Median arcuate ligament syndrome: A case report of a rare disease

Abstract

Introduction Median arcuate ligament syndrome (MALS) is a rare condition caused by the compression of the celiac trunk by the median arcuate ligament, which may trigger a typical symptom triad: postprandial abdominal pain, weight loss, nausea and vomiting. Clinical case A 58-year-old male was admitted to the emergency department due to abdominal pain, vomiting, fever and diarrhea. On admission, the patient was tachycardic, had abdominal distension, no peritoneal irritation signs, and a serum lactate level of 5 mmol/L. The computed tomography (CT) scan showed gastric and intestinal pneumatosis associated with pneumoportia. Conservative treatment with intravenous fluids, antibiotics and low molecular weight heparin, was initiated. The reassessment CT scan showed resolution of the gastric and intestinal pneumatosis and a severe stenosis of the initial portion of the celiac trunk with associated angulation. After recovery from the acute episode, an abdominal duplex ultrasound and an aortoiliac arteriography in forced inspiration and expiration was performed, corroborating the diagnosis of MALS. A laparoscopic decompression of the celiac trunk was performed. Discussion Symptoms of MALS closely mimic other abdominal disorders, and it should be considered in the differential diagnosis. Currently, there are no international guidelines on MALS diagnostic criteria. Treatment is focused on decompression of the median arcuate ligament constriction of the celiac artery, with or without celiac lymph node removal. Conclusion MALS diagnostic and therapeutic approach must be patient focused. Laparoscopic decompression is an effective treatment and can provide immediate symptomatic relief, associated with the benefits of the less invasive nature of the procedure.

A Case Series of Median Arcuate Ligament Syndrome With Varied Presentations

“…The clinical presentation of MALS can be vague and nonspecific, often leading to diagnostic challenges and numerous misdiagnoses all of which could lead to nonbeneficial trials of drugs and surgical interventions resulting in unwanted complications and delaying diagnosis. 2 In our study, all 3 cases were initially misdiagnosed, illustrating the diagnostic challenge (Table 1). The shared triad of symptoms, including postprandial epigastric pain, weight loss, nausea, and vomiting, concurs with previous literature.…”

“…1 A triad of symptoms including weight loss, nausea, vomiting, and postprandial epigastric pain, is frequently observed. 2 Despite its infrequency, MALS remains a subject of medical interest, yet its true prevalence remains uncertain due to its variable clinical expression. 2 The condition tends to favor a female phenotype (4:1) and typically emerges within the age range of 30 to 50 years.…”

“…2 Despite its infrequency, MALS remains a subject of medical interest, yet its true prevalence remains uncertain due to its variable clinical expression. 2 The condition tends to favor a female phenotype (4:1) and typically emerges within the age range of 30 to 50 years. 3 This syndrome presents a challenging diagnostic workup, partly owing to its varied clinical manifestations and poorly understood pathophysiological mechanism, requiring comprehensive investigations to exclude more common causes of abdominal pain.…”

“…3 This syndrome presents a challenging diagnostic workup, partly owing to its varied clinical manifestations and poorly understood pathophysiological mechanism, requiring comprehensive investigations to exclude more common causes of abdominal pain. 2,3 Various imaging modalities, such as Doppler ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), and mesenteric angiogram, play a crucial role in highlighting celiac axis compression. 1 The treatment goal revolves around celiac trunk decompression, using approaches like open, laparoscopic, or robotic methods, accompanied by adjunctive interventions like percutaneous transluminal angioplasty (PTA) and stenting.…”

“…1 The treatment goal revolves around celiac trunk decompression, using approaches like open, laparoscopic, or robotic methods, accompanied by adjunctive interventions like percutaneous transluminal angioplasty (PTA) and stenting. 1,2 In this context, we present a case series involving three distinct instances of MALS, each characterized by unique presentations and managed through laparoscopic decompression.…”