Displasia fibromuscular y síndrome de ligamento arcuato medio. Infrecuentesy simultáneos / Fibromuscular dysplasia and median arcuate ligament syndrome. Rare and simultaneous findings

Introducción: la displasia fibromuscular (DFM) es una patología poco frecuente de la capa muscular de las arterias. El síndrome de ligamento arcuato medio (SLAM) es una entidad infrecuente causada por la compresión extrínseca del tronco celíaco por el diafragma. Caso clínico: presentamos el caso de una mujer joven con DFM diagnosticada de afectación a nivel del tronco celíaco y de la arteria hepática común. Ante clínica de dolor abdominal, se solicita angio TC, que describe un SLAM asociado a la DFM. Se decide sección quirúrgica del ligamento arcuato y descompresión del tronco celíaco mediante abordaje robótico. Discusión: en ambas entidades la angiografía es el trataminto de referencia para el diagnóstico. El tratamiento de primera línea de la DFM es el endovascular mediante angioplastia, y del SLAM, el quirúrgico, seccionando el ligamento arcuato.(AU)

Introduction: fibromuscular dysplasia (FMD) is a rare disorder that affects the muscular layer of the arteries. The medianarcuate ligament syndrome (MALS) is also a rare disorder due to the extrinsic compression of the celiac trunk by thediaphragm.Case report: we report the case of a young woman with FMD and splachnic involvement of the celiac trunk and thecommon hepatic artery level. After presenting with abdominal pain, a CCTA was performed that revealed the presenceof FMD-related MALS. The surgical section of the arcuate ligament and decompression of celiac trunk were decided andperformed through robotic approach.Discussion: the gold standard for the diagnosis of both entities is angiography. However, while the first-line therapy ofFMD is endovascular, in the case MALS the best alternative is surgical treatment sectioning the arcuate ligament.(AU)

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Pancreaticoduodenal Artery Aneurysm Complicated by Median Arcuate Ligament Syndrome.

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

Celiac trunk thrombosis in a patient with antiphospholipid syndrome induced by median arcuate ligament compression: a case presentation and literature review.

Median arcuate ligament syndrome (MALS) is a rare disorder caused by the compression of the celiac axis by the fibrous structure of the diaphragm called the median arcuate ligament. Patients with MALS are usually undiagnosed unless characteristic symptoms such as nausea and vomiting, postprandial pain, and weight loss are presented. We report a case of a 29-year-old patient diagnosed with MALS and secondary antiphospholipid syndrome (APS) that developed celiac trunk, common hepatic artery and splenic artery thrombosis. There is not enough information on MALS as a trigger of thrombosis in predisposed patients such as those with APS. However, the case gives rise to suspicion and highlights the diagnostic processes, especially for patients with APS presenting postprandial abdominal pain and weight loss. This review likewise aims at the importance of Doppler ultrasonography as a screening tool and computer tomography (CT) or magnetic resonance (MR) both in the angiography variant, especially to diagnose confirmation and underlying treatment options.

A rare case of a combination of ectopic kidney and medial arcuate ligament syndrome: a case report.

BACKGROUND: Ectopic kidney and median arcuate ligament syndrome are both rare conditions. The clinical presentation and diagnosis of these conditions are not well studied. There are no reports on the combination of these two rare conditions. CASE PRESENTATION: We report a 24-year-old woman with fever, dysuria, urinary frequency and left flank pain for two days. The primary diagnoses in the clinic were left acute pyelonephritis and left hydronephrosis due to throbbing pain in the left costovertebral angle and pyuria. However, further computed tomography showed right ectopic pelvic kidney, left renal pelvis dilatation without definite ureteral lesion, good bilateral renal contrast enhancement, and compression of the celiac axis due to obstruction by the median arcuate ligament. Chronic abdominal symptoms were reported by the patient after repeat history taking. The patient's condition was fully explained and discussed with her and her family, but they refused further therapy. After the acute pyelonephritis began improving, the patient was discharged for follow-up at our outpatient clinic. CONCLUSION: We present an extremely rare case of a combination of two rare conditions: ectopic kidney and median arcuate ligament syndrome. No study to date has reported on the relationship between the two diseases. Given the rarity of the two conditions, no evidence or even a hypothesis exists to explain the possible etiology of their combination. More reports are required to enhance the understanding of these rare conditions.

Atypical case of coexistent vascular compression syndromes: median arcuate ligament syndrome and nutcracker syndrome.

Vascular compression syndromes, including median arcuate ligament syndrome (MALS) and nutcracker syndrome (NCS), are poorly understood and frequently delayed diagnoses. This case describes a young adult female presenting with chronic vomiting, abdominal pain and weight loss, with dependence on nasogastric feeding. Subsequent to her gastrointestinal symptoms, she developed haematuria and orthostatic intolerance. Investigations confirmed NCS and possible MALS, with superadded gastroparesis and bradygastria. Under the joint care of general and vascular surgeons, she underwent a gastrojejunostomy and panelled renocaval bypass which led to partial resolution of her symptoms. It is hypothesised that gastroparesis may have been caused by MALS via a neurogenic mechanism, or coexistent compression of the duodenum by the superior mesenteric artery. This case highlights the difficulty in diagnosis of vascular compression syndromes, the overlap between the conditions and the potential for multiple coexistent conditions which complicate diagnosis and lead to increased lead-time and morbidity for patients.

Prevalence and outcomes of select rare vascular conditions in females: A descriptive review.

Rare vascular conditions frequently pose a diagnostic and therapeutic dilemma for health care providers. Several of these conditions have distinct relevance to females populations but, due to their infrequency, there has been little reported on the outcomes of rare vascular conditions specifically in females populations. We performed a literature review of a selection of three rare vascular conditions known to either disproportionately affect females (median arcuate ligament syndrome and fibromuscular dysplasia) or have unique manifestations in females populations (vascular Ehlers-Danlos syndrome). We performed a descriptive review of the literature focused on these three vascular conditions and identified aspects of the current available research describing sex-based differences in prevalence, any pathophysiology explaining the observed sex-based differences, and the contribution of sex to outcomes for each disease process. In addition, considerations for pregnant females with respect to each rare vascular disease process are discussed.

A Case Series of Median Arcuate Ligament Syndrome With Varied Presentations.

Median arcuate ligament syndrome (MALS) is characterized by the constriction of the celiac trunk caused by fibrous connections originating from the median arcuate ligament (MAL) and diaphragmatic crura. It presents with symptoms often leading to misdiagnosis. In this study, we present three cases of MALS, with distinct manifestations. These cases were diagnosed through comprehensive investigations and managed successfully using laparoscopic decompression. The diagnosis of MALS poses challenges due to its variable presentations and overlap with other conditions. Diagnostic imaging techniques such as Doppler ultrasound, computed tomography (CT) scans and angiography play a role in confirming the diagnosis. Laparoscopic decompression has proven to be a treatment option that relieves symptoms and restores blood flow. This series highlights the importance of considering MALS as a cause for abdominal pain cases. Early detection and the use of diagnostic techniques can result in favorable outcomes.

A fatal case of massive hemobilia caused by invasive pancreatic cancer with median arcuate ligament syndrome: A case report.

INTRODUCTION: In median arcuate ligament syndrome (MALS), the celiac artery is compressed, causing an arcade to develop in the pancreatic head, leading to ischemic symptoms and aneurysms. PATIENT CONCERNS: The patient was diagnosed with borderline resectable pancreatic cancer (PC) and MALS. Endoscopic biliary drainage with a covered metal stent (CMS) was performed for the obstructive jaundice. After the jaundice improved, a modified FOLFIRINOX regimen was initiated. Several days later, cardiopulmonary arrest occurred after hematemesis occurred. Cardiopulmonary resuscitation was performed, his blood pressure stabilized, and emergent upper endoscopy was performed. The CMS was dislodged and active bleeding was observed in the papillae. The CMS was replaced, and temporary hemostasis was achieved. Contrast-enhanced computed tomography revealed a diagnosis of extravasation from the posterior superior pancreaticoduodenal artery (PSPDA) into the biliary tract. Transcatheter arterial embolization was performed. However, the patient was subsequently diagnosed with hypoxic encephalopathy and died on day 14 of hospitalization. DIAGNOSIS: Biliary hemorrhage due to invasion of pancreatic cancer from the PSPDA associated with MALS. INTERVENTION: None. OUTCOMES: Biliary hemorrhage from the PSPDA was fatal in the patient with invasive PC with MALS. LESSONS: Since MALS associated with PC is not a rare disease, the purpose of this study was to keep in mind the possibility of fatal biliary hemorrhage.

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.

Obstructive jaundice as a rare complication of multiple pancreaticoduodenal artery aneurysms due to median arcuate ligament syndrome: a case report and review of the literature.

BACKGROUND: Obstructive jaundice has various causes, and one of the rarest is pancreaticoduodenal artery aneurysm (PDAA), which is often associated with celiac axis stenosis caused by median arcuate ligament syndrome (MALS). CASE PRESENTATION: The patient was a 77-year-old Azeri woman who presented with progressive jaundice, vague abdominal pain, and abdominal distension from 6 months ago. The intra- and extrahepatic bile ducts were dilated, the liver's margin was slightly irregular, and the echogenicity of the liver was mildly heterogeneous in the initial ultrasound exam. A huge cystic mass with peripheral calcification and compressive effect on the common bile duct (CBD) was also seen near the pancreatic head, which was connected to the superior mesenteric artery (SMA) and had internal turbulent blood flow on color Doppler ultrasound. According to the computed tomography angiography (CTA) findings, the huge mass of the pancreatic head was diagnosed as a true aneurysm of the pancreaticoduodenal artery caused by MALS. Two similar smaller aneurysms were also present at the huge aneurysm's superior margin. Due to impending rupture signs in the huge aneurysm, the severe compression effect of this aneurysm on CBD, and the patient's family will surgery was chosen for the patient to resect the aneurysms, but unfortunately, the patient died on the first day after the operation due to hemorrhagic shock. CONCLUSION: In unexpected obstructive jaundice due to a mass with vascular origin in the head of the pancreas, PDAA should be considered, and celiac trunk should be evaluated because the main reason for PDAA is celiac trunk stenosis or occlusion by atherosclerosis or MALS. The treatment method chosen (including transarterial embolization, open surgery, or combined method) depends on the patient's clinical status and radiological findings, but transarterial embolization would be safer and should be used as a first-line method.

Transarterial embolization for ruptured pancreaticoduodenal artery aneurysm due to segmental arterial mediolysis combined with median arcuate ligament syndrome: a case report.

A 75-year-old female with a history of Parkinson's disease treatment and hypertension presented at the emergency section with sudden onset of right abdominal pain. Contrast-enhanced computed tomography revealed beaded irregular stenosis and dilation of the superior mesenteric artery (SMA) and an aneurysm in the branch of the pancreaticoduodenal artery (PDA) that communicates with the common hepatic artery and SMA. Additionally, a hematoma had formed in the retroperitoneal space, and extravasation of contrast medium from the pancreaticoduodenal artery aneurysm (PDAA) into the hematoma was observed. The celiac artery (CA) was compressed by the median arcuate ligament; stenosis of the CA at its origin and dilation on the distal side were observed. Based on the imaging findings, it was diagnosed that PDAA was ruptured, SMA developed segmental arterial mediolysis (SAM), and CA developed median arcuate ligament syndrome (MALS). The ruptured PDAA was thought to be caused by SAM combined with MALS. Transcatheter arterial embolization (TAE) was performed for the ruptured PDAA. To the best of our knowledge, there have been no reports of TAE for a ruptured PDAA caused by SAM and MALS. After TAE, the PDAA did not re-rupture.