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.

Median arcuate ligament syndrome complicated with gallbladder stones:A case report

BACKGROUND Median arcuate ligament syndrome(MALS)is a rare disease caused by compression of the celiac trunk artery by the median arcuate ligament(MAL).It can cause symptoms of postprandial abdominal pain,weight loss,and nausea and vomiting.CASE SUMMARY A 55-year-old woman was admitted due to abdominal pain,nausea and vomiting.On admission,the patient presented with epigastric pain that worsened after eating,without signs of peritoneal irritation.Computed tomography angiography of the upper abdomen showed compression of the proximal segment of the abdominal trunk,local luminal stenosis with angular“fishhook”changes,which changed significantly during forceful inspiration and expiration;gallbladder stones;and multiple cysts in the liver.Abdominal duplex ultrasonography showed that peak systolic velocity was 352 cm/s.After diagnosis of MALS was confirmed,an arch ligament release procedure was performed.MALS has no specific symptoms and can be misdiagnosed as other abdominal diseases.Awareness of MALS should be improved to avoid misdiagnosis.The commonly used treatment option is MAL release and resection of the peripheral ganglion of the celiac trunk artery.CONCLUSION The diagnosis and treatment of MALS must be individualized,and MAL release is effective and provides immediate symptomatic relief.

Arcuate ligament syndrome inducing hepatic artery thrombosis after liver transplantation

BACKGROUND: Hepatic artery thrombosis (HAT) is a frequent complication following liver transplantation, but it is rarely caused by arcuate ligament compression of the celiac artery. This article mainly describes our experience in managing a patient with celiac artery stenosis and HAT after liver transplantation. METHODS: A 44-year-old man with a 15-year history of hepatitis B was admitted to our hospital for hepatocellular carcinoma. Before the operatiori, he received transarterial chemoembolization once, and pretransplant MR angiography indicated a suspected stenosis at the initiation of the celiac artery, while color Doppler showed normal blood flow in the arterial system. In this case, orthotopic liver transplantation was performed for radical cure of hepatocellular carcinoma. However, B-ultrasonography detected poor blood flow in the intra- and extra-hepatic artery on the first posttransplant day, and during exploratory laparotomy a thrombus was found in the hepatic artery. Thus, re-transplantation was conducted with a bypass between the graft hepatic artery and the recipient abdominal aorta with the donor's splenic artery. RESULTS: The patient made an uneventful recovery and color Doppler showed good blood flow in the artery and portal system. Histology confirmed extensive thrombosis in the left and right hepatic artery of the explanted graft, indicating HAT. CONCLUSIONS: Although HAT caused by celiac trunk compression is rarely reported in liver transplantation, the diagnosis should be considered in patients with pretransplant hepatic artery stenosis on angiography and abnormal blood flow on B-ultrasonography. Once HAT is formed, treatment such as thrombectomy or retransplantation should be performed as early as possible.

Median arcuate ligament syndrome with retroperitoneal haemorrhage:A case report

BACKGROUND Median arcuate ligament syndrome(MALS)is relatively rare and is due to extraluminal compression of the coeliac artery by the median arcuate ligament of the diaphragm.Here,we report a case of MALS found in a patient with abdominal pain and retroperitoneal haemorrhage for education and dissemination.CASE SUMMARY This article describes a 46-year-old female patient who was admitted to our hospital with abdominal pain as her chief complaint.She had experienced no obvious symptoms but had retroperitoneal bleeding during the course of the disease.Contrast-enhanced computed tomography(CT)and noninvasive CT angiography(CTA)led to an initial misdiagnosis of pancreaticoduodenal artery aneurysm(PDAA)causing retroperitoneal hemorrhage.After intraoperative exploration and detailed analysis of enhanced CT and CTA images,a final diagnosis of MALS was made.The cause of the haemorrhage was bleeding from a branch of the gastroduodenal artery,not rupture of a PDAA.The prognosis of MALS combined with PDAA treated by laparoscopy and interventional therapy is still acceptable.The patient was temporarily treated by gastroduodenal suture haemostasis and was referred for further treatment.CONCLUSION MALS is very rare and usually has postprandial abdominal pain,upper abdominal murmur,and weight loss.It is diagnosed by imaging or due to complications.When a patient has abdominal bleeding or PDAA,we should consider whether the patient has celiac trunk stenosis(MALS or other etiology).When abdominal bleeding is combined with an aneurysm,we generally think of aneurysm rupture and hemorrhage first,but it may also be collateral artery rupture and hemorrhage.

Feasible management of median arcuate ligament syndrome in orthotopic liver transplantation recipients

BACKGROUND In orthotopic liver transplantation(OLT)recipients,median arcuate ligament syndrome(MALS)is considered a risk factor for hepatic arterial thrombosis(HAT),which is dreadful for OLT recipients.Different alternative surgical procedures have been proposed to overcome the impact of MALS on transplantation,but clinical evidence is still scarce.AIM To evaluate the feasible surgical management of MALS to reduce complications in OLT patients.METHODS Data for 288 consecutive patients who underwent OLT at The First Hospital of Jilin University between January 2017 and July 2020 were retrospectively reviewed.The surgical management of median arcuate ligament(MAL)and modifications to the arterial anastomosis were recorded.The perioperative and long-term prognosis of MALS recipients were noted.Detailed preoperative and postoperative data of patients were analyzed in a descriptive manner.RESULTS Eight patients with MALS were included in this study.The first patient with MALS received no intervention during the primary surgery and developed postoperative HAT.Salvage liver transplantation with MAL division was successfully performed.Gastroduodenal artery(GDA)preservation with splenic artery ligation was performed on three patients,only GDA preservation was performed on two patients,and no intervention was performed on two patients.No patient developed HAT after surgery and postoperative recovery was satisfactory.CONCLUSION The preservation of collateral circulation between the superior mesenteric artery and celiac trunk via the GDA with or without splenic artery ligation is a safe and feasible alternative to MAL division.

Treatment strategy for pancreatic head cancer with celiac axis stenosis in pancreaticoduodenectomy:A case report and review of literature

BACKGROUND During pancreaticoduodenectomy in patients with celiac axis(CA)stenosis due to compression by the median arcuate ligament(MAL),the MAL has to be divided to maintain hepatic blood flow in many cases.However,MAL division often fails,and success can only be determined intraoperatively.To overcome this problem,we performed endovascular CA stenting preoperatively,and thereafter safely performed pancreaticoduodenectomy.We present this case as a new preoperative treatment strategy that was successful.CASE SUMMARY A 77-year-old man with a diagnosis of pancreatic head cancer presented to our department for surgery.Preoperative assessment revealed CA stenosis caused by MAL.We performed endovascular stenting in the CA preoperatively because we knew that going into the operation without a strategy could lead to ischemic complications.Double-antiplatelet therapy(DAPT)–which is needed when a stent is inserted–was then administered in parallel with neoadjuvant chemotherapy(NAC).This allowed us to administer DAPT for a sufficient period before the main pancreaticoduodenectomy procedure while obtaining therapeutic effects from NAC.Subtotal stomach-preserving pancreaticoduodenectomy was thenperformed.The operation did not require any unusual techniques and was performed safely.Postoperatively,the patient progressed well,without any ischemic complications.Histopathologically,curative resection was confirmed,and the patient had no recurrence or complications due to ischemia up to six months postoperatively.CONCLUSION Preoperative endovascular stenting,with NAC and DAPT,is effective and safe prior to pancreaticoduodenectomy in potentially resectable pancreatic cancer.

Uncommon Ethiology in Abdominal Pain: Celiac Axis Compression Syndrome

Celiac axis compression syndrome, one of the reasons of mesenteric ischemia, is an extremely rare etiology of abdominal pain. Primary pathological mechanism is the external compression of the celiac trunk by median arcuate ligament. The diagnosis of this condition is usually difficult and depends on angiographic findings and computerized tomography (CT) evaluations. Here we report a celiac axis compression syndrome case, presenting with chronic abdominal pain and weight loss, which was successfully treated by surgery.