What is the diagnosis and treatment for Celiac (celiac) artery compression syndrome?

From the Guidelines

Celiac artery compression syndrome, also known as median arcuate ligament syndrome, should be treated with surgical decompression through laparoscopic or open release of the median arcuate ligament for severe cases, as it has been associated with symptomatic relief in 84.6% of patients 1.

Diagnosis and Treatment

The diagnosis of celiac artery compression syndrome typically involves a combination of clinical findings, such as abdominal pain, nausea, and vomiting that worsens with meals, as well as imaging findings, including a proximal narrowing of the celiac artery in a “J-shaped” configuration 1.

• Conservative management, including pain medications like NSAIDs (ibuprofen 400-600mg every 6-8 hours or naproxen 250-500mg twice daily), weight management, and small, frequent meals, is recommended for mild cases.
• For severe cases, surgical decompression is the definitive treatment, with post-surgery recovery typically requiring 2-6 weeks, and gradual return to normal activities.
• Diagnosis involves imaging studies such as Doppler ultrasound, CT angiography, or MR angiography to visualize the compression 1.

Surgical Intervention

Surgical release of the median arcuate ligament may be accompanied by reconstruction of the celiac artery, with either endovascular stent placement or surgical bypass creation, to provide complete symptomatic relief and prevent the development of splanchnic artery aneurysms 1.

• 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, compared with 53% of patients who received decompression alone 1.

Additional Interventions

Percutaneous transluminal angioplasty with stent placement may be performed if there is residual stenosis of the celiac artery of >30% after surgical release of the ligament 1.

• A multidisciplinary approach advocating stenting or surgical bypass as needed following surgical release suggests high rates of symptomatic relief of 75% and freedom from reintervention of 64% at 6 months 1.

From the Research

Diagnosis of Celiac Artery Compression Syndrome

• The diagnosis of celiac artery compression syndrome (CACS) can be made using various imaging techniques, including:
  • Splanchnic duplex ultrasound scanning 2
  • Gastric exercise tonometry (GET) 2
  • Multiplane selective splanchnic angiography 2
  • Computed tomography (CT) angiography 3, 4
  • Magnetic resonance angiography (MRA) 5
• Patients with CACS typically present with chronic abdominal symptoms, such as nausea, vomiting, postprandial abdominal pain, and weight loss 4

Treatment of Celiac Artery Compression Syndrome

• The treatment of CACS involves the release of the extrinsic compression on the celiac axis, which can be achieved through:
  • Open surgical approach by an upper midline or left subcostal incision 2
  • Laparoscopic approach, which has been shown to be feasible and safe in several studies 3, 6, 5
  • Endoscopic retroperitoneal approach, which has been shown to be effective in a study of 46 patients 2
• Additional treatment options include:
  • Percutaneous transluminal angioplasty (PTA) and stenting of the celiac artery, which can be used as a complementary procedure after the release of the extrinsic compression 3
  • Aortoceliac artery bypass grafting, which may be necessary in cases of restenosis or failed PTA and stenting 3

Outcomes and Complications

• The outcomes of treatment for CACS have been shown to be generally favorable, with:
  • Significant improvement or resolution of symptoms in the majority of patients 2, 3, 5
  • Anatomic success of the procedure, as confirmed by angiography, in a high percentage of patients 2
• Complications of treatment for CACS can include:
  • Bleeding or hemorrhage, which may require conversion to an open procedure 2, 3
  • Pneumothorax or other respiratory complications, which may require treatment 2
  • Restenosis or recurrence of the celiac artery compression, which may require additional treatment 3, 5