An Uncommon Variant of Nutcracker Syndrome Secondary to Left Renal Vein Compression Between the Right Renal Artery and The Proper Hepatic Artery.

Nutcracker syndrome refers to the compression of the left renal vein between the abdominal aorta and the superior mesenteric artery. The subsequent venous congestion of the left kidney, when symptomatic, could be associated with left flank pain, hematuria, varicocele, dyspareunia, dysmenorrhea, and proteinuria. Here we describe a 42-year-old female patient with simultaneous Dunbar syndrome and a rare variant of nutcracker syndrome in which the left renal vein (LRV) compression is secondary to the unusual path of the vein between the right renal artery and the proper hepatic artery. For both the nutcracker syndrome and the Dunbar syndrome, open approach by median mini-laparotomic access for transposition of LRV, and resection of the diaphragmatic pillars and arcuate ligament was attempted. During the intervention, due to anatomical issues, the LRV transposition was converted to endovascular stenting of the LRV, moreover the implanted stent was transfixed with an external non-absorbable suture to avoid migration. At the 12 months follow-up the patient was asymptomatic, and the duplex scan confirmed the patency of the celiac trunk without re-stenosis and a correct position of the LRV stent with no proximal or distal migration.

Long-term symptom improvement and health-related quality of life after operative management of median arcuate ligament syndrome.

OBJECTIVE: To investigate long-term symptom improvement and health-related quality of life (HRQOL) after operative intervention for median arcuate ligament syndrome (MALS). METHODS: Clinical data of all consecutive patients treated by operative management of MALS from 1999 to 2018 were reviewed. A cross-sectional questionnaire using the Visick score, the Gastrointestinal Quality of Life Index, and Short Form (SF)-12v2 questionnaires was performed to assess long-term outcomes. The SF-12 HRQOL domains were compared between symptom-free and symptomatic patient groups and to averages for the US general population. Treatment failure was defined as no relief after surgery and Visick category 3 to 4 symptoms. Freedom from symptoms was estimated at 5 years. RESULTS: A total of 100 patients were treated for MALS (mean age, 38 ± 18 years; 75% female). Open surgical release was performed in 81 and laparoscopic release in 19 patients. The most common presenting symptom was abdominal pain in 99 patients with postprandial exacerbation in 85. There was no mortality. Major adverse events at 30 days had occurred in 21 patients (open 19, laparoscopic 2) including myocardial infarction (n = 1), pancreatitis (n = 2), respiratory failure (n = 4), estimated blood loss of more than 1 L (n = 8), and postoperative ileus (n = 8). One patient treated by laparoscopic release required conversion for an aortic injury, which was treated by primary repair and splenectomy. Forty-six patients responded to the questionnaire with a mean follow-up of 8 ± 4 years. Initial symptom resolution or improvement was reported by 38 patients (83%), whereas 8 patients (17%) reported treatment failure. Seven of the 38 patients (18%) with initial treatment success reported symptom recurrence. The estimated 5-year freedom from symptoms was 67 ± 7%. All SF-12 HRQOL domains were significantly lower and below the average population range in symptomatic patients compared with those who were symptom free, in which all domains were within the average population range. The Gastrointestinal Quality of Life Index scores were also significantly lower in symptomatic patients. Forty respondents (87%) reported that they would still undergo operative management if given the choice, including all respondents who reported symptom recurrence. CONCLUSIONS: The operative management of MALS can be performed with a low rate of complications. Approximately two-thirds of respondents were free of symptoms 5 years after the procedure. Treatment success in symptom-free patients was associated with an improved HRQOL on par with the population average compared with symptomatic patients. The vast majority of respondents would opt to have the operation again if given a choice. However, patients should be well-informed about the possibility of failure to relieve symptoms and symptom recurrence.

Perioperative Microcirculatory Changes Detected with Gastroscopy Assisted Laser Doppler Flowmetry and Visible Light Spectroscopy in Patients with Median Arcuate Ligament Syndrome.

PURPOSE: Physiological tests may aid in diagnosing median arcuate ligament syndrome (MALS). MALS is a symptomatic compression of the celiac artery causing symptoms similar to chronic mesenteric ischemia (CMI) of atherosclerotic etiology. Simultaneous use of visible light spectroscopy (VLS) and laser doppler flowmetry (LDF) during upper endoscopy may detect microcirculatory changes in these patients. PATIENTS AND METHODS: In a single-center, prospective comparative cohort, 25 patients were evaluated for MALS. Patients with a consensus diagnosis of MALS (n=15) underwent a gastroscopy assisted, transmucosal microcirculatory assessment with LDF and VLS. Results were compared to individuals with normal intestinal circulation (n=38) evaluated with duplex ultrasonography, and to patients with chronic mesenteric ischemia (n=32). Treatment response was evaluated clinically at 1, 3, 6, and 12 months, and with ultrasound, VLS and LDF at three months. Health-related quality of life (QoL) was assessed with Euroqol (EQ-5D-5L), preoperatively, and 12 months postoperatively. RESULTS: Preoperative mean transmucosal oxygen saturation was significantly lower in patients with MALS (SO2 76±6), as compared to healthy individuals (SO2 81±4), p=0.02. An overall significant improvement in SO2 after surgical decompression of the celiac artery was found (SO2 81±3.7, p=0.05). Eleven (92%) patients with clinical improvement after laparoscopic decompression had a definitive diagnosis of MALS. Median follow-up was 18 months (4-24 months). Four of the five dimensions investigated with EQ-5D-5L improved. CONCLUSION: VLS detected a significantly lower baseline transmucosal SO2 in patients with MALS as compared to control subjects with normal intestinal circulation. An improvement in SO2 after laparoscopic decompression was found, supporting a possible ischemic etiology in our patient population.

Staged Treatment for an Unusual Case of Median Arcuate Ligament Syndrome Caused by Compression of the Celiac and Superior Mesenteric Arteries.

We report the unusual presentation of a patient with median arcuate ligament syndrome (MALS) and compression of both the celiac artery and the superior mesenteric artery (SMA). He underwent a staged treatment. First, a laparoscopic release of the median arcuate ligament was performed. In the second stage, due to persistent postprandial pain, the SMA was stented, resulting in complete symptom relief. Recognizing this rare anatomical presentation is very important to avoiding MALS misdiagnosis and providing the appropriate staged treatment.

Median arcuate ligament syndrome.

BACKGROUND: Median arcuate ligament syndrome (MALS) describes the clinical presentation associated with direct compression of the celiac artery by the median arcuate ligament. The poorly understood pathophysiologic mechanism, variable symptom severity, and unpredictable response to treatment make MALS a controversial diagnosis. METHODS: This review summarizes the literature pertaining to the pathophysiologic mechanism, presentation, diagnosis, and management of MALS. A suggested diagnostic workup and treatment algorithm are presented. RESULTS: Individuals with MALS present with signs and symptoms of foregut ischemia, including exercise-induced or postprandial epigastric pain, nausea, vomiting, and weight loss. Consideration of MALS in patients' diagnostic workup is typically delayed. Currently, no group consensus agreement as to the diagnostic criteria for MALS exists; duplex ultrasound, angiography, and gastric exercise tonometry are used in different combinations and with varying diagnostic values throughout the literature. Surgical management involves decompression of the median arcuate ligament's constriction of the celiac artery; robotic, laparoscopic, endoscopic retroperitoneal, and open surgical intervention can provide effective symptom relief, but long-term follow-up data (>5 years) are lacking. Patients treated nonoperatively appear to have worse outcomes. CONCLUSIONS: MALS is an important clinical entity with significant impact on affected individuals. Presenting symptoms, patient demographics, and radiologic signs are generally consistent, as is the short-to medium-term (<5 years) response to surgical intervention. Future prospective studies should directly compare long-term symptomatic and quality of life outcomes after nonoperative management with outcomes after open, laparoscopic, endoscopic retroperitoneal, and robotic celiac artery decompression to enable the development of evidence-based guidelines for the management of MALS.

Factors Associated with Symptomology of Celiac Artery Compression and Outcomes following Median Arcuate Ligament Release.

BACKGROUND: This study aims to identify potential risk factors for becoming symptomatic in patients with radiographic celiac artery compression (CAC) as well as prognostic factors for patients with median arcuate ligament syndrome (MALS) who underwent surgical ligament release. METHODS: This is a retrospective cohort study of patients with findings of CAC on computed tomography or magnetic resonance angiography (CT/MRA) who were asymptomatic and who were diagnosed with MALS at a single university hospital between January 2001 and 2018. RESULTS: Following a review of 1,330 CT/MRA reports, a total of 109 patients were identified as having radiographically apparent CAC. Among these, 48 (44.0%) patients were symptomatic. Univariate comparison between those with and without symptoms showed that symptomatic patients were more commonly younger than 30 years old [17/48 (35.4%) vs. 8/61 (13.1%), P = 0.006], had a history of prior abdominal surgery [25/48 (52.1%) vs. 18/61 (29.5%), P = 0.017], and had high-grade stenosis [32/43 (74.4%) vs. 25/61 (41.0%), P = 0.001]. Among 41 included patients who underwent surgical release of the median arcuate ligament including open, laparoscopic, and robotic approaches, 82.9% reported overall clinical improvement, 5/41 (12.2%) reported persistent pain, and 13/36 (36.0%) experienced pain recurrence. The only identified risk factor associated with symptom recurrence was American Society of Anesthesiologists class III [7/13 (53.8%) vs. 4/23 (17.4%), P = 0.029]. CONCLUSIONS: The severity of stenosis and prior abdominal surgery both contributed to symptom development in patients with radiographically apparent CAC from the median arcuate ligament.

Hybrid Laparoscopic and Endovascular Treatment for Median Arcuate Ligament Syndrome: Case Report and Review of Literature.

Median arcuate ligament syndrome is a rare cause of chronic gastrointestinal ischemia caused by compression of median arcuate ligament on the celiac trunk. A 38-year-old male presented at our institution with unspecific crampy abdominal pain. After several diagnostic examinations, he firstly underwent arcuate ligament resection by laparoscopic approach and 2 months later, he underwent percutaneous transluminal angioplasty with stenting of the stenotic vessel. Postoperatory and follow-up controls showed regular patency of the artery with complete relief of abdominal symptoms. We propose a review of the literature on this uncommon condition, describing different surgical approaches.

Median Arcuate Ligament Syndrome and Its Associated Conditions.

The risk factors and associated conditions of median arcuate ligament syndrome (MALS) have not been well characterized in the literature. In this study, we aim to investigate the presentation and outcomes of MALS patients with an emphasis on the prevalence of other uncommon disorders. To this end, data of patients with MALS who underwent surgery between 2013 and 2018 were collected and compiled into a retrospective database and analyzed. Eleven patients were identified. Seven of these eleven patients underwent diagnostics to evaluate gastric emptying. Five of these seven patients (71.4%) had radiographic evidence of delayed gastric emptying. Four of the eleven patients (36.4%) were found to have anatomic abnormalities of their visceral vasculature. Two of the eleven patients (18.2%) were found to have connective tissue disorders, both with Ehlers-Danlos syndrome. Three of the eleven (27.3%) had a diagnosis of postural orthostatic tachycardia syndrome. This is the first case series reporting on an association between MALS and delayed gastric emptying. We also explored the relationship between MALS and visceral vascular abnormalities, Ehlers-Danlos syndrome, and postural orthostatic tachycardia syndrome. It is notable that these conditions are more prevalent in the MALS population than in the general population, suggesting a possible pathophysiologic relationship.

Symptomatic Pancreaticoduodenal Artery Aneurysm in a Patient with Prior Aortic Surgery and Celiac Trunk Obstruction: Case Report.

Pancreaticoduodenal artery aneurysm (PDA) is a rare visceral aneurysm and represents 1.5% of all the visceral arterial aneurysms. An 81-year-old man was admitted with acute abdominal pain. He suffered 2 previous episodes of pancreatitis and underwent abdominal aortic aneurysm repair 11 years before. The computed tomography scan revealed a 92-mm aneurysm of inferior PDA artery with prerupture signs associated with the celiac trunk obstruction. The patient underwent a selective catheterization and embolization of superior PDA artery with coils, inferior PDA artery with 12-mm Amplatzer, and aneurysm sac embolization by Onyx and coils. The postoperative course was uneventful and the patient was discharged on the fourth postoperative day. Celiac trunk stenosis could be one of the possible etiology but atherosclerosis and pancreatitis are the 2 most common risk factors. Treatment of choice is yet to be established and it has to be properly defined "case by case"; surgery, endovascular embolization, or percutaneous thrombin injection are valuable approaches.

Laparoscopic Surgery in Chronic Mesenteric Ischemia: Release of the Superior Mesenteric Artery from the Median Arcuate Ligament Using the Transperitoneal Left Retrorenal Approach.

Median arcuate ligament (MAL) syndrome usually involves the celiac artery (CA) only. Far less frequently, both the CA and superior mesenteric artery (SMA) are compressed, leading to chronic mesenteric ischemia. We report the case of a 46-year-old woman with a 4-year history of permanent nausea, postprandial abdominal pain, and asthenia. A clear epigastric bruit was observed on physical examination. Duplex ultrasound and computed tomography angiography revealed an occlusion of the CA and a highly compressed proximal SMA by the MAL, with an important collateral mesenteric network. Laparoscopic release of the MAL using a transperitoneal retrorenal approach was performed, with excellent postoperative outcomes. Compression of the SMA by the MAL is a rare cause of chronic mesenteric ischemia. Laparoscopic release of the SMA from the MAL using a transperitoneal retrorenal approach is safe and effective. Long-term outcomes need to be further assessed.

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. METHODS: There were 299 patients who were evaluated for MAL syndrome (MALS) between January 2009 and November 2015. Of these, 29 underwent laparoscopic MAL release and completed 1-year follow-up. The patients' preoperative and postoperative symptoms, use of analgesics, and body mass index were recorded. Patients' demographics and DUS and CTA findings were reviewed. Fisher exact and Student t-tests were used to identify correlation between patient or imaging variables and clinical outcomes. RESULTS: There were 19 patients (66%) who reported improvement in symptoms, and 18 (62%) decreased their use of analgesics; average body mass index increased by 0.2 (standard deviation, 1.97; range, -3.35 to 5.11). No celiac artery DUS finding (peak celiac artery velocity, angle of deflection, or change in preoperative to postoperative velocity) was predictive of successful clinical outcomes (P > .05). Similarly, no CTA finding (characteristic morphology, cross-sectional area, diameter, or location of the focal stenosis of the celiac artery) was associated with clinical outcomes (P > .05). CONCLUSIONS: Clinical response to laparoscopic MAL release was favorable in two-thirds of patients; however, no specific imaging finding of stenosis was predictive of this response. Given that the severity of stenosis on conventional imaging had no impact on treatment efficacy, vascular compromise may not be the primary cause of pain in patients presenting with this syndrome. Future investigation incorporating the neurogenic basis of MALS pain, such as with diagnostic celiac ganglion blockade, would be helpful in further elucidating the enigmatic pathophysiologic process of MALS.

Clinical impact of collateral circulation in patients with median arcuate ligament syndrome.

PURPOSE: We aimed to analyze computed tomography (CT) findings and medical records of patients diagnosed with median arcuate ligament syndrome (MALS) and evaluate possible risk factors associated with vascular complications that develop in patients with MALS. METHODS: This retrospective study was approved by the institutional review board, and the requirement to obtain informed consent was waived. A total of 37 consecutive patients were diagnosed with MALS using both axial and sagittal CT reconstruction imaging at a single institution over a 7-year period. Dynamic contrast-enhanced CT data, medical records, and angiography results were reviewed. RESULTS: Thirty-two (86.5%) patients were asymptomatic and incidentally diagnosed with MALS using CT. Seventeen (45.9%) patients exhibited significant arterial collateral circulations and nine (24.3%) were found to have splanchnic artery aneurysms, including one (2.7%) with acute bleeding secondary to aneurysm rupture. Peripancreatic vascular network including pancreaticoduodenal arcades and dorsal pancreatic artery was the most common site for development of both collateral circulations (16/22, 72.7%) and aneurysms (9/16, 56.3%). Splanchnic artery aneurysms were significantly more common in patients with collateral circulations (8/17, 47.1%) compared with those without collateral circulations (1/20, 5%) (P < 0.01). At least one peripancreatic vascular aneurysm was found in five of nine patients with splanchnic artery aneurysms (55.6%). CONCLUSION: Splanchnic artery aneurysms are not uncommon in asymptomatic patients with collateral circulations caused by significant celiac trunk stenosis or obstruction due to median arcuate ligament. Therefore, careful imaging evaluation is necessary in patients with peripancreatic collateral circulations associated with MALS and regular follow-up is recommended for possibility of aneurysm development and rupture. Prophylactic endovascular treatment should be specifically performed in patients with pancreaticoduodenal arcade aneurysms to prevent life-threatening aneurysm rupture regardless of size.

Anaesthetic management of median arcuate ligament syndrome. / Manejo anestésico del síndrome del ligamento arcuato medio.

Median arcuate ligament syndrome, also known as celiac artery compression syndrome, is a rare and unusual clinical disorder. Its symptoms are non-specific, which complicates its diagnosis, and a multidisciplinary approach is required to treat the disorder. The ligament is circumferentially cleared by laparoscopy. Selective angiography and endovascular techniques may be used after laparoscopy. Vital organs and important vascular structures can be injured during the surgery. The combination of different procedures, as well as the high risk of damage, make this process a significant challenge for the anaesthetist. During corrective surgery for median arcuate ligament syndrome, general anaesthesia must be adapted to the various haemodynamic and ventilatory requirements, and strict control of pain established, as oral tolerance is a key factor in the post-operative recovery of these patients.

Tratamento laparoscópico da compressão do tronco celíaco pelo ligamento arqueado do diafragma associado a correção endovascular do aneurisma de artéria pancreatoduodenal: relato de caso / Laparoscopic treatment of celiac axis compression by the median arcuate ligament and endovascular repair of a pancreaticoduodenal artery aneurysm: case report

A compressão do tronco celíaco pelo ligamento arqueado mediano do diafragma pode causar sintomas inespecíficos como dor abdominal, vômitos e emagrecimento. Existe uma associação comprovada entre estenoses ou oclusões do tronco celíaco e aneurismas da artéria pancreatoduodenal. Nas situações em que essa associação ocorre, a estratégia de tratamento deve ser individualizada. Relatamos o caso de uma paciente com aneurisma de artéria pancreatoduodenal associado à compressão do tronco celíaco pelo ligamento arqueado, manejados, respectivamente, por técnicas endovasculares e laparoscópicas

Compression of the celiac axis by the median arcuate ligament of the diaphragm can cause nonspecific symptoms such as abdominal pain, vomiting, and weight loss. There is a known association between stenosis or occlusion of the celiac trunk and aneurysms of the pancreaticoduodenal artery. Treatment strategies for patients who have this association should be selected on a case-by-case basis. We describe the case of a patient with pancreaticoduodenal artery aneurysm associated with compression of the celiac trunk by the arcuate ligament, which were managed with endovascular and laparoscopic techniques, respectively

Treatment of the Median Arcuate Ligament Syndrome in a Patient with Behcet's Disease.

Compression of the celiac artery by the median arcuate ligament (MAL) and neurofibrous tissue is called MAL syndrome or Dunbar's syndrome. Postprandial abdominal pain and weight loss are the main symptoms. Surgical treatment still remains the gold standard therapy. Here, we present the first case to our knowledge in which MAL syndrome accompanies Behcet's disease.

Usefulness of four-dimensional flow-sensitive magnetic resonance imaging to evaluate hemodynamics in the pancreaticoduodenal artery.

Pancreaticoduodenal artery (PDA) aneurysm associated with celiac axis compression by the median arcuate ligament (MAL) is a rare disorder, but may be lethal if ruptured. Therefore, prophylactic surgical treatments need to be considered when MAL compresses the celiac axis. We herein report the usefulness of an arterial flow analysis for objectively evaluating this pathophysiology under four-dimensional flow-sensitive magnetic resonance imaging (4D-flow MRI). Celiac artery stenosis was incidentally found under contrast-enhanced CT in a 50-year-old woman with symptomatic solitary pancreatic insulinoma. Under 4D-flow MRI, retrograde blood flow and aberrant wall shear stress were detected in the pancreaticoduodenal artery arcade. After obtaining informed consent, enucleation for insulinoma concomitant with MAL dissection was performed. Hypoglycemic attack completely resolved immediately after surgery. One month after surgery, 4D-flow MRI revealed normalized vectorial flow and wall shear stress in the PDA arcade without hypoglycemic attack. 4D-flow MRI is a very useful and non-invasive modality for objectively evaluating visceral artery hemodynamics.