Identical anatomy, different symptoms: a comparative case study of median arcuate ligament syndrome in monozygotic twins.

Median arcuate ligament syndrome (MALS) is a rare clinical entity arising from the extrinsic compression of the coeliac axis by the median arcuate ligament. In this report, we detail a unique presentation involving monozygotic twins, both of whom demonstrated anatomical extrinsic compression of the coeliac axis by the median arcuate ligament. Intriguingly, only one twin manifested clinical symptoms consistent with MALS, despite comparable anatomical compression of the coeliac axis observed in both. This case highlights the potential interplay of a genetic or anatomical predisposition to coeliac axis compression and secondary, possibly environmental, factors that lead to the development of clinical symptoms. In this report, we explore various determinants potentially influencing symptomatology in MALS and advocate for the publication of similar case studies to further elucidate this rare condition.

Comprehensive analysis of splenic artery variations using computed tomography angiography: development of the IPALGEA classification system.

BACKGROUND: The splenic artery, an essential component of abdominal vascular anatomy, exhibits significant variations with clinical implications in surgical and radiological procedures. The lack of a standardized classification system for these variations hinders comparative studies and surgical planning. This study introduces the IPALGEA classification system, based on computed tomography angiography (CTA) findings, to address this gap. METHODS: A retrospective analysis was conducted on 302 patients who underwent CTA at a tertiary university hospital between August 2021 and January 2022. The study focused on the evaluation of splenic artery variations, including the origin, course, terminal branching patterns, and the relationship between the inferior polar artery and the left gastroepiploic artery. The IPALGEA classification was developed to standardize the reporting of these variations. RESULTS: The study highlighted a significant prevalence of splenic artery variations, with the most common pattern being a superior course relative to the pancreas. The IPALGEA classification effectively categorized these variations, emphasizing the relationship between the inferior polar artery and the left gastroepiploic artery. The findings revealed that the bifurcation distance of the celiac trunk varied significantly between genders and that the presence of an inferior polar artery correlated with a shorter hilus distance. CONCLUSION: The IPALGEA classification offers a comprehensive and standardized approach to categorize splenic artery variations. This system enhances our understanding of abdominal vascular anatomy and has significant implications for surgical and radiological procedures, potentially reducing surgical complications and improving patient outcomes.

Novel embryological classifications of hepatic arteries based on the relationship between aberrant right hepatic arteries and the middle hepatic artery: A retrospective study of contrast-enhanced computed tomography images.

BACKGROUND: Variations in hepatic arteries are frequently encountered during pancreatoduodenecomy. Identifying anomalies, especially the problematic aberrant right hepatic artery (aRHA), is crucial to preventing vascular-related complications. In cases where the middle hepatic artery (MHA) branches from aRHAs, their injury may lead to severe liver ischemia. Nevertheless, there has been little information on whether MHA branches from aRHAs. This study aimed to investigate the relationship between aRHAs and the MHA based on the embryological development of visceral arteries. METHODS: This retrospective study analyzed contrast-enhanced computed tomography images of 759 patients who underwent hepatobiliary-pancreatic surgery between January 2011 and August 2022. The origin of RHAs and MHA courses were determined using three-dimensional reconstruction. All cases of aRHAs were categorized into those with or without replacement of the left hepatic artery (LHA). RESULTS: Among the 759 patients, 163 (21.4%) had aRHAs. Five aRHAs patterns were identified: (Type 1) RHA from the gastroduodenal artery (2.7%), (Type 2) RHA from the superior mesenteric artery (SMA) (12.7%), (Type 3) RHA from the celiac axis (2.1%), (Type 4) common hepatic artery (CHA) from the SMA (3.5%), and (Type 5) separate branching of RHA and LHA from the CHA (0.26%). The MHA did not originate from aRHAs in Types 1-3, whereas in Type 4, it branched from either the RHA or LHA. CONCLUSIONS: Based on the developmental process of hepatic and visceral arteries, branching of the MHA from aRHAs is considered rare. However, preoperative recognition and intraoperative anatomical assessment of aRHAs is essential to avoid injury.

Intraoperative assessment of blood flow using transabdominal ultrasound during laparoscopic surgery of celiac artery compression syndrome.

Surgical treatment of celiac artery (CA) compression syndrome (CACS) is to release the median arcuate ligament (MAL) by removing the abdominal nerve plexus surrounding CA. In laparoscopic surgery of CACS, objective intraoperative assessment of blood flow in CA is highly desirable. We herein demonstrate a case of laparoscopic surgery of CACS with use of intraoperative transabdominal ultrasound. A 52-year-old woman was presented with epigastric pain and vomiting after eating. Contrast-enhanced computed tomography demonstrated significant stenosis at the origin of CA. Doppler study of CA was also performed, and she was diagnosed as CACS. Laparoscopic surgery was performed, and the MAL was divided. And then, Doppler study using intraoperative transabdominal ultrasound confirmed the successful decompression of CA. This patient was discharged on postoperative day 11, and her symptoms was improved. Intraoperative assessment of blood flow in CA using transabdominal ultrasound was a simple and useful method for laparoscopic surgery of CACS.

Management of Spontaneous Isolated Celiac Artery Dissection.

BACKGROUND: Spontaneous isolated celiac artery dissection (SICAD) is uncommon, with very few series reported in the literature. The present study aims to describe the clinical characteristics and treatment outcomes of patients with SICAD treated at a single Chilean institution over 20 years. METHODS: A retrospective review of all patients from a single academic hospital with SICAD diagnosed between January 2003 and March 2023 was performed. Conservative treatment included antiplatelets, anticoagulation, or both. The normal size of a celiac artery in our population was 7.9 ± 0.79 mm in females and 8.3 ± 1.08 mm in males. We defined a celiac artery with a diameter equal to or more than 12.5 mm as an aneurysmal celiac artery. RESULTS: The cohort included 27 patients; 77.8% (n = 21) were males. The median age was 51.0 years (range: 38-84 years). Fourteen (51.8%) patients presented with aneurysmal dilatation. Fourteen (51.8%) patients were treated with antiplatelets, 6 (22.2%) patients with anticoagulation, and 7 (25.9%) with anticoagulation and antiplatelets. One patient was treated with endovascular therapy due to a pseudoaneurysm of the celiac artery detected 10 days after conservative treatment with antiplatelets. The median length of hospital stay was 5 days (range: 2-14 days). Complete remodeling was seen in 6 (22.2%) patients, partial remodeling in 10 (37.0%) patients, and no change was seen in 8 (26.9%) patients. Three (11.5%) patients were lost to follow-up. There were no significant differences between treatments and remodeling outcomes (P = 0.729). The median celiac artery diameter of patients with aneurysmal dilatation was 13.5 mm (range: 12.5-20.5 mm). Systemic arterial hypertension was found more commonly in patients who presented with aneurysmal dilatation than in patients without (87.5% vs. 12.5%, respectively, P = 0.016). Mean follow-up was 41.5 months and median follow-up was 16 months (range: 6-204 months). CONCLUSIONS: Most patients with SICAD can be treated conservatively with excellent outcomes. Hypertension was more commonly found in patients with SICAD and aneurysmal dilatation.

Superior mesenteric artery revisited using magnetic resonance angiography.

PURPOSE: We aimed to examine the superior mesenteric artery in detail by magnetic resonance angiography to provide an alternative to other imaging methods, to reduce the exposure time of patients and physicians to X-rays and the time spent in catheter angiography, to determine the variations, positions, and locations of the celiac trunk, and to provide detailed information for surgeons and interventional radiologists using this method. METHODS: The procedures were approved by the Kocaeli University Medical School Non-Interventional Clinical Research Ethics Committee (10.04.2023, approval number: 2021/51). MR angiography images of 185 patients with abdominal imaging in PACS (Picture Archiving Communication Systems) were retrospectively registered. The level of origin of the superior mesenteric artery according to the vertebral column, angle of origin, distance between the superior mesenteric artery and branches of the abdominal aorta, and branching pattern of the superior mesenteric artery were evaluated. Parameters were evaluated according to gender and age using SPSS version 25. RESULTS: The distance between superior mesenteric artery-inferior mesenteric artery and superior mesenteric artery-aortic bifurcation in males was higher than in females, and the difference was statistically significant. In females and the whole study group, a low, positive and significant relationship was found between age and superior mesenteric artery-sagittal angle. The most common origin site for the superior mesenteric artery, according to the vertebral column was found to be at L1 middle for males and L1 upper for females. The most common superior mesenteric artery branching pattern was classical type in both genders. CONCLUSION: Individual evaluation of the superior mesenteric artery could reduce the risks during surgical interventions, considering the relationship of the superior mesenteric artery, especially with distally located vessels, and the gender differences for the angle of origin. Furthermore, considering that interventional radiologists choose the catheter according to the angle of origin of the artery during catheter angiography procedures, individual evaluation of patients taking into account gender and age is of utmost importance.

Median arcuate ligament release at the time of pancreaticoduodenectomy or total pancreatectomy.

BACKGROUND: Celiac artery compression can complicate the performance of pancreaticoduodenectomy or total pancreatectomy due to the need for ligation of the gastroduodenal artery. Median arcuate ligament release restores normal arterial flow to the liver, spleen, and stomach and may avoid complications related to poor perfusion of the foregut. METHODS: All patients who underwent median arcuate ligament release for celiac artery compression at the time of pancreatectomy between 2009 and 2023 were reviewed. Pre- and postoperative computed tomography was used to categorize celiac artery compression by the extent of compression (types A [<50%], B [50%-80%], and C [>80%]). RESULTS: Of 695 patients who underwent pancreatectomy, 22 (3%) had celiac artery compression, and a majority (17) were identified on preoperative imaging. Median celiac artery compression was 52% (interquartile range = 18); 8 (36%) patients had type A and 14 (64%) had type B compression with a median celiac artery compression of 39% (interquartile range = 18) and 59% (interquartile range = 14), respectively (P < .001). Postoperative imaging was available for 20 (90%) patients, and a reduction in the median celiac artery compression occurred in all patients: type A, 14%, and type B, 31%. Complications included 1 (5%) death after hospital discharge, 1 (5%) pancreatic fistula, 1 (5%) delayed gastric emptying, and 4 (18%) readmissions. No patient had evidence of a biliary leak or liver dysfunction. CONCLUSION: Preoperative computed tomography allows accurate identification of celiac artery compression. Ligation of the gastroduodenal artery during pancreaticoduodenectomy or total pancreatectomy in the setting of celiac artery compression requires median arcuate ligament release to restore normal arterial flow to the foregut and avoid preventable complications.

Clinical Outcomes after Median Arcuate Ligament Release in Patients Responsive to Celiac Plexus Block.

PURPOSE: To determine if symptom relief with celiac plexus block (CPB) is associated with favorable clinical outcomes after median arcuate ligament release (MALR) surgery. MATERIALS AND METHODS: A retrospective review was performed from January 2000 to December 2021. Fifty-seven patients (42 women, 15 men; mean age, 43 years [range, 18-84 years]) with clinical and radiographic features suggestive of median arcuate ligament syndrome (MALS) underwent computed tomography (CT)-guided percutaneous CPB for suspected MALS. Clinical outcomes of CPB and MALR surgery were correlated. Adverse events were classified according to the Society of Interventional Radiology (SIR) guidelines. RESULTS: CT-guided percutaneous CPB was successfully performed in all 57 (100%) patients with suspected MALS. A cohort of 38 (67%) patients showed clinical improvement with CPB. A subset of 28 (74%) patients in this group subsequently underwent open MALR surgery; 27 (96%) responders to CPB showed favorable clinical outcomes with surgery. There was 1 (4%) CPB-related mild adverse event. There were no moderate, severe, or life-threatening adverse events. CONCLUSIONS: Patients who responded to CPB were selected to undergo surgery, and 96% of them improved after surgery.

Evaluation of the celiac trunk and its branching pattern by magnetic resonance angiography.

OBJECTIVE: The study aimed to examine the celiac trunk (CT) in detail by magnetic resonance angiography, to determine the locations, positions and variations of the CT, to provide detailed information for surgeons and interventional radiologists, and to reduce the time spent in the catheter angiography. MATERIAL AND METHODS: MR angiography images of 185 patients with abdominal imaging in PACS (Picture Archiving Communication Systems) were retrospectively analyzed. The level of origin of CT, according to the vertebral column, angle of origin, distance between CT and branches of the abdominal aorta (AA), and the branching pattern of CT were evaluated. Parameters were evaluated according to gender and age. RESULTS: The most common origin site for CT, according to the vertebral column, was found to be at T12-L1 in both genders. There was a low, positive correlation between age and CT-Sagittal angle (SA) in the whole study group (p<0.05). The most common CT branching pattern was the hepatogastrosplenic trunk in both genders, according to Adachi and Uflkacker's classification. The distance between CT and the inferior mesenteric artery (IMA) and CT and the aortic bifurcation (AB) of males was greater than in females, and the differences were statistically significant (p<0.05). CONCLUSION: Knowledge of the locations, positions, and variations of CT is essential in the diagnosis, differential diagnosis and decision-making mechanisms regarding the type of intervention to be performed for this vessel and related structures. In addition, the fact that data on these vessels can be obtained by MR angiography due to the improved image quality will prevent patients and physicians from the problems caused by the ionizing radiation of computed tomography. The data presented will constitute a basis for detailed and individualized interpretation and evaluation of each patient, as they provide important details about the configuration of the CT concerning gender and age using MRA.

Use of selective visceral angiography in surgical strategy planning for celiac artery aneurysm in the celiacomesenteric trunk.

BACKGROUND: The celiacomesenteric trunk (CMT) is a common duct of the celiac artery (CA) and the superior mesenteric artery originating from the aorta, which is an uncommon anatomical variant of visceral artery circulation. Because of the variety of visceral circulation in those with CMT, the visceral circulation associated with each branch should be evaluated prior to surgical treatment of visceral artery aneurysm in the CMT. CASE PRESENTATION: A 64-year-old woman was diagnosed with a CA aneurysm in the CMT. Aneurysmectomy of the aneurysm was performed successfully. On preoperative selective visceral angiography, the CA was seen to bifurcate into the common hepatic and splenic artery. The left gastric artery was directly isolated from the aorta and perfused to the common hepatic and splenic artery through collateral circulation. These findings showed that celiac artery embolization is anatomically feasible, even in cases of celiac artery aneurysm rupture. CONCLUSIONS: Selective visceral angiography can contribute to surgical strategy planning for CA aneurysm with CMT.

Laparoscopic Median Arcuate Ligament Release: Surgical Technique and Clinical Outcomes.

BACKGROUND: Median arcuate ligament syndrome (MALS) is characterized by a constellation of symptoms related to the compression of the celiac artery trunk. Laparoscopic release of the ligament has demonstrated its effectiveness in alleviating these symptoms while showing lower postoperative complication rates, reduced hospital stays, and improved clinical outcomes. This study describes a single institution's experience with this procedure and reports on the preoperative assessment, surgical technique, and clinical outcomes of patients with MALS. METHODS: We performed a retrospective chart review of all patients who underwent a primary laparoscopic MAL release (MALR) at a single high-volume academic institution from June 2021 to July 2023. Patient demographics, preoperative assessment, postoperative complications, and resolution of preoperative symptoms data were collected. RESULTS: A total of 30 patients underwent laparoscopic MALR, with 76.7% being female and a mean age of 33.4±16.3 years. The most common presenting symptom was postprandial epigastric pain (100%), followed by abdominal pain and nausea (83.3%), among others. The preoperative evaluation for all patients included a duplex mesenteric doppler and CT angiogram during inspiration and expiration and 3D reconstruction. Successful laparoscopic decompression of the celiac artery was achieved in 96.6% of cases, with only one conversion to an open procedure. There was only one reported early (<30 d postoperatively) complication with no subsequent late complications or mortality. None of the patients required reintervention or reoperation. Only 1 patient required postoperative celiac plexus/splanchnic block injection to alleviate pain. CONCLUSIONS: MALS can be effectively and safely managed using a laparoscopic approach when performed by an experienced minimally invasive surgeon. Further studies with longer follow-ups are needed to confirm the long-term effectiveness of this technique.

Dissection of the coeliac trunk.

This is a case report of two men aged 39 and 43 years with dissection of the coeliac trunk involving the splenic arteries causing splenic infarction. One case was associated with an increase in abdominal pressure during defaecation and the other occurred during treatment with methylphenidate. Based on the published 43 cases, risk factors include male sex, increased intraabdominal pressure or increased vascular pressure. Methylphenidate most likely increased the blood pressure, and dissections of other arteries have been described during treatment with this and the similar drug amphetamine.

First description of a histopathologic grading system and relationship to outcomes after robotic median arcuate ligament release with celiac ganglionectomy and lymphadenectomy.

BACKGROUND: Two dominating theories regarding median arcuate ligament syndrome include vascular and neurogenic etiologies from celiac artery and ganglion compression, respectively. Celiac ganglionectomy is not routine during surgery, and specimens are rarely excised; therefore, the extent of nerve involvement and histopathology are unknown. Our study aims to characterize histopathologic findings in median arcuate ligament syndrome, establish a histopathologic grading system, and correlate with clinical outcomes. METHODS: Robotic median arcuate ligament release, celiac ganglionectomy, and lymphadenectomy were performed with specimens excised and stained using hematoxylin & eosin, trichrome, and S100. Neurofibrosis, adiposity, and reactive changes were described, a grading scale was developed, and results were analyzed with clinical outcomes. RESULTS: Fifty-four patients were evaluated, of whom 36 met inclusion criteria (81% female, 34.9 [25.9-47.5] years, body mass index 23.5 [19.6-28.1] kg/m2). Histopathologic evaluation revealed fibrosis (hematoxylin & eosin and trichrome median score 1.5 [0-2.5]), reactive lymphadenopathy (89%), intraparenchymal nerves (31%), and lipogranulomas (31%). Greater fibrosis was associated with a lack of preoperative celiac plexus block relief (100% vs. 30%, P = .044) and lower postoperative celiac artery velocities (198 vs 323 cm/s, P = .02). Intraparenchymal nerves were associated with greater decreases in pre to postoperative velocities (161 vs 84 cm/s, P = .037). Symptoms improved in 28 patients (78%). CONCLUSION: We developed the first histopathologic grading system and identified unique findings of intraparenchymal nerves and lipogranulomas. Histopathologic abnormalities were associated with objective improvement and symptomatic relief postoperatively. These findings support nerve compression and inflammation as predominant contributors to median arcuate ligament syndrome pain, celiac ganglia resection to treat symptoms, and continued histopathologic analysis to better elucidate median arcuate ligament syndrome etiology.

[Diagnosis and treatment of Dunbar syndrome]. / Diagnostik und Therapie des Dunbar-Syndroms.

The rare Dunbar syndrome or medial arcuate ligament syndrome (MALS) is defined as compression of the celiac trunk and/or ganglion by the medial arcuate ligament. It is often diagnosed after patients have suffered for a long time and is characterized by intermittent food-related pain, nausea, and unexplained weight loss. After exclusion of other causes of the above symptoms by gastroscopy, colonoscopy, CT, or MRI, the gold standard for diagnosis is dynamic color-coded duplex sonography, which may be supplemented by CT or MR angiography. The treatment of choice is a laparoscopic division of the arcuate ligament at the celiac trunk, although percutaneous transluminal angioplasty (PTA) with stent implantation may be performed in cases of postoperative persistence of symptoms or recurrent stenosis. Since symptoms persist postoperatively in up to 50% of cases, strict indication and complete diagnosis in designated centers are of great importance for successful treatment.

Variant origins of the middle colic artery from the coeliac trunk and its branches.

The middle colic artery usually arises from the superior mesenteric artery, but in rare cases it may arise from the coeliac trunk or its branches. The aim of this study was to investigate variant origins of the middle colic artery on computed tomography and anatomical dissection. Variant middle colic arteries were identified on computed tomography as part of an ongoing study investigating anatomical variations of vessels of the upper abdomen. Three-dimensional reconstructions were made to demonstrate the variant findings. Cadaveric dissections were performed as part of a routine dissection course. We report five cases of rare variant origins of the middle colic artery arising from the coeliac axis. Among these sites of origin were the coeliac trunk, the gastrosplenic trunk, the splenic artery, and the common hepatic artery. Four cases were identified on multi-detector computed tomography and one in a cadaver. In all cases, the vessels passed posterior to the body of the pancreas before entering the transverse mesocolon. Knowledge of middle colic artery variations is important to prevent inadvertent injury in digestive surgery, especially in the hepatopancreatic area. Variant origins of the middle colic artery are rare, and their knowledge is crucial to prevent unnecessary iatrogenic injury during abdominal surgery.

Pancreaticoduodenal Artery Aneurysm Complicated by Median Arcuate Ligament Syndrome.

PURPOSE: To highlight median arcuate ligament syndrome as a potential cause for celiac artery stenosis and pancreaticoduodenal artery aneurysm, and describe treatment options in this setting. CASE REPORT: A 63-year-old male presented with a pancreaticoduodenal artery aneurysm and concomitant celiac artery stenosis that was treated with celiac artery stenting and aneurysm coiling. He subsequently developed stent fracture and celiac artery occlusion secondary to previously unrecognized median arcuate ligament syndrome causing reperfusion of the aneurysm. This was treated with open median arcuate ligament release and aorta to common hepatic artery bypass with good clinical result and stable 20-month surveillance imaging. CONCLUSION: It is critical to recognize median arcuate ligament syndrome as a cause of celiac artery stenosis in the setting of pancreaticoduodenal artery aneurysm given the high risk of failure of endovascular stenting. Open aorto-hepatic artery bypass and endovascular aneurysm coiling should be the preferred approach in these patients.

Celiac trunk stenosis by median arcuate ligament in orthotopic liver transplantation: a potential hidden foe to the biliary tree.

BACKGROUND: Celiac trunk compression by the median arcuate ligament (MAL) increases the risk of ischemic complications following gastrointestinal surgical procedures. Previous studies suggest increased risk of hepatic artery thrombosis (HAT) in orthotopic liver transplant (OLT) recipients. The aim of this study is to investigate the impact of untreated MAL compression (MAL-C) on biliary complications in OLT. METHODS: Contrast-enhanced imaging was used to classify celiac trunk stenosis by MAL-C. Medical records were reviewed to extract pre-transplant, transplant and post-transplant data. Patients were divided into two groups: no MAL compression (nMAL-C) and MAL-C. The primary endpoint was biliary complications. Secondary endpoints were HAT and graft survival. RESULTS: 305 OLT were performed from 2010 to 2021, of which 219 were included for analysis: 185 (84.5%) patients without and 34 (15.5%) with MAL-C. The incidence of HAT was 5.9% in both groups. Biliary complications were more common in the MAL-C group (35.3% vs. 17.8%, p = 0.035). Graft survival was decreased in patients with MAL-C (p = 0.035). CONCLUSIONS: MAL-C of the celiac trunk was associated with increased risk of biliary complications and inferior graft survival in OLT patients. These findings highlight the importance of preoperative screening and treatment of MAL in this population.

A gastroduodenal artery that branched from the celiac artery in gastrectomy: A rare case of an anatomical variant.

Understanding anatomical anomalies of the branch of the celiac artery for safe gastrectomy is important. We report a case of laparoscopic distal gastrectomy with D1+ lymph node dissection for early gastric cancer with a vascular anatomical anomaly of the celiac artery. A 45-year-old woman was referred to our hospital because of early gastric cancer. Computed tomography showed an anatomical variation of the gastroduodenal artery, which branched from the celiac artery. The celiac artery also branched into the left gastric artery, the splenic artery, and the common hepatic artery. Preoperative understanding of an unusual branch of the celiac artery enabled a safe laparoscopic surgery. There were no postoperative complications. The Adachi classification or Michel classification is used for an anatomical anomaly of the celiac artery, but to the best of our knowledge, this case has not been previously classified and is the first reported case.

Bilateral pulmonary sequestrations: Distinct types sharing a common celiac artery origin, presented with pneumonia and associated findings in a pediatric case.

A 14-year-old boy presented with shortness of breath, cough, and mild chest pain, with a history of intermittent milder symptoms. Physical examination and initial tests showed mild iron deficiency anemia, elevated C-reactive protein, and normal vital signs. Chest radiograph revealed abnormalities in the left lower zone, leading to contrast-enhanced chest CT. The CT scan revealed bilateral intrapulmonary sequestrations, both deriving blood supply from a common trunk originating from the celiac artery. The patient's symptoms initially attributed to a pulmonary infection improved with antibiotic therapy. Pulmonary sequestration is a congenital anomaly characterized by aberrant lung tissue lacking connections to bronchial tree or pulmonary arteries. It can lead to recurrent pulmonary infections and postinfectious sequelae. This case presented a unique bilateral sequestration, both originating from the celiac artery. Radiologists should be aware of sequestration types and associated anomalies, even in atypical locations. Blood supply can originate from various arteries, not just the aorta.