A 45-year-old man with sudden onset of severe abdominal pain was transferred to our emergency department. He had no previous significant gastrointestinal or cardiovascular disease. He also denied any drug abuse, lead poisoning, allergies, or abdominal injury. However, he disclosed he had been suffering from three-year history of undiagnosed abdominal distension and poor appetite. Physical examination showed severe tenderness in the epigastrium without guarding or rebound tenderness. He presented with tachycardia and tachypnea. Laboratory findings revealed hemoglobin of 7.7 g/dL (reference range 13.7–16.8 g/dL) and D-dimer of 3.1 μg/mL (reference range 0–1 μg/mL). A contrast-enhanced computed tomography (CT) demonstrated a large amount of retroperitoneal fluid with multiple pancreaticoduodenal artery aneurysms (Fig. 1A, B). Emergency transcatheter arterial embolization was performed. Sagittal contrast-enhanced CT reconstruction also demonstrated stenosis of the celiac trunk (Fig. 1C). Based on the clinical findings, we made a diagnosis of retroperitoneal bleeding from the rupture of pancreaticoduodenal artery aneurysms in patient with celiac artery compression syndrome (CACS). Subsequently, he underwent laparoscopic release of the median arcuate ligament. Six months after the procedure, his abdominal symptoms vanished completely without remission. CACS is a rare disease due to external compression of the celiac artery by the median arcuate ligament. Symptoms of CACS are often vague and non-specific, including intermittent epigastric crampy pain, nausea, vomiting, bloating, weight loss, and poor appetite. These symptoms are sometimes confused with other abdominal disorders, and hence, they are often overlooked. The mechanism of severe epigastric crampy pain associated with CACS includes foregut ischemia related to celiac artery compression, midgut ischemia triggered by vascular steal syndrome, and overstimulation of the celiac plexus with subsequent splanchnic vasoconstriction [1]. Although the incidence of this disease is not known, it is more frequently reported with developments of diagnostic imaging examinations. Approximately 80% of patients with CACS are asymptomatic due to sufficient collateral blood flow from the superior mesenteric circulation [2]. However, alteration of blood flow in the pancreaticoduodenal arcade sometimes leads to involvement of arterial aneurysms, which can eventually cause spontaneous rupture. Although surgical intervention, especially laparoscopic decompression of the median arcuate ligament has become a primary treatment for CACS [3], therapeutic guideline or criteria for median arcuate ligament release has still not been established. Cienfuegos et al. advocated the following selection criteria for surgical intervention for CACS in their study: young woman, intense postprandial pain, greater than 70% stenosis of the trunk, and development of collateral circulation [4]. Early diagnosis is essential for timely surgical intervention for preventing serious complication. Therefore, clinicians should consider CACS in the differential diagnosis of patients with a chronic history of non-specific abdominal symptoms with multiple aneurysms of pancreaticoduodenal artery, and multilateral assessment of the celiac trunk with contrast-enhanced CT, invasive selective angiography, and magnetic resonance angiography on sagittal sequence.
WHO estimates that half of all patients worldwide are non-adherent to their prescribed medication. The consequences of poor adherence can be catastrophic, on both the individual and population level.
Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.