Celiac Axis Compression Syndrome Research Articles

MDCT Evaluation of Celiac Axis Compression SyndromeA Rare Case Series

Celiac axis compression syndrome is an uncommon entity of abdominal vascular compression syndrome which results from the compression of the proximal celiac artery by the hypertrophied median arcuate ligament. The patients present clinically with postprandial abdominal pain predominantly in the epigastric region and weight loss. The use of thin-section Multidetector Computed Tomography (MDCT) and 3-Dimensional (3D) image reconstruction has greatly improved the ability to obtain detailed images and helped to assess the abdominal vascular system non invasively. Five cases of celiac axis compression syndrome with its characteristic MDCT imaging features like hooked appearance, thickened median arcuate ligament more than 4 mm, poststenotic dilatation and posterior and inferior displacement of celiac artery with indentation on aorta have been described in the present case series and the imaging findings described by other authors in their reported studies have also been compared.

Pancreatoduodenectomy co-morbid with celiac axis compression syndrome: a report of three cases

BackgroundCeliac axis compression syndrome (CACS) is a relatively rare disease. Because of the nature of the blood flow in the celiac region when a pancreatoduodenectomy (PD) is performed for CACS, the celiac region can become ischemic. The aim of this study is to report on the importance of pre-operative diagnosis of CACS in terms of the outcomes for patients post-operatively. In this study, three 3 cases of PD co-morbid with CACS are reported: one intra-operative diagnosis case and two pre-operative diagnosis cases.Case presentationThe one case, not diagnosed with CACS prior to the operation, had a hard post-operative course because of complication caused by ischemia of the celiac region compared with the two cases diagnosed prior to the operation, who had a good post-operative course because of pre-operative or intra-operative intervention.ConclusionsPost-operative complications due to CACS are preventable by pre-operative diagnosis and appropriate interventions.

Utility of Doppler Ultrasonography in Liver Transplantation With Celiac Axis Compression Syndrome: A Case Report

BackgroundCeliac axis compression syndrome (CACS) blocks adequate hepatic arterial flow and is a risk factor for hepatic artery thrombosis after liver transplantation. We report a case of living donor liver transplantation in a 65-year-old Brazilian male with liver cirrhosis of Child-Pugh class C and hepatocellular carcinoma caused by hepatitis C virus infection. ResultsThe patient underwent living donor liver transplantation using the graft of his 34-year-old daughter. Stenosis of the celiac artery was detected on preoperative computed tomography (CT), and CACS was suspected. Maintaining blood supply through the hepatic artery to prevent potential graft loss is essential in liver transplantation. A decrease in common hepatic artery (CHA) flow due to CACS could disturb graft blood supply or lead to hepatic artery thrombosis. In this case, we confirmed CACS through dynamic CT and used intraoperative Doppler ultrasonography (US) to plan the surgical procedure. Three types of hepatic artery reconstruction have been described in liver transplantation for CACS, namely the release of the median arcuate ligament (MAL), aorto-hepatic graft reconstructions, and reconstruction preserving the gastroduodenal artery (GDA) without MAL release. We found that clamping the GDA completely abolished hepatic inflow, but clamping the CHA did not change the hepatic inflow. Therefore, we performed arterial reconstruction without division of the GDA. The patient's postoperative course was good, with excellent hepatic artery flow, as assessed by Doppler US. ConclusionsPreoperative dynamic CT evaluation, adequate preparation of surgical procedures, and intraoperative evaluation by Doppler US is recommended in liver transplantation patients with CACS.

A case of negative computed tomography in liver transplant recipient with medium arcuate ligament syndrome (MALS). When to discard radiology in favor of surgical experience?

Introduction: MALS, also known as celiac axis compression syndrome or Dunbar syndrome, is a rare and nonspecific disorder, when the median arcuate ligament compresses the celiac trunk due to the abnormal position of either the celiac trunk or the median arcuate ligament. Diagnosis of MALS is confirmed with positive findings on computer tomography (CT) or magnetic resonance angiography combined with duplex ultrasonography (USG). MALS is treated with the surgical release of the median arcuate ligament and celiac ganglionectomy. Case: 41 year old female underwent a liver transplantation for unresectable epithelioid hemangioendothelioma. Following the transplantation, abdominal USG and CT showed no flow within the left and right hepatic arteries. No evident stricture in 3D CT reconstruction was found. Complete thrombosis of the common hepatic artery was assumed and the patient was retransplanted six days later. During retransplantation, blood flow through the initially thrombosed hepatic artery, was not achieved until the dissection of the median arcuate ligament. Conclusion: Duplex USG may be superior to CT for diagnosing MALS as it reveals real time images where functional compression may be the case. When patient performs full expiration, USG shows this compression, if present, on the celiac trunk. CT during full inspiration will commonly show undisturbed arterial alignment and blood flow. Early diagnosis and treatment of MALS is a graft saving procedure. The surgeon should bare in mind that it’s the patient we ought to treat and not the radiology.

Laparoscopic Division of Median Arcuate Ligament for the Celiac Axis Compression Syndrome-Two Case Reports with Review of Literature.

Median arcuate ligament (MAL) syndrome is an uncommon condition caused by the external compression of the celiac trunk by the median arcuate ligament. In the current era of technological advancement, this syndrome may be corrected through the laparoscopic approach. We report two patients who were diagnosed as MAL syndrome and underwent laparoscopic division of MAL fibers at our institute. Both the patients improved symptomatically following the procedure and were discharged on the fourth post-operative day. Also, they remained symptom free during subsequent follow-up period of 1year and 8months, respectively. Laparoscopic approach to correct the MAL syndrome is feasible and safe. It may be the preferred modality of treatment in view of its superior visualization and lack of morbidity. However, adequate experience in advanced laparoscopic surgery is required before attempting this procedure.

Uncommon Ethiology in Abdominal Pain: Celiac Axis Compression Syndrome

Celiac axis compression syndrome, one of the reasons of mesenteric ischemia, is an extremely rare etiology of abdominal pain. Primary pathological mechanism is the external compression of the celiac trunk by median arcuate ligament. The diagnosis of this condition is usually difficult and depends on angiographic findings and computerized tomography (CT) evaluations. Here we report a celiac axis compression syndrome case, presenting with chronic abdominal pain and weight loss, which was successfully treated by surgery.

Celiac axis compression syndrome: laparoscopic approach in a strange case of chronic abdominal pain in 71 years old man.

Celiac Axis Compression Syndrome by the Median Arcuate Ligament is a very rare condition characterized by chronic postprandial abdominal pain (angina abdominis), nausea, vomiting, which occurs mostly in young patients. The main treatment is a surgical procedure that consists of the division of the arcuate ligament combined with the section of the close diaphragmatic crus and the excision of the celiac plexus. Actually laparoscopic management is feasible and safe.

Exercise-induced Abdominal Pain: Median Arcuate Ligament Syndrome

Introduction: The median arcuate ligament joins the right and left crura of the diaphragm while allowing passage of the aorta into the abdomen. Median arcuate ligament syndrome (MALS) or celiac axis compression syndrome (CACS) is a result of a low lying median arcuate ligament compressing the proximal celiac trunk (branch of aorta supplying primarily the stomach) causing intermittent ischemic symptoms of abdominal pain, weight loss and exercise intolerance. Case Report: A 70-year-old Caucasian with presented with a one year history of intermittent aching abdominal pain located in the epigastric and left hypochondriac region. The pain is constant with frequent exacerbations provoked by eating (variable) and exercising (consistent), relieved by cessation of eating and by physical rest respectively. Exacerbations led to alternating changes in bowel movements from constipation to diarrhea along with weight loss of ten pounds over the last two months. The patient denied any nausea, vomiting, fevers or bloody bowel movements. Multiple colonoscopies and endoscopies have been negative. Physical exam was unremarkable except for mild tenderness in the epigastric region. Computerized Tomography (CT) abdomen with contrast showed poor contrast flow through the celiac artery with a “hooked” appearance. The diagnosis of MALS was made and the patient was referred to surgery for medial arcuate ligament division. Discussion: Patient with chronic abdominal pain associated with weight loss and exacerbations with exercise should have MALS in the differential. Eating stimulates increased metabolic demand for the stomach leading to mismatched perfusion via a compromised celiac artery. During exercise, the diaphragm expands at a higher frequency and force to accommodate for increased oxygen demand. This leads to further reduction of celiac artery flow in patient with MALS. CT Sagital images demonstrating acute angulation and narrowing of the proximal celiac artery causing a “hooked” appearance (arrow) is characteristic of median arcuate ligament syndrome.Figure: Arrow indicates “hooked” appearance of celiac artery due to compression by medial arcuate ligament from above.

An unusual origin of the celiac trunk and the superior mesenteric artery in the thorax

The authors report a case of a 44-year-old male found to have unusual origins of the celiac trunk (CT) and superior mesernteric artrery (SMA) as revealed by routine multidetector computed tomograph (MDCT) angiography. The CT and SMA originate from the thoracic aorta (TA) 21 mm and 9 mm above the aortic hiatus, respectively. The median arcuate ligament (MAL) is located at the level of the L1-L2 intervertebral disc. The course of the CT descends in the thoracic cavity making a 14° acute downward angle in front of the TA; below the level of the MAL, the CT descends, making an angle of 47°. The course of the SMA descends at both the thoracic and abdominal level making an angle of 17°, and having an aortomesenteric distance of 9 mm at the level of the third part of the duodenum. In the present case, the supradiaphragmatic origin of the CT and the SMA was determined by their incomplete caudal descent, associated with a pronounced apparent descent of the diaphragm. A thoracic origin of the CT and SMA and the acute downward aortomesenteric angle (17°) associated with a reduced aortomesenteric distance at the level of the third part of the duodenum (9 mm), although no clinical signs are present, may predispose the patient to develop simultaneously a triple syndrome: the compression of CT by MAL (celiac axis compression syndrome), the compression of SMA by MAL (superior mesenteric artery compression syndrome), and the compression of the duodenum by the SMA (superior mesenteric artery syndrome).

A Severe Case of Median Arcuate Ligament Syndrome with Successful Angioplasty and Stenting

Median arcuate ligament syndrome (MAL) or celiac axis compression syndrome (CACS) is a rare etiology of chronic abdominal pain. Traditional treatment of this syndrome is surgery. We report a case of median arcuate ligament syndrome with a severe compression of the celiac trunk, which was successfully treated by angioplasty with stenting.

Laparoscopic release of median arcuate ligament

Median arcuate ligament (MAL) syndrome, also known as the celiac axis compression syndrome, is rare. It is a diagnosis of exclusion, characterised by the clinical triad of postprandial abdominal pain, weight loss and vomiting. Computed tomographic angiography is the gold standard for making the diagnosis of MAL and colour Doppler is essential to confirm the diagnosis. The classic management involves the surgical division of the MAL fibres. We report successful management of two patients diagnosed as MAL syndrome and treated by laparoscopic release of the MAL.

The celiac axis compression syndrome (CACS): critical review in the laparoscopic era

The celiac axis compression syndrome (CACS) due to median arcuate ligament (MAL) was first described by Harjola in 1963; originating postpandrial abdominal pain, weight loss, epigastric bruit and celiac axis stenosis > 75% in angiographic studies. This clinical condition has been the origin of controversies about its pathogenesis, diagnosis and its long term clinical results. Advances in diagnostic imaging as 64 multidetector-row CT (MDCT), 3-D reconstruction, magnetic resonance (MR) and color duplex ultrasonography, provide better understanding of the syndrome and allow to identify the best candidates for surgical division of MAL fibers. Since the introduction of laparoscopic approach, and also endovascular procedures, in 2000, a new perspective has established in this challenging syndrome. With the occasion of our own experience, a critical review of the syndrome is presented.

Eleven Cases of Postoperative Hepatic Infarction Following Pancreato-Biliary Surgery

BackgroundPostoperative hepatic infarction is rare; therefore, clinical characteristics and outcomes of postoperative hepatic infarction after pancreatobiliary surgery have not been obvious. MethodsEleven patients encountered hepatic infarction after pancreato-biliary surgery. Management, clinical course, and outcome of these 11 patients were retrospectively analyzed. ResultsPossible causes of the hepatic infarction were inadvertent injury of the hepatic artery during lymph node dissection in five patients, right hepatic artery ligation in two patients, long-term clamp of the hepatic artery during hepatic arterial reconstruction in two patients, suturing for bleeding from the right hepatic artery in one patient, and celiac axis compression syndrome in one patient. Five of the 17 infarcts extended for one whole section of the liver, and distribution of the other 12 was less than one section. Ten patients discharged from hospital; however, one patient died of sepsis of unknown origin. ConclusionsAttention should be paid to inadvertent injury of hepatic artery to prevent hepatic infarction. Hepatic infarctions after pancreato-biliary surgery seldom extend to the entire liver and most of them are able to be treated without intervention.

Superior mesenteric artery syndrome caused by celiac axis compression syndrome: a case report and review of the literature

Celiac axis compression syndrome (CACS) is a rare entity of mesenteric ischemia, secondary to inadequate blood supply to the intestine, resulting in weight loss because of postprandial abdominal pain. Superior mesenteric artery (SMA) syndrome is an uncommon cause of intestinal obstruction manifesting with epigastric pain, bilious vomiting, and postprandial discomfort. Although the coexistence of both syndromes is very rare and has been reported only in eight patients in the literature, the CACS as a rare etiology of SMA syndrome has not yet been reported. Herein, we describe an uncommon case of SMA syndrome secondary to the CACS. The 27-year-old woman presented with epigastric pain, postprandial vomiting, and rapid body weight loss. The diagnosis of SMA syndrome was made by hypotonic duodenography and multidetector computer tomographic angiography. The CACS was also suspected by multidetector computer tomographic angiography. Surgical intervention was performed and the presence of CACS was confirmed. Her symptoms subsided shortly after operation and she was in good health at 1-year follow-up.

Diagnosis and management of splanchnic ischemia.

Splanchnic or gastrointestinal ischemia is rare and randomized studies are absent. This review focuses on new developments in clinical presentation, diagnostic approaches, and treatments. Splanchnic ischemia can be caused by occlusions of arteries or veins and by physiological vasoconstriction during low-flow states. The prevalence of significant splanchnic arterial stenoses is high, but it remains mostly asymptomatic due to abundant collateral circulation. This is known as chronic splanchnic disease (CSD). Chronic splanchnic syndrome (CSS) occurs when ischemic symptoms develop. Ischemic symptoms are characterized by postprandial pain, fear of eating and weight loss. CSS is diagnosed by a test for actual ischemia. Recently, gastro-intestinal tonometry has been validated as a diagnostic test to detect splanchnic ischemia and to guide treatment. In single-vessel CSD, the complication rate is very low, but some patients have ischemic complaints, and can be treated successfully. In multi-vessel stenoses, the complication rate is considerable, while most have CSS and treatment should be strongly considered. CT and MR-based angiographic reconstruction techniques have emerged as alternatives for digital subtraction angiography for imaging of splanchnic vessels. Duplex ultrasound is still the first choice for screening purposes. The strengths and weaknesses of each modality will be discussed. CSS may be treated by minimally invasive endoscopic treatment of the celiac axis compression syndrome, endovascular antegrade stenting, or laparotomy-assisted retrograde endovascular recanalization and stenting. The treatment plan is highly individualized and is mainly based on precise vessel anatomy, body weight, co-morbidity and severity of ischemia.

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.

A Rare Cause of Mesenteric Ischemia: Celiac Axis Compression Syndrome

Mesenteric ischemia is an uncommon etiology of abdominal pain. Celiac axis compression syndrome is an extremely rare cause of mesenteric ischemia. The primary pathological mechanism is the external compression of the celiac trunk by median arcuate ligament. The clinical manifestation of celiac axis compression syndrome includes postprandial pain, diarrhea, and body weight loss. The diagnosis of this disease is usually difficult and depends on the angiography findings. For treatment, only percutaneous transluminal angioplasty and surgical intervention have been suggested in reviews in the literature. We, herein, report an unusual case of celiac axis compression syndrome and also review the literature pertaining to this disease.

Gastric exercise tonometry: The key investigation in patients with suspected celiac artery compression syndrome

Controversy continues about the mere existence of the celiac artery compression syndrome. Earlier results of treatment of unselected patients groups showed varying, mostly disappointing, results. The recently introduced gastric exercise tonometry test is able to identify patients with actual gastrointestinal ischemia. We prospectively studied the use of gastric exercise tonometry as a key criterion for revascularization treatment in patients with otherwise unexplained abdominal complaints and significant stenosis of the celiac artery by compression of the arcuate ligament. Patients were prospectively selected using abdominal artery angiography and gastric exercise tonometry. Patients with a significant compression of the celiac artery, typical abdominal complaints, and abnormal tonometry were considered for revascularization. Over a 7-year period, 43 patients with significant celiac artery compression were included in this study, and 30 patients were diagnosed as ischemic. Twenty-nine patients had revascularization, 22 (76 %) had a trunk release only. After a median follow-up of 39 months, 83% of patients were free of symptoms. The repeated tonometry after treatment improved in 100% of patients free of symptoms, compared with 25% in patients with persistent complaints after revascularization. The results of this study suggest that the celiac axis compression syndrome exists and that the actual ischemia can be detected by gastric exercise tonometry and treated safely, with success.

Gastrointestinal: Celiac axis compression syndrome

A woman, aged 78 years, was investigated because of a 3-month history of nausea, anorexia and epigastric discomfort. She was known to have ischemic heart disease and had been previously treated by angioplasty and a coronary artery stent. On examination she had an epigastric bruit. Multi-slice computed tomography angiography (MSCT) of the coronary arteries did not reveal any significant stenoses. Because of the bruit, MSCT was extended to include the abdominal arteries. Images obtained with 40 × 0.625 mm slice collimation and a gantry rotation time of 50 milliseconds were reconstructed at 3 mm thickness with overlapping 1.4 mm slices. The lateral aortogram shows stenosis and poststenotic dilatation of the main celiac trunk apparently caused by the median arcuate ligament of the diaphragm (Fig. 1). There was also compensatory dilatation of the gastroduodenal artery, the superior pancreaticoduodenal artery and the inferior pancreaticoduodenal artery (Fig. 2). The latter arises from the superior mesenteric artery. As an initial attempt to dilate the stenosis with high-pressure balloon angioplasty was unsuccessful, she was subsequently treated with an Express Vascular stent using an Amplatz Super Stiff 0.035 guidewire (Boston Scientific, Natick, MA, USA). This appeared to result in a significant improvement in her symptoms. Stenosis of the celiac axis has been attributed to atheromatous plaques, fibrosis of the celiac ganglion and compression by the median arcuate ligament of the diaphragm. The latter is a fibrous arch that connects the right and left crura of the diaphragm and defines the anterior margin of the aortic hiatus. The ligament usually inserts superior and anterior to the celiac trunk. However, when it crosses the celiac trunk at a lower level, it may result in celiac axis compression and displacement of the celiac trunk towards the superior mesenteric artery. Compression can be accentuated during expiration. Whether celiac axis compression results in abdominal pain because of ischemia or other mechanisms is debated, particularly as many patients have enlarged collateral arteries. However, some patients appear to improve with a variety of operative procedures or with vascular stents. In the patient described above, images of the celiac trunk obtained by MSCT were of similar quality to those obtained by conventional angiography.

Reversible Gastroparesis: Functional Documentation of Celiac Axis Compression Syndrome and Postoperative Improvement

Celiac axis compression syndrome has generated much controversy since its original description in 1963. The main symptoms are postprandial epigastric abdominal pain, regurgitation of undigested food, and weight loss, all of which are caused by gastric ischemia from impingement of the celiac axis by the median arcuate ligament of the diaphragm. These symptoms are seen in other common disorders such as chronic mesenteric ischemia and gastroparesis. This makes the diagnosis of celiac axis compression syndrome a true challenge for the clinician. We present data on three patients successfully treated. The pre- and postoperative studies clearly demonstrate a resolution of the condition. The duplex ultrasound images clearly show variable compression on the celiac axis. The angiogram presented shows a classic image of the disease. A review of the data has enabled us to develop an algorithm for the diagnosis of this disease.