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Median arcuate ligament syndrome (MALS) is a rare gastroenterological illness that arises from the compression of the celiac trunk by the fibrous arch known as the median arcuate ligament, which connects the muscular tendon of the diaphragm to the vertebral column. It is hypothesized that this syndrome arises due to the inadequate caudal migration of the celiac trunk during embryogenesis, although the exact pathophysiology behind this disease process remains unclear. While MALS is classically associated with a triad of post-prandial pain, weight loss, and epigastric bruit, the triad is often incomplete due to variations in vascular structures with collateral circulation from adjacent vessels. When symptoms are present, they can be vague and often characterized as unexplained nausea, vomiting, diarrhea, or flatulence. Frequently, MALS is identified incidentally upon imaging of the abdomen in response to these nonspecific complaints. We present the case of a patient suffering from MALS in which a rare anatomic variant of the celiac trunk was identified.
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Median arcuate ligament syndrome (MALS, also known as celiac artery compression syndrome, celiac axis syndrome, celiac trunk compression syndrome, Dunbar syndrome, or Harjola-Marable syndrome) is a rare condition characterized by abdominal pain attributed to the compression of the celiac artery and celiac ganglia by the median arcuate ligament. Pain can occur post-prandially and may be accompanied by weight loss, nausea, or vomiting. Following angiographic diagnosis, current definitive treatment may include open or laparoscopic decompression surgery with celiac ganglion removal (if affected), which has been found to provide relief. In this case report, we outline a young female patient with a MALS diagnosis and subsequent surgery, but whose pain recurred in various stress-related instances even after surgical intervention. After a particular pain episode, osteopathic manipulative treatment (OMT) was applied, with a focus on restoring autonomic balance through the use of various gentle osteopathic treatment techniques. A significant reduction in pain was reported post-treatment, followed by complete pain resolution, indicating a great benefit to the incorporation of OMT into the treatment plan of MALS patients in future osteopathic practice.
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Celiac artery compression syndrome is not frequent in the pediatric population. The syndrome may entail long-standing abdominal pain, recurrent vomiting, bloating, weight loss, and an abdominal bruit, which in the case of our patient, was an incidental finding. Notably, patients may be asymptomatic. Our patient is a 16-year-old male who presented with concerns about multiple, non-tender chest lymph nodes lasting for two weeks. He had also lost 80 lbs. over one year. On examination, however, an abdominal bruit was discovered, and a diagnostic workup was significant for celiac artery compression following a magnetic resonance angiography of the abdomen. Due to his significant weight loss and mediastinal lymphadenopathy, a chest computed tomography (CT) scan was done to rule out malignancy. The chest CT scan was reported as normal. Additionally, a renal duplex ultrasound was done to rule out renal artery stenosis, considering he had presented with elevated blood pressure; this was also unremarkable. Although this patient had a history of marijuana use, his assessment did not show marked dependence. Substance abuse and atherosclerotic vascular disease can be predisposing factors for celiac artery compression syndrome in older individuals. However, compression of the celiac trunk by the median arcuate ligament is a congenital anomaly more appreciated in younger age groups. The patient was referred to vascular surgery for possible median arcuate ligament release.
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Pulmonary sequestration is a rare congenital pulmonary anomaly where a portion of the lung parenchyma is supplied by an anomalous systemic artery, usually originating from the thoracic or abdominal aorta. Traditionally surgical resection and ligation of the aberrant feeding vessel are the gold standard treatments of this disease. Hybrid operations consisting in endovascular arterial embolization and surgical resection is a promising treatment option. We report a case of a 69-years-old man with symptomatic intralobular sequestration successfully treated by hybrid approach.
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Celiac trunk and superior mesenteric artery (SMA) are the main blood supply to the liver and pancreas. The data of anatomical variations in these arteries or their branches are very important clinically and surgically. The aim of this study was to describe the different variants in these arteries through the examination of the angiographs of a large series of Egyptian individuals. This research involved 389 selective angiographies to celiac artery, its branches, and the SMA. Anatomy of the target arteries of people who experienced visceral angiograph was reviewed and the data were recorded. From the total available angiograms in this work, 286 patients (73.52%) had the standard anatomy of celiac trunk and superior mesenteric arteries, and 103 patients (26.47%) had a single or multiple vessel variation. The inferior phrenic artery originates from celiac trunk in 2.05% of patients, while quadrifurcation of the celiac trunk was noticed in only 0.51% of patients. Absence of celiac trunk is also found in 0.51% of patients. Left gastric artery showed an abnormal origin from the splenic artery in 0.51% of patients. Quadrifurcation of common hepatic artery was also noticed. Variant anatomy of the left hepatic artery (LHA) was seen in 9.51% of patients, while variations of the right hepatic artery (RHA) were 14.13%. With the different origin of hepatic arteries, the gastroduodenal artery arose either from the LHA (2.82%), RHA (2.31%) or even from the celiac trunk (1.79%).
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Median arcuate ligament syndrome (MALS) is an uncommon condition characterized by the compression of the celiac trunk by the median arcuate ligament. Due to the anatomical proximity to the foregut, MALS has significant implications in hepato-pancreato-biliary (HPB) surgery. It can pose complications in pancreatoduodenectomy and orthotopic liver transplantation, where the collateral arterial supply from the superior mesenteric artery is often disrupted. The estimated prevalence of MALS in HPB surgery is approximately 10%. Overall, there is consensus for a cautious approach to MALS when embarking on complex foregut surgery, with a low threshold for intraoperative median arcuate ligament release or hepatic artery reconstruction. The role of endovascular intervention in the management of MALS prior to HPB surgery continues to evolve, but more evidence is required to establish its efficacy. Recognizing the existing literature gap concerning optimal management in this population, we describe our tertiary center experience as a clinical algorithm to facilitate decision-making. Research question: What is the significance and management of median arcuate ligament syndrome in patients undergoing hepato-pancreato-biliary surgery?
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The celiac trunk and hepatobiliary anatomy often display variations in origin and branching patterns. A particularly rare variant involving the cystic artery giving rise to a common trunk for the supraduodenal and an aberrant right gastric artery, with an additional accessory right gastric artery originating from the splenic artery, has not been previously documented. We report a unique variation in the branching pattern of the celiac trunk and the cystic artery revealed during routine dissection of the hepatobiliary region of a male cadaver at Louisiana State University, Health Sciences Center, Shreveport. In this case, the cystic artery originated from the gastroduodenal artery and gave rise to a common trunk of an aberrant right gastric artery and the supraduodenal artery. Additionally, the cadaver lacked a proper hepatic artery, and an additional (accessory) right gastric artery originated from the splenic artery. This report is the first documented instance of such combined variations in the celiac and hepatobiliary arterial anatomy. Recognizing potential variations in these anatomies is crucial for radiological and surgical interventions in the hepatobiliary area to avoid iatrogenic hemorrhage or biliary complications.
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PURPOSE: To assess anatomical variations in the celiac trunk (Ct) in patients with Median Arcuate Ligament Syndrome (MALS) using computed tomography (CT). The primary objectives were to investigate the celiac trunk angle (CtA), origin level, length (CtL), and their relationships with the superior mesenteric artery (SMA) in MALS patients. Additionally, the study intended to evaluate gender differences in these parameters and explore correlations between variables. METHODS: Retrospectively, reports of abdominal CT scans taken between January 2018, and Sepmtember 2021, in the hospital image archive were screened vey two observers independently for MALS diagnosis. Parameters such as CtA, CtL, Ct-SMA distance, SMA angle (SMAA), and median arcuate ligament thickness (MALT) were measured. Statistical analyses were conducted using SPSS software. RESULTS: Among the 81 patients (25 females, 56 males), significant differences were observed in MALT between genders (p = 0.001). CtA showed a negative correlation with CtL and Ct-SMA (p < 0.001), and a positive correlation was found between CtL and Ct-SMA (p = 0.002). CtL was measured as 25 mm for the all group. Origin levels of Ct and SMA were evaluated in comparison to vertebral levels. Ct-SMA distance was relatively shorter (9.19 mm) compared to the literature. SMAA findings were consistent with normal population values. CONCLUSION: This study provided valuable insights into the anatomical parameters of the Ct ans SMA in MALS patients. Despite some differences compared to normal population parameters, no evidence supported the hypothesis of a superiorly placed Ct contributing to MALS.
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Variação Anatômica , Artéria Celíaca , Síndrome do Ligamento Arqueado Mediano , Tomografia Computadorizada por Raios X , Humanos , Masculino , Feminino , Artéria Celíaca/diagnóstico por imagem , Artéria Celíaca/anormalidades , Síndrome do Ligamento Arqueado Mediano/diagnóstico por imagem , Estudos Retrospectivos , Adulto , Pessoa de Meia-Idade , Artéria Mesentérica Superior/diagnóstico por imagem , Artéria Mesentérica Superior/anormalidades , Artéria Mesentérica Superior/anatomia & histologia , Idoso , Adulto Jovem , Fatores Sexuais , AdolescenteRESUMO
In median arcuate ligament syndrome (MALS), the median arcuate ligament compresses the celiac trunk and surrounding nerves leading to chronic functional abdominal pain and vague gastrointestinal symptoms. MALS can be effectively treated by dividing the arcuate ligament through open surgery or laparoscopy. This is a rare vascular condition and mostly encountered in adult patients. We hereby report a case of a pediatric patient diagnosed with MALS and treated successfully by laparoscopic approach. An 11-year-old girl presented with severe abdominal cramps for 3 months, accompanied by nonbilious vomiting. Computed tomography (CT) angiography demonstrated clear images of celiac trunk compression suggesting MALS. Laparoscopic surgery to cut the ligament and decompress the celiac artery was performed. The patient was discharged on day 7 postoperative with no recurrence of symptoms after 12 months of follow-up. This report suggested the diagnostic value of CT scan, and the safety and the feasibility of laparoscopic surgical techniques to treat MALS in children.
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A retroperitoneal ganglioneuroma is an exceptionally rare surgical entity, even more so in pancreaticoduodenal tumors. These well-differentiated neuroepithelial tumors originate in the neural crest, emerge in the sympathetic nervous system, and consist of ganglion cells and stromal Schwann cells. Generally, these tumors, despite being mostly benign, may be associated with venous or arterial vascular involvement. The symptomatology presented will depend on the mass effect due to tumor growth, and surgical excision is the only therapeutic option offered today to these patients. However, encapsulation of the main vessels represents a great surgical complexity. Various surgical approaches have been employed throughout history; however, the current preferred method is an open midline laparotomy, involving an extensive Kocher maneuver and an artery-first approach, aiming for an R0 resection of the tumor with total vascular preservation to the greatest extent possible. We present a case of an R2 resection involving a 95 mm x 85 mm retroperitoneal peripancreatic ganglioneuroma with double vascular involvement (celiac trunk and superior mesenteric artery). The procedure utilized an artery-first approach with total vascular preservation in a 17-year-old woman who had long-standing gastrointestinal symptoms due to the mass effect.
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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.
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Artéria Celíaca , Angiografia por Ressonância Magnética , Humanos , Masculino , Feminino , Estudos Retrospectivos , Artéria Celíaca/diagnóstico por imagem , Aorta Abdominal/diagnóstico por imagem , Tomografia Computadorizada por Raios XRESUMO
The celiac trunk is the first branch of the abdominal aorta which arises from the anterior surface of the abdominal aorta and usually extends 1-2 cm before it branches into 3 branch arteries: the left gastric artery, the common hepatic artery, and the splenic artery. It is essential to comprehend the intricate arterial branch anatomy of the abdominal aorta to carry out abdominal surgeries as well as interventional radiology procedures safely. A 63-year-old woman who had been experiencing right upper quadrant adnominal pain for 2 months along with weight loss and appetite presented to the surgical unit of Tikur Ambessa Hospital. On exams, her vital signs were stable. Her abdominal exams were unremarkable. She was evaluated with an abdominal ultrasound which detected a mass arising from the gallbladder. With the impression of gallbladder mass contrast-enhanced computed tomography was done and revealed a rare anatomic variant of the celiac artery where the celiac artery was absent and the left gastric, the common hepatic, and the splenic arteries arise independently from the abdominal aorta. Clinicians should be aware of the presence of rare arterial variations because these changes can have a variety of therapeutic implications when performing surgical procedures like liver transplant, celiac artery resection such as the Appleby procedure for pancreatic and stomach cancer, or during interventional radiology procedures like transarterial chemoembolization.
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Median arcuate ligament (MAL) syndrome (MALS) is a rare condition caused by compression of the celiac artery by the MAL. Symptoms include abdominal pain, nausea, and weight loss. Rarely, the MAL can compress both the celiac artery and the superior mesenteric artery (SMA). We describe the case of a young man with MALS involving the celiac artery and SMA. Laparoscopic release of the MAL was performed, and the patient had resolution of his symptoms at 6 months of follow-up. A review of the literature identified only six cases of MALS involving the SMA and celiac artery, making this a rare occurrence.
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SUMMARY: The stomach receives a rich blood supply from five sets of arteries, all of which originate from the celiac trunk. During the dissection of a female cadaver that had been fixed with formalin, an atypical branching pattern was observed. An accessory left gastric artery was found to originate from the left hepatic artery and send small branches to the esophagus, cardia, and fundus of the stomach. However, there was no anastomosis between the lower accessory left gastric artery and the left gastric artery. This is a rare variant of the gastric artery that has not been previously described in detail. It is important to recognize this variation for safe and effective interventional diagnosis and treatment techniques if dealing with the liver or gastric arteries.
El estómago recibe un rico suministro de sangre de cinco conjuntos de arterias, todas las cuales se originan en el tronco celíaco. Durante la disección de un cadáver femenino que había sido fijado con formalina, se observó un patrón de ramificación atípico. Se encontró una arteria gástrica izquierda accesoria que se originaba en la arteria hepática izquierda y enviaba pequeñas ramas al esófago, el cardias y el fondo del estómago. Sin embargo, no hubo anastomosis entre la arteria gástrica izquierda accesoria inferior y la arteria gástrica izquierda. Se trata de una variante rara de la arteria gástrica que no se ha descrito previamente en detalles. Es importante reconocer esta variación para la aplicación de técnicas de diagnóstico y tratamiento intervencionistas seguras y efectivas a nivel del hígado o las arterias gástricas.
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Humanos , Feminino , Idoso , Variação Anatômica , Artéria Gástrica/anatomia & histologia , CadáverRESUMO
Acute pancreatitis can lead to both local and systemic complications, including pseudocysts, biliary obstruction, duodenal obstruction, sepsis, necrosis, vascular complications, and multiorgan failure. Vascular complications following acute pancreatitis are associated with a high risk of morbidity and mortality due to their thrombotic and hemorrhagic effects. When thrombosis is present, it usually involves the splanchnic venous system, but it is rarely seen in the arterial system. Celiac artery thrombosis is rare with only a few cases reported in the literature. In this case, we present a 65-year-old Hispanic female who presented to the emergency department with abdominal pain and nausea, with computed tomography angiography (CTA) of the abdomen revealing acute pancreatitis with thrombosis of the celiac artery, which was managed with anticoagulation.
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OBJECTIVE: The objective of the current study was the examination of possible variants of the celiac trunk. METHODS: An advanced review of the literature search was undertaken by means of the PubMed database and Google Scholar, searching for new studies published up to October 2022. Additional articles provided useful information in relation to the aim of this review. Hence, articles that met the inclusion criteria were included in this review and the collected data were organized into a table. RESULTS: The search of the literature retrieved 10 articles that referred to the anatomical variations of the celiac trunk. According to the available literature, the most common anatomical variations are: hepatosplenic trunk where the left gastric artery originates from the abdominal aorta, hepatosplenic trunk, where the left gastric artery originates from the splenic artery, and hepatogastric trunk and splenic artery origin from the superior mesenteric artery. Many other anatomical variations of the celiac trunk may exist, such as tetrafurcation, pentafurcation and hexafurcation, that refer to the division of the celiac trunk into four, five or six branches, respectively, and should be reported as they can affect surgical approaches and the development of the appropriate treatment strategy in patients. CONCLUSION: Every visceral surgeon, interventional radiologist and abdominal imager should be familiar with these variants.
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Artéria Celíaca , Humanos , Artéria Celíaca/anatomia & histologiaRESUMO
Management of small bowel obstruction (SBO) in patients with symptomatic chronic mesenteric ischemia is a phenomenon that has not been previously described in the literature. This is an index case report describing the utilization of a multidisciplinary approach in a patient that suffered from SBO from cecal perforation with history of chronic mesenteric ischemia attributed to superior mesenteric artery (SMA) and celiac trunk stenosis. The patient was a 70-year-old female with recent diagnosis of ischemic colitis and chronic mesenteric ischemia, found to have high-grade SBO with transition point in the right lower quadrant. Computerized tomography angiogram showed occluded SMA, and severe celiac artery stenosis. Interventional radiology revascularized the celiac trunk with stent placement prior to right hemicolectomy for management of her high-grade SBO. Prospective research should ascertain whether revascularization indeed leads to improved post-operative outcomes.
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BACKGROUND AND AIMS: Variations in the branches of the abdominal aorta are relatively prevalent and can impact certain surgeries. The accurate identification and differentiation of these variations pre- and intraoperatively are crucial to avoid negative clinical sequelae. This study aimed to investigate the prevalence of variations in some branches of the abdominal aorta and to identify the most frequent variants as well as any rare variants not previously classified in the existing classification systems. The study's findings may help improve the understanding and management of these variations. MATERIALS AND METHODS: This retrospective study was conducted at the Department of Radiology at An-Najah National University Hospital (NNUH) and included 550 abdominal computed tomography (CT) angiographic scans for patients (51.5% males, 48.5% females) performed between January 2017 and January 2023. RESULTS: Variations were most common in the hepatic arteries (34.7%), followed by the renal arteries (31.3%). Variations in the celiac trunk were the least frequent (9.8%). The gastro-splenic trunk (type V) was the most common celiac trunk variant. The most common hepatic artery variant was the replacement of the right hepatic artery (type III). Accessory renal arteries were more frequent on the left side and among males (P = 0.01). The celiac trunk variations had a significant association with the hepatic artery variations (P = 0.001) and the renal artery variations (P = 0.011), respectively. CONCLUSION: There is a high prevalence of anatomical variations in the described vessels, and it matches the results in the reported literature. Our findings also suggest the possible coexistence of variants. We have also encountered rare variants, especially in the hepatic arterial system. Some of the hepatic arterial system variants are not included in the older classification systems, calling for an extension of the old systems (Michel's and Hiatt classification systems) or replacement with the newer (CRL or EX-CRL classification systems) to account for rare variants not previously classified. Radiologists and surgeons should be proficient in identifying and differentiating these variations to take precautions and actions for each variant individually.
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BACKGROUND: Anatomical research based on deceased body specimens is a time-consuming process that requires a great deal of skill and time to perform correctly. Three-dimensional medical image analysis is an excellent tool for anatomic evaluation, but it often includes patients with comorbidities in the study group, which can skew the results. The purpose of this study was to develop and evaluate methods for anatomic research based on postmortem contrast-enhanced computed tomography angiography 3D reconstruction of the celiac trunk. MATERIALS AND METHODS: Postmortem contrast-enhanced computed tomography angiography of 105 (28.6% female, age 50.8±18.7) decedents without abdominal trauma or tumor was analyzed. The abdominal portion of the aorta and the celiac trunk with its branches were reconstructed and evaluated. The type of celiac trunk was evaluated. The results were analyzed. RESULTS: The celiac trunk, splenic artery, and common hepatic artery were visualized in all cases. The left gastric artery was visible in 97.1% of cases. The dorsal pancreatic artery was visualized in 61.0% of cases. The most common type of celiac trunk was 1 (88.6%), and the rarest types were 2, 3, and 6 (1.0%). We observed 4 morphologies of the truncus celiacus that did not fit the classification presented previously. CONCLUSIONS: This study has demonstrated that three-dimensional reconstruction of postmortem contrast-enhanced computed tomography is an excellent tool for performing accurate morphometric analyzes for anatomic research purposes. This method can serve as a source for anatomic studies in the healthy population.
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Dunbar syndrome is diagnosed by excluding other possible causes of abdominal pains. Surgical treatment comprises complete dissection of the ligament and the surrounding nerve ganglion. This report describes the case of a previously healthy 45-year-old male patient who presented with epigastric abdominal pain irradiating to the back and weakness. Initially, abdominal computed tomography was ordered, showing arteriopathy of the celiac trunk and mesenteric artery with stenosis. The patient underwent surgical treatment because of the refractory pain, but findings were nonspecific. It was necessary to continue workup with serial angiotomography to follow the case. After around 6 months, thickening of the arcuate ligament was found, with compression of the proximal third of the celiac trunk and 80% stenosis. The patient therefore underwent laparoscopy to relieve celiac trunk compression, with satisfactory postoperative recovery.