Laparoscopic Treatment of a Symptomatic Young Woman With Median Arcuate Ligament Syndrome.

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.

Post-surgical Median Arcuate Ligament Syndrome (MALS) Symptom Exacerbation Treated With Osteopathic Manipulative Treatment (OMT): A Case Report.

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.

Celiac Artery Compression Syndrome in an Adolescent Male.

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.

Median arcuate ligament syndrome: A rare case report from Nepal.

INTRODUCTION: 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. CASE PRESENTATION: 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. DISCUSSION: 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. CONCLUSION: 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.

Recurrent aneurysmatic bleeding of pancreaticoduodenal aneurysm due to median arcuate ligament syndrome: a case report.

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.

Supradiaphragmatic origin of the celiac trunk leading to median arcuate ligament syndrome with superior mesenteric artery involvement.

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.

Combination between Dunbar Syndrome and May-Thurner Syndrome: A Rare Case Report.

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.

Prognostic factors for the long term outcome after surgical celiac artery decompression in MALS.

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.

Median arcuate ligament syndrome in an old male: a case report with 3-year follow-up.

Introduction and importance: Median arcuate ligament syndrome (MALS) or Dunbar syndrome is a rare compression syndrome that poses a challenge to many clinicians due to its ambiguous symptoms. It is predominantly common in females in their 30s to 50s. Case presentation: A 74-year-old male presented with generalized chronic postprandial abdominal pain, anorexia, and weight loss of 6 kg for the past 2 months. Physical examination, abdominal ultrasound, endoscopy, and colonoscopy were all unremarkable. His laboratory workup and tumor marker tests were within normal ranges. Finally, a multi-slice computed tomography (MSCT), an advanced computed tomography with multiple detectors resulting in faster and higher resolution imaging, outlined external compression on the celiac artery (CA) by the median arcuate ligament (MAL). The release of the CA from the MAL was done laparoscopically. Symptoms improved significantly postoperatively. During the follow-up period of 3 years, the patient did not regain his lost weight but had no other complaints. Clinical discussion: Due to its vague manifestations, MALS is diagnosed only after extensive evaluation and exclusion. This challenging diagnosis outlines the need for refined diagnostic guidelines. An MSCT plays a crucial role in confirming the diagnosis. Currently, more physicians prefer laparoscopic release of the MAL compared to an open approach. Conclusion: Despite MALS predominance in females, the diagnosis of MALS should be considered in males with postprandial abdominal pain and unexplained weight loss. An MSCT, along with other imaging modalities, can provide a comprehensive view of celiac compression. Laparoscopic decompression of the CA is an ideal treatment option.

Median arcuate ligament syndrome often poses a diagnostic challenge: A literature review with a scope of our own experience.

The median arcuate ligament syndrome (MALS) is recognized as a rare clinical entity, characterized by chronic post-prandial abdominal pain, nausea, vomiting, and unintentional weight loss. Due to its vague symptomatology, it is mainly regarded as a diagnosis of exclusion. Patients can often be misdiagnosed for several years before a correct diagnosis is established, also due to a medical team's clinical suspicion. We present a case series of two patients who suffered from MALS and were treated successfully. The first patient is a 32-year-old woman, presenting with post-prandial abdominal pain and weight loss that have lasted for the past ten years. The second patient, a 50-year-old woman, presented with similar symptomatology, with the symptoms lasting for the last five years. Both cases were treated by laparoscopic division of the median arcuate ligament fibers, which alleviated extrinsic pressure from the celiac artery. Previous cases of MALS were retrieved from PubMed, to assemble a better diagnostic algorithm and propose a treatment method of choice. The literature review suggests an angiography with a respiratory variation protocol as the diagnostic modality of choice, along with the laparoscopic division of the median arcuate ligament fibers as the proposed treatment of choice.

Enigma of Bowel Angina: Unraveling Celiac Trunk Stenosis.

The celiac axis is commonly involved in conditions that cause the narrowing or blockage of a celiac artery atherosclerosis and other vascular disease. Celiac artery compression syndrome is defined as chronic abdominal pain occurring because of compression of the celiac artery commonly in middle-aged (40 to 60 years) females. Various etiologies include atherosclerosis of mesenteries vessels, pancreatitis, median arcuate ligament syndrome, and tumor invasion. It is an uncommon condition, and symptoms include post-prandial abdominal pain mostly in the epigastrium, weight loss, nausea, diarrhea, anorexia, and bloating. Patients are asymptomatic for a prolonged duration due to collateral blood supply to the bowel from the patent superior mesenteric and inferior mesenteric arteries. We present a case of a 67-year-old female who initially presented with signs and symptoms suggestive of small bowel obstruction, however, due to persistent abdominal pain, she underwent a CT scan suggestive of severe celiac trunk stenosis causing abdominal angina. The patient was managed conservatively and responded well with close follow-up.

Systematic hybrid laparoscopic and endovascular treatment of median arcuate ligament syndrome: A single-center experience.

Background: Median 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 method: This 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. Results: Seven 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. Conclusions: A 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.

The Ultrasound Findings in a Rare Case of Nutcracker Syndrome, Wilkie's Syndrome, and Dunbar Syndrome Combination.

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.

Pancreaticoduodenal artery aneurysm associated with coeliac trunk occlusion treated with aorto-hepatic bypass and aneurysm resection.

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.

Systematic Review of the Efficacy of Treatment for Median Arcuate Ligament Syndrome.

OBJECTIVE: 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. METHODS: 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). RESULTS: 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. CONCLUSION: 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.

Gabapentin as a Symptomatic Modifier in Median Arcuate Ligament Syndrome: A Case Report and Assessment of Treatment Modalities.

Median arcuate ligament syndrome (MALS) is a rare constellation of neurogenic gastrointestinal (GI) symptoms resulting from compression of the celiac trunk and celiac plexus by the median arcuate ligament. MALS is characterized by nonspecific symptoms including nausea, vomiting, diarrhea, bloating, unintentional weight loss due to food aversion, and postprandial epigastric abdominal pain. We present a case of atypical, chronic MALS that confounded clinicians for over a decade and led to various misdiagnoses, including early-onset Parkinson's disease. Of the constellation of symptoms that MALS may present with, postprandial epigastric pain is a classic symptom and increases the index of suspicion for the diagnosis; however, the absence of the classic symptom of postprandial epigastric pain and the predomination of nonspecific GI symptoms and syncope in our patient further clouded clinicians' ability to diagnose MALS. Upon further investigation, we elucidated a link between gabapentin, which our patient was chronically prescribed, and its efficacy in decreasing neurogenic hypersensitivity in the GI tract. Our case and the implications of gabapentin use to decrease neurogenic pain from MALS represents a novel addition to the literature on MALS treatment modalities and elucidates new avenues for continued research in the use of gabapentin as a symptom-modifying agent in the nonoperative and preoperative treatment of MALS.

DUNBAR SYNDROME: CLINICAL MANIFESTATION IN ADULTS, DIAGNOSTIC PROBLEMS (CASE REPORT).

In this case we used CBC and biochemical tests, ECG, ultrasound of the abdominal cavity and heart, CT scan with and without IV contrast. Women with complains on recurrent severe abdominal pain in epigastric region and right part of the abdomen radiating to the back, nausea, vomiting. Making laboratory and instrumental tests for confirmation of the diagnosis. Surgical treatment of DS was performed and after one year of the follow up there were no complications. DS may mimic other medical conditions such as gallbladder diseases, gastritis/peptic ulcer, appendicitis, colorectal malignancy, hepatitis, atherosclerotic diseases etc. That is why DS is a diagnosis of exclusion. This case illustrates pathway to find correct diagnosis and improve management tactic.

Vascular compression syndromes: a pictorial review.

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.

Surgical Treatment of Wilkie's Syndrome by Vascular Transposition.

Introduction Superior mesenteric artery syndrome (SMAS), also called mesenteric duodenal compression syndrome, Wilkie's syndrome, chronic duodenal ileus or cast syndrome, is a rare clinical condition defined as a compression of the third portion of the duodenum in between the SMA and abdominal aorta (AA), due to narrowing of the space between them. SMAS is primarily attributed to loss of the intervening mesenteric fat pad, leading to partial or complete duodenal obstruction. Its manifestations are complex and non-specific, including postprandial epigastric pain, nausea, vomiting, early satiety, weight loss and anorexia. SMAS may present as an acute syndrome, or it may have an insidious onset with chronic symptoms. SMAS mainly affects females between 10 and 40 years of age. This study aims to discuss the safety and efficacy of vascular decompression of the duodenum by infrarenal transposition of SMA. Methods This single-centre prospective clinical study analysed 37 patients with Wilkie's syndrome who underwent infrarenal transposition of the SMA between January 2012 and December 2021. The indications for the surgery were severe weight loss, uncontrolled upper abdominal pain, vomiting and other gastrointestinal (GI) symptoms that were severely debilitating to patients' daily lives, along with radiological findings such as aortomesenteric angle < 25°, aortomesenteric distance <8 mm and distention of proximal part of the duodenum and the stomach. Ten patients (27%) concurrently had Nutcracker syndrome and seven patients (18.9%) had Dunbar syndrome (median arcuate ligament syndrome). Three female patients (8.1%) had all three above-mentioned vascular compression syndromes, which were treated in the same surgery. One male patient (2.7%) was after a laparoscopic duodenojejunostomy with symptoms that relapsed three months postoperatively, which was cured after the infrarenal transposition of SMA. Results Technical operative and clinical success were achieved in all patients. There were no cases of anastomotic failure, SMA thrombosis or intestinal ischemia. All of the patients are currently living symptom-free. One patient (2.7%), four days postoperatively, had a lymphocele formed in the retroperitoneum, which was successfully drained by a CT-guided percutaneous pigtail catheter. Another patient (2.7%) after three months of surgery needed a re-laparotomy for adhesive obstruction of the second part of the duodenum and was treated by adhesiolysis and omentoplasty. One patient (2.7%), 2-year postoperatively, had a proximal SMA stenosis up to 60% where drug-eluting balloon percutaneous transluminal angioplasty (DEB PTA) was performed successfully. Finally, the upper GI symptoms were resolved in all 37 patients (100%). Conclusion Wilkie's syndrome, although rare, is frequently late-diagnosed or underdiagnosed. In cases of failure of conservative therapy, infrarenal transposition of the SMA can be considered a safe and feasible surgical option with more physiologically favourable outcomes comparable to gastrointestinal bypasses, especially in patients concurrently suffering from Nutcracker syndrome. Simultaneously, it also restores physiologic duodenal passage of gastroduodenal content without the need of creating a digestive tract anastomosis. To our best knowledge, we have the highest number of SMA transposition surgeries performed in a single centre for the treatment of Wilkie's syndrome.