Celiac revascularization as a requisite for treating the median arcuate ligament syndrome

Median arcuate ligament syndrome (MALS) is a rare and often misunderstood condition characterized by compression of the proximal portion of the celiac artery by the median arcuate ligament. The existence of MALS, also called "celiac artery compression syndrome" or "Dunbar syndrome," has been questioned since compression of the celiac artery is often demonstrated on aortography in asymptomatic patients. Additionally, isolated stenosis of the celiac artery can be compensated for by collateral circulation from the superior mesenteric artery. Despite these dissenting arguments, there are many cases of patients with this syndrome whose symptoms have been relieved by surgical division of the median arcuate ligament. We report on a case involving a patient with similar symptoms not only to provide further support for the validity for this syndrome but also to emphasize the critical need for revascularization once pathological changes develop in the celiac artery.     

Aneurysm and stenosis of the celiomesenteric trunk: a rare anomaly

A celiomesenteric trunk (CMT) anomaly is extremely rare, occurring in less than 0.5% of people. We describe two cases of CMT anomaly treated surgically for median arcuate ligament compression with stenosis causing intestinal angina and an asymptomatic aneurysm. Disease involving a CMT is extremely uncommon. This comprises the first report of symptomatic median arcuate ligament compression of a CMT. Only four cases of aneurysmal disease are reported so far. Indications and technique of surgical repair of CMT aneurysms are similar to those used for splanchnic artery aneurysms.     

Multimedia article. Laparoscopic management of median arcuate ligament syndrome

Median arcuate ligament syndrome is a rare disorder resulting from luminal narrowing of the celiac artery by the insertion of the diaphragmatic muscle fibers or fibrous bands of the celiac nervous plexus. The syndrome is characterized by weight loss, postprandial abdominal pain, nausea, vomiting, and an epigastric bruit. Surgical management entails complete division of the median arcuate ligament. The video demonstrates the laparoscopic release of the median arcuate ligament in a patient with median arcuate ligament syndrome. The patient is a 22-year-old male with a 6-month history of epigastric abdominal pain, nausea, vomiting, a 140-lb. weight loss, and an epigastric bruit on physical exam. Aortography demonstrated a >or=90% extrinsic compression of the celiac artery. A full laparoscopic skeletonization of the celiac artery and branch vessels was performed. Intraoperative duplex U/S demonstrated flow rate reduction after the median arcuate ligament release. A postoperative CT angiogram demonstrated no residual stenosis. The patient was discharged on postoperative day 3 and remained asymptomatic after 7 months of follow-up. Laparoscopic release of the median arcuate ligament is a novel approach to the management of celiac artery compression syndrome. The role of minimally invasive techniques to manage median arcuate ligament syndrome is evolving but they appear to be a safe alternative to open surgery.     

Das Syndrom des Lig. arcuatum medianum mit Zweigefäßbeteiligung

In median avcuate ligament syndrome, compression of the celiac artery is caused by an abnormally low insertion of the median arcuate ligament in the presence of normally located celiac and superior mesenteric arteries. A lot of controversy exists concerning the indication for operation and the best mode of treatment. Depending on the presence and quality of collateral pathways, the symptoms vary considerably. We report a rare case of arcuate ligament syndrome with two-vessel involvement. In such cases surgical treatment seems to offer substantial benefit. Usually transsection of the compressing arcuate ligament is sufficient. Bypass procedures should remain limited to cases in which transsection does not enable a significant increase in splanchnic blood flow.     

External compression of the superior mesenteric artery by the median arcuate ligament

The median arcuate ligament can compress the proximal portion of the celiac artery causing symptoms of chronic mesenteric ischemia. This rare condition typically affects young women and often poses a diagnostic challenge. Compression of the superior mesenteric artery (SMA) in addition to the celiac artery represents an unusual variant of median arcuate ligament syndrome (MALS). We present a case of MALS resulting predominantly from external compression of the SMA. Diagnostic and therapeutic options are discussed.     

Laparoscopic management of median arcuate ligament syndrome

Median arcuate ligament syndrome is a rare disorder resulting from luminal narrowing of the celiac artery by the insertion of the diaphragmatic muscle fibers or fibrous bands of the celiac nervous plexus [1, 3]. The syndrome is characterized by weight loss, postprandial abdominal pain, nausea, vomiting, and an epigastric bruit [2]. Surgical management entails complete division of the median arcuate ligament [4]. The video demonstrates the laparoscopic release of the median arcuate ligament in a patient with median arcuate ligament syndrome.The patient is a 22-year-old male with a 6-month history of epigastric abdominal pain, nausea, vomiting, a 140-lb. weight loss, and an epigastric bruit on physical exam. Aortography demonstrated a ≥90% extrinsic compression of the celiac artery. A full laparoscopic skeletonization of the celiac artery and branch vessels was performed. Intraoperative duplex U/S demonstrated flow rate reduction after the median arcuate ligament release. A postoperative CT angiogram demonstrated no residual stenosis. The patient was discharged on postoperative day 3 and remained asymptomatic after 7 months of follow-up.Laparoscopic release of the median arcuate ligament is a novel approach to the management of celiac artery compression syndrome [2]. The role of minimally invasive techniques to manage median arcuate ligament syndrome is evolving but they appear to be a safe alternative to open surgery.Video presented at the annual meeting of the society of American Gastrointestinal Endoscopic Surgeons (SAGES) annual scientific session in Denver, Colorado, March 31–April 3, 2004     

Pediatric surgical image. A rare case: celiac artery compression syndrome in an asymptomatic child

Celiac artery compression syndrome is a rare disorder and charecterized by postprandial intestinal angina caused by insufficient blood supply to the gastrointestinal organs in symptomatic patients. This pathology is also known as median arcuate syndrome. The authors report a case of 12-year-old boy with severe celiac artery compression by the median arcuate ligament that was diagnosed by Magnetic Resonance Angiography (MRA) and color dupplex ultrasonography (US). There was only a bruit in the upper midepigastrium, and there were no gastrointestinal symptoms despite severe compression of celiac trunk. This clinicopathologic entity is very rare but represents severe complaints and clinical results caused by a decreased rate of the intestinal blood flow. In this report the authors suggest that the diagnosis of celiac artery compression should be considered in asymptomatic pediatric patients representing abdominal bruit or with palpated thrill in the upper epigastrium. In comparison with case reports present in the English-language literature, the patient reported on here is the youngest diagnosed.     

Median arcuate ligament syndrome: vascular surgical therapy and follow-up of 18 patients

Introduction  

The median arcuate ligament syndrome (MALS) or celiac artery compression syndrome is a rare vascular disorder caused by an extrinsic compression of the celiac artery from the median arcuate ligament, prominent fibrous bands, and ganglionic periaortic tissue. Clinical symptoms are postprandial abdominal pain, nausea, vomiting, unintentional weight loss, and sometimes, abdominal pain during body exercise caused by an intermittent visceral ischemia. The aim of this study was to evaluate the operative management of patients with MALS in our institution, especially in consideration of various vascular reconstructive techniques.     

True celiac artery aneurysm secondary to median arcuate ligament syndrome

Median arcuate ligament syndrome (MALS) is a rare disorder associated with chronic, postprandial abdominal pain and radiographic evidence of celiac artery compression. True arterial aneurysms distal to sites of chronic vascular compression due to post-stenotic dilatation have been described in other anatomic locations such as thoracic outlet syndrome and popliteal entrapment syndrome. We present the case of a patient who presented to vascular surgery clinic with a 35 year history of epigastric abdominal pain and bloating and was found to have a large celiac artery aneurysm with severe compression by the MAL.     

Laparoscopic treatment of Dunbar syndrome: A case report

BackgroundDunbar syndrome is a rare disorder due to external compression of the celiac trunk by the median arcuate ligament. The symptoms include abdominal pain, nausea and vomiting. Laparoscopic treatment was suggested as a safe procedure. The aim of this study is to present a case of Dunbar syndrome underwent laparoscopic treatment.CasepresentationA 17-year-old female patient presented at emergency room with upper abdominal pain and dyspepsia, related to food intake. A selective arteriography of the celiac trunk revealed stenosis due to compression of the celiac artery. The decompression of the celiac trunk by the median arcuate ligament was performed. Postoperative course was uneventful and the patient was discharged on the 5th postoperativeConclusionsLaparoscopic division of the arcuate ligament in patients with Dunbar syndrome is feasible and safe. This procedure can be performed uneventful in order to restore quality of life of the patient.     

Median arcuate ligament syndrome: open celiac artery reconstruction and ligament division after endovascular failure

Median arcuate ligament syndrome (MALS) is a rare disorder resulting from extrinsic compression and narrowing of the celiac artery, and--less often--the superior mesenteric artery, by the relatively low insertion of the ligament and/or prominent fibrous bands or ganglionic periaortic tissue of the celiac nervous plexus. We report on a young woman who after three consecutive attempts of endovascular therapy with balloon angioplasty and stenting for MALS, each followed by gross symptom recurrence and a cumulative weight loss of 10 kg, underwent open surgical division of the ligament and reconstruction of the celiac artery. Despite the initial response of MALS to endovascular therapy, the extrinsic pressure exerted on the celiac artery by the surrounding dense fibrous/ganglionic tissue resulted in slippage of the stents and/or failure of their material. These findings militate against the use of balloon angioplasty and stenting primarily in patients with MALS without prior release of the extrinsic compression on the celiac (and/or superior mesenteric) artery by dividing the surrounding median arcuate ligament and/or ganglionic tissue with open or laparoscopic surgery.     

Median arcuate ligament compression syndrome in monozygotic twins

Twin 27-year-old women had symptomatic mesenteric ischemia caused by median arcuate ligament compression. Arteriography demonstrated severe celiac artery stenosis in one twin, celiac artery occlusion in the other, and proximal superior mesenteric artery narrowing with retrograde filling from a meandering mesenteric artery in both. Division of the ligament and direct celiac artery revascularization completely relieved symptoms in both patients. Median arcuate ligament compression of the celiac and superior mesenteric arteries can result in mesenteric ischemia. Documentation of this unusual syndrome in monozygotic twins suggests that the responsible anatomic relationships are congenital. (J VASC SURG 1994;19:934-8.)     

Celiac compression syndrome and liver transplantation.

OBJECTIVE: The authors assessed the prevalence and clinical significance of the celiac compression syndrome in liver transplantation patients. SUMMARY BACKGROUND DATA: Compression of the celiac axis by the median arcuate ligament of the diaphragm, causes a decrease in celiac artery blood flow which may lead to hepatic artery thrombosis in patients undergoing orthotopic liver transplantation. METHODS: From July 1991 to July 1992, 17 (10%) cases of celiac compression syndrome were identified among 164 consecutive adult patients who underwent liver transplantation. The diagnosis was confirmed by blood flow recording demonstrating a typical pattern of accentuated decrease in celiac blood flow during expiration. RESULTS: Surgical transection of the median arcuate ligament resulted in normalization of the hepatic artery blood flow. In two cases (11.7%), an interposition iliac graft from the recipient supra-celiac aorta was used for the arterial reconstruction. During the follow-up period of up to 15 months, there was no incidence of hepatic artery thrombosis. CONCLUSIONS: The clinical significance of the celiac compression syndrome is evident in liver transplantation in which the collateral circulation to the liver is compromised and the celiac artery remains the only source of arterial blood. It is imperative to identify and remove the obstruction of the celiac axis to prevent severe complications and potential graft loss.     

Pediatric median arcuate ligament syndrome: first report of familial pattern and transperitoneal laparoscopic release

Median arcuate ligament syndrome (MALS) is a rare disorder resulting from luminal narrowing of the celiac artery by the insertion of the diaphragmatic muscle fibers. Surgical management entails division of the median arcuate ligament with or without celiac artery reconstruction. We are presenting an interesting case of a 16-year-old girl with postprandial abdominal pain and weight loss. Her mother also had MALS treated via open celiotomy with complete median arcuate ligament division and patch angioplasty of the celiac artery owing to persistent stenosis at our institution. After a diagnosis of MALS was confirmed in our patient, a transperitoneal laparoscopic release of the median arcuate ligament with skeletonization of the celiac artery and branch vessels was performed. The postoperative course was uneventful, and she was dismissed on postoperative day 2. She remains asymptomatic at 12-months follow-up. This represents the first report of a transperitoneal laparoscopic approach to MALS in an adolescent and the first report of a familial/generational component to MALS.     

Celiac artery compression syndrome

Celiac artery compression syndrome occurs when the median arcuate ligament of the diaphragm causes extrinsic compression of the celiac trunk. We report a case of a 65-year-old woman who presented with a three-month history of postprandial abdominal pain, nausea and some emesis, without weight loss. There was a bruit in the upper mid-epigastrium and the lateral aortic arteriography revealed a significant stenosis of the celiac artery. At operation, the celiac axis was found to be severely compressed anteriorly by fibers forming the inferior margin of the arcuate ligament of the diaphragm. The ligament was cut and a vein by-pass from the supraceliac aorta to the distal celiac artery was performed. The patient remains well and free of symptoms two and a half years since operation.In this report we discuss the indications and the therapeutic options of this syndrome as well as a review of the literature is being given.     

Median arcuate ligament syndrome: a nonvascular, vascular diagnosis

Median arcuate ligament syndrome (MALS) is often diagnosed when idiopathic, episodic abdominal pain is associated with dynamic compression of the proximal celiac artery by fibers of the median arcuate ligament. The character of the abdominal pain is often postprandial and associated with gradual weight loss from poor food intake, suggestive of chronic mesenteric ischemia. However, the pathognomonic imaging feature of dynamic, ostial celiac artery compression with expiration does not consistently predict clinical improvement from revascularization. Proposed but unproven pathophysiological mechanisms include neurogenic pain from compression of the splanchnic nerve plexus and intermittent ischemia from compression of the celiac artery. Alterations in blood flow and ganglion compression are both associated with delayed gastric emptying, another physiological correlate of the clinical syndrome. Published reports describe a variable response to revascularization and nerve plexus resection suggest a need for translational research to better characterize this poorly understood clinical entity. We illustrate the current gaps in our knowledge of MALS with the case of a 51-year-old woman with a 4-year history of chronic abdominal pain who responded to a combination of ganglion resection and celiac artery reconstruction.     

Hemodynamic benefits of celiac artery release for ruptured right gastric artery aneurysm associated with median arcuate ligament syndrome: a case report

Background

It has been reported that median arcuate ligament syndrome is closely associated with gastric or pancreaticoduodenal artery aneurysms. Hemodynamic state plays an important role in the formation of the aneurysms. These aneurysms are treated with open resection or endovascular exclusion. However, whether revascularization of the celiac artery can prevent the aneurysm formation is unknown. This report indicated a possibility that prophylactic revascularization for celiac artery stenosis resulted in decreased shear stress on the collaterals, which may otherwise be susceptible to new aneurysms.

Case presentation

This report describes a 51-year-old man who presented with epigastric pain at our hospital. According to contrast enhanced computed tomography (CT), he was diagnosed with a ruptured right gastric artery aneurysm and celiac artery stenosis caused by the median arcuate ligament (MAL). He had a vascular anomaly of the common hepatic artery arising from the superior mesenteric artery (SMA). His vital signs were stable. We informed him of the situation and he chose open surgery rather than endovascular treatment. Following, we resected the aneurysm and transected the MAL. Intraoperative angiography after transection of the MAL showed the antegrade blood flow to the splenic artery instead of the retrograde flow via the prominent collaterals. Follow-up CT confirmed narrowed collateral vessels between the SMA and the celiac artery without de-novo aneurysms.

Conclusion

While the necessity of celiac artery release could be questioned, the present case supports the hemodynamic benefits of MAL transection in terms of de-novo aneurysm prevention.

     

Laparoscopic release of celiac artery compression syndrome facilitated by laparoscopic ultrasound scanning to confirm restoration of flow

A 43-year-old woman presented with symptomatic mesenteric ischemia caused by median arcuate ligament compression of her celiac artery. Magnetic resonance angiography clearly demonstrated stenosis of the proximal celiac artery. She underwent laparoscopic decompression by division of the ligament and excision of the celiac plexus. Laparoscopic Doppler ultrasound scanning demonstrated markedly improved flow in the artery. She was discharged in 15 hours and reported complete resolution of her symptoms at the 3-month postoperative visit. Laparoscopy provides a less invasive but equally effective method for decompressing the celiac artery as well as assessing adequacy of flow after its release.     

Pancreaticoduodenectomy and the celiac artery compression syndrome

Celiac compression is usually a benign condition, but when surgery necessitates division of collaterals from the superior mesenteric artery, it may cause life-threatening gut ischemia. We report a case of cholangiocarcinoma necessitating pancreaticoduodenectomy in a patient with celiac artery compression by the median arcuate ligament. Preoperative duplex scanning confirmed the celiac stenosis and revealed retrograde flow through collaterals from the superior mesenteric artery. Intraoperative continuous wave Doppler examination revealed that gastric blood flow disappeared with compression of the superior mesenteric artery. This maneuver no longer affected gastric flow after transection of the compressing structures at the celiac origin. Preoperative identification of celiac artery stenosis is crucial to prevent small bowel ischemia and possible anastomotic breakdown or liver failure. Duplex scanning can provide important insight about collateral circulation, and intraoperative Doppler testing can assess the adequacy of revascularization.