A thin 25‑year‑old female with chronic postprandial epigastric pain, nausea, early satiety, unintentional weight loss and an epigastric bruit that increases on expiration—what is the most likely diagnosis and optimal management?

Median Arcuate Ligament Syndrome (MALS)

Most Likely Diagnosis

This clinical presentation—a thin young female with chronic postprandial epigastric pain, nausea, early satiety, unintentional weight loss, and an epigastric bruit that increases on expiration—is pathognomonic for Median Arcuate Ligament Syndrome (MALS). 1, 2

Clinical Presentation and Diagnostic Features

The classic triad of MALS consists of:

• Postprandial abdominal pain occurring 15-30 minutes after eating, present in nearly 100% of symptomatic patients 3
• Fear of eating (sitophobia) developing as a learned response to avoid pain, despite preserved appetite 3
• Progressive unintentional weight loss due to reduced oral intake from pain avoidance, not malabsorption 3

Additional key diagnostic features include:

• Epigastric bruit that worsens on expiration is highly characteristic, as expiration causes the diaphragm to descend and increases compression of the celiac artery 4
• Young, thin females are the most commonly affected demographic 5
• Early satiety and nausea are frequently present 1, 2

Diagnostic Workup

CT angiography (CTA) is the initial imaging modality of choice, with sensitivity and specificity of 95-100% 2. Look for:

• Proximal narrowing of the celiac artery in a "J-shaped" configuration 1
• Dynamic compression that worsens on expiration 1, 4

Mesenteric angiography with lateral projection during both inspiration and expiration is the diagnostic gold standard, demonstrating dynamic worsening of stenosis on expiration and relief on inspiration 1, 4. This respiratory variation is pathognomonic for MALS 4.

Duplex ultrasound can be used as an alternative screening tool, with peak systolic velocity cutoffs of 240 cm/s indicating ≥70% celiac artery stenosis 2.

Critical Diagnostic Pitfall

Celiac artery compression is present in approximately 20% of the normal population and does not always cause symptoms 1, 2. MALS remains a diagnosis of exclusion—extensive workup to rule out other gastrointestinal, cardiovascular, and musculoskeletal causes must be completed first 6, 7.

Optimal Management Algorithm

First-Line Treatment

Surgical release of the median arcuate ligament is the definitive first-line treatment for MALS, with symptomatic relief achieved in 84.6% of patients 1, 5. The laparoscopic approach is safe and effective with low morbidity 5.

The surgical procedure should include:

• Laparoscopic division of the median arcuate ligament to decompress the celiac artery 5, 6
• Celiac plexus neurolysis to address the neurogenic component of pain 4

When to Add Revascularization

Additional revascularization should be considered if residual stenosis of the celiac artery is greater than 30% after ligament release 1. Options include:

• Endovascular stent placement 1
• Surgical bypass creation 1

What NOT to Do

Endovascular stent placement alone (without surgical ligament release) is contraindicated due to persistent extrinsic compression, chronic vessel wall changes, and high risk of stent fracture or migration 1. One study confirmed that a patient who received postoperative celiac stent placement alone remained symptomatic 5.

Systemic anticoagulation alone has limited data and should not be used as sole therapy 1.

Predictors of Surgical Success

The following factors predict successful outcomes after surgical intervention:

• Postprandial pain pattern: 81% cure rate 1
• Age 40-60 years: 77% cure rate 1
• Weight loss ≥20 pounds: 67% cure rate 1

Important Caveat

Patients with atherosclerotic cardiovascular risk factors have poorer outcomes—in one series, 67% of nonresponders had cardiovascular risk factors versus only 27.3% of responders 5. This suggests that MALS symptoms are not primarily related to vascular compromise but rather to neurogenic pain from celiac plexus compression 5.

Role of Diagnostic Celiac Plexus Block

For patients with atypical presentations, preoperative diagnostic celiac plexus block using local anesthetic can predict surgical success 5. In one study, 4 of 5 patients who reported symptom relief after diagnostic block also experienced postoperative symptom relief 5, 6.

Initial Conservative Management

Before proceeding to surgery, supportive treatment with analgesics and continued diagnostic evaluation for alternate causes of abdominal pain are reasonable first steps 1. However, given this patient's classic presentation with all diagnostic features present, surgical consultation should not be delayed.

Follow-Up Considerations

Postoperative duplex ultrasound of the celiac axis should be performed to confirm decompression 5. However, symptomatic relief can occur even with residual stenosis or occlusion on imaging—in one series, 5 patients with residual celiac stenosis and 1 with occlusion reported complete symptom resolution 5. This reinforces that the neurogenic component, not vascular insufficiency, drives symptoms in most cases.