Program Director and Professor of Clinical Surgery University of Pennsylvania Philadelphia, Pennsylvania
Objectives: Median arcuate ligament compression of the celiac artery (MALS) can be found as an incidental finding on imaging studies. Postural Orthostatic Tachycardia Syndrome (POTS) is a syndrome of autonomic dysfunction that can be associated with finding of MALS. The objective of this study is to see if celiac artery compression by MALS is contributory to pathophysiology of POTS in these group of patients
Methods: We describe our series of 3 cases with MALS and POTS that underwent provocative vasodilator angiography and subsequent surgical decompression. We reviewed their symptoms before and after the surgery.
Results: Patients were predominantly female in the 3rd decade of life. Symptoms of abdominal pain with associated nausea and weight loss typically last from 6 months to 2 years. All 3 patients with MALS and POTS had complete resolution of symptoms of POTS after surgical decompression.
Conclusions: Surgical decompression of the median arcuate ligament with careful neurolysis of the celiac plexus is associated with complete symptomatic relief of POTS in these group of patients. Multidisciplinary approach involving primary care, gastroenterology, cardiology, psychiatry, general surgery, and vascular surgery are essential in providing accurate diagnosis and treatment of this rare association of inherently challenging conditions.