Laparoscopic treatment of celiac axis compression syndrome (CACS) and hiatal hernia: Case report with bleeding complications and review

INTRODUCTIONMedian arcuate ligament (MAL) malposition is a rare cause of celiac axis compression syndrome (CACS) or Dunbar syndrome.PRESENTATION OF CASEA 26-year-old female presented with severe postprandial epigastric pain, weight loss, heartburn and regurgitation unresponsive to medical therapy. CT angiography and duplex ultrasound demonstrated the MAL crossing anterior to the celiac artery (CA). Reconstructions demonstrated CA compression, while the superior mesenteric artery (SMA) was normal. The MAL was laparoscopically divided, releasing the celiac axis. A concomitant Nissen fundoplication was performed. At 3-months follow-up, the CT-scan demonstrated no evidence of CACS with complete symptom resolution.DISCUSSIONDunbar's syndrome can be treated with endovascular surgery, laparoscopic MAL division or vascular surgery.Six anatomical and morphologic variations of aortic and esophageal hiatus are described. The result of the analysis of these anatomical data leads to the conclusion that hiatus hernia, Dunbar's syndrome and GERD have a common etiopathogenesis and physiopathology.CONCLUSIONLaparoscopic treatment is useful and feasible in centers with experience in majorlaparoscopic surgery with reduced invasiveness, better cosmetic effect and shorter postoperative course.     

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.

     

Screening for mesenteric vascular insufficiency and follow-up of mesenteric artery bypass procedures

Duplex ultrasonography is the preferred noninvasive screening test for superior mesenteric artery (SMA) and celiac artery (CA) stenosis. Although postprandial increases in SMA peak systolic velocity (PSV) are known to occur, the principal duplex criteria for hemodynamically significant SMA and CA stenosis are based on fasting flow velocities. In the SMA, a PSV > or =275 cm/s predicts a > or =70% angiographic stenosis with a sensitivity of 92% and a specificity of 96%, whereas a CA PSV of > or =200 cm/sec predicts a > or =70% stenosis with a sensitivity of 87% and a specificity of 80%. Patients with appropriate symptoms of chronic visceral ischemia and a duplex scan showing high-grade stenosis of the SMA, especially when combined with CA stenosis, should have a confirmatory mesenteric angiogram. This approach will facilitate prompt intervention in these patients. If the duplex examination indicates widely patent mesenteric arteries, alternative diagnoses should be investigated. Other applications of mesenteric duplex scanning include evaluation of median arcuate ligament syndrome and postoperative surveillance of mesenteric artery revascularizations.     

Median Arcuate Ligament in Orthotopic Liver Transplantation: Relevance to Arterial Reconstruction

Median arcuate ligament (MAL) syndrome results from luminal narrowing of the celiac artery by the insertion of the diaphragmatic muscle fibers or by fibrous bands of the celiac nervous plexus. In 10% to 50% of cases it is responsible for significant angiographic celiac trunk compression. In orthotopic liver transplantation (OLT), the presence of celiac compression by MAL is considered to be a risk factor for hepatic arterial thrombosis (HAT); it may lead to graft loss. Various surgical procedures have been proposed to overcome the impact of MAL in OLT, but their impact is still ill defined. The aim of our study was to compare standard hepatic artery reconstruction and graft reconstruction (aortohepatic bypass) in terms of HAT among patients with MAL undergoing OLT. We retrospectively reviewed 168 adult recipients of OLT performed from January 1991 to December 1998. Ten cases (5.6%) of celiac compression by MAL were identified after celiomesenteric arteriography. There was no significant difference in terms of HAT incidence when aortohepatic bypass was performed compared to a standard anastomosis; moreover, this was greater in the graft reconstruction group (25% vs 17%; P = .67). In our opinion, the presence of an arcuate ligament should not contraindicate a routine hepatic artery reconstruction.     

Inability of conventional imaging findings to predict response to laparoscopic release of the median arcuate ligament in patients with celiac artery compression

Objective

The objective of this study was to identify duplex ultrasound (DUS) or computed tomography angiography (CTA) imaging findings that can predict clinical response to laparoscopic release of the median arcuate ligament (MAL) in patients with celiac artery compression.

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.     

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.     

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.     

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.     

Use of hemodynamic parameters in the diagnosis of mesenteric insufficiency

To evaluate the hemodynamic characteristics of the normal mesenteric circulation, five parameters of the velocity waveforms were measured in 15 normal subjects in the celiac and superior mesenteric arteries (SMA) in the pre- and postprandial periods. It was noted that changes in celiac artery flow after eating was minimal, indicating that this vessel's major supply function is not to the gut. SMA parameters showing the most significant and consistent changes after a meal were the diastolic reverse flow and diastolic forward flow (DFF). Four patients referred with symptoms of intestinal angina underwent scanning and subsequent angiography of their mesenteric circulation. All four exhibited loss of reverse flow in the SMA. The change in DFF in the SMA was statistically significant (p = 0.01). Change in peak systolic velocity in the celiac artery was marginally significant (p = 0.05). Angiography revealed that three patients had greater than 90% stenosis of both vessels. The fourth patient had a 90% celiac artery and 65% SMA stenosis. The technique described offers the first noninvasive means of identifying mesenteric insufficiency. It is an effective screening method for a disease entity difficult to verify without selective arteriography. The use of velocity waveform parameters giving good discrimination between normal subjects and those with stenoses of the visceral arteries should reduce both the incidence of missed diagnosis and unnecessary angiography.     

Isolated inferior mesenteric artery revascularization for chronic visceral ischemia.

PURPOSE: Complete visceral artery revascularization is recommended for the treatment of chronic visceral ischemia. However, in rare cases, it may not be possible to revascularize either the celiac or superior mesenteric (SMA) arteries. We have managed a series of patients with isolated revascularization of the inferior mesenteric artery (IMA) and now report our experience gained over a period of three decades. METHODS: Records were reviewed from 11 patients with chronic visceral ischemia who underwent isolated IMA revascularization (n = 8) or who, because of failure of concomitant celiac or SMA repairs, were functionally left with an isolated IMA revascularization (n = 3). All the patients had symptomatic chronic visceral ischemia documented with arteriography. Five patients had recurrent visceral ischemia after failed visceral revascularization, and two patients had undergone resection of ischemic bowel. The celiac or the SMA was unsuitable for revascularization in five cases, and extensive adhesions precluded safe exposure of the celiac or the SMA in five cases. IMA revascularization techniques included: bypass grafting (n = 4), transaortic endarterectomy (n = 4), reimplantation (n = 2), and patch angioplasty (n = 1). RESULTS: There was one perioperative death, and the remaining 10 patients had cured or improved conditions at discharge. One IMA repair thrombosed acutely but was successfully revascularized at reoperation. The median follow-up period was 6 years (range, 1 month to 13 years). Two patients had recurrent symptoms develop despite patent IMA repairs and required subsequent visceral revascularization; interruption of collateral circulation by prior bowel resection may have contributed to recurrence in both patients. Objective follow-up examination with arteriography or duplex scanning was available for eight patients at least 1 year after IMA revascularization, and all underwent patent IMA repairs. There were no late deaths as a result of bowel infarction. CONCLUSION: Isolated IMA revascularization may be useful when revascularization of other major visceral arteries cannot be performed and a well-developed, intact IMA collateral circulation is present. In this select subset of patients with chronic visceral ischemia, isolated IMA revascularization can achieve relief of symptoms and may be a lifesaving procedure.     

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.     

Median arcuate ligament syndrome in the pediatric population

Objectives

Median arcuate ligament syndrome (MALS) is a vascular compression syndrome with symptoms that overlap chronic functional abdominal pain (CFAP). We report our experience treating MALS in a pediatric cohort previously diagnosed with CFAP.

Patients and Methods

We prospectively evaluated 46 pediatric (< 21 years of age) patients diagnosed with MALS at a tertiary care referral center from 2008 to 2012. All patients had previously been diagnosed with CFAP. Patients were evaluated for celiac artery compression by duplex ultrasound and diagnosis was confirmed by computed tomography. Quality of life (QOL) was determined by pre- and postsurgical administration of PedsQL™ questionnaire. The patients underwent laparoscopic release of the median arcuate ligament overlying the celiac artery which included surgical neurolysis. We examined the hemodynamic changes in parameters of the celiac artery and perioperative QOL outcomes to determine correlation.

Results

All patients had studies suggestive of MALS on duplex and computed tomography; 91% (n = 42) positive for MALS were females. All patients underwent a technically satisfactory laparoscopic surgical release resulting in a significant improvement in blood flow through the celiac artery. There were no deaths and a total of 9 complications, 8 requiring a secondary procedure; 33 patients were administered QOL surveys. 18 patients completed the survey with 15 (83%) patients reporting overall improvement in the QOL. Overall, 31/46 patients (67%) reported improvement of symptoms since the time of surgery.

Conclusions

MALS was found to be more common in pediatric females than males. Laparoscopic release of the celiac artery can be performed safely in the pediatric population. Surgical release of the artery and resultant neurolysis resulted in significant improvement in the blood flow, symptoms, and overall QOL in this cohort. The overall improvement in QOL outcome measures after surgery leads us to conclude that MALS might be earlier diagnosed and possibly treated in patients with CFAP. We recommend a multidisciplinary team approach to care for these complex patients.     

Risk factor analysis and treatment outcome of patients with spontaneous isolated celiac axis or superior mesenteric artery dissection

BackgroundSpontaneous isolated mesenteric artery (celiac axis or superior mesenteric artery [SMA]) dissection (IMAD) is a rare clinical entity. The aim of the present study was to examine the patient demographics, comorbidities, clinical and radiologic features, management, and prognosis and to identify the risk factors predictive of symptoms.MethodsWe performed a single-center, retrospective review from November 2005 to November 2021 of prospectively collected data from patients with a diagnosis of IMAD. The clinical data and radiologic images were reviewed, and statistical analysis was performed to compare the symptomatic and asymptomatic groups.ResultsA total of 78 patients were identified. Of the 78 patients, 24 (31%) had had celiac dissections, 51 (65%) had had SMA dissections, and 3 (4%) had had both celiac and SMA dissections. The mean age was 57.7 years (range, 36-84 years), with a male predominance (86%). More than one half (55%) of the patients had had hypertension. In addition, 29 patients (37%) were symptomatic, and 24 (31%) had been admitted to the hospital. The symptomatic patients with celiac axis dissections were more likely to have thrombosis (P = .02), significant stenosis (P = .01) or branch extension (P = .02). The symptomatic patients with SMA dissections were more likely to have a smaller artery diameter (P = .07), a longer dissection length (P = .05), thrombosis (P < .001), significant stenosis (P < .001), or branch extension (P = .003). The symptomatic patients were more likely to have been treated with antiplatelet or anticoagulant therapy (P < .001). Only three patients had undergone an intervention. Seven patients (9%) had died of unrelated causes. The Kaplan-Meier survival analysis showed a 5-year survival rate of 96% and 10-year survival rate of 91%.ConclusionsIMAD is an uncommon disease entity with a risk of visceral ischemia. Nonetheless, most of these patients can be treated conservatively with medication, with only a small minority requiring emergency surgery.     

Comparison of spiral CT scan and arteriography for evaluation of renal and visceral arteries

Renal and visceral artery images obtained concurrently with spiral CT and conventional arteriography were compared for 32 patients. Indications for imaging were occlusive disease (n=12), aneurysmal disease (n=9), and renal or visceral artery disease (n=11). Conventional arteriography enabled visualization of 64 renal arteries and 15 accessory renal arteries. Lateral aortograms obtained in 15 patients enabled visualization of 14 superior mesenteric (SMA) and 14 celiac arteries. Spiral CT enabled visualization of 60 renal arteries, 12 accessory renal arteries, 27 SMAs, and 22 celiac arteries. Calcification or a disparity in timing of contrast material injection and scanning prevented visualization of the celiac artery in 10 patients and the SMA in four patients. With conventional arteriography as the standard for comparison, spiral CT had a sensitivity of 67% and a specificity of 95% for depiction of at least 75% stenosis in the main renal artery. By means of the Pearson correlation coefficient, significant correlation (p < 0.001) was confirmed between spiral CT and arteriography for evaluation of stenosis of the main renal artery, SMA, and celiac artery. This early experience suggests that spiral CT may be useful in evaluation of renal and visceral arteries and their relationship to aortic disease.Presented at the Twentieth Annual Meeting of the Peripheral Vascular Surgery Society, New Orleans, La., June 10, 1995.     

经腹小切口超声引导系膜穿刺逆向开通治疗肠系膜上动脉开口全堵病变

背景与目的：对于肠系膜上动脉（SMA）闭塞患者,尤其是存在开口处动脉粥样硬化性无残端病变,开腹逆行肠系膜动脉支架置入术（ROMS）是一种有效的治疗方法,但该手术创伤较大,一些身体条件差的患者并不适合。笔者通过报告1例SMA全堵病变病例的治疗过程介绍一种改良的微创ROMS技术,以期为临床治疗方法的选择提供参考。 方法：回顾复旦大学附属中山医院厦门医院血管外科2019年10月收治的1例SMA全堵病变患者的临床资料。患者为64岁女性,诊断为SMA闭塞引起的慢性肠系膜缺血,行杂交手术再通SMA。 结果：患者SMA开口处为无残端完全闭塞病变且无侧支血管与腹腔干动脉及脾动脉沟通,顺行或逆行血管腔内开通均无法进行。因患者全身条件较差,难以耐受开放血运重建手术和传统的ROMS。遂做腹部做小切口,超声引导下经系膜穿刺SMA远端建立通路,导丝顺利逆向通过SMA闭塞处进入降主动脉;右侧肱动脉入路导管和逆向导丝对接后顺利正向通过病变,完成球囊扩张和支架置入术。术后患者恢复良好,症状消失,3个月后随访CTA示,支架形态、位置良好,血流通畅。 结论：对于血管腔内治疗失败且全身条件较差的SMA闭塞患者,通过经腹小切口超声引导系膜穿刺逆向开通SMA是可行的。     

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 superior mesenteric artery dissecting aneurysm and simultaneous celiac artery compression

Spontaneous dissections of visceral arteries are rare, but when they do occur, they most commonly involve the superior mesenteric artery (SMA). We present a case of intestinal ischemia caused by a spontaneous dissection of the SMA in a patient with simultaneous celiac artery occlusion. The patient was a 45-year-old woman who presented with intestinal angina of sudden onset. Arteriography revealed the classic findings of SMA dissection and occlusion of the celiac artery. The patient underwent repair of both visceral vessels and made a full recovery. The 18 previously reported cases of isolated, spontaneous dissection of the SMA are reviewed. No previous case has been associated with celiac compression syndrome. The reported experience with symptomatic dissections of the SMA would suggest that prompt surgical repair is indicated and yields excellent results.     

腹腔干、肠系膜上动脉共干部动脉瘤的切除和血管重建探讨

目的探讨腹腔干、肠系膜上动脉畸形共干部真性动脉瘤的切除以及血管重建的手术治疗方法。方法回顾性总结1998年2月至2006年4月6例患者临床资料,均在全身麻醉下行动脉瘤切除,肾下主动脉与肝动脉、脾动脉、肠系膜上动脉行转流手术5例,行主动脉肝动脉转流、肠系膜上动脉成形术1例。结果均获得临床治愈,随访观察2月~8年,无一例复发。结论腹腔干、肠系膜上动脉畸形共干部动脉瘤切除,主动脉与内脏动脉转流或重建是一种安全有效的治疗方法。     

Celiac territory ischemic syndrome in a patient on chronic hemodialysis

Mesenteric ischemia among chronic dialysis patients is usually of the nonocclusive type. Chronic occlusive mesenteric ischemia has been reported rarely in the dialysis population. The subset of"celiac-territory ischemic syndrome" has not been described in dialysis. The current report involves a 66-year-old female on chronic dialysis for 11 years. She experienced abdominal pain following sessions of hemodialysis, that later became more pronounced after eating. Abdominal angiography showed heavily calcified aorta, celiac trunk and superior mesenteric artery (SMA), with a 50% narrowing of the celiac and superior mesenteric arteries. During the following 9 months the symptoms worsened and weight loss set in. She was admitted with an episode of upper abdominal pain. Acalculous cholecystitis was found, along with multiple gastric and duodenal erosions including the second part, with an antral ulcer and multiple duodenal bulb ulcers. Repeated abdominal angiography showed progression of the stenotic lesions with significant narrowing of both the celiac trunk and the SMA. A stent was placed in the SMA. Following the procedure, the patient noted marked symptomatic improvement. On follow-up gastroduodenoscopy, all ischemic ulcers had healed completely. Serum albumin rose from a nadir of 31 to 40 g/l, and an extremely elevated c-reactive protein of 205,000 microg/l returned to normal (8,000 microg/l). The diagnosis of chronic occlusive mesenteric ischemia should be suspected among dialysis patients with post-prandial pain and weight loss in the face of calcified vessels. Predominant celiac territory ischemic syndrome presents as gastric and duodenal erosions and ulcers with or without acalculous cholecystitis.