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Arcuate ligament syndrome (ALS) is caused by extrinsic compression of the celiac trunk by the median arcuate ligament. It has an estimated incidence of 2 per 100,000 people, preferentially affecting female individuals in the age range of 30 and 50 years. The clinical feature of SLA can manifest with chronic postprandial abdominal pain, nausea and/or vomiting, weight loss and abdominal murmur, although there are patients who do not develop symptoms due to increased collateral circulation. The diagnosis of SLA is based on the presence of clinical symptoms, imaging findings, and the exclusion of other causes of abdominal pain. Within imaging techniques, magnetic resonance angiography (MRA) and computed tomography angiography (AngioCT) are the modalities of choice for the diagnosis of SLA. The management of SLA is surgical with the purpose of restoring the flow in the celiac trunk and whose approaches are described in this article. Finally, this pathology is a challenge for the specialist since the symptoms can be non-specific and overlap with other abdominal disorders, making diagnosis and management difficult. The preparation of this review article was based on the search for systematic reviews preferably less than 10 years of publication in scientific journals, using the words “arcuate ligament syndrome”, “celiac artery compression syndrome” and other related ones [ABSTRACT FROM AUTHOR]

Median arcuate ligament syndrome (MALS) involves coeliac artery compression, causing a range of symptoms from chronic pain to life-threatening complications. This case features a 52-year-old patient with recurrent retroperitoneal bleeding from MALS-related inferior pancreaticoduodenal artery aneurysms (PDAAs). Emergency interventions, including surgical bleeding control, angioplasty, percutaneous drainage, and median arcuate ligament release, were conducted. The case highlights challenges in diagnosing and managing MALS-related PDAA, emphasizing the importance of early identification and tailored interventions based on clinical symptoms and imaging. Surgical intervention to release the ligament is the primary treatment, with considerations for prophylactic intervention in PDAA cases. Lack of established PDAA management protocols underscores the need for prompt intervention to prevent complications. In conclusion, this report stresses the association between MALS and PDAA, advocating for early identification and tailored management to mitigate complications. [ABSTRACT FROM AUTHOR]

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.

Abstract Background The median arcuate ligament syndrome (MALS) is a rare disease caused by compression of the celiac artery (ORPHA: 293208). Surgical treatment of MALS aims to restore normal celiac blood flow by laparoscopic celiac artery decompression. However, surgical success rates vary widely between patients, therefore adequate selection of patients is essential to improve surgical outcome. Symptoms of MALS might also overlap with other chronic multi-system disorders such as mast cell activation syndrome (MCAS). So far, no clinical or radiological parameter was found to be predictive of the postoperative outcome. We, therefore, aim to study preclinical parameters in one of the largest MALS cohorts with the focus to identify patients that would benefit from surgical MAL release. Results By analyzing 20 MALS patients that underwent surgical celiac artery decompression, we found 60% of patients (12/20) had a postoperative relief of their symptoms and a simultaneous decrease of analgetic use. No demographic, radiologic or operative parameter was able to predict postoperative symptom relief. However, mast cell activation syndrome correlated significantly (p = 0.04) with persistent symptoms after the operation. Conclusions Overall, laparoscopic MAL release can provide immediate symptomatic relief. Despite the missing predictive value of demographic and imaging data, our data show a correlation between persistent symptoms and a co-existing mast cell activation syndrome. This suggests that MCAS symptoms might be interpreted as MALS symptoms in the presence of celiac artery stenosis and therefore surgical treatment should be evaluated carefully. Overall, the selection of patients who are most likely to respond to surgical MAL release may best be accomplished by an interdisciplinary team of gastroenterologists, radiologists and surgeons.

Background: The median arcuate ligament syndrome (MALS) is a rare disease caused by compression of the celiac artery (ORPHA: 293208). Surgical treatment of MALS aims to restore normal celiac blood flow by laparoscopic celiac artery decompression. However, surgical success rates vary widely between patients, therefore adequate selection of patients is essential to improve surgical outcome. Symptoms of MALS might also overlap with other chronic multi-system disorders such as mast cell activation syndrome (MCAS). So far, no clinical or radiological parameter was found to be predictive of the postoperative outcome. We, therefore, aim to study preclinical parameters in one of the largest MALS cohorts with the focus to identify patients that would benefit from surgical MAL release. Results: By analyzing 20 MALS patients that underwent surgical celiac artery decompression, we found 60% of patients (12/20) had a postoperative relief of their symptoms and a simultaneous decrease of analgetic use. No demographic, radiologic or operative parameter was able to predict postoperative symptom relief. However, mast cell activation syndrome correlated significantly (p = 0.04) with persistent symptoms after the operation. Conclusions: Overall, laparoscopic MAL release can provide immediate symptomatic relief. Despite the missing predictive value of demographic and imaging data, our data show a correlation between persistent symptoms and a co-existing mast cell activation syndrome. This suggests that MCAS symptoms might be interpreted as MALS symptoms in the presence of celiac artery stenosis and therefore surgical treatment should be evaluated carefully. Overall, the selection of patients who are most likely to respond to surgical MAL release may best be accomplished by an interdisciplinary team of gastroenterologists, radiologists and surgeons. [ABSTRACT FROM AUTHOR]

Dunbar syndrome (DS) and May–Thurner syndrome (MTS) are part of a group of rare vascular disorders known as "vascular compression syndromes." Dunbar's syndrome is caused by the median arcuate ligament of diaphragm, which, due to an abnormal course, causes celiac artery compression. MTS is caused by the left common iliac vein compression pushed against the spine by the right common iliac artery causing progressive flow congestion and leading to thrombosis. Ultrasound is the first-level examination for the diagnosis of these rare pathologies and allows to recognize vascular compressions and to obtain an estimate of stenosis degree. We describe a very rare case of DS and MTS combination in a young man with postprandial pain and left lower limb thrombosis. [ABSTRACT FROM AUTHOR]

Pancreaticoduodenal artery aneurysms are rare visceral artery aneurysms that may be associated with stenosis of the coeliac trunk. We present a case of a 48-year-old woman with a symptomatic aneurysm of the inferior pancreaticoduodenal artery caused by occlusion of the coeliac trunk due to median arcuate ligament syndrome. The patient was treated with an aorto-hepatic bypass and excision of the aneurysm. We discuss the case and the complexities and controversies in the treatment of this disease. [ABSTRACT FROM AUTHOR]

Dunbar syndrome (DS) and May–Thurner syndrome (MTS) are part of a group of rare vascular disorders known as “vascular compression syndromes.” Dunbar's syndrome is caused by the median arcuate ligament of diaphragm, which, due to an abnormal course, causes celiac artery compression. MTS is caused by the left common iliac vein compression pushed against the spine by the right common iliac artery causing progressive flow congestion and leading to thrombosis. Ultrasound is the first-level examination for the diagnosis of these rare pathologies and allows to recognize vascular compressions and to obtain an estimate of stenosis degree. We describe a very rare case of DS and MTS combination in a young man with postprandial pain and left lower limb thrombosis.

Vascular compression syndromes represent a group of rare and poorly understood diseases. Dunbar syndrome (DS) is caused by the median arcuate ligament of diaphragm originating lower than normal and causing compression of celiac artery. The Nutcracker is caused by the superior mesenteric artery (SMA) originating from aorta at an acute angle causing a restriction of aortomesenteric space that is traversed by the left renal vein and duodenum; if the compression involves only the left renal vein and becomes symptomatic it is called Nutcracker syndrome; if the symptomatic compression involves only the duodenum it is called Wilkie's syndrome or SMA syndrome. The knowledge of these rare pathologies is essential to reduce the false negatives which still remain very high; it is, therefore, necessary to promote greater knowledge as the lack of diagnosis can be very dangerous for the patient's health. We describe a rare case of a combination of DS, Nutcracker, and SMA or Wilkie's syndrome in a young patient.

Dunbar syndrome is caused by the compression of the celiac artery by the median arcuate ligament (MAL) and the diaphragmatic crura, presenting with abdominal pain. The treatment is surgical, with the aim of dissecting and separating the diaphragmatic crura from the celiac axis, restoring blood flow in the celiac trunk. We propose the use of transesophageal echocardiography to measure the peak systolic velocity in the celiac trunk and to evaluate, in real time, the efficacy of the surgery during MAL resection. Two clinical cases with practical implications are reported. In one of these, the ultrasound exam was useful in providing an intraoperative confirmation of the outcome of the resection.

Vascular compression syndromes include a group of rare vascular changes due to extrinsic compression of veins or arteries by surrounding structures. These pathologies are often underestimated due to their rarity, clinicians’ poor level of knowledge, and the non-specificity of their symptoms. The best known are Eagle syndrome, thoracic outlet syndrome, nutcracker syndrome, May-Thurner syndrome, Dunbar syndrome, and popliteal entrapment syndrome. This work summarizes the main ultrasonographic characteristics, symptoms, and treatments of choice for these syndromes. Knowledge of these conditions’ characteristic signs is essential for the differential diagnosis. Failure to diagnose these rare diseases can expose patients to serious complications and risks to their health.

BackgroundMedian arcuate ligament syndrome (MALS) is caused by celiac trunk (CT) compression by the median arcuate ligament. Clinically, this pathology varies from postprandial pain (Dunbar syndrome) to a life-threatening hemorrhage because of a rupture of a gastroduodenal artery aneurysm (GAA). Due to the low prevalence of this disease, there is no standard management for MALS.Material and methodThis was a single-center, retrospective study of 13 patients. Two groups were identified on the basis of the initial clinical presentation: those operated for a GAA rupture (bleeding group—BG) and those operated electively for Dunbar syndrome (Dunbar syndrome group—DG). The primary endpoint was 30-day postoperative complications of a systematic laparoscopic release of the median arcuate ligament and stenting during the same procedure.ResultsSeven patients (54%) underwent elective surgery. Six patients (46%) underwent semiurgent repair under elective conditions post-embolization for GAA bleeding. The total operative time was longer in the BG (p = 0.06). Two patients in the BG suffered early major complications and needed reintervention, and those in the DG had a lower comprehensive complication index. No mortality was reported at 30 days. Overall median length of stay was 5 days (IQR: 3.5–15.3). Patients in the DG had a significantly shorter length of stay (p = 0.02). At 6 months, the primary and secondary CT stent patencies were 82% and 100%, respectively. There were no high-flow GAA recurrences.ConclusionsA combined approach of laparoscopic release of the median arcuate ligament and stenting during the same procedure is feasible and safe, and this approach must be systematically discussed in symptomatic patients.

Vascular compression syndromes represent a group of rare and poorly understood diseases. Dunbar syndrome (DS) is caused by the median arcuate ligament of diaphragm originating lower than normal and causing compression of celiac artery. The Nutcracker is caused by the superior mesenteric artery (SMA) originating from aorta at an acute angle causing a restriction of aortomesenteric space that is traversed by the left renal vein and duodenum; if the compression involves only the left renal vein and becomes symptomatic it is called Nutcracker syndrome; if the symptomatic compression involves only the duodenum it is called Wilkie's syndrome or SMA syndrome. The knowledge of these rare pathologies is essential to reduce the false negatives which still remain very high; it is, therefore, necessary to promote greater knowledge as the lack of diagnosis can be very dangerous for the patient's health. We describe a rare case of a combination of DS, Nutcracker, and SMA or Wilkie's syndrome in a young patient. [ABSTRACT FROM AUTHOR]

Since the first description of the median arcuate ligament syndrome (MALS), the existence for the syndrome and the efficacy of treatment for it have been questioned. A systematic review conforming to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) statement was conducted, with a broader view on treatment for MALS including any kind of coeliac artery release, coeliac plexus resection, and coeliac plexus blockage, irrespective of age. Online databases were used to identify papers published between 1963 and July 2021. The inclusion criteria were abdominal symptoms, proof of MALS on imaging, and articles reporting at least three patients. Primary outcomes were symptom relief and quality of life (QoL). Thirty-eight studies describing 880 adult patients and six studies describing 195 paediatric patients were included. The majority of the adult studies reported symptom relief of more than 70% from three to 228 months after treatment. Two adult studies showed an improved QoL after treatment. Half of the paediatric studies reported symptom relief of more than 70% from six to 62 months after laparoscopic coeliac artery release, and four studies reported an improved QoL. Thirty-five (92%) adult studies and five (83%) paediatric studies scored a high or unclear risk of bias for the majority of the Quality Assessment of Diagnostic Accuracy Studies 2 (QUADAS-2) items. The meaning of coeliac plexus resection or blockage could not be substantiated. This systematic review suggests a sustainable symptom relief of more than 70% after treatment for MALS in the majority of adult and paediatric studies; however, owing to the heterogeneity of the inclusion criteria and outcome parameters, the risk of bias was high and a formal meta-analysis could not be performed. To improve care for patients with MALS the next steps would be to deal with reporting standards, outcome definitions, and consensus descriptions of the intervention(s), after which an appropriate randomised controlled trial should be performed. [ABSTRACT FROM AUTHOR]

Celiac axis compression syndrome is an uncommon entity of abdominal vascular compression syndrome which results from the compression of the proximal celiac artery by the hypertrophied median arcuate ligament. The patients present clinically with postprandial abdominal pain predominantly in the epigastric region and weight loss. The use of thin-section Multidetector Computed Tomography (MDCT) and 3-Dimensional (3D) image reconstruction has greatly improved the ability to obtain detailed images and helped to assess the abdominal vascular system non invasively. Five cases of celiac axis compression syndrome with its characteristic MDCT imaging features like hooked appearance, thickened median arcuate ligament more than 4 mm, poststenotic dilatation and posterior and inferior displacement of celiac artery with indentation on aorta have been described in the present case series and the imaging findings described by other authors in their reported studies have also been compared. [ABSTRACT FROM AUTHOR]

Dunbar syndrome (DS) (or median arcuate ligament [MAL] syndrome) is a rare entity of the vascular compression syndrome, where there is focal proximal coeliac axis compression by MAL. It results in an insufficient supply of blood to the respective organs of the gastrointestinal tract. Multidetector computed tomography is a very convenient, noninvasive modality in diagnosing this condition and helpful in distinguishing it from other conditions, such as atherosclerotic disease. DS can further be treated disorder surgically by relieving the compression and sometimes may need vascular reconstruction. We present five cases of the DS.

Celiac axis compression syndrome is an uncommon entity of abdominal vascular compression syndrome which results from the compression of the proximal celiac artery by the hypertrophied median arcuate ligament. The patients present clinically with postprandial abdominal pain predominantly in the epigastric region and weight loss. The use of thin-section Multidetector Computed Tomography (MDCT) and 3-Dimensional (3D) image reconstruction has greatly improved the ability to obtain detailed images and helped to assess the abdominal vascular system non invasively. Five cases of celiac axis compression syndrome with its characteristic MDCT imaging features like hooked appearance, thickened median arcuate ligament more than 4 mm, poststenotic dilatation and posterior and inferior displacement of celiac artery with indentation on aorta have been described in the present case series and the imaging findings described by other authors in their reported studies have also been compared.

Background: The pathophysiology of median arcuate ligament syndrome (MALS) is poorly understood. The diagnostic process remains inadequately standardized, with an absence of precise criteria to guide therapeutic management., Methods: We studied consecutive subjects referred to the Department of Angiology at the University Hospital of Zurich over the past 17 years due to suspected MALS. We focused on (1) the imaging criteria that led to diagnosis, notably the results of color duplex ultrasound and the consistency with different imaging tests; (2) the clinical consequences focusing on symptom resolution., Results: We included 33 subjects; in 8 subjects (24.2%), the diagnosis of MALS was retained. The median expiration peak systolic velocity (PSV) on ultrasound was 3.05 (Q1; 2.1-Q3; 3.3). To confirm the sonographic results, either a CT or MRI was performed on all patients, with consistent findings confirming a significant stenosis. Seven patients underwent surgery, all involving arcuate ligament release. Four procedures were laparoscopic, one was via laparotomy, and two were robot-assisted. Additionally, two patients required angioplasty with stenting as a secondary intervention. Only two (28.6%) of the seven operated patients experienced a relief of symptoms. None experienced a relief of symptoms following secondary angioplasty, despite stent patency. The prevalence of psychiatric disorders was comparable between patients with retained and rejected diagnoses, 38% and 36%, respectively., Conclusions: Our study confirmed sonography and CT/MRI consistency. However, most patients with MALS did not benefit from invasive treatment. The majority (83%) of patients without MALS were diagnosed with alternative conditions, mainly functional disorders.

Median arcuate ligament syndrome (MALS), also known as Dunbar syndrome, is a rare but significant cause of chronic abdominal pain resulting from the extrinsic compression of the celiac trunk. This condition typically manifests with symptoms such as postprandial pain, nausea, vomiting, and weight loss, often leading to a diagnostic challenge due to its mimicry of other abdominal disorders. Diagnosis is based on exclusion and requires a high index of suspicion combined with precise imaging findings. This case report presents a 44-year-old female patient presenting with chronic abdominal pain, diaphoresis, and nausea, underscoring the complexity and diagnostic challenge of MALS, highlighting the significance of early intervention to mitigate morbidity and novelty treatment utilizing robotic-assisted surgical techniques. This report aims to contribute to the understanding of clinical presentations, diagnostic challenges, and treatment modalities of Dunbar syndrome, especially the option of minimally invasive robotic-assisted surgery for the treatment of this condition., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Barron-Cervantes et al.)

Vascular compression syndromes include a group of rare vascular changes due to extrinsic compression of veins or arteries by surrounding structures. These pathologies are often underestimated due to their rarity, clinicians' poor level of knowledge, and the non-specificity of their symptoms. The best known are Eagle syndrome, thoracic outlet syndrome, nutcracker syndrome, May-Thurner syndrome, Dunbar syndrome, and popliteal entrapment syndrome. This work summarizes the main ultrasonographic characteristics, symptoms, and treatments of choice for these syndromes. Knowledge of these conditions' characteristic signs is essential for the differential diagnosis. Failure to diagnose these rare diseases can expose patients to serious complications and risks to their health. [ABSTRACT FROM AUTHOR]

Chronic abdominal pain has several aetiologies, and many a times, the aetiology is one of the exclusions. Median arcuate ligament syndrome (MALS) also called coeliac artery compression syndrome is a form of chronic mesenteric ischaemia, although rare is an important cause. A 20-year-old male presented with recurrent episodes of pain abdomen, with a history of multiple admissions for the same, before the surgery. His physical examination and laboratories were normal, but the computed tomography (CT) done initially gave a doubtful diagnosis of MALS. A CT angiogram was done, which showed a definite kinking of the coeliac axis. After all the other causes of abdominal pain were ruled out, the patient was taken up for surgery with a provisional diagnosis of MALS. Intraoperatively, there was fibrosis around the coeliac axis with kinking. The fibrosis was cleared, and pulsations of the artery were confirmed. Postoperatively, the patient had an uneventful recovery.

Abstract Vascular compression syndromes are rare alterations that have in common the compression of an arterial and/or venous vessel by contiguous structures and can be congenital or acquired. The best known are the Thoracic Outlet Syndrome, Nutcracker Syndrome, May–Thurner Syndrome, and Dunbar Syndrome. The incidence of these pathologies is certainly underestimated due to the non-specific clinical signs and their frequent asymptomaticity. Being a first-level method, Ultrasound plays a very important role in identifying these alterations, almost always allowing a complete diagnostic classification. If in expert hands, this method can significantly contribute to the reduction of false negatives, especially in the asymptomatic population, where the finding of the aforementioned pathologies often happens randomly following routine checks. In this review, we briefly discuss the best known vascular changes, the corresponding ultrasound anatomy, and typical ultrasound patterns.

Median arcuate ligament syndrome (MALS) is a rare condition caused by the compression of the celiac trunk by the median arcuate ligament, leading to a typical symptom triad: postprandial abdominal pain, weight loss, nausea, and vomiting. A 41-year-old female patient presented to our center with mild postprandial abdominal pain over the epigastric region, and bloating sensation. Ultrasonography of the abdomen showed multiple stones in the gall bladder lumen, and the computed tomography scan showed median arcuate ligament impingement along the proximal aspect of the celiac trunk causing moderate narrowing with post-stenotic dilation. Laparoscopic release of the median arcuate ligament with laparoscopic cholecystectomy was performed. The diagnosis of Median Arcuate Ligament Syndrome is based on the classical post-prandial symptoms and abdominal imaging technologies like Doppler ultrasonography, computed tomography angiography, or magnetic resonance angiography. Exclusion of other intestinal disorders should be considered before making the diagnosis. Celiac artery decompression through different means is the principle of treatment of this condition. The diagnosis of median arcuate ligament syndrome should be considered in patients with postprandial abdominal pain that does not have an established etiology. Celiac artery decompression by releasing the median arcuate ligament is the treatment. • A rare condition caused by the compression of the celiac trunk by the median arcuate ligament • Classical post-prandial symptoms like post-prandial abdominal pain • Diagnosis: doppler ultrasonography, Computed tomography angiography, or magnetic resonance angiography • Decompression of celiac artery is the standard treatment of choice. [ABSTRACT FROM AUTHOR]

Dunbar syndrome (DS) (or median arcuate ligament [MAL] syndrome) is a rare entity of the vascular compression syndrome, where there is focal proximal coeliac axis compression by MAL. It results in an insufficient supply of blood to the respective organs of the gastrointestinal tract. Multidetector computed tomography is a very convenient, noninvasive modality in diagnosing this condition and helpful in distinguishing it from other conditions, such as atherosclerotic disease. DS can further be treated disorder surgically by relieving the compression and sometimes may need vascular reconstruction. We present five cases of the DS. [ABSTRACT FROM AUTHOR]

Introduction: Median arcuate ligament syndrome (MALS) is a rare cause of chronic epigastric pain. The presentation might be unclear and non-specific. Diagnosing the syndrome requires interdisciplinary methods and specialists. Treatments consist of celiac axis release performed laparoscopically or robotically, and intraluminal stenting. Aim: The aim of the study was to report the medium-term postoperative follow-up results for four patients with MALS. Materials and methods: We performed 5 laparoscopic celiac axis releases in patients with MALS in our department in 2018. The study included 4 patients who were admitted 16–23 months after surgery for computed tomography angiography. Results: Patients constituted 4 women aged 28–63 with a mean body mass index of 22.4 kg/m2. The diagnosis of MALS was confirmed by computed tomography angiography, which showed severe (>70%) narrowing of the celiac axis. Patients underwent laparoscopic celiac axis release, and were all discharged on the first postoperative day with no postoperative complications. The patients’ quality of life improved and their symptoms subsided completely. Follow-up computed tomography angiography confirmed full decompression of the celiac axis in all 4 patients, with no stenosis caused by scarification of the celiac axis. Conclusion: Laparoscopy is a valuable and safe method to treat patients with MALS. [ABSTRACT FROM AUTHOR]

Introduction: Endoscopic measures have continued to be the primary procedures in the management of ulcer bleeding. Nevertheless, in cases of failed endoscopic hemostasis and re-bleedings, endovascular techniques have gradually gained increased acceptance as an alternative to surgery, allowing to avoid surgical intervention in some cases. Case report: A case of a 42-year-old patient presenting to the authors’ institution with massive bleeding from the duodenal ulcer, sprang from a pathologically enlarged gastroduodenal artery is reported. This vascular anomaly was a consequence of occlusion of the coeliac trunk (Dunbar syndrome), which was shown on an angio-CT scan. In spite of several endoscopic and endovascular measures, as well as three operations, the bleeding persistently recurred (a total of 6 episodes) and the patient eventually died. The article presents details of operative and endovascular treatments. Contemporary trends in management in cases of failed endoscopic interventions and re-bleedings form peptic ulcers are shown in the discussion. [ABSTRACT FROM AUTHOR]

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., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Rogando et al.)

Median arcuate ligament syndrome (MALS), also known as Dunbar syndrome, celiac axis syndrome, or celiac artery compression syndrome, is caused by a band of tissue called the median arcuate ligament that compresses the celiac artery and sometimes the celiac plexus too. MALS does not always cause symptoms, but when symptoms occur, surgery is the treatment of choice. This case report focuses on the case of a 27-year-old woman presenting with postprandial episodes of abdominal pain and vomiting accompanied by loss of weight, which was found to be MALS., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Nitsa et al.)

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., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Volokitin et al.)

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., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Don-Pedro et al.)

Dunbar syndrome, also known as median arcuate ligament syndrome, is a rare clinical condition due to the external compression of the celiac trunk by the median arcuate ligament causing abdominal angina. We report a case of Dunbar syndrome and its borderline imaging findings focused on the crucial diagnostic role of color-Doppler ultrasound. We also reviewed the current literature, delineating the clinical manifestations and the diagnostic workup of the Dunbar syndrome with the objective to increase the knowledge of this clinical entity as a cause of postprandial abdominal pain and to underline the pivotal role of color-Doppler ultrasound to avoid incorrect or delayed diagnosis. [ABSTRACT FROM AUTHOR]

Abstract Patients with recurrent unclear causes of postprandial abdominal pain should have median arcuate ligament syndrome as a differential diagnosis which is thought to be caused by celiac artery compression. Diagnosis is by imaging such as CT angiography.

Vascular compression syndromes are rare alterations that have in common the compression of an arterial and/or venous vessel by contiguous structures and can be congenital or acquired. The best known are the Thoracic Outlet Syndrome, Nutcracker Syndrome, May–Thurner Syndrome, and Dunbar Syndrome. The incidence of these pathologies is certainly underestimated due to the non-specific clinical signs and their frequent asymptomaticity. Being a first-level method, Ultrasound plays a very important role in identifying these alterations, almost always allowing a complete diagnostic classification. If in expert hands, this method can significantly contribute to the reduction of false negatives, especially in the asymptomatic population, where the finding of the aforementioned pathologies often happens randomly following routine checks. In this review, we briefly discuss the best known vascular changes, the corresponding ultrasound anatomy, and typical ultrasound patterns. [ABSTRACT FROM AUTHOR]

BACKGROUND: Median arcuate ligament syndrome (MALS) is a rare condition due to compression of the celiac artery (CA) by an anatomically abnormal median arcuate ligament. With ultrasonography (US) as first-line diagnostic modality in patients with unclear abdominal pain, there is limited data on its diagnostic performance in MALS. OBJECTIVE: To investigate the value of CA peak systolic velocity (PSV) in the workup of patients with suspected MALS. METHODS: Patients with diagnosis of MALS between 2009 and 2019 were referred by Department of Visceral Surgery after clinical and gastroenterological workup. Diagnosis was confirmed by surgery or further cross-sectional imaging. B-mode US findings and PSV in the CA during various respiratory states were compared between patients with a final MALS diagnosis and patients not meeting the diagnostic criteria. RESULTS: Patients with proven MALS (n = 10) had higher median CA PSV during normal inspiratory breath-hold (239 [IQR, 159–327] vs. 138 [IQR, 116–152] cm/s; p < #x003C;< #x200A;0.001), and expiratory breath-hold (287 [IQR, 191–412] vs. 133 [IQR, 115–194] cm/s; p < #x003C;< #x200A;0.001) compared to patients without MALS (n = 26). CA PSV in both inspiratory breath-hold (AUC 0.88, 95% CI 0.77–1.00) and expiratory breath-hold (AUC 0.89, 95% CI 0.78–1.00) was of diagnostic value for confirming MALS. The best diagnostic performance (100% sensitivity, 80% specificity) was found for the combination of CA PSVexpiration + 2.4 · PSVinspiration > 550 cm/s. CONCLUSIONS: Since results on optimal cutoff values are inconsistent, a combination of CA PSVs during breathing maneuvers may help to diagnose or rule out MALS. [ABSTRACT FROM AUTHOR]

Myhre syndrome is a rare condition caused by a mutation in the SMAD4 gene, which leads to a defective TGF-β/BMP signaling, resulting in the proliferation of abnormal fibrous tissues. Clinically, patients with Myhre syndrome manifest with defects of connective tissue (skin, muscles, joints), and cardiovascular and neurological impairment. In our report, we present a case of a 16-year-old female with skeletal abnormalities, reduced articular mobility, skin, and muscular hypertrophy and cardiovascular defects characteristic of Myhre syndrome. Long-term pulmonary hypertension and arterial hypertension were persistent in spite of antihypertensive treatment. Our patient was also diagnosed with chronic kidney disease and Dunbar syndrome, which is an external compression of the coeliac trunk or coeliac artery by the surrounding tissues. Until now, only a few cases of renal complications in Myhre syndrome have been published. We describe for the first time a female patient with genetically confirmed Myhre syndrome caused by the p.Ile500Val SMAD4 mutation presenting with an unusual occurrence of congenital vesicoureteral reflux, proteinuria with a decreased renal function, and a condition recognized as Dunbar syndrome.

Chronic abdominal pain has several aetiologies, and many a times, the aetiology is one of the exclusions. Median arcuate ligament syndrome (MALS) also called coeliac artery compression syndrome is a form of chronic mesenteric ischaemia, although rare is an important cause. A 20-year-old male presented with recurrent episodes of pain abdomen, with a history of multiple admissions for the same, before the surgery. His physical examination and laboratories were normal, but the computed tomography (CT) done initially gave a doubtful diagnosis of MALS. A CT angiogram was done, which showed a definite kinking of the coeliac axis. After all the other causes of abdominal pain were ruled out, the patient was taken up for surgery with a provisional diagnosis of MALS. Intraoperatively, there was fibrosis around the coeliac axis with kinking. The fibrosis was cleared, and pulsations of the artery were confirmed. Postoperatively, the patient had an uneventful recovery. [ABSTRACT FROM AUTHOR]

We report a case of Dunbar syndrome, May–Thurner syndrome and Nutcracker syndrome diagnosed in one patient with clinical presentation and imaging findings on Doppler ultrasonography. Dunbar syndrome or truncus coeliacus compression syndrome is an under-diagnosed vascular compression syndrome with a lot of controversy around it because of insufficient differentiation from celiac artery stenosis. May–Thurner syndrome or iliac vein compression syndrome is an anatomically variable condition of venous outflow obstruction caused by extrinsic compression by the right common iliac artery as it crosses the iliac vein anteriorly. In Nutcracker syndrome due to compression the outflow from the left renal vein into the inferior vena cava is obstructed. The combination of all these syndromes in one patient was not described before. [ABSTRACT FROM AUTHOR]

Celiac artery compression syndrome (CACS), also known as Dunbar syndrome or median arcuate ligament syndrome (MALS), is a condition that can result from a fibrous ligament, the median arcuate ligament, passing superior to the celiac axis causing extrinsic compression or entrapment of sympathetic neural fibers near its takeoff from the abdominal aorta. This uncommon disorder is characterized clinically by the triad of postprandial abdominal pain, weight loss, and sometimes abdominal bruit. In the literature, most cases of CACS are from Western countries. The diagnosis is generally considered after more common conditions have been ruled out. In particular, an extensive evaluation of GI tract and biliary system have been done in an effort to identify the cause of pain. For patients in whom celiac artery compression is suspected, the diagnosis requires vascular imaging with a respiratory maneuver to confirm CACS. The author reported a 27-year-old woman with CACS, along with results of cross-sectional CTA, 3D reconstruction, combined with intra-operative duplex ultrasound of the celiac artery to demonstrate stenosis, and an attempt at decompression by aggressive resection of median arcuate ligament and nerve fibers. [ABSTRACT FROM AUTHOR]

• Median Arcuate Ligament syndrome (MALS) is also known as Durban syndrome. • MALS is a rare condition. • MALS must be considered in case of refractory post-surgical abdominal pain. • Angiotomography is accessible and reliable for making the diagnosis. Dunbar syndrome is a rare anatomical abnormality characterized by the extrinsic compression of the celiac trunk by the median arcuate ligament (MAL). Though it is rarely misdiagnosed, the clinical diagnosis may be difficult, especially after complex visceral surgery such as esophagectomy. A 62-year-old male patient with a squamous cell carcinoma of the distal esophagus, placed under trimodal treatment (chemotherapy, radiotherapy followed by hybrid minimal invasive 2-field esophagectomy) presented with abdominal pain refractory to analgesics, anti-spasmodic, opioids, and neuronal celiac plexus ablation in the late post-operative period. He was diagnosed with extrinsic celiac trunk compression based on abdominal angiotomography findings. Retrospectively, similar images were found in conventional abdominal tomography at pre-operative staging, but this time, the patient had only dysphagia. After surgical treatment of MAL, the patient had total relief of pain and symptoms. Abdominal pain after complex surgical procedures is very frequent and its investigation is mandatory, even more after refractory clinical management. Dunbar syndrome is related to ambiguous abdominal pain. It is uncommon and its diagnosis with angiotomography is accessible. Vascular disorders should be investigated in cases of abdominal pain after complex surgical procedures. [ABSTRACT FROM AUTHOR]

Patients with recurrent unclear causes of postprandial abdominal pain should have median arcuate ligament syndrome as a differential diagnosis which is thought to be caused by celiac artery compression. Diagnosis is by imaging such as CT angiography. [ABSTRACT FROM AUTHOR]

Abstract Background Median arcuate ligament syndrome is a rare condition with abdominal symptoms. Accepted treatment options are open release of median arcuate ligament, laparoscopic release of edian arcuate ligament, robot-assisted release of median arcuate ligament and open vascular treatment. Here we aimed to evaluate the central priority of open vascular therapy in the treatment of median arcuate ligament syndrome. Methods We conducted a monocentric retrospective study between January 1996 and June 2016. Thirty-one patients with median arcuate ligament syndrome underwent open vascular surgery, including division of median arcuate ligament in 17 cases, and vascular reconstruction of the celiac artery in 14 cases. Results In a 20-year period, 31 patients (n = 26 women, n = 5 men) were treated with division of median arcuate ligament (n = 17) or vascular reconstruction in combination with division of median arcuate ligament (n = 14). The mean age of patients was 44.8 ± 15.13 years. The complication rate was 16.1% (n = 5). Revisions were performed in 4 cases. The 30-day mortality rate was 0%. The mean in-hospital stay was 10.7 days. Follow-up data were obtained for 30 patients. The mean follow-up period was 52.2 months (range 2–149 months). Patients were grouped into a decompression group (n = 17) and revascularisation group (n = 13). The estimated Freedom From Symptoms rates were 93.3, 77.8, and 69.1% for the decompression group and 100, 83.3, and 83.3% for the revascularisation group after 12, 24 and 60 months respectively. We found no significant difference in the Freedom From Re-Intervention CA rates of the decompression (100% at 12, 24 and 60 months post-surgery) and revascularisation (100% at 12 months, and 91.7% at 24 and 60 months post-surgery) groups during follow-up (p = 0.26). Conclusions Open vascular treatment of median arcuate ligament syndrome is a safe, low mortality-risk procedure, with low morbidity rate. Treatment choice depends on the clinical and morphological situation of each patient.

Introduction : Celiac trunk (CT) compression syndrome caused by the median arcuate ligament (MAL) is a rarely diagnosed disease because of its nonspecific symptoms, which cause a delay in the correct diagnosis. Intestinal ischemia occurs, which causes symptoms of abdominal angina. One method of treatment for this disease is surgical release of the CT – the intersection of the MAL. Laparoscopy is the first step of the hybrid technique combined with percutaneous angioplasty and stenting of the CT. Aim: To demonstrate the usefulness and advantages of the laparoscopic approach in the treatment of Dunbar syndrome. Material and methods : Between 2013 and 2016 in the General and Minimally Invasive Surgery Department of the Medical Sciences Faculty of the University of Warmia and Mazury in Olsztyn, 6 laparoscopic procedures were performed because of median arcuate ligament syndrome. During the laparoscopy the MAL was cut with a harmonic scalpel. One month after laparoscopy 5 patients had Doppler percutaneous angioplasty of the CT with stent implantation in the Vascular Surgery Department in Pomeranian Medical University in Szczecin. Results : In one case, there was a conversion of laparoscopic surgery to open due to unmanageable intraoperative bleeding. In one case, postoperative ultrasound examination of the abdominal cavity demonstrated the presence of a large hematoma in the retroperitoneal space. All patients reported relief of symptoms in the first days after the operation. Conclusions : The hybrid method, combining laparoscopy and angioplasty, seems to be a long-term solution, which increases the comfort of the patient, brings the opportunity for normal functioning and minimizes the risk of restenosis.

Celiac artery compression syndrome, also referred to as median arcuate ligament syndrome, celiac axis syndrome or Dunbar syndrome is a rare disorder consequent to extrinsic compression of the celiac trunk by the median arcuate ligament. Doppler ultrasound, multi-slice computed tomography angiography, magnetic resonance angiography, or invasive selective angiography can identify stenosis of the initial segment of the celiac artery and confirm diagnosis. Treatment options include open surgical or videolaparoscopic section of the median arcuate ligament and the fibers of the celiac plexus, or percutaneous transluminal angioplasty via an endovascular approach. We report herein an interesting case of a 38-year-old woman diagnosed with this rare condition and successfully treated with the surgical strategy. [ABSTRACT FROM AUTHOR]

Background: Despite previous reports of robotic-assisted laparoscopic release for median arcuate ligament syndrome (MALS), the safety and efficacy profile of this approach has been difficult to establish due to the rarity of this diagnostic entity. We aim to present our experience from a tertiary minimally invasive surgery referral center.Methods: A case series was performed whereby all patients who underwent robotic-assisted MAL release from July 2010 to July 2017 at our institution were included. Diagnosis of MALS was made based on consideration of symptom presentation, celiac artery duplex ultrasound, and corresponding findings on Computed Tomography (CT) or Magnetic Resonance Angiography (MRA). Outcomes up until the most recent clinic follow-up were reviewed.Results: A total of 13 patients underwent robotic-assisted MAL release. Patients' age ranged from 16 to 71 years (mean 38 years) and consisted primarily of females (76.9%). Most common presenting symptoms included postprandial pain (76.9%), weight loss (76.9%), nausea and vomiting (76.9%). Mean symptom duration was 3 years (range 1-10 years). No intraoperative complications. None required conversion to open surgery. One case required a conversion back to laparoscopy due to anatomical complexity. The mean operative time for successfully completed robotic cases was 94.6 min (range 52-120 min), and for all cases including converted case was 103.5 min (52-210 min). Mean follow-up duration was 19.7 months (range 1-77 months). During subsequent follow-up, a 30-day readmission rate of 23.1% was observed. All but one of the patients experienced prompt symptom improvement. Four patients had symptom recurrence during follow-up.Conclusions: Our experience demonstrates that the robotic-assisted approach to MAL release may be safe and efficacious in selected patients. Prospective comparative studies are required to further evaluate its outcomes against conventional laparoscopic approach, the current gold standard. [ABSTRACT FROM AUTHOR]

Zusammenfassung Hintergrund Das „median arcuate ligament syndrome” (MALS) ist eine seltene Erkrankung der A. coeliaca (CA). Die Kompression dieser Arterie wird durch einen untypischen Verlauf des Ligamentum arcuatum medianum über dem Hiatus aorticus verursacht. Die Patienten mit einem MALS klagen über Oberbauchbeschwerden sowie über einen lumbalen Rücken- und Flankenschmerz. Betroffen sind überwiegend junge hochgewachsene Frauen (w:m 4:1). Die Stenose der CA kann mittels sonographischer Untersuchung erkannt werden. Dabei stehen die Bestimmung des Winkels zwischen der abdominellen Aorta und der CA sowie dessen Veränderung bei In- und Exspiration (DA+) im Vordergrund. Ein weiterer wichtiger Parameter ist die Bestimmung der systolischen Spitzenflussgeschwindigkeit (PV) in der CA. Ein PV ≥350 cm/s in der CA und ein Unterschied von ≥250% zwischen der Ein- und der Ausatmung sind mit einen PPV („positive predictive value”) von 83% hinweisend für ein MALS. Falldarstellung Berichtet wird über die primär osteopathische Behandlung von sieben Patienten mit einem MALS über einen Zeitraum von 6,2 (2–12) Monaten. Zwei der sieben Patienten unterzogen sich jedoch im Lauf der Therapie einer chirurgischen Durchtrennung des Ligamentum arcuatum medianum bzw. einer Angioplastie mit Stentversorgung. Die verbleibenden fünf Patienten erhielten im Mittel 5,7 (2–11) osteopathische Behandlungen und zeigten eine deutliche Reduktion ihrer Beschwerden. Die objektiven Marker (PV und DA+) blieben jedoch weiterhin über dem Cut-off-Wert. Fazit In Anbetracht der Tatsache, dass bis zum heutigen Tag die Ätiologie und Pathophysiologie des MALS nicht mit letzter Sicherheit definiert werden können und die operative Therapie keine Beschwerdefreiheit garantieren kann, ist die osteopathische Behandlung eine Option für jene Patienten, die sich nach Diagnosestellung nicht spontan für eine operative Behandlung entscheiden. Background The „median arcuate ligament syndrome” (MALS) is a rare disease of the celiac artery (CA). The compression of this artery, caused by an atypically positioned median arcuate ligament, leads to abdominal pains in the URQ and in the ULQ and low back pain as well as flank pain. The gender relation (f/m) is 4:1 and most often young leptosome are affected by MALS. The stenosis of the CA can be diagnosed by ultrasonography and by MRI. In patients with MALS the deflexion-angle (DA+), the angle between the abdominal aorta (AA) and the CA, increases during expiration. With a positive predictive value (PPV) of 83% a peak velocity (PV) ≥350 cm/s in the CA during expiration and a delta between PV during expiration and inspiration ≥250% is indicative for a MALS. Case Report We report a case series of seven patients with MALS treated initially with OMT („osteopathic manual medicine”) over a mean period of 6,2 (2–12) months. Two patients out of seven had operative treatment, the remaining five patients were treated with OMT. After a mean of 5,7 (2–11) treatment sessions the complaints were reduced to an acceptable level for all five patients. Also, the PV of the CA during expiration droped, but the DA+ remained over the cut-off-value of 50°. Conclusion Based on the fact, that the etiology and the pathophysiology of MALS is not fully understood and the surgical treatment of MALS does not guarantee the total symptom relief the probative treatment with OMT is a treatment option for patients who decide against surgery. [ABSTRACT FROM AUTHOR]