Celiac artery compression syndrome (CACS), also known as median arcuate ligament syndrome, can sometimes represent a diagnostic challenge. Here, we present the case of a 29-year-old man who presented with recurrent diabetic ketoacidosis (DKA), abdominal pain, and vomiting thought to be due to cyclical vomiting syndrome. However, the lack of a clear precipitant for DKA, the presence of chronic gastrointestinal symptoms, and a revealing physical examination of abdominal bruit led to clinical suspicion of CACS and its diagnosis after appropriate investigations. While angiography has traditionally been considered the gold standard diagnostic test, hemodynamic and geometric ultrasound criteria can, however, be diagnostic. The patient was managed by releasing the celiac artery through robotic surgery and serial monitoring as an outpatient revealed resolution of his symptoms and no further readmissions for DKA. This case highlights how a presumptive and erroneous diagnosis (cyclical vomiting syndrome) can misguide clinicians, especially when dealing with a rare diagnosis of exclusion.
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