Abstract

Celiac artery compression syndrome (CACS) or median arcuate ligament (MAL) syndrome is a rare vascular disease. The clinical manifestations of CACS include the triad of postprandial pain, vomiting, and weight loss. The pathogenesis of CACS is the external compression of celiac artery by the MAL or celiac ganglion. Moreover, some authors also reported the compression with different etiologies, such as neoplasms of pancreatic head, adjacent duodenal carcinoma, vascular aneurysms, aortic dissection, or sarcoidosis. In the literature, most cases of CACS were reported from Western countries. In contrast, this disease was seldom reported in Oriental countries or regions, including Taiwan. Superior mesenteric artery syndrome (SMAS) is also a rare disease characterized by compression of the third portion of the duodenum by the SMA. The clinical features of SMAS are postprandial pain, vomiting, and weight loss. To date, there are no guidelines to ensure the proper treatment of patients with CACS because of its low incidence. Thus, tailored therapy for patients with CACS remains a challenge as well as the prediction of clinical response and prognosis. The aim of our present study was to investigate the clinical features, the association with SMAS, treatments, and outcomes of patients with CACS in a single institution in Taiwan.

Highlights

  • Celiac artery compression syndrome (CACS), called median arcuate ligament (MAL) syndrome or Dunbar syndrome, is a rare vascular disease [1,2,3]

  • The clinical manifestations of CACS include the triad of postprandial pain, vomiting, and weight loss

  • From January 2003 to March 2011, we retrospectively reviewed the medical records of patients who were diagnosed as CACS in the outpatient department and inpatient department of China Medical University Hospital, a tertiary referral hospital in the middle of Taiwan

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Summary

Introduction

Celiac artery compression syndrome (CACS), called median arcuate ligament (MAL) syndrome or Dunbar syndrome, is a rare vascular disease [1,2,3]. As early as the 1960s, Dunbar first described the stenotic anomaly of celiac trunk through visualization angiographically. He correlated the image findings and clinical manifestations of patients presenting with abdominal angina, such as postprandial pain, nausea/vomiting, and weight loss. He found that those patients became symptom-free after successful surgical release of the compression by sectioning the MAL. For patients with SMAS, the aortomesenteric angle is less than 25 degrees (6◦–25◦) or the aortomesenteric

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