Diagnosis of MALS is one of exclusion because many people are found to have incidental celiac artery compression on imaging studies (CT angiography). As many as 10% to 24% of people in the general population may have various degrees of celiac artery compression, but only a few will present with symptoms.1 The evolution of the surgical approach to this syndrome is interesting. Until the 2000s, an open surgical approach with dissection of the ligament around the aorta was the only reported operative method. Concerns regarding a laparoscopic approach were raised due to the potential risk of major vessel injury and subsequent bleeding. However, in the last decade, an increasing number of laparoscopic operations for MALS were reported, with satisfactory results.4 We present here a new laparoscopic approach for MALS that was used by our team in a young patient suffering from this syndrome. We believe that our suggested approach has potential advantages over the standard one.
Disclosure Information: Nothing to disclose.
1. Isolation of the hepatic artery with a vessel loop for retraction; 2. Dissection of the tissue overlying the hepatic artery on the anterior surface of the celiac trunk; 3. Isolation of the esophagus at the hiatus/circling and retraction with a Penrose; 4. Dissection of the tissue and exposure of the supraceliac aorta posterior to the esophagus; 5. Continued dissection toward the origin of the celiac artery; and 6. Identification of the fibers of the median arcuate ligament and resection and release of the fibrous tissue.
Received July 8, 2014; Revised August 11, 2014; Accepted August 19, 2014. From the Vascular Surgery Unit (Fajer, Cornateanu) and the General Surgery Dept B (Ghinea, Inbar, Avital), Meir Medical Center, Kfar Saba, Israel. Correspondence address: Simone Fajer, MD, Vascular Surgery Unit, Meir Medical Center, 59 Tschernichovsky St, Kfar Saba, Israel 44281. email: [email protected]
The postoperative recovery was uneventful and the symptoms were relieved. The patient developed symptoms associated with gastroesophageal reflux, which was treated successfully with a proton pump inhibitor. After 3 months, follow-up with CT angiography (Fig. 2) revealed a patent celiac artery with no significant stenosis.
Case and technique presentation A healthy, 45-year-old female patient presented to the emergency room with abdominal epigastric pain that had exacerbated over the course of several months. The
ª 2014 by the American College of Surgeons Published by Elsevier Inc.
e1
http://dx.doi.org/10.1016/j.jamcollsurg.2014.08.009 ISSN 1072-7515/14
e2
Fajer et al
Laparoscopic Resection of Arcuate Ligament
J Am Coll Surg
Figure 2. Median arcuate ligament syndrome: 3 months after laparoscopic resection of median arcuate ligament. Red arrow, treated celiac artery.
Figure 1. Severe stenosis of celiac artery (red arrows) and poststenotic dilation on CT angiography.
DISCUSSION Median arcuate ligament syndrome classically presents with postprandial abdominal pain, weight loss, and an epigastric bruit. The pathophysiology of pain in this syndrome may be due to limited blood flow through the celiac artery, causing a syndrome of chronic intestinal angina. A second explanation for the pain may be related to the inflammatory fibrosis involving the ligament and celiac axis. There is a controversy regarding the actual existence of this syndrome. It has been reported that a large number of asymptomatic individuals present with varying degrees of celiac artery stenosis. 5-7 However, when there is a critical stenosis with the typical associated symptoms and no other pathology, as in our case, it is reasonable to assume that the symptoms are related to the anatomic findings and a surgical approach should be considered.
During investigation of these patients, it is important to exclude other causes of visceral pain such as biliary and peptic disease using abdominal ultrasound, esophagogastroduodenoscopy (EGD), and a Helicobacter pylori breath test.8 In the past, after the initial work-up, angiography was the gold standard in diagnosing MALS, which can show the indentation in the proximal celiac artery with post-stenotic dilatation.9 Recently, duplex ultrasound (DUS) has also been reported as helpful in determining the degree of stenosis. Imaging of the celiac artery during inspiration and expiration is a sensitive exam in diagnosing MALS.10 Computed tomography with 3-dimensional reconstruction (Fig. 3) is presently the gold standard to evaluate MALS. It adds useful information regarding the regional anatomy and can rule out other causes of celiac artery stenosis (ie, atherosclerosis).9 Mensink and colleagues11 proposed using gastric exercise tonometry because it can detect gastrointestinal ischemia in patients with MALS who would benefit from treatment.11 Regarding therapy, the general principle is to surgically decompress the artery. Several different techniques have been reported in the literature.9,12-14 Percutaneous transluminal angioplasty (PTA) and stenting have also been used; however, they were associated with early restenosis due to external compression from the ligament.12 Stenting can be complementary to excision of the median arcuate ligament at a later stage if needed. Historically, median arcuate ligament release was accomplished by an open surgical technique with higher
Vol.
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No.
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2014
Fajer et al
Laparoscopic Resection of Arcuate Ligament
e3
Figure 3. Three-dimensional reconstruction of CT angiography before (blue arrow) and 3 months after (green arrow) laparoscopic resection of the median arcuate ligament.
morbidity (compared with laparoscopy) associated with laparotomy. The first minimally invasive approach to treating MALS was reported by Roayaie and associates13 in 2000. Since then, the laparoscopic approach has become more widespread. In a case series, by Baccari and coworkers9 from Milan, 16 patients with MALS were treated laparoscopically and all patients had complete resolution of symptoms at long-term follow-up (28.3 months on average), with no residual celiac artery stenosis seen on CT angiography. Two of 16 patients’ operations were converted to open procedures due to bleeding.9 In this publication, the approach to the median arcuate ligament was performed by a dissection along the anterior surface of the aorta.9 In a recent publication by El-Hayek and colleagues,14 15 patients were treated with laparoscopic release of the median arcuate ligament. Ten of the 15 patients had a significant improvement in celiac artery stenosis. There were no conversions to open surgery. Of the 12 patients who followed up in the outpatient clinic, 11 of 12 reported complete resolution of their symptoms.14 In this case, the surgical approach was similar to the previous case, with dissection along the anterior surface of the aorta and moving distally to the celiac trunk.14 In our study, we decided to approach the anterior part of the celiac trunk from 2 directions. Initially we proceeded toward the celiac trunk by dissection of the anterior surface of the hepatic artery and toward the median arcuate ligament, which compressed the celiac artery. Subsequently, we proceeded with dissection along the anterior surface of the aorta toward the origin of the celiac artery from the aorta (2-direction dissection). Through this approach, we were better able to isolate, control, and resect the fibers of the median arcuate ligament with optimal visualization of the regional anatomy.
CONCLUSIONS Laparoscopic release of the median arcuate ligament is a safe and effective alternative to an open surgical approach. Symptomatic relief is seen in a majority of patients after undergoing minimally invasive treatment, as was seen in our patient. Our combined approach of dissecting the celiac trunk from above and below may facilitate this approach. Author Contributions Study conception and design: Fajer, Cornateanu, Ghinea, Inbar, Avital Acquisition of data: Fajer, Cornateanu, Ghinea, Inbar, Avital Analysis and interpretation of data: Fajer, Cornateanu, Ghinea, Inbar, Avital Drafting of manuscript: Fajer, Cornateanu, Ghinea, Inbar, Avital Critical revision: Fajer, Cornateanu, Ghinea, Inbar, Avital
REFERENCES 1. Duffy AJ, Panait L, Eisenberg D, et al. Management of median arcuate ligament syndrome: a new paradigm. Ann Vasc Surg 2009;23:778e784. 2. Harjola PT. A rare obstruction of the celiac artery. Report of a case. Ann Chir Gynaecol Fenn 1963;52:547e550. 3. Dunbar JD, Molnar W, Beman FF, Marable SA. Compression of the celiac trunk and abdominal angina. Am J Roentgenol Radium Ther Nucl Med 1965;95:731e744. 4. A-Cienfuegos J, Rotellar F, Valentı´ V, et al. The celiac axis compression syndrome (CACS): critical review in the laparoscopic era. Rev Esp Enferm Dig 2010;102:193e201. 5. Szilagyi DE, Rian RL, Elliott JP, Smith RF. The celiac artery compression syndrome: does it exist? Surgery 1972;72: 849e863.
e4
Fajer et al
Laparoscopic Resection of Arcuate Ligament
6. Lindner HH, Kemprud E. A clinicoanatomical study of the arcuate ligament of the diaphragm. Arch Surg 1971;103:600e605. 7. Park CM, Chung JW, Kim HB, et al. Celiac axis stenosis: incidence and etiologies in asymptomatic individuals. Korean J Radiol 2001;2:8e13. 8. Lainez RA, Richardson WS. Median arcuate ligament syndrome: a case report. Ochsner J 2013;13:561e564. 9. Baccari P, Civilini E, Dordoni L, et al. Celiac artery compression syndrome managed by laparoscopy. J Vasc Surg 2009;50: 134e139. 10. Erden A, Yurdakul M, Cumhur T. Marked increase in flow velocities during deep expiration: a duplex Doppler sign of celiac artery compression syndrome. Cardiovasc Intervent Radiol 1999;22:331e332.
J Am Coll Surg
11. Mensink PBF, van Petersen AS, Kolkman J, et al. Gastric exercise tonometry: the key investigation in patients with suspected celiac artery compression syndrome. J Vasc Surg 2006;44:277e281. 12. Allen RC, Martin GH, Rees CR, et al. Mesenteric angioplasty in the treatment of chronic intestinal ischemia. J Vasc Surg 1996;24:415e421. 13. Roayaie S, Jossart G, Gitlitz D, et al. Laparoscopic release of celiac compression syndrome facilitated by laparoscopic ultrasound scanning to confirm restoration of flow. J Vasc Surg 2000;32:814e817. 14. El-Hayek KM, Titus J, Bui A. Laparoscopic median arcuate ligament release: are we improving symptoms? J Am Coll Surg 2013;216:272e279.
Laparoscopic Repair of Median Arcuate Ligament Syndrome: A New Approach Simone Fajer,
MD,
Randall Cornateanu,
BsC,
Ronen Ghinea,
MD,
Roye Inbar,
MD,
Shmuel Avital,
MD
pain was most pronounced after meals and occasionally woke her up at night. There was a 3-kg weight loss during this time period. On examination, her abdomen was mildly tender, with no peritoneal signs. Her laboratory tests were normal, with no evidence of metabolic acidosis. Before her admission she underwent gastroscopy, which showed no indication of upper gastrointestinal pathology, including peptic disease. A breath test for Helicobacter pylori was negative. Continued investigation with abdominal ultrasound demonstrated severe stenosis of the celiac artery with post-stenotic dilatation. The superior mesenteric artery was patent and normal. Computed tomography angiography demonstrated severe stenosis with post-stenotic dilatation of the celiac artery distal to the origin (Fig. 1). Additionally, there were several small intestinal loops near the right colon, which suggested a possible internal hernia, but with no signs of obstruction. Diagnostic laparoscopy was decided on to rule out the presence of an internal hernia, and it was normal. Due to these results, we considered treatment for MALS and decided on a laparoscopic treatment. We approached the celiac artery from 2 directions: supraceliac aortic dissection from proximal to distal down the aorta and dissection from distal to proximal toward the celiac trunk via the hepatic artery. The steps were as follows:
Median arcuate ligament syndrome (MALS), also known as celiac artery compression syndrome, is a rare disorder characterized by postprandial epigastric pain and nausea, weight loss, and an epigastric bruit. The median arcuate ligament is a fibrous band that crosses over the aorta and connects the right and left crura of the diaphragm. Symptoms are related to an extrinsic compression and result in a narrowing of the celiac artery due to a low insertion of the median arcuate ligament or high origin of the celiac artery.1 This syndrome was first described by Harjola2 in 1963, and the first surgical treatment was reported 2 years later by Dunbar and colleagues.3 Diagnosis of MALS is one of exclusion because many people are found to have incidental celiac artery compression on imaging studies (CT angiography). As many as 10% to 24% of people in the general population may have various degrees of celiac artery compression, but only a few will present with symptoms.1 The evolution of the surgical approach to this syndrome is interesting. Until the 2000s, an open surgical approach with dissection of the ligament around the aorta was the only reported operative method. Concerns regarding a laparoscopic approach were raised due to the potential risk of major vessel injury and subsequent bleeding. However, in the last decade, an increasing number of laparoscopic operations for MALS were reported, with satisfactory results.4 We present here a new laparoscopic approach for MALS that was used by our team in a young patient suffering from this syndrome. We believe that our suggested approach has potential advantages over the standard one.
Disclosure Information: Nothing to disclose.
1. Isolation of the hepatic artery with a vessel loop for retraction; 2. Dissection of the tissue overlying the hepatic artery on the anterior surface of the celiac trunk; 3. Isolation of the esophagus at the hiatus/circling and retraction with a Penrose; 4. Dissection of the tissue and exposure of the supraceliac aorta posterior to the esophagus; 5. Continued dissection toward the origin of the celiac artery; and 6. Identification of the fibers of the median arcuate ligament and resection and release of the fibrous tissue.
Received July 8, 2014; Revised August 11, 2014; Accepted August 19, 2014. From the Vascular Surgery Unit (Fajer, Cornateanu) and the General Surgery Dept B (Ghinea, Inbar, Avital), Meir Medical Center, Kfar Saba, Israel. Correspondence address: Simone Fajer, MD, Vascular Surgery Unit, Meir Medical Center, 59 Tschernichovsky St, Kfar Saba, Israel 44281. email: [email protected]
The postoperative recovery was uneventful and the symptoms were relieved. The patient developed symptoms associated with gastroesophageal reflux, which was treated successfully with a proton pump inhibitor. After 3 months, follow-up with CT angiography (Fig. 2) revealed a patent celiac artery with no significant stenosis.
Case and technique presentation A healthy, 45-year-old female patient presented to the emergency room with abdominal epigastric pain that had exacerbated over the course of several months. The
ª 2014 by the American College of Surgeons Published by Elsevier Inc.
e1
http://dx.doi.org/10.1016/j.jamcollsurg.2014.08.009 ISSN 1072-7515/14
e2
Fajer et al
Laparoscopic Resection of Arcuate Ligament
J Am Coll Surg
Figure 2. Median arcuate ligament syndrome: 3 months after laparoscopic resection of median arcuate ligament. Red arrow, treated celiac artery.
Figure 1. Severe stenosis of celiac artery (red arrows) and poststenotic dilation on CT angiography.
DISCUSSION Median arcuate ligament syndrome classically presents with postprandial abdominal pain, weight loss, and an epigastric bruit. The pathophysiology of pain in this syndrome may be due to limited blood flow through the celiac artery, causing a syndrome of chronic intestinal angina. A second explanation for the pain may be related to the inflammatory fibrosis involving the ligament and celiac axis. There is a controversy regarding the actual existence of this syndrome. It has been reported that a large number of asymptomatic individuals present with varying degrees of celiac artery stenosis. 5-7 However, when there is a critical stenosis with the typical associated symptoms and no other pathology, as in our case, it is reasonable to assume that the symptoms are related to the anatomic findings and a surgical approach should be considered.
During investigation of these patients, it is important to exclude other causes of visceral pain such as biliary and peptic disease using abdominal ultrasound, esophagogastroduodenoscopy (EGD), and a Helicobacter pylori breath test.8 In the past, after the initial work-up, angiography was the gold standard in diagnosing MALS, which can show the indentation in the proximal celiac artery with post-stenotic dilatation.9 Recently, duplex ultrasound (DUS) has also been reported as helpful in determining the degree of stenosis. Imaging of the celiac artery during inspiration and expiration is a sensitive exam in diagnosing MALS.10 Computed tomography with 3-dimensional reconstruction (Fig. 3) is presently the gold standard to evaluate MALS. It adds useful information regarding the regional anatomy and can rule out other causes of celiac artery stenosis (ie, atherosclerosis).9 Mensink and colleagues11 proposed using gastric exercise tonometry because it can detect gastrointestinal ischemia in patients with MALS who would benefit from treatment.11 Regarding therapy, the general principle is to surgically decompress the artery. Several different techniques have been reported in the literature.9,12-14 Percutaneous transluminal angioplasty (PTA) and stenting have also been used; however, they were associated with early restenosis due to external compression from the ligament.12 Stenting can be complementary to excision of the median arcuate ligament at a later stage if needed. Historically, median arcuate ligament release was accomplished by an open surgical technique with higher
Vol.
-,
No.
-, -
2014
Fajer et al
Laparoscopic Resection of Arcuate Ligament
e3
Figure 3. Three-dimensional reconstruction of CT angiography before (blue arrow) and 3 months after (green arrow) laparoscopic resection of the median arcuate ligament.
morbidity (compared with laparoscopy) associated with laparotomy. The first minimally invasive approach to treating MALS was reported by Roayaie and associates13 in 2000. Since then, the laparoscopic approach has become more widespread. In a case series, by Baccari and coworkers9 from Milan, 16 patients with MALS were treated laparoscopically and all patients had complete resolution of symptoms at long-term follow-up (28.3 months on average), with no residual celiac artery stenosis seen on CT angiography. Two of 16 patients’ operations were converted to open procedures due to bleeding.9 In this publication, the approach to the median arcuate ligament was performed by a dissection along the anterior surface of the aorta.9 In a recent publication by El-Hayek and colleagues,14 15 patients were treated with laparoscopic release of the median arcuate ligament. Ten of the 15 patients had a significant improvement in celiac artery stenosis. There were no conversions to open surgery. Of the 12 patients who followed up in the outpatient clinic, 11 of 12 reported complete resolution of their symptoms.14 In this case, the surgical approach was similar to the previous case, with dissection along the anterior surface of the aorta and moving distally to the celiac trunk.14 In our study, we decided to approach the anterior part of the celiac trunk from 2 directions. Initially we proceeded toward the celiac trunk by dissection of the anterior surface of the hepatic artery and toward the median arcuate ligament, which compressed the celiac artery. Subsequently, we proceeded with dissection along the anterior surface of the aorta toward the origin of the celiac artery from the aorta (2-direction dissection). Through this approach, we were better able to isolate, control, and resect the fibers of the median arcuate ligament with optimal visualization of the regional anatomy.
CONCLUSIONS Laparoscopic release of the median arcuate ligament is a safe and effective alternative to an open surgical approach. Symptomatic relief is seen in a majority of patients after undergoing minimally invasive treatment, as was seen in our patient. Our combined approach of dissecting the celiac trunk from above and below may facilitate this approach. Author Contributions Study conception and design: Fajer, Cornateanu, Ghinea, Inbar, Avital Acquisition of data: Fajer, Cornateanu, Ghinea, Inbar, Avital Analysis and interpretation of data: Fajer, Cornateanu, Ghinea, Inbar, Avital Drafting of manuscript: Fajer, Cornateanu, Ghinea, Inbar, Avital Critical revision: Fajer, Cornateanu, Ghinea, Inbar, Avital
REFERENCES 1. Duffy AJ, Panait L, Eisenberg D, et al. Management of median arcuate ligament syndrome: a new paradigm. Ann Vasc Surg 2009;23:778e784. 2. Harjola PT. A rare obstruction of the celiac artery. Report of a case. Ann Chir Gynaecol Fenn 1963;52:547e550. 3. Dunbar JD, Molnar W, Beman FF, Marable SA. Compression of the celiac trunk and abdominal angina. Am J Roentgenol Radium Ther Nucl Med 1965;95:731e744. 4. A-Cienfuegos J, Rotellar F, Valentı´ V, et al. The celiac axis compression syndrome (CACS): critical review in the laparoscopic era. Rev Esp Enferm Dig 2010;102:193e201. 5. Szilagyi DE, Rian RL, Elliott JP, Smith RF. The celiac artery compression syndrome: does it exist? Surgery 1972;72: 849e863.
e4
Fajer et al
Laparoscopic Resection of Arcuate Ligament
6. Lindner HH, Kemprud E. A clinicoanatomical study of the arcuate ligament of the diaphragm. Arch Surg 1971;103:600e605. 7. Park CM, Chung JW, Kim HB, et al. Celiac axis stenosis: incidence and etiologies in asymptomatic individuals. Korean J Radiol 2001;2:8e13. 8. Lainez RA, Richardson WS. Median arcuate ligament syndrome: a case report. Ochsner J 2013;13:561e564. 9. Baccari P, Civilini E, Dordoni L, et al. Celiac artery compression syndrome managed by laparoscopy. J Vasc Surg 2009;50: 134e139. 10. Erden A, Yurdakul M, Cumhur T. Marked increase in flow velocities during deep expiration: a duplex Doppler sign of celiac artery compression syndrome. Cardiovasc Intervent Radiol 1999;22:331e332.
J Am Coll Surg
11. Mensink PBF, van Petersen AS, Kolkman J, et al. Gastric exercise tonometry: the key investigation in patients with suspected celiac artery compression syndrome. J Vasc Surg 2006;44:277e281. 12. Allen RC, Martin GH, Rees CR, et al. Mesenteric angioplasty in the treatment of chronic intestinal ischemia. J Vasc Surg 1996;24:415e421. 13. Roayaie S, Jossart G, Gitlitz D, et al. Laparoscopic release of celiac compression syndrome facilitated by laparoscopic ultrasound scanning to confirm restoration of flow. J Vasc Surg 2000;32:814e817. 14. El-Hayek KM, Titus J, Bui A. Laparoscopic median arcuate ligament release: are we improving symptoms? J Am Coll Surg 2013;216:272e279.
