Truncus-coeliacus-Kompressionssyndrom

The celiac axis compression syndrome is a rare disease in adults. In childhood it is extremely uncommon. Vascular reasons or chronic irritation of the celiac ganglion are discussed as causes for this syndrome, leading to chronic abdominal pain. Lateral aortography is acknowledged to be the best method for diagnosis of the celiac axis compression syndrome. New duplex scanning methods are gaining more and more importance. The indication for surgery is the subject of controversy in the current literature. We present the case of a 15-year-old girl who recovered completely after transsection of the ligamentum arcuatum medianum and resection of the celiac ganglion.     

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

INTRODUCTION: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.     

Hepatic ischemia, caused by celiac axis compression, complicating pancreaticoduodenectomy.

OBJECTIVE: In the course of pancreaticoduodenectomy, profound hepatic ischemia developed in two patients (one with ampullary carcinoma, the other with chronic pancreatitis). This article addresses the diagnosis and correction of the celiac axis compression responsible in this complication. SUMMARY BACKGROUND DATA: Since hepatic ischemia appeared immediately after division of the gastroduodenal--pancreaticoduodenal arcade, which provides mesenteric to celiac collateral circulation, celiac axis narrowing or occlusion was suspected. Previous reports have indicated that celiac axis disease may be present in about 10% of such patients. METHODS: Doppler flow studies, and in the second patient, intraoperative angiography were performed. The celiac axis was exposed and mobilized in both. RESULTS: Initially, no flow could be detected in the celiac axis. Dense fibrous tissue was found encasing it. Division of the entrapping tissue restored flow to the upper abdominal viscera. CONCLUSIONS: The anatomic deformation of the celiac axis predisposing to this complication is detectable on the lateral projection of a preoperative celiac angiogram. If, however, an angiogram has not been done, an initial test occlusion of the gastroduodenal artery before its division permits anticipation of the complication, correction of the celiac impingement, and hence, avoidance of hepatic ischemia.     

The Use of Gastric Tonometry in the Assessment of Celiac Artery Compression Syndrome

This report describes the use of gastric tonometry to measure gastric mucosal ischemia/intestinal mucosa pH (pHi) in a patient treated for celiac artery compression syndrome. Significant gastric mucosal ischemia was demonstrated prior to celiac artery decompression as indicated by a pHi of 7.29. The ischemia was relieved by celiac artery decompression, with an increase in the pHi to 7.48. The patient experienced complete relief of his symptoms after surgical decompression and remains asymptomatic 14 months after surgery. Gastric tonometry provides an objective measurement of intestinal perfusion and ischemia in the treatment of celiac artery compression syndrome.     

Median arcuate ligament syndrome with severe two-vessel involvement

Intestinal angina is an unusual condition caused by decreased blood supply to the abdominal viscera. It has been hypothesized that at least two of the three vessels supplying the viscera need to be compromised to cause ischemia. On the other hand, compression of the celiac axis by the medium arcuate ligament, causing symptoms, has been reported. We described a severely symptomatic patient in whom this ligament completely occluded the celiac axis and severely narrowed the superior mesenteric artery. The condition was cured by division of the ligament.     

Laparoscopic Treatment of Celiac Artery Compression Syndrome: Case Series and Review of Current Treatment Modalities

Introduction  Compression of the celiac artery by the diaphragmatic crura, the median arcuate ligament, or the fibrous periaortic ganglionic tissue results in a rare constellation of symptoms known as celiac artery compression syndrome (CACS). Anatomy  First described in 1963 by Harjola in a patient with symptoms of mesenteric ischemia, it remains an elusive diagnosis. Clinical Presentation  Patients commonly present with a wide variety of symptoms resulting in multiple diagnostic tests. Diagnosis  A firm diagnosis is difficult to establish, and treatment is equally challenging. These challenges are illustrated by the following case series, and evidence supporting current treatment modalities is reviewed. Treatment  We describe a laparoscopic approach to decompression of the celiac artery facilitated by intraoperative ultrasound.     

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 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.     

Celiac axis compression syndrome: factors predicting a favorable outcome

There is still considerable doubt about the existence of the celiac axis compression syndrome. Asymptomatic compression or narrowing of the celiac artery is common, and it is difficult to understand why some patients have significant symptoms while others have none. One major problem emerges from a study of the literature, in that there is no precise definition of the clinical features of a syndrome. Patients with all kinds of abdominal symptoms and varying psychiatric and medical backgrounds have been included in previous studies. It is not surprising, then, that there is no agreement on whether surgery is ever justified. We reported a small series of 11 patients whose clinical features have been carefully documented. Regression analysis of results suggests that good surgical results can be achieved in selected patients by decompression of the artery and restoration of the normal arterial lumen. Patients likely to benefit are those with epigastric pain related to food or hunger who do not have a galaxy of other unrelated symptoms. It is suggested that these factors be included in subsequent definitions of the celiac axis compression syndrome, so that some uniformity be introduced into the writing and thinking about this confusing problem.     

Arcuate ligament vascular compression syndrome in infants and children

Background

Arcuate ligament vascular compression syndrome has not been described previously in the pediatric or pediatric surgical literature. However, it is mentioned in the literature of vascular and general surgery and in journals of radiology and orthopedics. In this review, the intraoperative pathological anatomy and the principles of treatment for 8 children will be presented.

Methods

The chart records and the anatomical sketches that were documented by the surgeon immediately after each procedure were analyzed retrospectively. In addition, preoperative courses and long-term follow-up (range, 3-18 years) were evaluated by a defined program.

Results

The diagnosis of celiac artery compression by an arcuate ligament was suspected in children presenting with a history of several years of recurrent acute abdominal pain associated with a typical arterial bruit in the midline of the epigastric region.

Conclusions

Other diseases with recurrent abdominal pain and an arterial bruit must be excluded before making the decision for an operative intervention. Duplex ultrasound and angiography are possibly helpful tools to establish the respective diagnosis, but in the patients of the present series, these techniques neither confirmed compression of the celiac axis nor demonstrated decreased perfusion of the superior mesenteric artery. However, as the clinical symptoms clearly announce the disease, these diagnostic measures are not mandatory.     

Mesenteric vascular problems. A 26-year experience.

Mesenteric vascular problems are infrequent, but may be catastrophic. During a 26-year period, 55 private patients were treated for the following disorders: (1) 12 patients with visceral artery aneurysms, (2) 8 with celiac compression syndrome, (3) 13 with chronic mesenteric ischemia, (4) 12 with acute mesenteric ischemia, and (5) 10 with mesenteric ischemia associated with aortic reconstructions. Splenic artery aneurysms were managed by excision and splenectomy, while celiac and hepatic had excision with graft replacement. Patients with celiac compression syndrome underwent lysis of the celiac artery. Two patients had compression of both celiac and superior mesenteric artery (SMA). One patient required vascular reconstruction of both arteries for residual stenoses. Patients having chronic mesenteric ischemia were treated with bypass grafts, with one death (7.7% mortality) and good long-term results. Those with acute mesenteric ischemia were treated by SMA embolectomy, bowel resection, or both, with a mortality of 67%. When associated with aortic reconstructions, mesenteric ischemia carried a mortality of 100% if bowel infarction occurred after operation, but when prophylactic mesenteric revascularization was performed at the time of aortic surgery, prognosis was greatly improved, with only one death among six patients. An aggressive approach including prompt arteriography with early diagnosis and surgical therapy is advocated for these catastrophic acute mesenteric problems.     

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.     

Surgical treatment of the celiac axis compression syndrome

Six patients with symptomatic celiac axis compression syndrome are reported on. Four were treated surgically with arterial reconstruction. None of these four patients was benefited more than temporarily by the corrective surgery. One patient had an abdominal exploration and highly selective vagotomy unrelated to the celiac axis and is symptom-free ten months after surgery. One patient had no surgery and remains symptomatic. Angiographic evaluation in all patients demonstrated that despite high grade stenosis of the celiac axis, there was no radiographically discernible reduction in contrast flow to the celiac axis because of well developed collaterals from the superior mesenteric artery. These results indicate that compression of the celiac axis may be merely an incidental angiographic finding, so this syndrome needs cautious evaluation.     

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.     

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.     

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.     

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.     

Definitive test to diagnose median arcuate ligament syndrome: injection of vasodilator during angiography

The diagnosis of median arcuate ligament (MAL) syndrome and its correlation with symptoms has been controversial since the disease entity was described. The authors describe a technique that will identify patients who will benefit from intervention. Eight patients with isolated celiac artery compression from MAL were identified by the authors. Their technique involved selective cannulation of the superior mesenteric artery (SMA) and injection of a vasodilator during angiography. Symptom reproduction and loss of collateral filling of the celiac territory represented a positive test: 4 of the 8 patients had a positive test and underwent successful surgical treatment of the condition; 3 of them remained asymptomatic at follow-up; 1 patient continues to have mild abdominal discomfort. Of the 4 patients with a negative test, 2 were found to have other conditions causing their symptoms. Vasodilator injection into the SMA is a useful diagnostic test to identify patients with symptomatic MAL syndrome.     

Late results following operative repair for celiac artery compression syndrome

The clinical significance of celiac artery compression by the median arcuate ligament of the diaphragm remains unsettled. The controversy stems from an undefined pathophysiologic mechanism and the existence of celiac compression in asymptomatic patients. This study was therefore conducted to evaluate the late results of operative therapy among our patients and possibly to identify parameters that might correlate with sustained symptom relief. Among 51 patients (12 men and 39 women) (mean age 47 years) who underwent operative treatment for symptomatic celiac artery compression, 44 (86%) were available for late follow-up. Their clinical status was determined between 1 and 18 years postoperatively (mean 9.0 years) by patient interview (36) or chart review (7). Operative treatment consisted of celiac axis decompression only (16 patients), celiac decompression and dilatation (17 patients), or celiac decompression and reconstruction by primary reanastomosis or interposition grafting (18 patients). Sustained symptom relief occurred more often with a postprandial pain pattern (81% cure), age between 40 and 60 years (77%), and weight loss of 20 pounds or more (67%). A negative correlation with clinical improvement was demonstrated for an atypical pain pattern with periods of remission (43% cure), a history of psychiatric disorder or alcohol abuse (40%), age greater than 60 years (40%), and weight loss of less than 20 pounds (53%). Eight of 15 patients (53%) treated by celiac decompression alone remained asymptomatic at late follow-up in contrast to 22 of 29 patients (76%) treated by celiac decompression plus some form of celiac revascularization. Late follow-up arteriograms (18 studies) showed a widely patent celiac artery in 70% of asymptomatic patients but a stenosed or occluded celiac axis in 75% of symptomatic patients. These findings suggest that persistent clinical improvement in patients with symptomatic celiac axis compression can be achieved by an operative technique that ensures celiac axis patency. Although some clinical features are identified that correlate with long-term benefit, reliable diagnosis of the symptomatic patient awaits definition of the pathophysiologic mechanisms involved in this syndrome.     

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.