A 52-year-old man with a medical history of asthma and hypertension presented with low chest pain, which started six hours prior to his visit. He reported nausea and vomiting, bilateral back pain, and three days of dry cough. He did not have a fever but did have chills. He denied any sick contacts, abdominal pain, urinary symptoms, or blood in his stool. He tried to alleviate his symptoms with his inhaler, but it didn't help. He denied a history of blood clots or coronary artery disease.
Chart review revealed that he had presented to the ED seven times before in the previous three months for similar symptoms, each time with an unremarkable workup.
His oxygen saturation was 98% on room air, pulse was 76 bpm, blood pressure was 193/114 mm Hg, and temperature 36.8°C. He was oriented to person, place, and time, but he had episodic diaphoresis while in the room.
His heart rate and rhythm were regular without murmurs or gallops, and his lungs were clear bilaterally to auscultation. His abdomen was soft.
When asked where his pain was, he pointed to his epigastric area but showed no worsening tenderness on exam. He was intermittently and mildly diaphoretic. His initial cardiac workup was negative. Given the systemic symptoms and multiple similar visits for the same symptoms and very high blood pressure, the team did a CT angiogram to rule out dissection.
What does this patient have? What treatment should he get?
Find the diagnosis and case discussion on the next page.
Diagnosis: Median Arcuate Ligament Syndrome
Median arcuate ligament syndrome is also referred to as celiac artery compression syndrome, celiac axis compression syndrome, or Dunbar syndrome. Patients often have chronic abdominal pain that is caused by the compression of the celiac artery by the median arcuate ligament. The condition is uncommon and usually identified through by exclusion. It was first seen in 1917, and later described in the 1960s by Pekka-Tapani Harjola and J. David Dunbar. (Ann Chir Gynaecol Fenn 1963;52:547; Am J Roentgenol Radium Ther Nucl Med 1965;95:731.)
The etiology is not completely understood, but many believe it is related to the ischemic or neuropathic pathways. The symptoms are characterized as postprandial epigastric abdominal pain, nausea, vomiting, and even idiopathic gastroparesis. The median arcuate ligament at the base of the diaphragm usually crosses the aorta right above the branch off of the celiac artery. Some individuals' median arcuate ligament passes right in front of the celiac artery, however, and it seems to cause problems in about one percent of the 10 to 24 percent of these individuals. (Radiographics 2005;25:1177.)
The celiac plexus is adjacent to the median arcuate ligament. One theory suggests that the pain is caused by the ligament compressing the blood flow through the celiac artery. Another says the pain is caused by the ligament's proximity to the celiac plexus. Yet another suggests that the compression of the celiac artery causes delayed emptying of the stomach and causes another type of neuropathic pain. (Am J Gastroenterol 1997;92:519.)
The presentation is usually epigastric abdominal pain, nausea, and vomiting. Some of these patients have significant weight loss as well. Abdominal pain is the most common presenting symptom. (J Vasc Surg 2012;56:869.) These patients often have a normal physical exam and have been seen multiple times for similar symptoms. They can have a mid-epigastric abdominal bruit on exam on rare occasions.
The diagnosis of this syndrome is often a surprise or a diagnosis of exclusion. It has often been found on CT angiograms while looking for another cause for the abdominal pain. Ultrasound can also be used to aid in the diagnosis. The patient should undergo inspiratory and expiratory arteriography if he presents with symptoms consistent with median arcuate ligament syndrome and has no other cause for his abdominal pain. Inspiration should take the pressure off of the celiac artery while expiration should exacerbate the symptoms by causing more compression. Remember to make sure there is no other cause because this is a diagnosis of exclusion and needs further workup.
This condition is managed with e management for this is surgery, which can decompress the celiac artery by dividing or separating the median arcuate ligament. Surgery sometimes combines decompression with celiac artery stenting. (Ann Vasc Surg 2009;23:778.) This surgery can be done in an open or laparoscopic approach. The outcomes of these surgeries have varied: Some case studies report significant symptomatic relief while others didn't achieve significant benefit. (Can J Surg 1982;25:377.)
Many case studies have shown patients getting better after celiac axis decompression and imaging has shown changes to the celiac artery, but it remains unclear whether the condition truly exists. (Arch Surg 1971;103:252; Perspect Vasc Surg Endovasc Ther 2007;19:259.)That said, a few long-term studies seem to show the benefits of surgical intervention. This study showed that 75 percent of 51 patients who underwent surgical intervention didn't have the symptoms on follow-up. (J Vasc Surg 1985;2:79.) This patient is awaiting follow-up with gastroenterology and surgery. He has presented two more times to the emergency department with similar symptoms.
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