Case Presentation: A 48-year-old woman presented with a history of intermittent abdominal pain for eight years, with an extensive workup that had been negative. She ended up being labeled as having irritable bowel syndrome. The pain was localized to the epigastrium, was present during most of the day, but was aggravated by large meals. Upon specific questioning, she mentioned that she had some relief upon lying curled up on her right side. This specific clinical history led to focused ultrasound doppler studies being done. Ultrasound results suggested hemodynamically significant celiac artery stenosis. She then underwent a laparoscopic release of the arcuate ligament with immediate relief of her pain. A year later, the pain recurred. She then underwent an angiogram, was found to have a re-stenosis of the celiac artery and underwent angioplasty with complete relief of pain. The pain relief was noted to be persistent upon follow up at 2 years.
Discussion: An indentation of the celiac artery caused by a mal-positioned MAL is seen in 10-24% of people. However, only a minority have hemodynamically significant stenosis. MALS is a clinically rare disease resulting from extrinsic compression of the celiac trunk by fibrous attachments of the diaphragmatic crura, median arcuate ligament. Most celiac artery compressions do not produce symptoms, probably due to collateral supply from the superior mesenteric artery. However, in some cases, the ligament may compress the celiac artery, compromising blood flow and causing symptoms, including postprandial abdominal pain (sometimes leading to a ‘food fear’), vomiting, and weight loss. It typically occurs in young patients (20-40 years of age) with a low body mass index (BMI). The clinical features can be vague and ill-defined and can overlap many other possible diagnoses. Also, radiological proof of stenosis is often difficult to obtain. As a result, MALS is a disease entity that has a diagnosis of exclusion after ruling out other more common causes of symptoms. Doppler Ultrasound is one of the main imaging modality for the diagnosis of MALS. A flow velocity measurement on Doppler ultrasound performed at a compressed or narrowed segment of a celiac artery reveals the variation of peak systolic velocity (PSV) during respiration with a marked increase during expiration in PSV to greater than 200 cm/s. A greater than 3:1 ratio of PSV in the celiac artery in expiration compared with the PSV in the abdominal aorta just below the diaphragm is a useful criterion to diagnose MALS.Therapy options for MALS include surgical or laparoscopic division, i.e., a “release” of the median arcuate ligament to restore normal blood flow in the celiac artery. Angioplasty or stent placement are other options and are often used in surgical failures. Unfortunately, complete pain relief is not achieved in a significant minority of patients.
Conclusions: In conclusion, this case report shows MALS to be a rare cause for postprandial abdominal pain. Most patients have had extensive workups or various surgical procedures for postprandial abdominal pain. Our patient went undiagnosed for more than 8 years, and only upon careful history taking was the diagnosis suspected. Surgery was initially successful, but a relapse 2 years later required angioplasty to achieve pain relief. This syndrome should be kept in the differential for a patient with abdominal pain once other common diagnoses have been excluded.