From the Guidelines
Surgical release of the median arcuate ligament is the recommended treatment for celiac artery compression syndrome, with a reported symptomatic relief in 84.6% of patients, and subsequent reconstruction of the celiac artery may be necessary to provide complete symptomatic relief. The diagnosis of celiac artery compression syndrome, also known as median arcuate ligament syndrome, typically requires imaging studies like CT angiography or Doppler ultrasound to visualize the compression of the celiac artery by the median arcuate ligament 1.
Key Points to Consider
- The condition results from an anatomical variation where the diaphragm's median arcuate ligament sits lower than normal, compressing the celiac artery during respiration and causing symptoms like postprandial abdominal pain, weight loss, and nausea due to intestinal ischemia during digestion when blood flow demands increase.
- Treatment depends on symptom severity, with mild cases managed conservatively through dietary modifications and pain medications like NSAIDs.
- For severe or persistent cases, surgical intervention is recommended, typically laparoscopic release of the median arcuate ligament to decompress the celiac artery, which has been associated with symptomatic relief in 84.6% of patients 1.
- Subsequent reconstruction of the celiac artery, with either endovascular stent placement or surgical bypass creation, may be necessary to provide complete symptomatic relief, restore normal hemodynamics, and prevent the development of splanchnic artery aneurysms in the setting of recurrent symptoms or persistent celiac stenosis 1.
Post-Surgery Care
- Post-surgery, patients may require temporary dietary modifications and pain management with acetaminophen or NSAIDs as needed.
- Physical therapy focusing on core strengthening may help prevent recurrence.
- A study evaluating long-term outcomes in 44 patients who received operative management for MAL syndrome reported persistent resolution of clinical symptoms in 76% of patients who underwent some form of revascularization, such as primary reanastomosis or interposition grafting, in addition to decompression, compared with 53% of patients who received decompression alone 1.
From the Research
Diagnosis of Celiac Artery Compression Syndrome
- Celiac artery compression syndrome (CACS) is diagnosed using various imaging techniques, including digital subtraction angiography 2, computed tomography (CT) angiography 2, 3, and splanchnic duplex ultrasound scanning 4.
- The diagnosis is based on the presence of respiratory-dependent celiac artery stenosis and abnormal gastric exercise tonometry (GET) results 4.
- A thorough workup for other pathology, including upper and lower endoscopy, CT scanning, gastric and gallbladder emptying studies, upper gastrointestinal series, and small-bowel follow-through studies, is necessary to rule out other causes of symptoms 2.
Treatment of Celiac Artery Compression Syndrome
- Laparoscopic decompression of the celiac artery is a useful therapy for CACS, with significant improvement in symptoms reported by most patients 2, 3, 5.
- The laparoscopic approach involves division of the median arcuate ligament and complete lysis of the celiac artery from its origin on the aorta to its trifurcation 2.
- Open surgical therapy is also a safe and reliable procedure, with no mortality and low morbidity, and may be necessary for patients with fixed stricture or stenosis of the celiac trunk 6.
- Endovascular treatment with percutaneous transluminal angioplasty and insertion of a stent may not solve the underlying problem of extrinsic compression of the celiac trunk and often requires open procedures during the long-term course 6.
- Vascular co-procedures, such as aorto-celiac vein interposition, aorto-hepatic vein interposition, resection of the celiac artery and end-to-end anastomosis, patchplasty of the celiac artery with vein, and transaortic removal of a stent of the celiac artery, may be necessary in some cases 6.
Outcomes and Follow-up
- Most patients experience significant improvement in symptoms after laparoscopic or open surgical therapy, with some patients gaining weight and becoming asymptomatic 2, 3, 6.
- Follow-up is necessary to monitor for recurrence of symptoms and to assess the long-term efficacy of treatment, with a mean follow-up duration of 20-40 months reported in some studies 2, 4.
- The choice of treatment must depend on the specific clinical situation for each patient, and no guidelines can do justice to all patients sufficiently due to the low incidence of CACS 6.