|Year : 2021 | Volume
| Issue : 1 | Page : 12-16
Addition of antireflux procedure to median arcuate ligament release: A significant difference?
Rajkumar Janavakula Sankaran, Jayakrishna Reddy Aluru, Rajkumar Shreya, Rajkumar Anirudh
Department of Surgical Gastroenterology, Lifeline Institute of Minimal Access, Chennai, Tamil Nadu, India
|Date of Submission||02-Dec-2020|
|Date of Acceptance||14-Dec-2020|
|Date of Web Publication||14-Jun-2022|
Dr. Jayakrishna Reddy Aluru
Lifeline Institute of Minimal Access, No. 47/3 New Avadi Road, Kilpauk, Chennai - 600 010, Tamil Nadu
Source of Support: None, Conflict of Interest: None
Introduction: Median arcuate ligament syndrome (MALS) is a rare but debilitating gastrointestinal disorder. Division of the ligament (obstructing flow to the celiac artery) cures the symptoms, and a celiac ganglionectomy is also performed. Retroesophageal dissection causes dilatation of the hiatus. Severe gastroesophageal reflux disease (GERD) in the first six patients (Group A) led us to add an antireflux procedure in (Group B) 18 patients. Manometric and 24 h pH data support this. This article has been reported in line with SQUIRE guidelines.
Materials and Methods: Over 7 years, we encountered 24 patients with MALS. The diagnosis was clinched by CT angiography, by the characteristic “Hook sign”. We have devised a standard operating strategy, involving retroesophageal mobilization, diaphragmatic crural exposure dividing the MAL, clearing out the perivascular neural tissue around the celiac axis, and exposing the celiac artery origin and its branches. The procedure was completed by crural approximation; Toupet fundoplication was performed in Group B patients. Relief from MALS symptomatology was obtained in all patients. Group A patients had severe reflux.
Results: All patients were pain free within the 1st month of follow-up and regained normal weight by 6 months. Postoperative reflux symptoms initially were seen in four patients in Group B and all six patients in Group A. By the 6th month postprocedure, all Group B patients were symptom free and all Group A patients had persistent GERD.
Conclusion: To our knowledge, there is no reported series in English literature with a formal protocol for MALS. Our approach provides an efficacious solution to a debilitating disorder. The addition of fundoplication has shown significant improvement in reflux symptoms.
Keywords: Gastroesophageal reflux disease, Hook sign, median arcuate ligament syndrome, Toupet fundoplication
|How to cite this article:|
Sankaran RJ, Aluru JR, Shreya R, Anirudh R. Addition of antireflux procedure to median arcuate ligament release: A significant difference?. Saudi J Laparosc 2021;6:12-6
|How to cite this URL:|
Sankaran RJ, Aluru JR, Shreya R, Anirudh R. Addition of antireflux procedure to median arcuate ligament release: A significant difference?. Saudi J Laparosc [serial online] 2021 [cited 2022 Jun 30];6:12-6. Available from: https://www.saudijl.org/text.asp?2021/6/1/12/347475
| Introduction|| |
The median arcuate ligament syndrome (MALS) is a congenital anomaly resulting from a low lying ligament, causing compression of the celiac artery.,, The vessel hooks upward distal to the compression, causing “Hook sign” [Figure 1]. Symptoms are postcibal abdominal pain and severe weight loss with or without vomiting.,, The diagnosis is best made by computed tomography (CT) angiography, which demonstrates the hook sign of the celiac artery with or without poststenotic dilatation., Angiography may demonstrate retrograde compensatory flow in the superior mesenteric artery (SMA). Doppler studies are also useful., Surgery involves the release of the ligament along with celiac ganglionectomy.,, Severe gastroesophageal reflux disease (GERD) in the first six patients (Group A) prompted us to add a posterior partial fundoplication in the remaining 18 patients (Group B).
The aim of this study was to develop a formal technique to treat MALS, which also addresses the postoperative GERD which occurs due to extensive retroesophageal dissection.
| Materials and Methods|| |
Over 7 years, we encountered 24 patients who were diagnosed with MALS over a 7-year period, from 2013 to 2019, of which 15 were female and nine were male. The age range was from 21 to 55 years. All patients presented with significant weight loss; in addition, 20 patients (5 patients from Group A and 15 patients in Group B) had postcibal abdominal pain, and 6 patients (Group A, 2 patients and 4 patients in Group B) had vomiting. The presenting symptoms are detailed in [Chart 1]. A detailed upper gastrointestinal (GI) workup (UGI scopy and UGI manometry) was performed, including ultrasound of the abdomen, routine blood, and a Doppler of the celiac artery. When suspicion of MALS was significant, CT angiography was performed, which clinched the diagnosis in all the cases.
Operative technique used
This strategy was adopted after understanding the anatomy and available scientific data.,
The esophagogastric (EG) junction was first exposed through five ports as for standard upper GI laparoscopic surgery. A wide retroesophageal space was created behind the EG junction. The right and left crura were dissected out completely from the anterior to posterior decussation. A sling was placed around the EG junction for anterior traction, and the fibers of the median arcuate ligament between the crura were released and progressively, this dissection was continued caudally along the anterior surface of the aorta. All neural and vascular tissue around the aorta and its branches were released, to expose the adventitia of the vessel. Anterior traction on the EG junction with the sling facilitated this step of dissection.
In the second step of the dissection, the common hepatic artery was identified above the head of the pancreas. It was followed proximally toward the celiac axis, dissecting off all the fibrous and neural tissues from the artery.
In the third step, the splenic artery was identified along the border of the body of the pancreas [Figure 2]. Complete perivascular dissection was performed, and the vessel followed proximally toward its origin from the celiac.
Next, the left gastric artery was followed to its origin from the celiac axis [Figure 3], and the periceliac tissue was thoroughly cleared out in order to expose the short broad celiac artery.
Completion of these four steps facilitates an entirely bared celiac – aortic junction and a bilateral celiac ganglionectomy.
Then, the crura were approximated with nonabsorbable sutures, and the fundus was released by taking down the upper two short gastric vessels. The fundus was then passed behind the EG junction, and a Toupet fundoplication, covering 270° of the esophageal circumference, was fashioned with three nonabsorbable sutures on either side, between the fundus and the esophagus.
The MALS release procedure and crural approximation that was carried out in both groups (24 patients) was identical, barring the Toupet wrap, which was done in Group B (all 18 patients) alone.
| Discussion|| |
The MALS was first described by Harjola and Semerable in 1963, and by Dunbar in 1965. The classic triad of weight loss, postprandial abdominal pain, and an epigastric bruit was described by these early investigators,, who also described the procedure of the MALS release. The syndrome is still a controversial entity.
Mesenteric ischemia alone may not be the cause of symptoms in MALS as there are no reports of intestinal infarction caused by MALS, though intermittent foregut ischemia caused by postcibal steal of blood away from the mesenteric circulation may be causative of symptoms. Compression and irritation of celiac ganglionic fibers may also cause pain directly, or indirectly by the splanchnic vasoconstriction that they cause. Higher origin of the celiac axis, above the 12th vertebra, may contribute to compression.
Due to the difficult approach to the area, there was reluctance to surgically treat this condition, until the era of minimally invasive surgery. Like the Western investigators, we found a positive correlation between poststenotic dilatation, more than 10 kg weight loss, and postprandial pain, with postoperative pain relief. Ganglia of the celiac plexus are merged over the adventitia of the left edge of the aorta. Extensive crural dissection and widening of the hiatus led to the addition of Toupet fundoplication in Group B.
The diagnosis has been described with Doppler, CT, or magnetic resonance angiography, but in our series, we found that the Doppler identified only six of the 24 patients (25%), with CT angiography diagnosing all the patients (100%). Superior mesenteric retrograde flow of contrast described on dynamic angiography in literature was not seen in any of our patients. However, poststenotic dilatation, seen in six patients, correlated to slightly higher weight loss and relief from symptoms.
Despite the release of the MAL, in the early studies, a fair proportion of these patients developed recurrent abdominal pain and symptomatology, and researchers over the next three decades pointed to celiac ganglion compression and stimulation as the cause of persistent pain. It was theorized that the ligamentous fibers set off an inflammatory process along the bilateral celiac ganglia and neural fibers, causing retroperitoneal pain, as has been postulated with chronic pancreatitis., In light of these findings, celiac ganglionectomy was advocated by several researchers. Complete exposure of the median arcuate ligament and the base of the crura was facilitated by anterior traction to the EG junction. Complete clearance of all the celiac ganglionic fibers was made possible by perivascular dissection, and freeing the branches of the celiac axis, just as in a D2 lymphadenectomy. Excellent pain relief was achieved by those who added the celiac ganglionectomy, and we incorporated this too, in the steps of our procedure. The peri-esophageal dissection, retroesophageal mobilization, crural, and fundoplication possibly caused transient dysphagia in patients (3 in Group A and 13 in Group B). Few patients (Group A, 1 patient and Group B, 4 patients) presented with bloating, possibly secondary to extensive celiac ganglionectomy, or the release of the upper short gastric vessels. Manometry revealed decreased motility 3 months after surgery in these patients. With prokinetics, by 6 months, all patients were completely asymptomatic.
Comparable with the other investigators, we followed a strict operative strategy for every one of our patients, and the results have been very satisfying.
Finally, some investigators have suggested a therapeutic testing with a temporary celiac ganglionic block, and to proceed with ganglionectomy if effective. With definite vascular compression seen in CT angio, we preferred a direct laparoscopic intervention in our group. Perhaps when the origin of the symptomatology is doubtful, the test would be useful to identify those subsets more likely to respond to ligament release.
| Results|| |
The diagnosis of MALS was suggested by the ultrasound in no patient (0%), by a Doppler of the celiac plexus in six patients (25%), and by CT angiography in all 24 patients (100%). Reversal of SMA flow on dynamic angiography was not found in any of the patients.
All Group A patients (who did not undergo Toupet) presented with severe reflux postoperatively for which their repeat manometry (5 patients) and 24 h pH study (5 patients) showed severely hypotensive lower esophageal sphincter (LES) and reflux [Table 1]. A multidisciplinary team meeting was held which led us to add the Toupet fundoplication.
|Table 1: Average 24 hour pH study and Lower Esophageal Sphincter pressure|
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- Only 19 patients were available for follow-up at 36 months: one at 24 months, two at 6 months, one patient at 3 months, and one at 1 month were seen postoperatively at the time of article submission [Table 2]
- There were no immediate major postoperative complications and zero mortality
- Postoperative CT angiography was performed in 20 patients at 1st month's follow-up which showed disappearance of the hook sign [Figure 4], with weight regain to normalcy in all 24 patients, and with postoperative reflux in 10 patients (Group A; 6 patients, Group B; 4 patients) and bloating in five patients (Group A; 1 patient and Group B; 4 patients) over 3-month follow-up [Chart 2]
- Total 17 patients (5 in Group A and 12 in Group B) underwent follow-up (3rd month) upper GI manometric studies. This showed a lowered resting LES pressure 3–10 mmHg (MMS solar manometry) in all Group A patients. In Group B, 12 patients who had follow-up manometry showed pressures in the normal range (15–50 mmHg). Reflux and slightly decreased gastric motility was noted in the patients with bloating (Group A; 1 patient and Group B; 4 patients)
- Four patients refused postoperative CT-angiography investigations and seven refused 24 h pH study and manometry in Group B
- In Group-B, four patients had dysphagia but regained normal swallowing by the 6-month postoperative follow-up.
|Figure 4: Post-operative CT-Angiogram showing disappearance of Hook sign and Metallic clips near the root of celiac artery|
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| Conclusion|| |
MALS is a complex syndrome for which there is no Level – I recommendation or protocol for surgical management, given its rarity. The jury is still out on whether the symptomatology arises from vascular factors, neural factors, or a combination of both. In our series, we found adding extensive perivascular dissection with celiac ganglionectomy to be a very useful tool to relieve symptoms. Additional Toupet fundoplication appears to prevent postoperative gastroesophageal reflux and is certainly worth further evaluation, so that it can be generalized as a treatment protocol.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]
[Table 1], [Table 2]